Neuroradiology Companion Methods Guidelines and Imaging Fundamentals Zamor PDF

Neuroradiology
Companion
Methods, Guidelines, and
Imaging Fundamentals
2
Neuroradiology
Companion
Methods, Guidelines, and
FIFTH EDITION
Carlos Zamora, MD, PhD

Assistant Professor of Radiology
Division of Neuroradiology, Department of
Radiology
The University of North Carolina School of Medicine
Chapel Hill, North Carolina
Mauricio Castillo, MD, FACR

James H. Scatliff Distinguished Professor
Chief of Neuroradiology
Division of Neuroradiology, Department of
Radiology
The University of North Carolina School of Medicine
Chapel Hill, North Carolina
3
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Fifth edition
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Library of Congress Cataloging-in-Publication Data

Names: Castillo, Mauricio, author. | Zamora, Carlos, 1978- , author.
Title: Neuroradiology companion : methods, guidelines, and imaging fundamentals
/ Carlos Zamora, Mauricio Castillo.Description: Fifth edition. | Philadelphia :
Wolters Kluwer, [2017] | Preceded by Neuroradiology companion / Mauricio
Castillo. 4th ed. c2012. | Includes bibliographical references and index.
Identifiers: LCCN 2016012005 | ISBN 9781496322135Subjects: | MESH: Central
Nervous System Diseases—radiography | Central Nervous System— radiography |
Neuroradiography—methods | Handbooks
Classification: LCC RC349.R3 | NLM WL 39 | DDC 616.8/047572—dc23 LC
record available at http://lccn.loc.gov/2016012005
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To Hajnalka, Sophie, David, Moisés, and Ninette.
C.Z.
7
Preface
There is no doubt that neuroimaging plays a central role in the ever-

advancing field of the neurosciences. The extensive body of knowledge
that has been generated over the past several years is not only daunting but
certainly impossible to assimilate in its entirety. Updating the previous
edition of the Neuroradiology Companion to include not only advances in
radiology but also basic current concepts of neuropathology, neurosurgery,
and neurology, all while keeping the text relevant to the neuroradiologist,
has been a challenging but at the same time interesting and hopefully
fruitful task.
The fifth edition of the Companion continues to be focused on
residents and fellows and includes fundamental information that trainees
should be familiar with by the end of their program. I am confident that
they will benefit from reading the book from cover to cover. At the same
time, the pathologies presented here are extracted from every day practice
and likely to be encountered in both private and academic settings, such
that a practicing radiologist will most likely find it valuable for quick
reference. As with prior editions, the content of this book is presented in a
succinct fashion. Because of the ever-increasing breadth of
neuroradiology, the reader is encouraged to make the most of the resources
listed as suggested reading, which have been thoroughly updated, as they
will expand on the topics covered in each section.
The first part of the book has been updated to reflect some of the more
relevant protocols utilized at our institution, again noting that these are
continuously changing along with advances in technology and that they
will vary from place to place and according to the individual needs and
different vendors. The section on contrast media reactions has been
updated to reflect the most recent guidelines. We have removed the
chapter on sample dictations as the use of macros is commonplace at most
institutions and many of these can be found online.
Finally, in an attempt to make the book more physically manageable,
some of the sections have been merged and a few less relevant or
8
redundant topics and images have been removed. On the other hand, some
of the individual sections have been expanded. Overall, the book will be
somewhat thinner than the prior edition. Care has also been taken to
present the most illustrative images and to make the best use of labeling as
an educational tool and to highlight subtle findings. This edition of the
Companion features many recent cases and includes more than 800 new
images.
I hope that you will enjoy this book as much as have I enjoyed going
through every detail of the prior edition.
9
Acknowledgments
First and foremost, I am thankful to Dr. Castillo for his mentorship and for
giving me the opportunity to contribute to this project whose preparation I
have found intellectually stimulating and educational. Some of the new
cases presented here come from pathology that I encountered during my
training at Johns Hopkins Hospital and for that I am grateful to this
institution and to its faculty, particularly Dr. Nafi Aygun and Dr. David
Yousem, educators and clinicians extraordinaire. I would like to thank my
new colleagues at the Neuroradiology Division who have welcomed me to
UNC and whose tremendous support has proven instrumental for my
academic endeavors. They are the best group of individuals I could
possibly ask to work with and I have found my interactions with them to
be enriching and rewarding. I am also grateful to Dr. Matthew Mauro and
Dr. Paul Molina for their continued support to our division. Finally, I
would like to acknowledge our supporting staff, whose tireless daily work
makes ours much easier, and our trainees, whose keen curiosity continues
to be an endless source of inspiration.
10
Contents
Preface
Acknowledgments
PART 1 ■ IMAGING PROTOCOLS AND

GUIDELINES
1 CT Protocols
Brain without Contrast
Brain with Contrast Administration
Deep Brain Stimulator Head Protocol
Paranasal Sinus, Screening
Paranasal Sinuses with Contrast
Paranasal Sinuses, Preoperative for Computer Navigation
Maxillofacial without Contrast
Maxillofacial with IV Contrast
Orbits
Temporal Bones
Neck
CSF Leak
Craniosynostosis
Routine C-Spine
Routine T-/L-Spine
CTA Head
CTA Neck/Carotids
CT Perfusion
Pituitary Protocol
2 MRI Protocols
Brain without and with Contrast
11
Neonatal Brain
Brain, Stroke
Brain, Tumor
Brain, Trauma
Brain, Perfusion
Carotid Arteries, Neck
Venogram
Brain, Pulsatile Tinnitus
Pituitary
Neck, General
Temporomandibular Joints
Cervical Spine
Thoracic Spine
Lumbar Spine
Brachial Plexus
3 Myelography Protocols
General Guidelines
4 Digital Subtraction Angiography Protocols
General Guidelines
5 Sedation and Anxiolysis Protocols
Conscious Sedation
Anxiolysis
6 Medications in Neuroradiology
Medications for Contrast Media Reactions
Endotracheal Tubes
Prevention of contrast reactions in allergic patients
Management of contrast reactions
Medications That May Affect the Performance of Invasive Procedures
Medications (Generic Names) That May Lower Seizure Threshold
Over-the-Counter Medications That Increase Bleeding Time
CT Contrast Allergy
Iodinated Contrast in Renal Insufficiency
MR Contrast Administration in Adults (>18 Years of Age)
MR Contrast Administration in Children (<16 and >2 Years of Age)
Contrast Extravasation
12
PART 2 ■ IMAGING FUNDAMENTALS
SECTION A ■ Brain Imaging

7 Trauma
Arterial Dissection
Child Abuse
Contusions
Diffuse Axonal Injury and Intermediary Injuries
Epidural Hematoma
Pneumocephalus
Skull Fractures
Subdural Hematoma and Hygroma
Traumatic Subarachnoid Hemorrhage
8 Stroke
Acute Cerebellar Infarct
Acute (<24 Hours) Middle Cerebral Artery Infarct, CT
Acute (<24 Hours) Middle Cerebral Artery Infarct, MRI
Acute Anterior (ACA) and Posterior (PCA ) Cerebral Artery Infarcts
Basilar Artery Occlusion
Cadasil
Corpus Callosum Infarctions
Cortical Vein Thrombosis
Deep Venous System Occlusion
Generalized Brain Hypoxia/Ischemia
Fibromuscular Dysplasia (FMD)
Hemorrhagic Infarct and Hemorrhagic Transformation
Hypertensive Encephalopathy
Stenosis, Extracranial ICA
Lacunar Infarctions
Moyamoya
Subacute (2 to 21 Days) Middle Cerebral Artery Infarct, CT
Subacute Infarction, MRI
Cerebral Vasculitis
Venous Sinus Occlusion
Wallerian Degeneration
Watershed Cerebral Infarctions
13
9 Nontraumatic Hemorrhage
Acute Hypertensive Hemorrhages
Cerebral Amyloid Angiopathy
Cerebral Microhemorrhages
Hemorrhage in the Premature Brain
Periventricular Leukomalacia (PVL)
10 Aneurysms
Anterior Communicating (AComm) Artery Aneurysms
Basilar Artery Tip Aneurysms
Giant Aneurysms
Infratentorial Aneurysms
Middle Cerebral Artery Bifurcation Aneurysms
Multiple Intracranial Aneurysms
Posterior Communicating Artery Aneurysms
Pseudoaneurysms
Vasospasm (after SAH)
11 Vascular Malformations
Arteriovenous Malformations (AVM)
Capillary Telangiectasias
Carotid Artery–Cavernous Sinus Fistulas
Cavernous Malformations
Developmental Venous Anomalies (DVA)
Dural Arteriovenous Fistulas
Vein of Galen Malformations
12 Extra-Axial Masses
Arachnoid Cyst
Choroid Plexus Tumors
Colloid Cyst
Craniopharyngioma
Dermoid
Epidermoid
Lipoma
Meningioma
Pineal Gland Tumors
Pituitary Adenoma
13 Intra-Axial Tumors
14
Supratentorial Tumors
Anaplastic Astrocytoma
Ependymomas and Subependymomas
Glioblastoma
Gliomatosis Cerebri
Astrocytoma, Low Grade
Lymphoma
Metastases
Neuronal Tumors
Oligodendroglioma
Infratentorial Tumors
Brainstem Astrocytoma
Ependymoma and Subependymoma
Hemangioblastoma
Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease)
Medulloblastoma
Pilocytic Astrocytoma
14 Infections and Inflammations
Acute Disseminated Encephalomyelitis (ADEM)
Cerebral Pyogenic Abscess
Creutzfeldt-Jakob Disease (CJD)
Cryptococcus
Cysticercosis
Human Immunodeficiency Virus Infection
Meningitis, Complications
Meningitis (Uncomplicated)
Multiple Sclerosis
Neuromyelitis Optica
Progressive Multifocal Leukoencephalopathy (PML)
Rasmussen Encephalitis
Sarcoidosis
Tuberculosis
Toxoplasmosis
Viral Encephalitis
15 Leukodystrophies
Adrenoleukodystrophy
Alexander Disease
Canavan Disease
15
Krabbe Disease
Metachromatic Leukodystrophy
Pelizaeus-Merzbacher Disease
16 Metabolic Disorders
Amino Acid Disorders
Cerebral Calcifications
Mitochondrial Disorders
Mucopolysaccharidoses
Wernicke Encephalopathy
Wilson Disease (Hepatolenticular Degeneration)
17 Degenerative and Iatrogenic Disorders
Alzheimer Disease
Amyotrophic Lateral Sclerosis
Carbon Monoxide and Methanol Intoxication
Hippocampal Sclerosis
Huntington Disease (Chorea)
Nonketotic Hyperglycemia
Intracranial Hypotension
Liver Insufficiency and Total Parenteral Nutrition
Multisystem Atrophy
Osmotic Demyelination Syndrome
Pantothenate Kinase Deficiency (Hallervorden-Spatz Syndrome)
Pseudotumor Cerebri
Radiation Injury
18 Congenital Malformations
Agenesis of Corpus Callosum
Agyria/Pachygyria Complex and Band Heterotopia
Anterior Nasal Masses
Aqueductal Stenosis
Chiari Malformation Type I
Chiari Malformation Type II
Cortical Dysplasias
Craniosynostoses
Dandy-Walker Complex
Focal Gray Matter Heterotopias
Holoprosencephalies
Joubert Syndrome
16
Posterior (Occipital and/or Parietal) Encephaloceles
Schizencephalies
Septooptic Dysplasia
19 Neurocutaneous Syndromes
NF-1
NF-2
PHACE(S) Syndrome
Sturge-Weber Syndrome
Schwannomatosis (NF-3)
Tuberous Sclerosis
von Hippel-Lindau Disease
SECTION B ■ Spinal Imaging
20 Brachial Plexus
Inflammatory and Infectious Plexitis
Traumatic Brachial Plexus Injuries
Brachial Plexus Tumors
21 Congenital Malformations
Caudal Agenesis (Regression) Syndrome
Diastematomyelia (Split-Cord Malformation)
Dermoid and Epidermoid
Lipomas
Lipomyelomeningocele and Lipomyelocele
Myelomeningocele and Myelocele
22 Degenerative Spine
Annular Fissures
Facet Cysts
Herniated Disc
Ossified Posterior Longitudinal Ligament (OPLL)
Paget Disease
Schmorl Nodes
Spondylolysis and Spondylolisthesis, Lumbar Spine
23 Infection Inflammation
Ankylosing Spondylitis (Seronegative Spondyloarthropathy)
Arachnoiditis
Guillain-Barré Syndrome
17
Hypertrophic Polyneuropathies
Rheumatoid Arthritis
Spondylitis, Discitis, and Epidural Abscess, Pyogenic
Tuberculosis
24 Spine Tumors and Tumor-like Conditions
Aneurysmal Bone Cyst (ABC)
Spinal Cord Astrocytoma
Chordoma
Spinal Cord Cysts, Nonneoplastic
Eosinophilic Granuloma
Spinal Cord Ependymoma
Vertebral Body Hemangioma
Osteoid Osteoma
Perineural (Tarlov) Cysts
Spinal Schwannoma and Meningioma
Spinal Cord and Leptomeningeal Metastases
Subacute Combined Degeneration
Vertebral Metastases
25 Trauma
Occipitoatlantal Separation
Benign Compression Fractures
Pathologic (Malignant) Compression Fractures
Chance-Type Fractures
Facet Dislocation
Hangman Fracture
Jefferson Fracture
Odontoid Fractures
Spinal Cord Injury
Vertebral Artery Injury
26 Vascular Disorders
Spinal Cord Infarction
Spinal Epidural and Subdural Hematomas
Spinal Arteriovenous Fistula
Spinal Cord Arteriovenous Malformations
Spinal Cord Cavernous Malformation
SECTION C ■ Ear, Nose, and Throat Imaging
18
27 Neck Masses
Branchial Cleft Cysts
Buccal Space Masses
Carotid Space Masses
Fissural Cysts
Hemangiomas
Laryngeal Masses
Masticator Space Masses
Nasopharyngeal Masses
Nasopharyngeal Angiofibroma
Nodal Metastases
Oral Cavity and Oropharyngeal Space Masses
Paragangliomas (Skull Base)
Parapharyngeal Space Masses
Parotid Space Masses
Retropharyngeal Space Masses
Sialolithiasis
Slow-Flow Vascular Malformations
Sublingual and Submandibular Space Masses
Thyroglossal Duct Cyst
Thyroid Masses
Tornwaldt Cyst
28 Orbits
Globe Calcifications
Orbital Dermoid
Graves Ophthalmopathy
Orbital Cavernous Malformation (Hemangioma, Adult Type)
Infantile and Congenital Hemangiomas
Inflammatory Pseudotumor
Lacrimal Gland Masses
Orbital Lymphatic Malformation
Melanoma
Optic Neuritis
Orbital Cellulitis and Abscess
Orbital Cavity Trauma
Persistent Hyperplastic Primary Vitreous (PHPV)
Posttreatment Findings
Detachments and Effusions
Retinoblastoma (PNET-RB)
19
Ocular Trauma
29 Sinuses
Acute (Uncomplicated) Sinusitis
Anatomical Variants of the Sinonasal Cavity
Choanal Narrowing
CSF Leaks
Fibro-osseous Lesions of Sinonasal Cavities
Fungal Sinusitis
Papilloma
Mucocele
Mucous Retention Cysts
Ostiomeatal Unit, Obstruction
Sinonasal Polyps
Paranasal Sinus Malignancy
Granulomatosis with Polyangiitis (Wegener Granulomatosis)
30 Temporal Bone
Bell Palsy
Cholesteatoma, Acquired (Secondary)
Cholesterol Granuloma
Facial Nerve Schwannoma
Temporal Bone Fractures
Glomus Tympanicum
Labyrinthitis Obliterans
Otospongiosis
Vascular Anomalies and Variants, Middle Ear
Enlarged Endolymphatic Sac (Large Vestibular Aqueduct) Syndrome
Vestibular Schwannoma
Index
20
PART 1
Imaging Protocols and
Guidelines
As in the prior editions of this book, the protocols for CT, MRI, DSA, and
myelography are provided in this section. These protocols should serve as
general guidelines, may vary from those utilized in other imaging centers,
and need to be adapted to the needs of the readers and their patients. Care
Dose refers to our equipment radiation reduction feature. Indications refer
to the most common ones and are not all-inclusive. We have also included
the charge codes that we use.
21
CHAPTER 1
CT Protocols
Brain without Contrast

Purpose: Evaluation of subdural hematoma, epidural hematoma,
stroke, bleed, headaches, initial workup of dementia, mental status
changes, fractures, trauma, shunt malfunction, hydrocephalus.
Patient Position: Supine
Scan Type: Preferably sequence, spiral for trauma or if scanning head
and face
Image Acq: 4.5 mm (seq) or 5 mm (spiral) × 1.2 mm collimator (0.75 mm
collimator for trauma)
Scan Extent: Skull bases through vertex
FOV: Sized to include entire skull area
Technique:
Adults: kV: 120 Ref mAs: 250 Care Dose: ON
Peds: kV: 120 Care Dose: ON
Ref mAs:
0 to 6 months: 100 mAs

18 months to 3 years: 150 mAs
3 to 10 years: 175 mAs
>10 years: 200 mAs
Reconstructions:
4.8 or 5 mm axial cerebrum
4.8 or 5 mm axial bone
For trauma:
22
4.8 or 5 mm coronal cerebrum
4.8 or 5 mm coronal bone
4.8 or 5 mm sagittal cerebrum
4.8 or 5 mm sagittal bone
PACS: Transmit all images.

Charge Codes: 3510100
CPT Code: 70450
Note: CT of the head is generally obtained 10 to 20 degrees from Reid
base line (infraorbital rim to top of external auditory meatus) or parallel to
the hard palate. Soft tissue views of the posterior fossa are presented with a
window width of 110 to 120 Hounsfield units (HU) and a center (level) of
43 HU. In the supratentorial region, window width of 80 HU and center of
43 HU are helpful. Bone windows are presented with a width of 3,500 HU
and a center of 700 HU. Please note that all pediatric scans are done with
the low-radiation protocols as determined by the manufacturer and may
vary by scanner. In our units the average dose is as follows: 0 to 6 months
(effective mAs of 90), 6 months to 3 years (effective mAs of 150), and 3 to
6 years (effective mAs of 220).
Brain with Contrast Administration

Purpose: Evaluation of tumors, metastases, infection, vascular
malformations
Scan Type: Preferably sequence, spiral for trauma or if scanning head
and face
Image Acq: 4.8 mm (seq) or 5 mm (spiral) × 1.2 collimator pre- and
postcontrast (both image sets must be acquired the same way)
Scan Extent: Skull bases through vertex
Technique:
Peds: kV: 120 Care Dose: ON
Ref mAs:

18 months to 3 years: 150 mAs
23
3 to 10 years: 175 mAs
>10 years: 200 mAs
IV Contrast: After completion of unenhanced scan

Adults: 75 mL bolus Omni 350
Peds: Omni 300 1 mL per pound
Start postcontrast scan at least 3 minutes after completion of injection.
Reconstructions:
Precontrast: 4.8 or 5 mm axial cerebrum
Postcontrast: 4.8 or 5 mm axial cerebrum
4.8 or 5 mm axial bone
CPT Code: 70470
Note: We use high-concentration iodinated contrast (350 mg/mL) for all
contrast-enhanced CT studies except for pediatric patients where we
continue to use that with a concentration of 300 mg/mL.
Deep Brain Stimulator Head Protocol

Scan Type: Spiral
Image Acq: 1.0 × 0.6 mm collimator
Scan Extent: Secure head if the patient has a frame; if the patient comes
w/o frame use the head holder to position the patient and make sure to scan
3 cm above the top of the head.
Gantry Tilt: NONE
FOV: 300 or 320. Include all nine rods inside FOV if the patient has a
frame. If the patient comes w/o frame, scan 3 cm above top of the head.
Technique: kV: 120 Ref mAs: 380 Care Dose: OFF
IV Contrast: Per doctor’s instructions.
Reconstructions: 1 mm axial cerebrum
PACS: Transmit axial images.
Charge Codes: 3510100 (without contrast) or 3510110 (with contrast)
CPT Code: 70450 (without contrast) or 70460 (with contrast)
Paranasal Sinus, Screening

24
Purpose: Evaluation for sinusitis
Patient Position: Supine (tabletop, no headholder, eyes closed)
Scan Type: Spiral
Scan Extent: Frontal sinuses through hard palate (scan craniocaudal)
FOV: Sized to include entire facial area, including tip of nose
Technique:
Adults: kV: 120 Ref mAs:120 Care Dose: ON
Peds: kV: 120 Ref mAs: 25 Care Dose: ON
IV Contrast: None
Reconstructions:
0.75 mm axial soft tissue
2 mm axial bone
2 mm coronal bone
2 mm sagittal Bone
CPT Code: 70486
Note: Coronal CT scans of the sinonasal cavities are presented with bone
windows at a width of 3,500 HU and a center of 700 HU and processed
with high-resolution bone algorithm.
Paranasal Sinuses with Contrast

Purpose: Evaluation of sinus cavity for tumors, masses, invasive
sinusitis, suspicion of abscess
Scan Extent: Hard palate through frontal sinuses
Scan Type: Spiral
Image Acq: 3 mm × 0.6 collimator
Care Dose: No in adults, yes in children
FOV: Sized to include entire facial area
Algorithm: Soft tissue. Bone algorithm
Bolus Tracking: None
IV Contrast: 75 mL bolus
Reconstructions:
2 mm axial soft tissue
25
2 mm axial bone
2 mm coronal soft tissue
2 mm coronal bone
2 mm sagittal bone
Charge Codes: 3511342 and 3510020
Paranasal Sinuses, Preoperative for

Computer Navigation
Purpose: Preoperative
Patient Position: Supine (tabletop, no headholder, eyes closed)
Scan Extent: Frontal sinuses through hard palate (scan top-down)
Scan Type: Spiral
Image Acq: 0.75 mm × 0.6 collimator
FOV: Sized to include entire facial area, including tip of nose
Technique:
Peds: kV 120 Ref mAs: 25 Care Dose: ON
IV Contrast: None
Reconstructions: 0.7 mm axial soft tissue
2 mm axial bone
2 mm coronal bone
2 mm sagittal bone
Maxillofacial without Contrast

Purpose: Evaluation for fractures
Scan Type: Spiral
Image Acq: 3 × 0.6 mm collimator
Scan Extent: Frontal supraorbital ridge through mandibular symphysis
FOV: Sized to include entire mandible and facial area
Technique:
26
IV Contrast: None
Reconstructions:
3 mm axial bone
3 mm coronal bone
3 mm sagittal bone
(Can create MPRs in 3-D)
CPT Code: 70486
Maxillofacial with IV Contrast

Purpose: Evaluation of face for tumors, masses, etc.
Scan Type: Spiral
Scan Extent: Frontal supraorbital ridge through mandibular symphysis
FOV: Sized to include entire mandible and facial area
Technique:
IV Contrast:
Reconstructions:
3 mm axial bone
3 mm coronal bone
3 mm sagittal bone
(Can create MPRs in 3D)
CPT Code: 70487
27
Orbits
Purpose: Evaluation of fractures, tumors, cellulitis
Scan Type: Spiral
Scan Extent: Supraorbital ridge through hard palate.
Include entire orbit and all pathology.
FOV: Sized to include entire orbital/facial area
Technique:
IV Contrast: If suspected infection, tumor, cellulitis.
Adults: 75 mL Omni 350
Reconstructions:
2 mm axial bone
2 mm coronal bone
2 mm sagittal bone
Charge Codes: 3510150 (with) and 3511402 or 3510140 (without)
CPT Code: 70481 (with) or 70480 (without)
Temporal Bones
Purpose: Evaluation for cholesteatoma, hearing loss, fractures, etc.
Scan Type: Spiral
Scan Extent: At least 15 mm above and below the IAC for both axials and
direct coronals
Control Scan: Through level of IACs
FOV: Use control scan to set up FOVs for both R and L temporal
Technique:
28
IV Contrast: Per protocol
Reconstructions:
Right T. bone 0.6 mm axial inner ear
Left T. bone 0.6 mm axial inner ear
Right T. bone 0.6 mm coronal inner ear
Left T. bone 0.6 mm coronal inner ear
CPT Code: 70480
Neck
Purpose: Evaluation of neck for tumors, masses, etc.
Scan Type: Spiral
Scan Extent: Top of sella to pulmonary hila
FOV:
Sized to include face and neck. (Do not clip chin or nose.)
Use butterflies to angle around dental fillings.
Technique:
IV Contrast:
Reconstructions:
3 mm axial bone
3 mm coronal soft tissue
3 mm sagittal soft tissue
CPT Code: 70491
Note: Neck studies are generally presented with soft tissue windows using
a width of 250 to 270 HU and a center of 70 to 90. Bone windows are
presented with a width of 3,500 to 4,000 and a center of 700.
29
CSF Leak
Purpose:
Evaluation of CSF leak
Usually done after intrathecal contrast
Scan Type: Spiral
Scan Extent: Posterior sphenoid sinus through frontal sinus
Technique: kV: 120 Ref mAs: 120 Care Dose: ON
IV Contrast: None
Reconstructions:
0.75 mm axial bone
0.75 mm coronal soft tissue
0.75 mm coronal bone
Charge Codes:
3511341 preintrathecal contrast
3511342 postintrathecal contrast
CPT Code: 70486 (preintrathecal) or 70487 (postintrathecal)
Craniosynostosis
Purpose: Evaluation of cranial sutures
Scan Type: Spiral
Scan Extent: Below skull bases through vertex (get air shot)
Technique:
IV Contrast: None
Reconstructions:
5 mm axial cerebrum
2 mm axial bone
30
1 × 0.5 mm soft tissue overlaps for 3D shaded-surface display (SSD)
Post-processing: From overlaps, recon SSD of skull.
PACS: Transmit axial ST, BW and 3D SSD images.
CPT Code: 70450, 76376
Routine C-Spine
Purpose: Evaluation for HNP, stenosis, fractures, etc.
Scan Type: Spiral
Scan Extent: Foramen magnum through T1
FOV: Sized to include entire vertebrae and spinous processes
Technique:
Reconstructions:
2 mm axial bone
2 mm coronal bone
2 mm sagittal bone
PACS: Transmit all images and topogram
CPT Code: 72125
Note: Spine CT studies are presented with bone windows using a width of
4,000 HU and a center of 700 HU. Soft tissue windows are presented with
a width of 400 HU and a center of 50 HU. These values are not absolute
and may need to be optimized in some cases. Same values apply to
postmyelogram spinal CT studies.
Routine T-/L-Spine
Purpose: Evaluation for HNP, stenosis, fxs, etc.
Scan Type: Spiral
31
Scan Extent: Include at least half of vertebra above and below area of
interest.
FOV: Sized to include entire vertebrae and spinous processes.
Technique:
Reconstructions:
3 mm axial bone window
3 mm coronal bone window
3 mm sagittal bone window
PACS: Transmit all images and topogram
Charge Codes:
T-spine 3510230 (without contrast)
T-spine 3510240 (with contrast)
L spine 3510190 (without contrast)
L-spine 3510200 (with contrast)
CTA Head
Purpose: Evaluation for arterial pathology
Scan Type: Spiral
Scan Extent: Caudocranial from the skull base to 2 in. above the frontal
sinuses. If evaluating a known hemorrhage or suspected vascular
malformation, be sure to include the entire extent of the lesion.
FOV: Decreased FOV to include circle of Willis
IV Contrast: 75 mL Omni 350 with a 50 mL saline chaser with an
injection rate of 4 mL/s (18 g PIV, 20 g PIV in the antecubital fossa or
access approved for the injection rate)
Bolus Tracking: Place ROI in the ascending aorta
(Do not place ROI on calcification or artifact)
Reconstructions:
32
0.6 × 0.3 mm thin MIP overlaps
A) 1 × 1 mm axial soft tissue
B) 1 × 1 mm coronal soft tissue
C) 1 × 1 mm sagittal soft tissue
D) 1 × 1 mm coronal soft tissue angled with basilar artery
E) 20 × 3 mm axial thin MIP
F) 20 × 3 mm coronal thin MIP
G) 20 × 3 mm sagittal thin MIP
H) VRT of the COW
PACS: 1 mm axials, MPRs, VRTs and all thin MIPS
CPT Code: 70496
CTA Neck/Carotids
Purpose: Evaluation for arterial pathology.
Scan Type: Spiral
Scan Extent: Scan caudocranial from the aortic arch to above sella
FOV: To include all anatomy of interest.
IV Contrast: 75 mL Omni 350 with a 50 mL saline chaser with an
injection rate of 4 mL/s (18 g PIV, 20 g PIV in the AC or access approved
for this injection rate)
Bolus Tracking: Place ROI in the ascending aorta
Do not place ROI on calcification or artifact.
Reconstructions:
0.6 × 0.3 mm thin MIP overlaps
A) 1.5 × 1.5 mm axial soft tissue
B) 1.5 × 1.5 mm coronal soft tissue
C) 1.5 × 1.5 mm sagittal soft tissue
D) 1.5 × 1.5 mm oblique sagittal soft tissue for both carotids to show
the bifurcation.
E) VRT of the carotids
33
PACS: Transmit 1.5-mm axial images, VRT, and all MPRs.
CT Perfusion
Purpose: Evaluate stroke and/or vasospasm.
Patient Position: Supine. Chin down. Make use of positioning
devices in order to reduce motion as much as possible. Try to position
head as symmetrically as possible.
Scan Type: Spiral and dynamic
Image Acq: 9.6 mm × 1.2 collimator (perfusion)
Scan Extent: Determined by radiologist. Scan takes 40 images at three
different levels for a total of 120 images.
Technique: kV: 80 mAs: 150 Care Dose: ON
IV Contrast: 40 mL Omni 350 (unless otherwise noted) at 5 mL/s max
per/s
*18GA antecubital IV mandatory
Bolus Tracking: Place ROI in the aortic arch.
Reconstructions: 9.6 mm axial cerebrum
Postprocessing: As per perfusion software provided by specific
manufacturer
PACS: All axial and perfusion images
CPT Code: 70496
Pituitary Protocol
Purpose: Evaluation of pituitary tumors in patients that cannot have
an MRI
Patient Position: Preferably prone for direct coronal acquisition. Do
supine if prone can’t be tolerated, patient has dental work or is unable
to hyperextend neck.
Scan Type: Preferably sequential for direct coronal acquisition
Spiral if images are acquired with patient supine
Image Acq: Direct coronal 1.2 mm × 0.6 collimator
Control Scan: Through level of sella to set FOV
Scan Extent: 30 mm centered on pituitary/sella
34
FOV: Include all pituitary.
IV Contrast: 75 mL Omni 350 w-30 second delay, injection rate of 2
mL/s
Reconstructions:
1.2 mm bone
1.2 mm soft tissue
If acquired supine and spiral, reformat 1 mm coronals in bone and soft
tissue
Charge Codes: 3510150, add 3511402 if you do coronal MPRs
CPT Code: 70481
35
CHAPTER 2
MRI Protocols
The following is a sample of the most commonly performed neuro-MRI

studies. They do not include all of the technical parameters but only those
that we feel should serve the reader to familiarize himself/herself with
them. They are based on what we do on our Siemens equipment and thus
may be different to those provided by other manufacturers. Because of the
“teaching” nature of our institution, they may be longer than those
obtained in other settings.
Brain without and with Contrast
#SL, number of slices; PH DIR, phase direction; FOW, field of view in millimeters;
THK, slice thickness in millimeters; TR, repetition time in milliseconds; TE, echo time
in milliseconds; AVG, number of averages; FLIP, flip angle; SAT, type of saturation;
TI, inversion time in milliseconds; RES, resolution in phase-encoding direction; RES%,
resolution in frequency encoding direction expressed as percentage of resolution (100%
results in rectangular FOV); BW, band width.
36
Neonatal Brain
Brain, Stroke
Brain, Tumor
37
*ASL, arterial spin–labeled perfusion may be used additionally to contrast-enhanced
perfusion or as a substitute to it.
†See perfusion-specific protocol.
Brain, Trauma
*ASL, arterial spin labeled perfusion may be used additionally to contrast-enhanced

perfusion or as a substitute to it.
†See perfusion-specific protocol.
Brain, Perfusion
Carotid Arteries, Neck
38
Venogram
Brain, Pulsatile Tinnitus
*MRA centered at base of skull.
Pituitary
*Startdynamic coronal scan with contrast injection, to be repeated every 30 seconds for
3 minutes.
39
Neck, General
Temporomandibular Joints
*Obtain with mouth in open and closed positions.
Cervical Spine
Thoracic Spine
40
Lumbar Spine
Brachial Plexus
41
Cranial nerves: MRI protocols for the evaluation of each of the cranial
nerves are beyond the scope of this book. For general information
regarding these protocols I refer the reader to the following article:
Mukherji SK, Castillo M, Daughtry LH. MR imaging protocols for
evaluation of cranial neuropathies. Top Magn Reson Imaging 1996;8:187.
42
CHAPTER 3
Myelography Protocols
General Guidelines
1. Most patients are scheduled for this procedure. If there are any
emergencies, discuss the case with the neuroradiology attending staff
before sending for the patient.
2. Include the following possible risks and complications on the consent
form and have the patient acknowledge appropriate areas: headache;
bleeding; infection; seizures; nausea; vomiting; damage to nerves;
paralysis; bowel, bladder, and muscle dysfunction; allergic reaction to
contrast; and death. If the patient has a clotting disorder or low
platelets (platelets need to be at least 50,000 to perform a lumbar
puncture), be especially careful. Myelography on anticoagulated
patients is usually avoided, if possible.
3. Check the patient’s chart for allergies, as well as whether the patient
is taking any of the medications listed in Chapter 6 that may lower the
seizure threshold (although not an absolute contraindication since the
introduction of nonionic hypo-osmolar contrast media). Some
physicians will stop these medications before myelography (the risk
of seizures is <1%), while some may perform the study without
discontinuing such medications. The inserts for iohexol and
iopamidol approach this issue in such a way that it leaves the decision
to the physician. We currently do not discontinue these medications
prior to myelography. Patients taking platelet inhibitors are at risk for
epidural hematoma and subarachnoid hemorrhage from a lumbar or
cervical puncture. Aspirin and Plavix should be discontinued at least
5 days before myelography. Thienopyridine derivatives such as
clopidogrel and ticlopidine should be discontinued 7 and 14 days
43
before myelography, respectively. Anti-inflammatory drugs with mild
platelet inhibition such as ibuprofen are not a contraindication to
lumbar puncture. If there is no opportunity to discontinue an
antiplatelet medication before myelography, then the use of a small-
bore needle (22 gauge) is advised. Low molecular weight heparin
should be discontinued for at least 12 hours if being given for
prophylaxis or 24 hours if treatment dosing. Unfractioned intravenous
heparin is withheld for 2 to 4 hours, and the procedure can be
performed after a normal aPTT is documented. Unfractioned
subcutaneous heparin is not a contraindication for doses <10,000
units per day; if higher, treat as if intravenous. INR should be 1.5 or
less.
4. One of the most common problems during a myelogram is a
vasovagal reaction, that is, the patient has nausea, sweating, cold
clammy appearance, hypotension, and bradycardia. Be prepared to
treat it with IV fluids and atropine. In some instances (particularly
with young males who seem to have more vasovagal reactions), it is
advisable to place an IV line before the myelogram.
5. Usually inject at L2-L3. Remember that the majority of pathology is
at the L4-L5 or L5-S1 levels. Stay away from higher levels because
that is where the conus is located (i.e., T12-L1 or L1-L2). When
injecting, use the “puff method” and look for the contrast to flow
downward outlining the thecal sac and nerve root sleeves with the
table angled slightly caudally. If there is a question about the injection
being subdural, leave the needle in, do not inject any more media, and
call attending staff. A lateral cross-table radiograph will clearly show
the position of the needle and of the injected contrast. We always
begin with a 22-gauge spinal needle. We use a standard bevel-tip
needle, but a blunt-tip needle may also be used.
6. For pediatric patients, talk with attending staff first because general
anesthesia is usually used. Find out if the patient has had an MR
imaging study first (usually, the MR should be done before the
myelogram as it almost always answers the question).
7. All patients have a CT study after the myelogram. Inform the CT
technologist what levels need to be scanned based on the findings of
the conventional myelogram.
8. Ask women about the possibility of pregnancy. If unsure, get a urine
pregnancy test stat.
9. When doing a cervical myelogram from the lumbar route, if you see
contrast outlining the basilar artery along the clivus, or if the patient
44
complains of headache, tilt the patient back down because contrast
media is going up into the head and may induce seizures.
10. Suggested usage of contrast media myelography in adults is shown
in the following table.
*Total dose of contrast should not exceed 3.0 g of iodine. For cervical
myelograms from a lumbar approach, use 10 mL of 300 mg/mL.
11. If a complete myelogram is requested, do the thoracic area last

(unless worried about a block, which usually occurs in the upper
thoracic area and is encountered when running contrast media up to
the cervical area). In cases of suspected spinal cord compression,
inject contrast, to rule out cord compression, before removing any
CSF. In the presence of a compressive lesion, removal of CSF may
worsen the symptoms. However, contrast mixed with CSF does not
hinder cytology of this fluid.
12. C1-C2 punctures may be done to outline the superior aspect in a
region of cord compression or when lumbar access is not available
(e.g., in patients with severe spondylosis). With the patient in a prone
position with the neck mildly flexed (so that the upper cord and brain
stem are displaced anteriorly) and using cross-table fluoroscopy, a 22-
g spinal needle is introduced into the posterior third of the spinal
canal at the C1-C2 interspace. If the needle is in the subarachnoid
space, there is always CSF return. Contrast is injected in order to fill
completely the cervical canal. The amount of contrast needed is
variable, but it is safe to stop injecting it when it reaches the inferior
tip of the clivus.
Lumbar Myelograms
1. Scout AP and lateral lumbar views (done by technologist).
2. Three spots (shallow and steep oblique and right lateral) and three
spots opposite side (shallow and steep oblique and left lateral).
Lateral decubitus positioning may be helpful in patients with
45
scoliosis.
3. Cross-table lateral views (done by technologist).
4. Spot lateral flexion and extension with patient standing (for those
with subluxations, spondylolysis, or any other pathology that affects
stability).
5. Supine AP of conus (done last by technologist). View of conus may
also be done with fluoroscopic spot film. This last radiograph may be
skipped if the patient has had a prior MR imaging study showing the
position of the conus medullaris.
6. Since all patients should have a postmyelogram CT, the radiographic
protocol at the time of the injection can be variable and can be
tailored from patient to patient. The above described technique can be
considered the “classic” one and should be routinely used when
patients will not undergo postmyelogram CT.
Cervical Myelograms
1. Place a number of towels (or special head holder) under the chin, and
take scout AP, lateral, and swimmer’s views (done by technologist).
Neck extension should be kept to a minimum to avoid compression of
the spinal cord by osteophytes or other lesions. Development or
worsening of the symptoms in this position may preclude this
examination. In these patients, we have performed the cervical
myelograms in a lateral decubitus position.
2. Prone spot cervical spine.
3. Coned spot film of occiput and foramen magnum.
4. Spots for both oblique cervical (shallow and steep obliques may also
be needed).
5. Overhead horizontal beam oblique is optional.
6. Overhead cross-table lateral and swimmer’s views (done by
technologist).
a. The two most important films in the cervical myelogram are the
prone PA spot done by the radiologist and the cross-table lateral
done by the technologist.
b. With cervical myelograms, the contrast dissipates fairly quickly,
so the films must be taken quickly. CT follows immediately.
c. If syringomyelia is suspected, scanning 6 hours after the
myelogram may demonstrate filling of the cavity with contrast
material. This is however rarely needed because most of these
patients are examined with MR imaging.
46
d. It is critical to perform this procedure with lateral fluoroscopy to
assure adequate placement of the needle. Be sure that the
projection is a true lateral one with the mandibles perfectly
aligned.
Thoracic Myelograms
1. Scout AP, lateral, and swimmer’s views to see upper T-spine (done
by technologist).
2. Usually just a thoracic myelogram will not be done, that is, it will be
part of a complete myelogram.
3. Injection of contrast material may be done with patient in lateral
decubitus position with the head elevated and with the shoulder
braced so that contrast goes to the thoracic area first and not to the
lumbar area, as this will dilute the contrast media. Contrast material
can also be guided fluoroscopically but may become diluted.
4. Lateral PA spots.
5. Right and left lateral decubitus cross-table, PA horizontal beam, and
supine PA overhead films (done by technologist).
6. If a block is encountered, mark the upper and lower level of the block
on skin surface with a permanent marker to aid in radiation therapy or
surgery.
7. If a complete block is encountered, a C1-C2 puncture may be needed
to outline the superior margin of the block. However, CT usually
shows some contrast cephalad to “blocks.”
9. If the examination is being performed to evaluate a potential
arachnoid cyst, the patient must be transported promptly to CT as the
cyst will progressively fill in with contrast and may not be visible. An
alternative for the assessment of arachnoid cysts is CISS imaging or
similar MRI techniques.
47
Postmyelogram Orders
Printed orders are available in many radiology departments. If they are
handwritten, be sure to include the following:
1. Seizure precautions for 8 hours.
2. Keep head elevated at least at 45 degrees for 8 hours or until
discharge, if outpatient.
3. Encourage PO fluids for 8 hours.
4. Avoid phenothiazines, tricyclics, MAO inhibitors, and other drugs
that lower the seizure threshold for 48 hours. They can usually be
restarted 24 hours after the procedure.
5. If the patient is an outpatient and the myelogram was performed in
the morning, we generally discharge them and let them go back home
in the late afternoon (usually after about a 4- to 6-hour period of
observation). Patients are instructed not to drive and to avoid weight
bearing that day.
Postmyelography Headache
1. It is the most common complication of myelography.
2. The headache is occipital or frontal; is postural, relieved by lying
supine, and exacerbated by sitting/standing; and may be accompanied
by nausea, vomiting, visual disturbances, tinnitus, and hearing
difficulties.
3. It is due to persistent drainage of CSF through the puncture site,
which results in compensatory dilatation of pain-sensitive intracranial
structures.
4. Most begin in the first 48 hours after the procedure and last 3 to 5
days.
5. For mild headaches, bed rest and symptomatic treatment are used.
6. For severe and/or persistent headaches, more aggressive symptomatic
treatment and an epidural blood patch may be used. An epidural
blood patch results in improvement of symptoms in over 80% of
patients. At our institution, the epidural blood patch is given by the
anesthesiologists, but in other centers, the radiologists administer this
treatment.
48
CHAPTER 4
Digital Subtraction
Angiography Protocols
General Guidelines
1. The persons performing the procedure should review the patient’s
chart and familiarize themselves with his/her problems. Particular
attention should be paid to prior noninvasive neurovascular studies
(i.e., MRA, CTA, and sonograms).
2. Consent should be obtained before the patient is brought to the
angiography suite. Risks and complications include bleeding,
infection, damage to nerves and blood vessels, paralysis, difficulty
with speech and vision, stroke, allergic reaction, and death.
3. If the patient is on heparin drip, have the drip discontinued ~4 hours
before the angiogram to allow the patient’s PT/PTT to normalize. If
possible, oral anticoagulants should be discontinued ideally 7 days
(minimum 3 days) before procedure and then the PT/PTT and INR
checked. If the angiogram needs to be performed on an emergency
basis, fresh frozen plasma may need to be administered to a patient on
warfarin. For patients taking aspirin, bleeding time should also be
obtained. If possible, discontinue aspirin 7 to 10 days before
procedure. Many angiograms need to be done in anticoagulated
patients to avoid ischemic complications; the risk of groin hematomas
is higher in such patients.
4. If previous allergic reactions have been severe (involving respiratory
or cardiovascular system or angioneurotic edema), arrange for
anesthesiologist to stand by.
49
5. Assess the patient’s femoral, popliteal, and dorsalis pedis pulses, and
as a rule of thumb, puncture the groin with the strongest pulse. We
generally puncture the right side due to the setup of the angiographic
equipment. If the patient is hemiplegic, puncture that side as it will be
least prone to motion after the angiogram.
6. Always inject first the vessels suspected to best demonstrate the
pathology.
7. As a rough rule, most neuroangiographic catheters measure between
90 and 120 cm in length. The use of the following preformed
catheters is suggested in the following situations:
a. Aortic arch—5-Fr (French) pigtail (however, any other 5-Fr
single-end–hole catheter being used usually suffices).
b. Cerebral angiogram in young adult patient—5-Fr or 7-Fr
Berenstein, H1H, or JB2.
c. Cerebral angiogram in older patient—5- or 7-Fr Simmons 1, 2, or
3, H1H, or Berenstein.
d. If you cannot get a vessel before switching catheters, try a
different wire first.
e. When having problems with the:
i. Left vertebral artery, try using H1H or Berenstein catheters.
ii. Left common carotid artery (CCA), try using a Newton or
Simmons 1 or 2 catheter.
f. In carotid stenoses workup, consider a reverse curve catheter, such
as a Simmons 2 or 3, in order to avoid passage of guide wire to or
through the stenotic bifurcation area. Be careful and avoid
formation of “knots.”
8. Nonionic contrast media is used for all neuroangiograms. Those with
the lowest osmolarity are best for injections of the external carotid
artery as they cause less pain. Be sure to eliminate microbubbles from
the syringe prior to injection.
a. Adults:
*Filming sequence is 2 to 4 frames per second using digital subtraction equipment. If

rotational digital angiography and/or 3-D are used, higher doses of contrast material and
longer injections are needed (check manufacturer’s instructions). Vascular
50
malformations and other high-flow lesions may require faster filming rates. Severely
stenotic or occluded vessels may require slower filming rates. CCA, common carotid
artery; ECA, external carotid artery; ICA, internal carotid artery.
b. Children:
Dosage may have to be tailored; remember that children have a
very dynamic flow and may require slightly higher doses than
those mentioned above.
Amounts of contrast media given above are the total dosage and
need to be given over 2 to 3 seconds.
Filming rates may need to be faster than those given for adults.
If the patient is intubated, hyperventilation may slow cerebral
blood flow and result in higher-quality films.
VA, vertebral artery.
9. If the patient is complaining of stroke-like symptoms, do no do

further catheter manipulations, pull the catheter out of the vessel to a
position in the lower descending aorta, call neuroradiology attending
staff, and also call the clinician taking care of the patient. We
generally transport these patients to the MR imaging unit and obtain
diffusion-weighted imaging to rule out an infarction.
10. Make sure all patients have the following laboratory tests before
doing a cerebral angiogram:
a. BUN/serum creatinine (NL: 8 to 20/0.8 to 1.0)
b. PT/PTT (NL: 10 to 12.4/21.5 to 31.9)
c. Bleeding time (total clotting time, NL: 9.8 to 14.0)
d. Hb/HCT and platelets (NL: 13.4 to 17.4/40 to 54 and 150 to 440).
e. INR (preferably ≤1.5)
11. In preangiogram note, briefly describe:
a. The reason for angiogram and what the CT, MR (MRA), or
carotid Doppler sonogram showed (if the patient has had these
studies).
b. Significant PMH (e.g., diabetes, hypertension, or migraine).
51
c. Allergies.
d. Above laboratory values (if not already available, order the
necessary tests).
e. Status of the patient’s peripheral pulses and if there are any
carotid bruits (if you have a stethoscope handy).
f. That the procedure was explained to the patient, as well as the
risks and benefits (including but not limited to bleeding, infection,
damage to the nerves and vessels, paralysis, difficulty with speech
and vision [i.e., stroke], and death). Indicate that all the patient’s
questions were answered and that he or she gave verbal and
written consent (signed, witnessed, and placed in the chart).
g. That if the patient is not capable of giving consent for the
angiogram (e.g., comatose) and there is no immediate family
available, the physicians responsible for the patient have acted on
his/her behalf and given consent. Policies vary from hospital to
hospital, so please refer to your institution’s guidelines.
h. Have the consent form witnessed.
12. If the patient is hypertensive, the diastolic pressure must be below
110 (if above, a cerebral angiogram is contraindicated because it will
be difficult to stop bleeding from the puncture site after the catheter is
removed).
13. In young females, check for the possibility of pregnancy. If unsure,
do a pregnancy test immediately.
14. Many radiology departments have printed preangiogram orders. The
following are suggested if these are not available:
a. Only clear liquids after midnight and NPO 2 hours before
procedure.
b. Void before being called to the radiology department.
c. Shave and prep site of expected puncture (this is sometimes done
in the radiology department).
d. Start IV fluids before angiogram, 1/2 normal saline at 75 mL/hour
to be continued for the duration of the procedure.
e. If not already available, order BUN/creatinine clearance, PT/PTT,
bleeding time, INR, Hb/HCT, and platelet tests.
15. For vasculitis workup, start by injecting one ICA; if abnormal the
study may be stopped, and if normal, continue with other vessels.
16. For carotid stenosis, arch study is optional, a minimum of frontal
and lateral views of the neck (obliques are suggested but not need of
rotational/3-D angiography is being performed), and frontal and
lateral views of the head (to evaluate the ICA siphon).
52
17. For aneurysm workups:
a. May need to do cross-compression of the ICA opposite to one
being injected (if there is no crossover flow via anterior
communicating artery to opposite anterior cerebral artery) in order
to better visualize the anterior communicating artery.
b. Also, to better visualize the anterior communicating artery and
anterior cerebral arteries near the midline, do an opposite oblique
to the carotid being injected, for example, right ICA injection with
the head turned to the left ~30 degrees.
c. May need 3-D DSA to clearly visualize neck and vessels arising
from the aneurysm.
18. For vertebral arteries, keep the catheter low in the vessel, and do the
test injection to observe the size and flow rate. If these are of medium
or small size or if a decreased flow rate is noted, pull the catheter out
of the vessel as soon as the injector has completed its full injection
(do not wait until film sequence is over). If the artery terminates in
the posterior inferior cerebellar artery (PICA), inject very gently.
Manipulation of catheters in the vertebral arteries commonly results
in vasospasm. In the presence of vasospasm, withdraw the catheter
from that vessel. Most vasospasm resolves spontaneously in 5 to 10
minutes.
19. In the postangiogram notes include the procedure performed,
preliminary findings, complications, and status of the patient’s
peripheral pulses (e.g., palpable or nonpalpable femoral, popliteal,
dorsalis pedis pulses). Also, mark the dorsalis pedis and posterior
tibial pulses with an ink pen on the patient’s feet to help the nurses
who check the vital signs and pulses after the procedure.
20. Postangiogram orders may be preprinted. If not available, the
following are suggested:
a. Enforce strict bed rest with leg or arm (whichever one was used)
straight for 6 hours.
b. Enforce bed rest with bathroom privileges for next 6 hours.
c. Order normal diet, and encourage fluids PO for 12 hours.
d. Take vital signs, and check the groin for hematoma or bleeding
and pulses peripheral to puncture site q15 minutes for 4 hours,
then q30 minutes for 4 hours, and then only routine vital signs.
e. Call radiology (or neuroradiology) house officer if complications
occur or with questions.
21. If patients are outpatients, they can be discharged to their home in
the late afternoon after an observation period of 6 hours and only if
53
they are stable.
22. Anticoagulation during a procedure is obtained in adults by giving a
bolus of 5,000 units of heparin IV. The optimal activated clotting
time is 1.5 to 2.5 times that of baseline. Anticoagulation is not
generally needed for diagnostic neuroangiography. Heparinization
may be reversed by giving 10 mg of protamine sulfate for every 1,000
units of heparin administered IV over a 10 minute period.
*Significant hematomas occur in 5% to 10% of all cerebral angiograms.
This risk is higher in brachial/axillary punctures than in groin ones.
Hematomas judged to contain the equivalent to one unit of blood generally
will require exploration for repair of the vessel.
54
CHAPTER 5
Sedation and
Anxiolysis Protocols
Conscious Sedation
Pre and Intraprocedural Care
At our institution, conscious sedation is now used routinely. It is
administered by either two registered nurses or anesthesia technologists
under supervision of a physician. The goals of conscious sedation are to
only minimally depress the level of the patient’s consciousness, to
maintain a patent airway, and to allow the patient to respond to commands.
Generally, emergency equipment such as oxygen, suction, monitors, bag-
valve, mask, and resuscitation drugs should be readily available. The
patient should have an empty stomach (adults: NPO 4 to 6 hours before
sedation, children: NPO 2 hours before sedation). During sedation,
continuous monitoring of vital signs and oxygen saturation (normally
above 95%) is needed. We record vital signs and oxygen saturation every
15 minutes. The level of consciousness of a patient is also continuously
evaluated and recorded every 15 minutes according to a sedation scale as
follows:
55
The following is a list of the most common drugs used for conscious
sedation at our institution. This list details drugs administered
intravenously. All drugs should be given slowly.
Parenteral Drugs
Pentobarbital Sodium (Nembutal)
This medication results in rapid sedation given via either IV or IM routes.
Its side effects include respiratory and cardiovascular depression. It should
be avoided in patients with hepatic insufficiency. If given IV, its onset is
immediate, and when given IM its onset is within 10 to 15 minutes. Its
effect lasts 15 to 20 minutes. We do not use it in children <6 months of
age.
Morphine Sulfate
This medication is used for sedation and/or severe pain. It may result in
nausea/vomiting and respiratory depression. Its antagonist is naloxone
(Narcan). It should not be given to patients with increased intracranial
pressure or respiratory depression. Its effects peak between 20 and 60
minutes and may last 3 to 5 hours.
56
Fentanyl
This medication is used for pain and/or sedation. It may result in similar
side effects as morphine, and its antagonist is also Narcan. Its onset is
immediate, and its effects last 30 to 60 minutes.
Meperidine (Demerol)
This medication is used for patients with moderate-to-severe pain. Its side
effects are similar to those of morphine and its antagonist is naloxone
(Narcan). Its onset is within 5 minutes of administration and its effect lasts
2 to 4 hours.
Midazolam (Versed)
The medication is used for sedation. It may result in cardiac and
respiratory depression. Its antagonist is flumazenil. Its onset of action is
within 1 to 5 minutes after administration, and its effect lasts 2 to 6 hours.
Diazepam (Valium)
57
Its indications, antagonist, and onset of action are similar to midazolam.
The duration of its effect is 1 to 2 hours.
Lorazepam (Ativan)
Its indications and antagonist are similar to midazolam. Its onset of action
is 15 minutes and its effect lasts 30 to 180 minutes.
Oral Drugs
In children <2 years of age we begin by giving oral sedation. If this type of
sedation fails, then we administer parenteral sedation. These medications
should be given 30 to 60 minutes before the procedure.
Chloral Hydrate
This drug is used for nonpainful procedures. Its side effects include
gastrointestinal upset and paradoxical excitement. It is contraindicated in
patients with liver/renal insufficiencies. Its effect peaks between 30 and 60
minutes and may last up to 8 hours. It may also be given per rectum.
58
Flumazenil (Romazicon)
This medication may reverse the effects of midazolam, diazepam, and
lorazepam. It may result in nausea/vomiting, cardiac arrhythmias, blurred
vision, and seizures.
59
Topical Medications
Use topical lidocaine particularly in children undergoing oral sedation. We
have found it very useful when inserting an IV line.
Lidocaine 2.5% and Prilocaine 2.5% (EMLA Cream)

This drug is to be used only on normal intact skin. Its side effects include
local and minor irritation and itching. It is not indicated in patients <1
month of age or in those with a known history of allergy to local
anesthetics of the amide type. The onset of its action is within 1 hour, its
peak effects within 2 to 3 hours, and its effect disappears 1 to 2 hours after
removal of the cream. Apply a thick layer of cream, and cover the area
with an occlusive dressing. Dose is 1/2 of the 5-g tube per site.
Post-procedure Care
We monitor and document vital signs, oxygen saturation, and sedation
scale scores every 15 minutes (total of four times), then every 30 minutes
(total of two times), and then every hour until release of the patient. This
monitoring continues until the patient returns to the preprocedure sedation
score.
Releasing the Patient

The supervising physician is in charge of releasing the patient. The
patients are advised that residual effects from sedation may persist for
some time (according to the medication given). Adult patients are advised
regarding the “seven D’s”:
1. Drive. Do not drive.
2. Dangerous. Do not operate dangerous equipment.
3. Decisions. Do not make important decisions.
4. Drink. Do not drink alcoholic beverages.
5. Diet. After sedation, have a light diet and then resume normal diet on
the following day.
6. Dizziness. Watch out for dizziness.
7. Discuss. Ask any questions you may have to your physician.
Our postsedation guidelines for children are as follows:
1. Provide a safe environment for your child. Keep your child inside and
under supervision by adults. The child needs to be safely secure in the
60
car on your trip back home.
2. Your child may remain sleepy for several hours after the procedure.
You should be able to wake your child up by calling his/her name
and/or touching him/her. The child may drift back to sleep again.
3. Give only clear liquids for the first 2 hours after the procedure.
Resume normal diet once the child is fully awake.
4. Your child should be completely recovered from the medication in 4
to 8 hours.
5. Call at the Department of Radiology if you have any questions or
concerns.
Anxiolysis
Children
Adults
(Instruct the patient to hold the tablet under the tongue [sublingual] for 1
minute and then swallow.)
61
62
CHAPTER 6
Medications in
Neuroradiology
Medications for Contrast Media Reactions

1. Diphenhydramine (Benadryl)
This antihistaminic produces relief of minor allergic reactions but
induces drowsiness and anticholinergic effects (therefore, do not use in
cases of glaucoma and prostatic hypertrophy).
Parenteral (IV/IM) dose:
Child: 1.25 mg/kg
Adult: 25 to 50 mg
Oral dose:
Child: 1.25 mg/kg q4–6h
Adult: 25 to 50 mg q4–6h
2. Epinephrine
These alpha- and beta-receptor agonists induce vasoconstriction and
increased peripheral vascular resistance, increase cardiac output, relax
bronchi, inhibit histamine release, and relieve pulmonary edema. Do not
use in cases of preexisting beta-blockade.
Dose for mild-to-moderate reactions:
Concentration: 1:1,000
Route: SQ/IM
Volume:
63
Child:0.1 to 0.3 mL/kg (<0.5 mL total)
Adult: 0.3 to 0.5 mL
Repeat:
Child:q30 minutes
Adult: q15 minutes
Dose for severe reactions:
Concentration: 1:10,000
Route: IV over 5 minutes
Volume:
Child:1 to 3 mL
Adult: 3 to 5 mL
Repeat:
Child:q30 minutes
Adult: q15 minutes
3. Aminophylline
Mechanism of action not certain but probably related to inhibition of
phosphodiesterase, leading to bronchodilation, CNS stimulation, and
cardiovascular effects. May produce hypotension and seizures.
Dose for acute bronchospasm:
5 to 6 mg/kg IV slowly
4. Dexamethasone sodium phosphate
This corticosteroid is easier to use in an emergency and has the fastest
effect. Inhibits production of allergic mediators.
Dose for acute reaction:
20 mg IV q2–6h PRN
5. Atropine
Produces anticholinergic effects thereby inhibiting vagal tone and is
useful for bradycardia accompanied by hypotension.
Dose for acute reactions:
Child: 0.2 mg/kg IV (<0.6 mg total)
Adult: 0.5 to 1 mg IV q5–10 minutes (<2 mg total)
6. Diazepam (Valium)
This GABA agonist inhibits neuronal firing and may be used to
64
control seizures. It may be cardiotoxic and cause respiratory depression.
Dose in acute situation:
Child: 0.2 to 0.5 mg/kg IV q20–30 minutes for a maximum of two
doses
Adult: 5 to 10 mg IV q20–30 minutes
7. Other medications, such as naloxone (Narcan), lidocaine, nitroprusside,
phentolamine, ranitidine, and sodium bicarbonate, are used less often in
cases of contrast media reaction and require more experience. Consult with
the nurse and/or anesthesiologist for correct use.
Endotracheal Tubes
Prevention of contrast reactions in allergic

patients
Prednisone, 50 mg PO q6h for three doses starting the day before the
procedure (13, 7, and 1 hour prior), or methylprednisolone, 32 mg PO 6 to
24 hours before and then repeat dose 2 hours before procedure.
Diphenhydramine, 50 mg PO or IV 1 hour before procedure.
For emergency situations, methylprednisolone 125 mg IV 4 hours before
procedure and diphenhydramine 1 hour before procedure.
Management of contrast reactions

Facial or Laryngeal Edema
1. Give O2 6 to 10 L/min (via mask).
65
2. Give epinephrine IM (1:1,000) 0.3 mL ( = 0.3 mg) or, especially if
hypotension evident, epinephrine (1:10,000) slowly IV 1 mL ( = 0.1
mg). Repeat as needed up to a maximum of 1 mg.
If not responsive to therapy or if there is obvious acute laryngeal edema,
seek appropriate assistance (e.g., cardiopulmonary arrest response team).
Bronchospasm
1. Give O2 6 to 10 L/min (via mask). Monitor electrocardiogram, O2
saturation (pulse oximeter), and blood pressure.
2. Give beta-agonist inhaler (albuterol) two puffs (can repeat up to three
times); repeat as necessary. If unresponsive to the inhaler, use IM or
IV epinephrine.
3. Give one dose of EpiPen/epinephrine IM (1:1,000) 0.3 mL (= 0.3 mg)
or, especially if hypotension, epinephrine (1:10,000) slowly IV 1 mL
(= 0.1 mg). Repeat as needed up to a maximum of 1 mg.
Call for assistance (e.g., cardiopulmonary arrest response team) for severe
bronchospasm or if O2 saturation <88% persists.
Hypotension with Tachycardia (Anaphylaxis)

1. Legs elevated 60 degrees or more (preferred) or Trendelenburg
position.
2. Monitor electrocardiogram, pulse oximeter, and blood pressure.
4. Rapid intravenous administration of large volumes of Ringer lactate
or normal saline.
If poorly responsive, epinephrine (1:10,000) IV 1 mL (=0.1 mg) slowly
(preferred over IM as extremities may not be adequately perfused).
Repeat as needed up to a maximum of 1 mg.
If still poorly responsive, seek appropriate assistance (e.g.,
cardiopulmonary arrest response team).
Hypotension with Bradycardia (Vagal

Reaction)
1. Remove offending stimulus.
2. Secure airway: give O2 6 to 10 L/min (via mask).
66
3. Monitor vital signs.
4. Legs elevated 60 degrees or more (preferred) or Trendelenburg
position.
5. Secure IV access: rapid administration of Ringer lactate or normal
saline.
6. Give atropine 0.6 to 1 mg IV (into running IV infusion of fluids) if
the patient does not respond quickly to steps 2 to 4.
7. Repeat atropine up to a total dose of 3 mg in an adult.
8. Ensure complete resolution of hypotension and bradycardia prior to
discharge.
Hypertension, Severe
2. Monitor electrocardiogram, pulse oximeter, and blood pressure.
3. Give labetalol 20 mg IV slowly over 2 minutes (dose can be doubled
every 10 minutes).
4. If labetalol not available, give nitroglycerine 0.4-mg tablet, sublingual
(may repeat every 5 to 10 minutes) and furosemide 20 to 40 mg IV
slowly over 2 minutes.
5. Transfer to intensive care unit or emergency department.
6. For pheochromocytoma, phentolamine 5 mg IV. (May use labetalol if
phentolamine is not available.)
Seizures
2. Consider diazepam (Valium) 2 to 4 mg IV slowly to maximum of 4
mg.
3. If longer effect needed, obtain consultation.
4. Careful monitoring of vital signs required, particularly of pO2
because of risk to respiratory depression with benzodiazepine
administration.
5. Consider using cardiopulmonary arrest response team for intubation if
needed.
Pulmonary Edema
2. Elevate the torso.
67
3. Give diuretics: furosemide (Lasix) 20 to 40 mg IV, slow push.
4. Transfer to intensive care unit or emergency department.
Urticaria
1. Discontinue injection if not completed.
2. No treatment needed in most cases.
3. Give H1-receptor blocker: diphenhydramine (Benadryl) PO (IM or IV
if moderate or severe) 25 to 50 mg. Fexofenadine 180 mg PO can be
given instead of diphenhydramine for mild-to-moderate reactions.
If severe or widely disseminated, give alpha agonist as outlined above.
(Source: ACR Manual on Contrast Media v 10.1
http://www.acr.org/quality-safety/resources/contrast-manual)
Medications That May Affect the

Performance of Invasive Procedures
As mentioned in Chapter 3, some physicians will stop these medications
before myelography (the risk of seizures is <1%), while some may perform
the study without discontinuing such medications. The inserts for iohexol
and iopamidol approach this issue in such a way that it leaves the decision
to the physician. We currently do not discontinue these medications prior
to myelography. Thienopyridine derivatives such as clopidogrel and
ticlopidine should be discontinued 7 and 14 days, respectively. Patients
taking platelet inhibitors are at risk for epidural hematoma and
subarachnoid hemorrhage from a lumbar or cervical puncture. Aspirin and
Plavix should be discontinued at least 5 days before myelography. Anti-
inflammatory drug with mild platelet inhibition such as ibuprofen is not a
contraindication to lumbar puncture. If there is no opportunity to
discontinue an antiplatelet medication before myelography, then the use of
a small-bore needle (22 gauge) is advised. Low molecular weight heparin
should be discontinued for at least 12 hours if being given for prophylaxis
or 24 hours if treatment dosing. Unfractioned intravenous heparin is
withheld for 2 to 4 hours, and the procedure can be performed after a
normal aPTT. Unfractioned subcutaneous heparin is not a contraindication
for doses <10,000 units per day; if higher, treat as if intravenous. INR
should be 1.5 or less for most procedures.
68
Medications (Generic Names) That May
Lower Seizure Threshold
*Note that the above list is not all inclusive; check for newer drugs that fall
into these categories.
Phenothiazine Derivatives
MAO Inhibitors
Tricyclic Antidepressants
CNS Stimulants
69
Antidepressants
Antipsychotics
Bronchodilators
aminophylline
Immunomodifiers
Narcotics
pethidine
Over-the-Counter Medications That

Increase Bleeding Time
Common Medications That Contain Aspirin
or Its Derivatives (Trade Names)
70
Common Medications That Contain
Nonsteroidal Anti-Inflammatory Agents and
May Affect Coagulation (Trade Names)
71
CT Contrast Allergy
*Contrast allergy premedication: prednisone 50 mg PO 13 hours, 7 hours,

and 1 hour before contrast load and Benadryl 50 mg PO/IV 1 hour before
procedure.
**Emergency premedication: methylprednisolone 125 mg IV 4 hours
before procedure and diphenhydramine 1 hour before procedure.
Based on UNC Radiology Intravascular Contrast Protocol and ACR
Contrast Manual Policy.
Iodinated Contrast in Renal Insufficiency
72
*Note that if the patient has acute renal insufficiency (i.e., recent bump in
creatinine) but is not quite at 1.8, be hesitant about administering contrast.
When in doubt about whether to give contrast, get a second opinion!
Based on UNC Radiology Intravascular Contrast Protocol.
Guidelines for Use of Gadolinium-Based

Contrast Agents
We generally use gadobenate dimeglumine because of its low to none
association with systemic nephrogenic sclerosis (NSF) in all adult patients
with normal renal function. In adult patients with renal impairment (and
children with normal renal function) who need contrast administration, we
use gadoterate meglumine (one of the macrocyclic agents, due to its
decreased rate of dissociation and decreased likelihood of release of free
gadolinium into the body) if GFR is <60 but >30 mL/min/1.73 m2. Adults
with GFR <30 or children with renal impairment would get a noncontrast
exam or, exceptionally, gadoterate meglumine if deemed absolutely
necessary and for urgent cases.
Lactating mothers receiving Gd-based contrast agents should stop
breast-feeding for 24 hours from the time of contrast injection. Our clinical
computer systems calculate glomerular filtration rates (GFR) for all
patients. Note that a serum creatinine level of over 1.8 mg/dL generally
indicates some degree of renal insufficiency, and thus a need for a GFR
value before any type (MR or CT) of contrast agent is given.
Remember that risk of NSF increases in the following situations:
Acute or chronic severe renal insufficiency as evidenced by GFR <30

mL/min/1.73 m2.
73
or
Acute renal insufficiency of any severity due to the hepatorenal

syndrome or in the perioperative liver transplantation period.
Adults with normal renal function Standard dose (0.1 mmol/kg)

gadobenate dimeglumine
Adults with renal impairment (GFR is <60 but >30 mL/min/1.73
m2)
Standard dose (0.1 mmol/kg) gadoterate meglumine
Children with normal renal function (2 years and older)
Standard dose (0.1 mmol/kg) gadoterate meglumine
Adults with GFR <30 and children with any renal impairment
Noncontrast exam or standard dose (0.1 mmol/kg) gadoterate
meglumine if deemed essential
Pregnancy
Gadobenate dimeglumine half dose (0.05 mmol/kg) used when
unavoidable.
Repeat gadolinium-enhanced MR
Not performed within 48 hours of initial study in patients without risk
factors unless essential
Performed after 48 hours in low-risk patients
Not performed in high-risk patients
Patients with NSF
Gadolinium-based agents should not be administered.
MR Contrast Administration in Adults

(>18 Years of Age)
74
MR Contrast Administration in Children
(<16 and >2 Years of Age)
Contrast Extravasation
Go evaluate the patient and look for diminished pulses, swelling,
decreased sensation/muscle strength, and decreased capillary refill. Ask
about pain.
75
The dictated report for the study should include information about the
extravasation (location, estimated amount, and follow-up). The appropriate
prompt should be found in the extravasation macro.
76
PART 2
77
SECTION A BRAIN
IMAGING
78
CHAPTER 7
Trauma
Arterial Dissection
Key Facts
Affects ICA or vertebral arteries and can be multivessel in 25% of
cases. May be induced by trauma (including chiropractic
manipulation) or be “spontaneous.”
Causes of nontraumatic dissection include fibromuscular dysplasia,
hypertension, migraine, oral contraceptives, collagen vascular
disorders, autosomal dominant polycystic kidney disease, and
pharyngeal infections.
Hematoma dissects between the media and adventitia and may create
a false lumen between these layers.
Patients present with neck pain, Horner syndrome, stroke (infarcts
may be embolic or watershed in ICA dissection), or cranial nerve
palsies.
Vertebral dissections may result in posterior circulation ischemia,
commonly with lateral medullary (Wallenberg) syndrome, or spinal
cord ischemia.
Location of ICA dissections: 2 to 3 cm above bifurcation, at the skull
base, and supraclinoid segment.
Location of vertebral artery dissections: at C6 (as it enters the
foramen transversarium), C1 loop, and at foramen magnum (as it
pierces the dura).
79
FIGURE 7-1. Axial fat-suppressed T1 in a patient with right ICA
dissection shows bright signal surrounding the vessel consistent with
subacute intramural/subintimal clot (methemoglobin crescent sign). This
patient had both fibromuscular dysplasia and hypertension.
80
FIGURE 7-2. Lateral DSA view, different patient, shows the typical
“flame” shape of an ICA dissection.
81
FIGURE 7-3. Curved planar CT reformat in a different patient shows the
classic location of an ICA dissection just below the skull base. There is
focal dilatation (arrow) with a longitudinal intimal flap.
82
FIGURE 7-4. Axial fat-suppressed T1 demonstrates circumferential mural
thickening in the left vertebral artery with a narrowed lumen due to
traumatic dissection.
83
FIGURE 7-5. Sagittal contrast-enhanced MRA in a different patient
shows irregular long-segment luminal narrowing in the left vertebral artery
in a patient with a dissection.
84
FIGURE 7-6. Corresponding DSA view shows an abrupt change in vessel
caliber at the mid V2 segment with narrowing distally.
SUGGESTED READINGS
Lum C, Chakraborty S, Schlossmacher M, et al. Vertebral artery dissection with a
normal-appearing lumen at multisection CT angiography: the importance of
identifying wall hematoma. AJNR Am J Neuroradiol 2009;30:787–792.
Rahme RJ, Aoun SG, McClendon J Jr, El Ahmadieh TY, Bendok BR.
Spontaneous cervical and cerebral arterial dissections: diagnosis and
management. Neuroimaging Clin N Am 2013;23:661–671.
85
Child Abuse
Key Facts
Brain injury is the leading cause of death in child abuse and is present
in 40% of all cases.
More commonly seen in children <2 years of age (peak incidence 6
months) and in premature and sick infants.
Subdural hematomas (particularly multiple, of different ages, and
located along the interhemispheric fissure and in the posterior fossa)
are the most common intracranial injury, followed by subarachnoid
hemorrhage.
Other imaging features include complex (often bilateral) skull
fractures with sutural extension, contusions, and shearing injuries.
Infarctions may be hemispheric because of dissection of the cervical
or supraclinoid internal carotid artery (ICA). Diffuse anoxia may
follow strangulation, cervicomedullary injury (compromise of the
respiratory center), or bilateral ICA dissections.
Diffuse cerebral edema is more common in young infants than in
older children.
MR imaging is better than CT in depicting and dating injuries of child
abuse. However, remember that on CT, even acute extra-axial
hematomas may have different densities when mixed with CSF
(hematohygromas).
Other findings: retinal hemorrhage, cervical spinal cord injury, and
shearing of the pituitary stalk.
86
FIGURE 7-7. Axial T2 shows large bilateral subdural hematomas with
different layering intensities indicating blood products of various ages.
There are multiple thin septations.
87
FIGURE 7-8. Axial T2 in a different patient shows large multiloculated
hemispheric subdural hematomas with layering hematocrit levels (arrow).
There is marked parenchymal volume loss.
88
FIGURE 7-9. Axial CT, different, ex-preterm patient, demonstrates
diffuse cerebral edema with accentuation of gray–white matter attenuation
and ventricular effacement, consistent with global anoxic injury. There is
overriding of lambdoid and right frontoparietal sutures.
89
FIGURE 7-10. Axial T2 shows a focal irregular lesion in the posterior left
globe compatible with a retinal hemorrhage (arrow) in a patient who
sustained vigorous shaking and blunt head trauma.
SUGGESTED READINGS
Barnes PD. Imaging of nonaccidental injury and the mimics: issues and
controversies in the era of evidence-based medicine. Radiol Clin North Am
2011;49:205–229.
Pinto PS, Meoded A, Poretti A, Tekes A, Huisman TA. The unique features of
traumatic brain injury in children. Review of the characteristics of the
pediatric skull and brain, mechanisms of trauma, patterns of injury,
complications, and their imaging findings—part 2. J Neuroimaging
2012;22:e18–e41.
Contusions
90
Key Facts
Commonly involve the cortex and are hemorrhagic in 50% of cases,
reflecting the rich vascularity of gray matter. CT obtained 24 to 48
hours after injury usually shows larger and more numerous lesions.
They are the most frequently encountered lesions in traumatic brain
injury, seen in ~44% of cases.
Commonly involve the basal and polar frontal and temporal lobes as
well as dorsolateral midbrain.
They occur in 5% to 10% of severe head trauma victims and are
multiple in 30% of cases.
Intraventricular hemorrhage is present in 1% to 5% of patients with
cerebral contusions due to tearing of subependymal veins and choroid
plexus or extension from an adjacent parenchymal hematoma.
91
FIGURE 7-11. Axial CT demonstrates multifocal hemorrhagic contusions
in the frontal lobes (arrows). There is a right hemispheric subdural
hematoma (arrowhead) and subarachnoid blood bilaterally.
92
FIGURE 7-12. Axial T2 in a trauma patient shows multiple hemorrhagic
contusions in the temporal lobes with edema. There is layering hematocrit
in a large hematoma on the left side.
93
FIGURE 7-13. Axial CT shows a large acute hemorrhagic contusion in
the right temporal lobe (arrow) with surrounding edema. Smaller
contusions, edema, and trace subarachnoid blood (arrowhead) are also
present in the basal frontal lobes.
94
FIGURE 7-14. In a different patient, axial CT shows typical contrecoup
injury with small cortical hemorrhagic contusions in the left frontal lobe
(arrowheads) and soft tissue hematoma in the occipital scalp (arrow).
SUGGESTED READINGS
Alahmadi H, Vachhrajani S, Cusimano MD. The natural history of brain
contusion: an analysis of radiological and clinical progression. J Neurosurg
2010;112:1139–1145.
Hijaz TA, Cento EA, Walker MT. Imaging of head trauma. Radiol Clin North Am
2011;49:81–103.
95
Diffuse Axonal Injury and Intermediary
Injuries
Key Facts
Most common traumatic brain injury caused by severe
acceleration/deceleration and rotational forces (also known as
“shearing injuries”).
Common locations: frontal and temporal lobes at gray–white
junctions (50%), basal ganglia, splenium of corpus callosum, and
dorsal midbrain.
Most common cause of posttrauma vegetative state with overall
mortality of 50%.
CT is initially normal in 50% to 85% of patients; lesions become
more prominent during the first 24 hours.
T2* and particularly SWI are very sensitive for detection of lesions
that contain hemorrhage (most are nonhemorrhagic). On DWI, most
are bright probably secondary to shearing of small arteries and
subsequent infarction.
Intermediary injuries (between coup and contrecoup) of the basal
ganglia are rare and secondary to shearing of lenticulostriate or
anterior choroidal vessels and have a poor prognosis as they are
generally accompanied by diffuse axonal injuries.
96
FIGURE 7-15. Axial CT shows multiple hemorrhagic shearing injuries
mostly at gray–white matter junctions and subarachnoid hemorrhage.
97
FIGURE 7-16. Axial CT, different patient, shows hemorrhagic shear
(arrow) in the midbrain.
98
FIGURE 7-17. Axial DWI in a 3-year-old shows shear injury in the
callosal splenium and cerebral hemispheres.
99
FIGURE 7-18. Axial SWI minIP in a different patient shows classic shear
injuries in the callosal splenium (arrow), bilateral frontal lobes at the gray–
white interface, and thalami.
100
FIGURE 7-19. Axial DWI, different patient, shows shear injuries in the
left fornix (arrow) and right splenium. Forniceal injury is rare but is
associated with cognitive and memory dysfunction.
101
FIGURE 7-20. Axial T2 shows a large hematoma in the right basal
ganglia consistent with an intermediary injury. There is also shear injury in
the callosal splenium (arrow).
SUGGESTED READING
Moen KG, Brezova V, Skandsen T, Håberg AK, Folvik M, Vik A. Traumatic
axonal injury: the prognostic value of lesion load in corpus callosum, brain
stem, and thalamus in different magnetic resonance imaging sequences. J
Neurotrauma 2014;31:1486–1496.
102
Epidural Hematoma
Key Facts
50% to 75% of severe head trauma victims will have an epidural
hematoma.
90% are due to arterial injury; about 70% to 75% occur in the
temporoparietal region secondary to laceration of the middle
meningeal artery.
CT identifies an underlying fracture in 85% to 95% of epidural
hematomas. Epidural hematomas do not cross cranial sutures unless
fracture or diastasis is present.
“Lucid” interval is seen in 50% of patients and precedes clinical
deterioration; overall mortality is 5%; delayed enlargement of
hematoma occurs in 10% to 30% of cases.
“Vertex” hematomas are usually venous and always epidural. They
cross the superior sagittal sinus and displace it inferiorly.
In children, epidural hematomas may more commonly arise from
laceration of the venous sinuses.
A venous sinus origin should be suspected when the hematoma abuts
both sides of the tentorium or is adjacent to a dural venous sinus.
103
FIGURE 7-21. Axial CT shows a large epidural hematoma in the right
frontoparietal region containing areas of low attenuation (swirl sign)
indicative of active bleeding.
104
FIGURE 7-22. Axial CT in a 4-month-old after a fall demonstrates a large
epidural hematoma in the left frontoparietal region containing areas of low
attenuation.
105
FIGURE 7-23. Axial CT shows a biconvex, hyperacute epidural
hematoma with low attenuation anteriorly (asterisk) corresponding to
unclotted blood.
106
FIGURE 7-24. Coronal CT reconstruction, in a different patient, shows a
convexity (vertex) epidural hematoma typically crossing the midline.
SUGGESTED READING
Selariu E, Zia E, Brizzi M, Abul-Kasim K. Swirl sign in intracerebral
haemorrhage: definition, prevalence, reliability and prognostic value. BMC
Neurol 2012;12:109.
Pneumocephalus
107
Key Facts
Defined as presence of air in any intracranial compartment.
Most are due to trauma (fracture of the frontal sinus) and surgery.
Occasionally, skull base tumors or infection with gas-producing
organisms may be responsible.
May occur due to barotrauma after air travel or rarely rapid
decompression after diving and is associated with frontal sinus
osteomas, congenital abnormalities of the middle and inner ear, and
large arachnoid granulations.
Most cases resolve spontaneously. Incidentally noted small amounts
of venous air in nontrauma patients (particularly cavernous sinus) are
usually of no clinical significance.
Tension pneumocephalus has high pressure, exerts mass effect,
results in symptoms, and needs to be evacuated.
Tension pneumocephali are large and occur in ~8% of patients with
fractures or surgery of the base of the skull.
108
FIGURE 7-25. Axial CT shows large air accumulations in a patient with
hydrocephalus resulting in the “Mount Fuji” (also called “double peak”)
sign.
109
FIGURE 7-26. Axial CT, different patient, shows extensive air in basilar
cisterns and frontotemporal regions following pituitary surgery.
110
FIGURE 7-27. Axial CT, different patient, shows a large left frontal
pneumatocele, which is under pressure and exerts mass effect upon the
brain; air is also present in the ventricles and subarachnoid space.
111
FIGURE 7-28. Axial CT, different patient, shows a low density in the
posterior left temporal region corresponding to air inside a fragment of
wood impaled in the skull.
SUGGESTED READING
Zee CS, Hovanessian A, Go JL, Kim PE. Imaging of sequelae of head trauma.
Neuroimaging Clin N Am 2002;12:325–338.
Skull Fractures
112
Key Facts
Linear fractures or sutural diastases with no underlying brain injury
are generally not clinically significant; formation of leptomeningeal
cysts and/or herniated brain (“growing fracture”) is rare.
Fractures through the skull base or posterior table of the frontal
sinuses may produce pneumocephalus, CSF leaks, and meningitis.
Sphenoid bone involvement increases the risk of vascular (carotid
canal) or cranial nerve injury.
Determination of otic capsule involvement or sparing is clinically
relevant in temporal bone fractures. Fracture through the temporal
bone may give origin to gas in the temporomandibular joint.
Depressed fracture: fragments are displaced by more than 0.5 cm;
most depressed fractures have underlying contusions, and contrecoup
injuries are present in 30% of patients.
Most depressed fractures are considered “open” and require
debridement. Those depressed greater than the thickness of the
cranium are surgically elevated.
Most skull fractures have no underlying brain injuries, and most
severe brain injuries have no skull fractures; plain radiographs are not
useful in suspected cerebral trauma.
CT thin slices (using bone kernel), multiplanar reformats, or skull
radiographs may be helpful to document fractures (which may be
missed on axial CT if oriented parallel to the slices).
113
FIGURE 7-29. Axial CT shows a comminuted and depressed left parietal
fracture extending to the left temporoparietal suture.
114
FIGURE 7-30. Axial CT in a different patient with multiple fractures
shows a right parietal fracture (arrow), which is depressed greater than the
thickness of the cranium.
115
FIGURE 7-31. 3-D volume–rendered CT demonstrates multiple fractures
crossing the midline, which risks injuring the superior sagittal sinus.
116
FIGURE 7-32. 3-D volume–rendered CT in a 7-week-old who fell onto
concrete shows a typical “ping-pong” parietal fracture due to pliable and
immature bones at this age.
117
FIGURE 7-33. Thin axial CT (0.75-mm bone kernel) shows a right
temporal fracture extending to the sphenoid bone (arrowheads) and
partially crossing the carotid canal. The patient had an extrinsic hematoma
narrowing the ICA (not shown).
118
FIGURE 7-34. Axial T2 in a different patient demonstrates herniation of
brain parenchyma through a progressively diastatic “growing fracture” in
the left parietal bone.
SUGGESTED READING
Baugnon KL, Hudgins PA. Skull base fractures and their complications.
Subdural Hematoma and Hygroma
119
Key Facts
Subdural hematomas are found in 10% to 20% of severe head trauma
victims with an overall mortality rate of 60% to 90%.
95% occur in the frontoparietal regions due to tearing of bridging
veins, most commonly in older, atrophic brains.
10% to 15% are bilateral; interhemispheric location or different-age
blood products in children suggest abuse.
CT: acute (<3 days) are hyperdense; subacute (3 to 21 days) are
isodense; chronic (>3 weeks) are hypodense.
Contrast enhancement (spot sign) within an acute subdural hematoma
predicts expansion. Both subacute and chronic hematomas may have
inner membrane contrast enhancement or loculations.
Visualization of small hematomas adjacent to the calvarium requires
intermediate CT window settings (width: 250; level: 40); generally,
these are small and not clinically significant but occasionally grow.
Cerebral contusions are seen in 50% of patients with subdural
hematomas.
Subdural hematomas may follow ventricular shunting or other causes
of intracranial hypotension.
Hygromas are hypodense subdural collections of nonbloody CSF
caused by an arachnoid tear. Most occur in older persons.
120
FIGURE 7-35. Axial CT shows acute dense right hemispheric subdural
hematoma with mass effect and mild midline shift to the left.
121
FIGURE 7-36. Axial CT shows a right subdural hematoma that is
isodense to the cortex. Note mass effect with displacement of white matter
(arrowheads), sulcal obliteration, and midline shift.
122
FIGURE 7-37. Superimposed acute bleed is seen as a dense fluid level in
the dependent portion of a right chronic subdural hematoma (supernatant is
hypodense) that has significant mass effect.
123
FIGURE 7-38. Axial CT in an elderly patient with recurrent bilateral
subdural hematomas shows blood products of different ages and brain
atrophy.
124
FIGURE 7-39. Corresponding axial T2 shows formation of thick
membranes and extensive loculation due to recurrent hemorrhages.
125
FIGURE 7-40. Axial FLAIR in a different patient demonstrates bilateral
subdural hygromas with CSF-like signal suppression.
SUGGESTED READING
Romero JM, Kelly HR, Delgado Almandoz JE, et al. Contrast extravasation on
CT angiography predicts hematoma expansion and mortality in acute
traumatic subdural hemorrhage. AJNR Am J Neuroradiol 2013;34:1528–1534.
Traumatic Subarachnoid Hemorrhage

126
Key Facts
Seen with most moderate and severe head injuries.
Usually more localized and lesser amount of blood than aneurysmal
traumatic subarachnoid hemorrhage (SAH); therefore, it almost never
induces vasospasm but may produce posttraumatic communicating
hydrocephalus.
CT shows a “pseudodelta” sign as hyperdense blood layers along the
posterior superior sagittal sinus (also described with subdural blood);
blood may also be seen in the interpeduncular cistern and needs to be
differentiated from brain stem hematoma or basilar artery aneurysm.
May arise from extension of superficial brain contusions.
Sensitivity with third-generation CT scanners is close to 100% within
the first 6 hours and overall >90% in the first 24 hours, but decreases
to <50% by the 3rd day.
FLAIR images allow for diagnosis of acute and early subacute SAH
seen as areas of high signal in CSF. FLAIR is hampered by CSF flow
artifacts in the basilar cisterns and thus is ideal for supratentorial
SAH.
High CSF signal on FLAIR is nonspecific and reflects increased
proteins (blood, meningitis, cancer); can also be seen in anesthetized
patients receiving oxygen (high FiO2), leptomeningeal collaterals,
after gadolinium administration (especially with renal failure), and
melanosis.
Remember that “convexity” SAH is a common cause of
“thunderclap” headache and in older individuals may be due to
vasculitis, venous thrombosis, or amyloid. Focal (“cortical”) SAH
may be a manifestation of reversible vasoconstriction syndrome
(RCVS), particularly in young women.
127
FIGURE 7-41. Axial CT shows a tiny amount of subarachnoid blood in
the deep left central sulcus (arrow) in a 71-year-old woman who fell onto
concrete.
128
FIGURE 7-42. Axial CT in a pedestrian struck by a motor vehicle shows
extensive subarachnoid hemorrhage along the posterior cerebral
hemispheres (arrowheads). Subdural blood is also present bilaterally, and
there are complex calvarial fractures posteriorly.
129
FIGURE 7-43. Axial CT in a different patient who developed both
subarachnoid and subdural hemorrhage after trauma shows the appearance
of a pseudodelta sign (arrow).
130
FIGURE 7-44. Corresponding FLAIR image shows bright signal
(nonsuppression) within sulci due to hemorrhage. There are also thin
subdural hematomas bilaterally.
131
FIGURE 7-45. Axial SWI image in a different patient demonstrates dark
signal along the left sylvian fissure and adjacent sulci in keeping with
subarachnoid blood.
SUGGESTED READINGS
Perry JJ, Stiell IG, Sivilotti ML, et al. Sensitivity of computed tomography
performed within six hours of onset of headache for diagnosis of
subarachnoid haemorrhage: prospective cohort study. BMJ 2011;343:d4277.
Stuckey SL, Goh TD, Heffernan T, Rowan D. Hyperintensity in the subarachnoid
space on FLAIR MRI. AJR Am J Roentgenol 2007;189:913–921.
132
CHAPTER 8
Stroke
Acute Cerebellar Infarct

Key Facts
The posterior inferior cerebellar artery (PICA) is more commonly
involved (and may produce Wallenberg syndrome), followed by the
superior and anterior inferior (AICA) cerebellar arteries.
Cerebellar infarcts occur in association with vertebral artery
dissection (particularly younger patients) or basilar artery disease
(older patients).
Cerebellar infarcts may lead to death rapidly by compression of
brainstem and acute obstructive hydrocephalus (secondary to
compression of the fourth ventricle).
Upward cerebellar herniation (seen as effacement of the
quadrigeminal plate cistern) and downward herniation of cerebellar
tonsils may also occur.
Emergent decompressive craniectomy with or without resection of
necrotic cerebellum (strokectomy) or ventriculostomy may be
required, particularly in full PICA territory “pseudotumoral” infarcts.
Symptoms are frequently nonspecific and may masquerade as more
benign conditions such as gastroenteritis or labyrinthitis.
133
FIGURE 8-1. Axial CTA shows acute right PICA territory infarct
(asterisk) effacing the fourth ventricle. Note sparing of the AICA territory
(arrowheads) and the patent contralateral PICA (arrow).
134
FIGURE 8-2. Axial DWI, in a different patient, shows a large acute right
PICA territory infarct involving the cerebellum as well as the right lateral
medulla.
135
FIGURE 8-3. Axial DWI, in a different patient, shows acute infarcts
involving median branches of the left PICA.
136
FIGURE 8-4. Axial DWI, in a different patient, demonstrates acute infarct
involving the left middle cerebellar peduncle in the territory of the AICA.
137
FIGURE 8-5. Axial T2 in a different patient who presented with right
vertebral artery dissection shows acute infarct in the paramedian right
cerebellum.
138
FIGURE 8-6. Axial contrast-enhanced T1, in a different patient, shows a
subacute infarct with enhancement mimicking a neoplasm in the right
cerebellum. Note subtle enhancing striations along the course of the folia.
SUGGESTED READING
Wijdicks EF, Sheth KN, Carter BS, et al. Recommendations for the management
of cerebral and cerebellar infarction with swelling: a statement for healthcare
professionals from the American Heart Association/American Stroke
Association. Stroke 2014;45:1222–1238.
Acute (<24 Hours) Middle Cerebral Artery

Infarct, CT
139
Key Facts
Cerebral stroke is the fifth leading cause of death in the United States;
mortality with each episode varies between 15% and 35%.
75% of all cerebral infarctions involve the MCA territory.
Emboli originating from atherosclerosis of the common and internal
carotid arteries are the most common cause of MCA occlusion.
Sensitivity of CT increases over time: 60% of patients show
abnormalities between 3 and 6 hours (earlier with perfusion imaging);
all show abnormalities by 24 hours.
87% of strokes are ischemic, and the rest are hemorrhagic.
The importance of CT is in establishing the presence or absence of
hemorrhage and therefore prescribing thrombolytic or conservative
treatment.
Findings that preclude thrombolysis: hemorrhage, involvement of
more than one third of MCA territory, over 4.5 hours from ictus, and
mass effect.
Early signs (0 to 24 hours) include hyperdense MCA (25% to 50%),
“disappearing” lentiform nucleus, and loss of insular cortex (may
require use of narrow or “stroke” windows). The presence of early
signs on plain CT correlates with worse functional outcomes.
CT and MR perfusion detect areas of impaired blood flow
immediately. DWI is the only technique to show the infarct “core”.
SWI may show venous prominence (pseudodilatation) as the earliest
sign of acute stroke due to increased deoxyhemoglobin content.
CTA and MRA may be used to detect occlusion at the M1 segment,
which allows treatment with mechanical clot retrieval.
140
FIGURE 8-7. Axial noncontrast CT shows hyperdense right MCA
(arrow).
141
FIGURE 8-8. Axial CT, in a different patient, shows obscuration of the
left caudate head and lentiform nucleus (arrowheads) and loss of their
interface with the internal capsule.
142
FIGURE 8-9. Axial CT, in a different patient, shows hypodensity in the
right basal ganglia and loss of the right insular ribbon. Compare to normal
insular ribbon on the left (arrowheads).
143
FIGURE 8-10. Axial CT, in a different patient, demonstrates a right MCA
stroke with mass effect and sulcal effacement.
144
FIGURE 8-11. Base CTA view, in a different patient, shows occlusion of
the left MCA (arrow). Note absent vessels in left temporal region
(asterisk).
145
FIGURE 8-12. Axial CT perfusion study, in a different patient, shows
prolonged mean transit time (MTT) corresponding to a left MCA infarct
(red and orange).
SUGGESTED READING
Heit JJ, Wintermark M. Imaging selection for reperfusion therapy in acute
ischemic stroke. Curr Treat Options Neurol 2015;17:332.
Acute (<24 Hours) Middle Cerebral Artery

Infarct, MRI
146
Key Facts
Conventional MRI:
Earliest abnormality is the “intravascular enhancement sign,”
which may be seen 2 hours after ictus and is probably caused by
sluggish intra-arterial flow.
Meningeal enhancement overlying infarcted territory is seen in
30% of cases during the first 3 days.
Cortical edema (hyperintensity, increased thickness, and a blurry
gray–white junction) may be seen as early as 3 hours,
particularly on FLAIR.
Occluded arteries may appear bright on FLAIR and of low signal
on T2* images. MRA shows lack of flow-related enhancement
in occluded arteries.
DWI:
All infarcts are seen between 10 and 60 minutes.
May miss small infarcts near the base of the skull.
Area of restricted diffusion on ADC map (infarct core) needs to
be compared to perfusion defect; mismatch represents ischemic
penumbra (tissue at risk).
Perfusion:
TTP, MTT, and Tmax maps are most helpful to judge
hypoperfused area (CBF is usually less conspicuous, and rCBV
may be falsely normal due to “luxury” and collateral perfusion).
Eventual size of infarct will be in between that as seen on ADC
and on perfusion studies.
Patients with large penumbras require more aggressive therapy
to save these regions.
SWI:
May show pseudodilatation of draining veins in region of
ischemia (due to increased deoxyhemoglobin content) as early as
DWI.
147
FIGURE 8-13. Axial DWI shows a large acute left MCA infarction.
148
FIGURE 8-14. Corresponding ADC maps show low signal in the same
territory confirming the presence of restricted diffusion.
149
FIGURE 8-15. Corresponding axial SWI minIP shows prominent cortical
draining veins in the same ischemic territory.
150
FIGURE 8-16. ADC maps, in a different patient, show an acute right
insular infarct (arrow).
151
FIGURE 8-17. Corresponding TTP maps demonstrate a larger area of
hypoperfusion indicating a significant mismatch.
152
FIGURE 8-18. Axial contrast-enhanced T1, in the same patient, shows
“intravascular enhancement sign” (arrowheads) corresponding to area of
ischemia.
153
FIGURE 8-19. Axial T2 GRE, in a different patient, shows low signal
(arrow) from right MCA clot.
154
FIGURE 8-20. Frontal MRA, in a different patient, shows occlusion of
the left MCA.
SUGGESTED READINGS
Copen WA, Schaefer PW, Wu O. MR perfusion imaging in acute ischemic stroke.
Fung SH, Roccatagliata L, Gonzalez RG, Schaefer PW. MR diffusion imaging in
ischemic stroke. Neuroimaging Clin N Am 2011;21:345–377.
Acute Anterior (ACA) and Posterior

(PCA) Cerebral Artery Infarcts
Key Facts
155
ACA infarcts:
Most occur in combination with ICA occlusion; isolated ACA
infarctions are rare (<1%). Relatively more favorable prognosis
than MCA infarctions.
Almost half of ACA infarctions result from cardioembolism.
May be secondary to subfalcine herniation and “clipping” of the
ACA under the falx cerebri.
Earliest sign is restricted diffusion (ADC) or prominence of
vessels on SWI along the medial cerebral convexity, followed by
hypodensity (CT) or hyperintensity (MR T2/FLAIR) at later
stages.
PCA infarctions:
Are most common after internal and middle cerebral artery ones;
may also be seen with downward transtentorial herniation
secondary to compression of PCA between temporal lobe and
edge of tentorium.
Involvement of the medial occipital lobe causes homonymous
hemianopsia.
In all young patients with stroke consider trauma, drug abuse
(cocaine, amphetamines), coagulopathy (sickle cell disease,
antiphospholipid syndrome), vasculitides (lupus, granulomatous
angiitis), oral contraceptives, and steroids.
156
FIGURE 8-21. Axial CT shows low density in the right anterior ACA
territory (arrow) and also in ipsilateral MCA territory (asterisk)
compatible with acute infarcts.
157
FIGURE 8-22. Axial DWI, in a different patient, shows a similar pattern
with extensive restricted diffusion in right ACA and MCA infarcts.
158
FIGURE 8-23. Axial DWI, in a different patient, demonstrates the typical
distribution of an ACA infarct.
159
FIGURE 8-24. Axial CT, in a different patient, shows low-density
infarction in the region supplied by the right PCA.
160
FIGURE 8-25. Base MRA view shows PCA occlusion with only partial
flow-related enhancement of the proximal P1 segment (arrow).
161
FIGURE 8-26. Axial DWI, in a different patient, shows a large area of
restricted diffusion in the right PCA territory compatible with acute
infarct.
SUGGESTED READINGS
Arboix A, García-Eroles L, Sellarés N, Raga A, Oliveres M, Massons J.
Infarction in the territory of the anterior cerebral artery: clinical study of 51
patients. BMC Neurol 2009;9:30.
Lee E, Kang DW, Kwon SU, Kim JS. Posterior cerebral artery infarction:
diffusion-weighted MRI analysis of 205 patients. Cerebrovasc Dis
2009;28:298–305.
162
Basilar Artery Occlusion
Key Facts
Mortality is 2.5 times higher than with occlusion of one ICA. Better
prognosis with distal clots; mortality in proximal occlusions
approaches 100%.
Two types: acute catastrophic (patients die nearly immediately) and
acute insidious (rapidly progressive, generally due to atherosclerosis
and/or low cardiac output leading to thrombosis).
Common etiologies: embolism, atherosclerosis, vascular
malformations of the base of the skull, syphilis, tuberculous and
fungal meningitis.
Earliest imaging signs of occlusion may be a hyperdense (CT) or
hyperintense (MRI T1/FLAIR) basilar artery, even in the absence of
restricted diffusion on ADC.
If only distal artery is occluded, “top-of-the-basilar” syndrome is
produced (infarcts of thalami, posterior limb of internal capsules, and
midbrain). Median thalamic infarcts (with or without involvement of
the rostral midbrain) may result from occlusion of the artery of
Percheron (single median thalamoperforator).
Infarcts of the pons may lead to “locked-in” syndrome (retained
consciousness and voluntary eye movements with quadriparesis).
Window for thrombolysis may be as long as 24 hours.
163
FIGURE 8-27. Axial CT shows a dense basilar artery (arrow) due to
thrombosis.
164
FIGURE 8-28. Axial FLAIR, in the same patient, demonstrates
corresponding bright signal within a tortuous basilar artery at the level of
the pons (arrow).
165
FIGURE 8-29. ADC map, in the same patient, shows restricted diffusion
in the pons confirming acute infarction.
166
FIGURE 8-30. TTP map from MRI perfusion study, in the same patient,
shows markedly delayed transit (red) to the entire posterior fossa.
Compare with the normally perfused temporal lobes (green).
167
FIGURE 8-31. Axial DWI, in a different patient, shows restricted
diffusion in the paramedian thalami due to artery of Percheron infarction.
168
FIGURE 8-32. Axial FLAIR demonstrates typical V-shaped
hyperintensity along the pial surface of the interpeduncular fossa (V sign)
in a different patient with artery of Percheron infarction.
SUGGESTED READINGS
Gawlitza M, Quäschling U, Hobohm C, et al. Hyperintense basilar artery on
FLAIR MR imaging: diagnostic accuracy and clinical impact in patients with
acute brainstem stroke. AJNR Am J Neuroradiol 2014;35:1520–1526.
Lazzaro NA, Wright B, Castillo M, et al. Artery of percheron infarction: imaging
patterns and clinical spectrum. AJNR Am J Neuroradiol 2010;31:1283–1289.
CADASIL
169
Key Facts
CADASIL = cerebral autosomal dominant arteriopathy with
subcortical infarctions and leukoencephalopathy.
Begins in young adults presenting with TIAs, strokes, migraines with
aura, and subcortical dementia.
Associated with mutation of the NOTCH3 gene in chromosome 19,
which results in abnormalities in the smooth muscle cells of cerebral
arteries.
Initially, MRI shows patchy areas of high T2 signal in temporal poles,
frontoparietal white matter, and external capsule, with or without
lacunar infarcts and/or microhemorrhages.
Later, MRI shows progressive involvement of posterior temporal and
occipital white matter, internal capsule, basal ganglia, thalami, and
pons; periventricular lesions become large and coalescent. Callosal
involvement is rare (as opposed to multiple sclerosis). The posterior
columns in the spinal cord may rarely be affected.
Main differential diagnosis: idiopathic vascular changes,
hypertension, vasculitis, postradiation/chemotherapy changes.
Remember: CADASIL is not associated with hypertension or high
cholesterol and may be misdiagnosed as multiple sclerosis. Anterior
temporal lobe or external capsule involvement is highly unusual in
chronic small vessel ischemic disease.
170
FIGURE 8-33. Axial FLAIR shows high signal intensity in white matter,
basal ganglia, and thalami, including characteristic lesions in both external
capsules (arrow).
171
FIGURE 8-34. Axial FLAIR, in the same patient, shows typical confluent
lesions in the temporal poles (arrows), with more nonspecific
hyperintensities in the pons.
172
FIGURE 8-35. Axial FLAIR demonstrates extensive periventricular white
matter lesions in a different patient with proven NOTCH3 mutation.
173
FIGURE 8-36. Axial FLAIR, in the same patient, shows white matter
signal abnormalities in centrum semiovale and subcortical lacunar infarcts
(arrow).
174
FIGURE 8-37. Axial SWI minIP, in the same patient, shows multiple
thalamic microhemorrhages.
175
FIGURE 8-38. Axial T2, in a different patient, demonstrates distinctive
involvement of the posterior columns of the spinal cord.
SUGGESTED READINGS
Liem MK, Lesnik Oberstein SA, Haan J, et al. Cerebral autosomal dominant
arteriopathy with subcortical infarcts and leukoencephalopathy: progression
of MR abnormalities in prospective 7-year follow-up study. Radiology
2008;249:964–971.
Singhal S, Rich P, Markus HS. The spatial distribution of MR imaging
abnormalities in cerebral autosomal dominant arteriopathy with subcortical
176
infarcts and leukoencephalopathy and their relationship to age and clinical
features. AJNR Am J Neuroradiol 2005;26:2481–2487.
Corpus Callosum Infarctions

Key Facts
Most focal infarcts are embolic or atherothrombotic; larger infarcts
may be “hypotensive” or watershed in nature.
Uncommon due to rich blood supply and perpendicular orientation of
penetrating vessels, but not as rare as previously believed.
Most common locations: splenium, body, entire corpus callosum, and
genu.
Generally in territories supplied by ACA, PCA, or anterior
communicating artery.
May affect entire width or only one side.
Best imaging method: DWI (restricted ADC); may show contrast
enhancement, mass effect, and extension beyond the midline and
should not be confused with tumor.
Main differential diagnosis for lesions in splenium: seizures,
vasculitis, postirradiation, demyelination, trauma (shearing injuries),
viral infections, extrapontine osmotic myelinolysis.
177
FIGURE 8-39. Axial CT shows a hypodense infarct in the genu of the
corpus callosum (arrow) following clipping of an anterior communicating
artery aneurysm with probable injury to the subcallosal artery.
178
FIGURE 8-40. DWI, in a different patient, demonstrates a bright acute
infarct in the splenium of the corpus callosum.
179
FIGURE 8-41. DWI, in a different patient, shows acute infarcts in the left
callosal splenium and bilateral parieto-occipital lobes.
180
FIGURE 8-42. DWI in an infant with hypoxic encephalopathy shows
multiple infarcts including entire corpus callosum, right MCA, left
watershed territory, and right caudate head nucleus.
181
FIGURE 8-43. DWI, in a different patient, shows a large watershed
infarct of the corpus callosum as a result of gastrointestinal hemorrhage in
a patient with severe atherosclerosis.
182
FIGURE 8-44. DWI in a different patient with extrapontine myelinolysis
shows diffuse increased signal in the callosal splenium.
SUGGESTED READINGS
Kasow DL, Destian S, Braun C, Quintas JC, Kagetsu NJ, Johnson CE. Corpus
callosum infarcts with atypical clinical and radiologic presentations. AJNR Am
J Neuroradiol 2000;21:1876–1880.
Uchino A, Takase Y, Nomiyama K, Egashira R, Kudo S. Acquired lesions of the
corpus callosum: MR imaging. Eur Radiol 2006;16:905–914.
183
Cortical Vein Thrombosis
Key Facts
Unlike venous sinus thrombosis, cortical vein thrombosis is
uncommonly associated with elevated intracranial pressures,
generally presents with encephalopathy and seizures, and may lead to
hemorrhagic infarctions.
Predisposing factors: inflammation/infection, trauma, dehydration,
pregnancy, oral contraceptives, coagulopathies, malignancy,
intracranial hypotension, lumbar puncture, collagen-vascular
disorders, and vasculitis.
Most typical finding (but not common): “cord” sign that represents
the clot-filled dilated cortical vein, hyperdense on CT, hyperintense
on T1WI or FLAIR, and of low signal on SWI.
Other findings: cortical and subcortical white matter edema,
hemorrhage, SAH, and/or leptomeningeal enhancement overlying the
abnormality.
DWI: may be variable due to recanalization of vein; perfusion studies
may show delayed TTP, prolonged MTT, and low rCBV.
T2* GRE sequences are highly sensitive and in SWI thrombus may
be accompanied by blooming artifact.
MRV is not very helpful due to anatomic variations of cortical veins
(difficult to find a missing vein).
184
FIGURE 8-45. Sagittal noncontrast CT reformat shows a bright
thrombosed cortical vein (cord sign, arrow) and a subjacent infarct
(arrowhead).
185
FIGURE 8-46. Corresponding TTP map shows delayed perfusion to the
posterior hemispheres, greater on the right.
186
FIGURE 8-47. Axial GRE, in a different patient, shows hypointense
thrombosed cortical veins bilaterally (arrowheads).
187
FIGURE 8-48. Axial GRE, in the same patient, demonstrates a
hemorrhagic infarct in the right frontoparietal region.
188
FIGURE 8-49. Axial noncontrast T1, in a different patient, shows a bright
thrombosed vein in the right opercular region (arrow).
189
FIGURE 8-50. Axial SWI, in a different patient, shows prominent dark
thrombosed cortical veins with blooming artifact (arrowheads). There is
susceptibility artifact on the right due to a subdural hematoma (asterisk) in
a child who sustained inflicted trauma.
SUGGESTED READINGS
Boukobza M, Crassard I, Bousser MG, Chabriat H. MR imaging features of
isolated cortical vein thrombosis: diagnosis and follow-up. AJNR Am J
Neuroradiol 2009;30:344–348.
Coutinho JM, Gerritsma JJ, Zuurbier SM, Stam J. Isolated cortical vein
190
thrombosis: systematic review of case reports and case series. Stroke
2014;45:1836–1838.
Deep Venous System Occlusion

Key Facts
Deep venous system occlusion is less common than occlusion of
dural sinuses or cortical veins.
More common in children (particularly neonates) than in adults.
Common etiologies in children: systemic illness, dehydration,
paranasal sinuses or mastoid infections, trauma (child abuse), and
hypercoagulable states.
Deep gray matter nuclei are involved and may hemorrhage; because
of its involvement of the central gray matter, it may simulate
generalized anoxia, both clinically and upon imaging.
Deep venous occlusion is an important cause of thalamic (unilateral
or bilateral), basal ganglia, and midbrain abnormalities. Unilateral
involvement may simulate a tumor.
Prompt recognition and therapy has great impact on outcome.
Remember that hemorrhage does not prevent anticoagulation or
thrombolytic treatment of venous thrombosis in general.
191
FIGURE 8-51. Axial noncontrast CT shows swelling of the thalami
bilaterally (arrow) and bright, thrombosed paired internal cerebral veins
(arrowhead).
192
FIGURE 8-52. SWI, in the same patient, demonstrates hemorrhagic
change in the thalami.
193
FIGURE 8-53. Sagittal noncontrast CT, in the same patient, shows bright
clot throughout the deep venous system (arrowheads) and low density in
the thalami (arrow).
194
FIGURE 8-54. Axial T2, in a different patient, shows bright signal and
swelling in the thalami with mass effect on the third ventricle.
195
FIGURE 8-55. Lateral view, MRV, a different patient, shows absence of
the entire deep venous system due to thrombosis.
196
FIGURE 8-56. Axial contrast-enhanced T1, in the same patient,
demonstrates patchy enhancement in the thalami compatible with infarcts
in the subacute stage.
SUGGESTED READINGS
Linn J, Pfefferkorn T, Ivanicova K, et al. Noncontrast CT in deep cerebral venous
thrombosis and sinus thrombosis: comparison of its diagnostic value for both
entities. AJNR Am J Neuroradiol 2009;30:728–735.
Meckel S, Reisinger C, Bremerich J, et al. Cerebral venous thrombosis:
diagnostic accuracy of combined, dynamic and static, contrast-enhanced 4D
197
MR venography. AJNR Am J Neuroradiol 2010;31:527–535.
Generalized Brain Hypoxia/Ischemia

Key Facts
Etiologies in adults: trauma, severe hypotension or hypertension,
acute radiation, and venous sinus occlusion; in children, dehydration,
neonatal anoxia, near-drowning, and child abuse are common causes.
The more metabolically active gray matter is most susceptible (deep
nuclei, “pericentral” cortex, hippocampi, cerebellum, brainstem).
Acute findings are best seen on DWI.
Diffuse edema with effacement of all gray–white junctions may be
seen by all imaging techniques during the first 24 hours.
In the subacute stage, cortical hyperintensities may be seen on
noncontrast T1WI with superimposed gyral enhancement; atrophy
and hypointense cortex on T2WI are seen in the chronic stage. On T2
and FLAIR, gliosis (bright) may mask laminar necrosis, which may
also be bright on DWI.
Perfusion studies show generalized abnormalities (> TTP, > MTT, <
rCBF, < rCBV); MRA and CTA show absent flow in ICA and
posterior circulation when intracranial pressure exceeds mean arterial
pressure.
As early as 3 days, DWI may show high signal throughout white
matter, and gray matter abnormalities “pseudonormalize” by about
the end of the first week (at which time T1 and T2 are most useful).
Generalized hypoxia may also give origin to watershed (border zone)
infarcts or laminar (deep layers of cortex) necrosis.
Laminar necrosis refers to ischemic changes in the cortex of
cerebrum and cerebellum.
Usually, the watershed zones in the parieto-occipitotemporal
regions are affected.
Gray matter layers 3, 5, and 6 are very sensitive to ischemia and
are affected in laminar necrosis.
T1 brightness is presumably due to the presence of lipid-laden
macrophages and not hemorrhage.
Main differential diagnosis (when clinical history is not typical):
hypoglycemia, mitochondrial diseases, CJD, hepatic encephalopathy.
198
FIGURE 8-57. Axial CT shows low density with gray–white matter
accentuation in the supratentorial brain and normal density in the
cerebellum (“white” or “dense” cerebellum sign, arrowheads).
199
FIGURE 8-58. ADC map, in a different patient, shows low signal in
pericentral cortex (arrows).
200
FIGURE 8-59. Axial DWI, in the same patient, also shows high signal in
the basal ganglia and thalami.
201
FIGURE 8-60. Axial DWI, in the same patient, shows high signal in the
mesial temporal lobes including hippocampi (arrows).
202
FIGURE 8-61. Axial CT, in a different patient, demonstrates diffuse
cerebral hypodensity and sulcal effacement with small ventricles. Note low
density in the basal ganglia.
203
FIGURE 8-62. Frontal MRA view, in a different patient, shows absence
of intracranial flow with only extracranial arteries visualized.
SUGGESTED READING
Huang BY, Castillo M. Hypoxic-ischemic brain injury: imaging findings from
birth to adulthood. Radiographics 2008;28:417–439.
Fibromuscular Dysplasia (FMD)
204
Key Facts
About 90% of patients are women aged 40 to 60 years.
May affect any artery but generally involves the high internal carotid
(95%) and/or vertebral (15% to 25%) arteries (at the C1-C2 level);
bilateral involvement is seen in 60% to 75% of patients.
It may be associated with intracranial aneurysms (20% to 50%),
spontaneous dissection (20%), stenosis, spontaneous arteriovenous
fistulas, and renal artery FMD.
FMD may cause headaches (50%), bruit, tinnitus, dizziness, neck
pain, and focal neurologic deficits (due to TIAs or stroke). Rarely,
carotid artery–cavernous sinus fistulae may occur.
The medial form is more common than the subadventitial, intimal, or
perimedial form. Atypical (septal) FMD may rarely present as a small
spur in an enlarged carotid bulb.
205
FIGURE 8-63. Lateral view from DSA shows typical “beaded”
appearance (arrow) of FMD in the ICA.
206
FIGURE 8-64. Lateral CTA shows FMD involving the distal ICA in
another patient (arrow).
207
FIGURE 8-65. CT volume-rendered surface display, in a different patient,
shows FMD in tortuous ICAs bilaterally (arrows), worse on the right.
208
FIGURE 8-66. Coronal MRA, in a different patient, demonstrates FMD in
the ICAs bilaterally (arrows).
SUGGESTED READING
Olin JW, Froehlich J, Gu X, et al. The United States Registry for fibromuscular
dysplasia: results in the first 447 patients. Circulation 2012;125:3182–3190.
Hemorrhagic Infarct and Hemorrhagic
209
Transformation
Key Facts
A hemorrhagic infarction is considered as such if blood is identified
within 24 hours of the ictus; hemorrhagic transformation of an
infarction occurs when blood is identified 2 to 14 days after the ictus.
Most hemorrhagic infarctions occur as a consequence of ischemic
infarctions and are due to sudden reperfusion (lysis of intra-arterial
clot) of damaged tissues (called “hemorrhagic transformation”).
Most common cause today is previous treatment with thrombolytic
drugs. Petechial hemorrhage usually does not preclude thrombolysis
or alter the prognosis of stroke.
<5% of all cerebral infarctions are initially hemorrhagic.
Poor pial collaterals, significant CT hypodensity, large infarct
volume, presence of microbleeds elsewhere, and advanced age all
indicate greater risk of hemorrhagic transformation.
CT shows hemorrhage in 15% to 50% of patients with large strokes,
especially when the middle cerebral artery territory is initially
involved.
Hemorrhagic infarctions in nonarterial distributions may be venous in
origin.
DWI may miss small hemorrhages (generally not clinically
important). Gradient echo imaging and in particular SWI are more
sensitive for detection of tiny bleeds.
210
FIGURE 8-67. Axial noncontrast CT shows a hyperdense right MCA
(arrow) due to thrombosis in hyperacute infarction.
211
FIGURE 8-68. Axial CT following mechanical thrombectomy shows
acute hemorrhage in the right MCA territory with intraventricular
extension. Note also acute on chronic left MCA infarct.
212
FIGURE 8-69. Axial CT, different patient, demonstrates a right MCA
infarct with hemorrhagic transformation in the caudate head and anterior
lentiform nucleus. Note also intraventricular blood.
213
FIGURE 8-70. SWI minIP, in the same patient, better delineates the
extent of hemorrhage.
SUGGESTED READING
Mullins ME, Lev MH, Schellingerhout D, Gonzalez RG, Schaefer PW.
Intracranial hemorrhage complicating acute stroke: how common is
hemorrhagic stroke on initial head CT scan and how often is initial clinical
diagnosis of acute stroke eventually confirmed? AJNR Am J Neuroradiol
2005;26:2207–2212.
214
Hypertensive Encephalopathy
Key Facts
Results from significant acute elevation of blood pressure with
extravasation of fluids and proteins leading to diffuse or focal
cerebral edema. Some believe that gray matter edema is also
“vasogenic” in this situation (explaining why it resolves in most
patients).
Etiologies: toxemia of pregnancy, renal insufficiency, hemolytic–
uremic syndrome, autoimmune disease, sepsis, and drugs (tacrolimus,
cyclosporine, VEGF inhibitors).
In preeclampsia and eclampsia, most lesions occur in the distribution
of posterior cerebral circulation (thus the acronym PRES [posterior
reversible encephalopathy syndrome]), are associated with visual
disturbances, and 90% are reversible; some, however, produce
permanent infarctions. Posterior circulation has little sympathetic
innervation and thus lacks autoregulation.
Most patients show cortical and white matter swelling, and 20% may
have hemorrhages (probably even higher incidence with SWI).
FLAIR and T2 show areas of increased signal in white/gray matter
and sometimes in the basal ganglia, thalami, and midbrain; contrast
enhancement may be minimal and patchy; there is slight-to-
moderately high DWI signal without ADC abnormalities (although in
some patients frank infarctions develop), and perfusion may show
normal or < rCBF, < rCBV, > TTP and > MTT in which cases the
incidence of permanent infarctions is higher. May rarely be seen in
the cervical cord.
Main differential diagnosis: ADEM or other demyelinating diseases,
venous thrombosis (particularly sinuses).
215
FIGURE 8-71. Axial FLAIR image shows areas of predominantly cortical
hyperintensity (edema) in the parietal and to a lesser degree frontal lobes.
216
FIGURE 8-72. Corresponding TTP map from MR perfusion study, in the
same patient, shows markedly slow blood flow in these regions.
217
FIGURE 8-73. Axial T2 in a different patient on a VEGF inhibitor for
renal cell cancer shows PRES involving the cerebellum.
218
FIGURE 8-74. Axial T2 in a different patient with eclampsia
demonstrates cortical and subcortical hyperintensities along the superior
frontal sulci and in the parietal lobes.
219
FIGURE 8-75. Axial FLAIR, in a different patient, shows PRES
involving the posterior cerebral hemispheres and callosal splenium.
220
FIGURE 8-76. Axial FLAIR, in the same patient, shows PRES involving
the brainstem.
SUGGESTED READINGS
Bartynski WS. Posterior reversible encephalopathy syndrome, part 1:
fundamental imaging and clinical features. AJNR Am J Neuroradiol
2008;29:1036–1042.
Junewar V, Verma R, Sankhwar PL, et al. Neuroimaging features and predictors
of outcome in eclamptic encephalopathy: a prospective observational study.
AJNR Am J Neuroradiol 2014;35:1728–1734.
221
Stenosis, Extracranial ICA
Key Facts
Chronic injury at zones of turbulent blood flow may lead to cellular
proliferation, development of fibrofatty plaques, and thrombus
formation due to platelet aggregation.
Types of plaque:
Unstable (“vulnerable plaque”): thin fibrous cap, ulceration,
hemorrhages, lipid core (may result in emboli regardless of size
and may need to be treated; often in nonstenotic vessels).
Stable: homogeneous and calcified (may not produce symptoms
until stenosis becomes critical).
Imaging of plaques may be done with sonography, high-
resolution contrast-enhanced MRI, or CT.
Most common sources of emboli that cause strokes include
atherosclerotic plaques in ICAs and the heart.
Vessel diameter needs to be reduced by over 60% by
atherosclerosis to produce symptoms.
Ulcers can be diagnosed by catheter angiography in 50% of
instances and carry an increased risk of cerebral infarction.
Use the NASCET measuring method: maximal stenosis over
ICA diameter distal to stenosis when walls return to parallel with
each other.
Retropharyngeal ICA is not necessarily narrowed but may cause local
symptoms of pulsatile mass secondary to looping and redundancy;
may also be at risk of surgical injury.
A retropharyngeal or palatine tonsil abscess may result in ICA
narrowing.
222
FIGURE 8-77. Lateral DSA view shows moderate but irregular stenosis
in the bulb of the ICA.
223
FIGURE 8-78. Lateral DSA view, in a different patient, shows severe
stenosis of the origin of the left ICA (arrow) with filling defect distally
due to thrombus (arrowheads).
224
FIGURE 8-79. Oblique angiography view, in a different patient, shows
intrastent restenosis.
225
FIGURE 8-80. Axial contrast-enhanced CT, in a different patient, shows
retropharyngeal course of the ICAs bilaterally (arrows). Such location may
be transient.
SUGGESTED READINGS
Jewells V, Castillo M. MR angiography of the extracranial circulation. Magn
Reson Imaging Clin N Am 2003;11:585–597.
Zavodni AE, Wasserman BA, McClelland RL, et al. Carotid artery plaque
morphology and composition in relation to incident cardiovascular events: the
Multi-Ethnic Study of Atherosclerosis (MESA). Radiology
226
2014;271:381–389.
Lacunar Infarctions
Key Facts
Probably the most common type of cerebral infarction, seen in 20%
of elderly individuals. Many are clinically silent but may result in
cognitive deficits and dementia especially when multiple.
Associated with increasing age and hypertension, generally due to
primary vessel disease (hyalinization and microatheromas) leading to
thrombosis and not to emboli.
Commonly affect small perforating and deep arteries (lenticulostriate
and thalamoperforators); thus, the basal ganglia, internal capsule, and
thalamus are typically involved.
The brainstem is also a common location, due to occlusion of
perforators arising from circumflex branches of the basilar artery.
Not hemorrhagic, generally about 1 cm in diameter.
Best imaging technique: DWI (bright acutely, overestimates final
infarct size); by CT, it is generally not possible to age them, unless
they cavitate (60% to 70% do so in the chronic stage, and the rest
evolve into nonspecific white matter lesions).
227
FIGURE 8-81. Axial DWI shows a bright lesion in the right corona
radiata corresponding to an acute lacunar infarction.
228
FIGURE 8-82. Axial DWI, in a different patient, shows a bright acute
infarction in the left paramedian pons (arrow).
229
FIGURE 8-83. Axial CT, in a different patient, shows a well-
circumscribed old lacunar infarct with low, near-CSF density.
230
FIGURE 8-84. Axial FLAIR, in a different patient, shows an old lacunar
infarct with low CSF-like signal and bright gliosis in the periphery
(arrow).
SUGGESTED READING
Koch S, McClendon MS, Bhatia R. Imaging evolution of acute lacunar infarction:
leukoariosis or lacune? Neurology 2011;77:1091–1095.
Moyamoya
231
Key Facts
Moyamoya disease is a primary progressive arteriopathy leading to
occlusion of intracranial ICAs and is mainly seen in the Orient.
Moyamoya syndrome is a nonspecific radiographic finding that is
identical to the disease but seen in association with sickle cell anemia,
collagen-vascular disorders (Ehlers-Danlos or Marfan syndrome,
homocystinuria), neurofibromatosis type I, Menke kinky hair
syndrome, atherosclerosis, and radiation injury.
By conventional angiography, MRI, or MRA, there is occlusion of
supraclinoid ICAs and proliferation of large and irregular perforating
vessels as well as transdiploic ECA collaterals supplying ischemic
brain regions. The posterior cerebral arteries are also commonly
affected, and the thalamoperforators enlarge while involvement of
infratentorial vessels does not occur. Bilateral involvement is
necessary for a diagnosis of moyamoya disease.
Imaging studies show infarcts in up to 80% of patients (particularly
children) and asymptomatic cerebral microhemorrhages (on MR T2*)
in 30%.
Many of these infarcts are in the deep and parasagittal watershed
regions.
FLAIR may show high signal in sulci and perivascular spaces (which
may also enhance) representing pial collateral circulation (“Ivy”
sign). Perfusion abnormalities are commensurate with degree of
disease.
The most common initial presentation in adults is intraventricular
hemorrhage. Other manifestations include parenchymal hemorrhage,
stroke, TIA, and epilepsy.
There is an association with cerebral aneurysms and arterial ectasia,
which are the major determinants of parenchymal hemorrhage.
232
FIGURE 8-85. Axial T2 image shows innumerable small collaterals
replacing the proximal MCAs bilaterally (arrowheads).
233
FIGURE 8-86. Lateral DSA view, in the same patient, shows stenosis of
the distal ICA (arrow) with proliferation of deep perforating arteries.
234
FIGURE 8-87. Axial postcontrast T1 in a different patient with
neurofibromatosis type I demonstrates marked pial enhancement due to
arterial collaterals (“ivy” sign).
235
FIGURE 8-88. Axial CT, in a different patient, shows a large
parenchymal hematoma with intraventricular extension. Note volume loss
and old infarcts in the frontal lobes.
SUGGESTED READINGS
Jang DK, Lee KS, Rha HK, et al. Clinical and angiographic features and stroke
types in adult moyamoya disease. AJNR Am J Neuroradiol
2014;35:1124–1131.
Strother MK, Anderson MD, Singer RJ, et al. Cerebrovascular collaterals
correlate with disease severity in adult North American patients with
236
Moyamoya disease. AJNR Am J Neuroradiol 2014;35:1318–1324.
Subacute (2 to 21 Days) Middle Cerebral

Artery Infarct, CT
Key Facts
Hemorrhage (especially in the basal ganglia and cortex) occurs
spontaneously in ~15% of all patients with middle cerebral artery
strokes (most common cause is previous administration of
thrombolytic drugs).
Hemorrhagic transformation may occur 1 to 4 days after onset of
infarction. Homogeneous hyperdensity within the infarct after
thrombolysis may be due to contrast extravasation, particularly if high
attenuation values (>100 HU); this may be difficult to differentiate
from hemorrhage.
Mass effect increases during first 3 days, and CT shows wedge-
shaped area of low density, which involves both gray and white
matter.
CT has a sensitivity of over 90% in detection of subacute infarcts of
the middle cerebral artery.
Gyral enhancement (attributed to luxury perfusion and blood–brain
barrier breakdown) begins between 3 and 7 days after ictus and may
correlate with a better prognosis than infarcts with no gyral
enhancement. Enhancement peaks at 2 to 3 weeks and can be seen as
late as 3 to 4 months.
237
FIGURE 8-89. Axial CT shows low density in the left posterior MCA
territory 6 days after ictus.
238
FIGURE 8-90. Axial CT, in a different patient, shows maximal mass
effect in a left MCA infarct 3 days following ictus. Note linear
hyperdensities compatible with mild hemorrhagic change.
239
FIGURE 8-91. Axial CT, in a different patient, shows a subacute left
posterior MCA infarct with bright areas of hemorrhagic conversion.
240
FIGURE 8-92. Axial CT, in a different patient, shows formation of a
hematoma and mass effect in a right MCA infarct following thrombolysis.
SUGGESTED READING
Mayer TE, Schulte-Altedorneburg G, Droste DW, Brückmann H. Serial CT and
MRI of ischaemic cerebral infarcts: frequency and clinical impact of
haemorrhagic transformation. Neuroradiology 2000;42:233–239.
Subacute Infarction, MRI

241
Key Facts
Conventional MRI:
Intravascular and meningeal enhancement disappear and are
followed by parenchymal (especially cortex) enhancement
beginning 3 to 7 days after ictus and may persist from 1 week to
6 months.
75% of all subacute infarctions show parenchymal contrast
enhancement.
Infarct becomes hyperintense on FLAIR and T2; adjacent CSF
may be also bright on FLAIR (protein extravasation). At about
14 days, infarct may become less obvious on T2 (“fogging”
effect, same may occur with CT).
T2 hypointensity in tissues surrounding infarct may represent
impaired axonal transport of iron.
DWI:
DWI signal begins to fade by 7 to 10 days but may remain bright
for several weeks primarily due to T2 shine through effects.
ADC becomes progressively less restricted and
pseudonormalizes by the second week.
Low signal on ADC generally means that an infarct is <10 days
old.
242
FIGURE 8-93. Axial FLAIR shows subacute right MCA infarct 10 days
after ictus.
243
cortical and deep enhancement within the infarcted territory.
244
FIGURE 8-95. Axial DWI, in the same patient, shows mild DWI signal,
which has begun to fade at this point.
245
FIGURE 8-96. MRS, long TE, shows high choline presumably due to
inflammation, low NAA due to neuronal damage, and inverted lactate peak
due to ischemia.
246
FIGURE 8-97. Axial contrast-enhanced MRI, in a different patient, shows
extensive enhancement related to bilateral subacute infarctions including
the left MCA territory.
SUGGESTED READING
Allen LM, Hasso AN, Handwerker J, Farid H. Sequence-specific MR imaging
findings that are useful in dating ischemic stroke. Radiographics
2012;32:1285–1297.
247
Cerebral Vasculitis
Key Facts
Cerebral vasculitis is characterized by nonatheromatous inflammation
and fibrinoid necrosis of arterial media and intima, leading to
occlusions, infarctions, and hemorrhages. Spinal cord is affected in
5% of cases.
Etiologies: bacterial, tubercular, viral, and fungal meningitis (mainly
involving vessels at the base of the brain) and syphilis (mainly
involving large-caliber vessels).
Noninfectious etiologies: giant cell arteritis; polyarteritis nodosa,
temporal arteritis, granulomatous angiitis (because very small vessels
are involved, the angiogram is typically normal), sarcoidosis,
collagen-vascular disorders (lupus, Wegener’s, Behçet’s), Takayasu
disease and chemical vasculitis (methamphetamine, ergot
derivatives). A severe postpartum vasculitis (oxytocin induced) has
been also described.
Serum inflammatory markers are usually normal in primary CNS
vasculitis, and 25% of biopsy-proven cases show evidence of cerebral
amyloid angiopathy.
Mortality after hemorrhage secondary to vasculitis approaches 50%.
Nearly all patients will have focal areas of high signal on FLAIR and
T2; vasculitis is highly unlikely if MRI is entirely normal (sensitivity
close to 100%). However, normal MRA or DSA does not exclude
vasculitis.
DSA shows segmental narrowings (predominantly at nonbranching
points), occlusions, slow vessel filling, areas of prolonged capillary
stain, and (rarely) microaneurysms. Despite these findings, biopsy
confirmation is required.
Main differential diagnosis for intra-axial lesions: atherosclerosis-
related disease, vasospasm, shearing injuries, viral encephalitis.
May be confused with reversible cerebral vasoconstriction syndrome
(RCVS), which presents more acutely with thunderclap headaches.
Distinction is important, as steroids appear to be harmful in RCVS.
248
FIGURE 8-98. Axial DWI in a patient with vasculitis shows multiple
acute infarctions in nonarterial distributions.
249
FIGURE 8-99. Oblique DSA view, in the same patient, shows diffuse
arterial irregularities with alternating segments of stenosis and dilatation.
250
FIGURE 8-100. Base MRA view, in a different patient, demonstrates
multiple arterial irregularities involving both the anterior and posterior
circulation bilaterally.
251
FIGURE 8-101. Axial FLAIR, in a different patient, shows increased
signal with mass effect in the left frontoparietal region extending to the
basal ganglia.
252
FIGURE 8-102. Sagittal contrast-enhanced T1, in the same patient,
demonstrates patchy perivascular enhancement consistent with biopsy-
proven vasculitis.
253
FIGURE 8-103. Axial FLAIR, in a different patient, shows bright sulci
due to subarachnoid hemorrhage in vasculitis.
SUGGESTED READINGS
Moharir M, Shroff M, Benseler SM. Childhood central nervous system vasculitis.
Salvarani C, Brown RD Jr, Hunder GG. Adult primary central nervous system
vasculitis. Lancet 2012;380:767–777.
254
Venous Sinus Occlusion
Key Facts
The superior sagittal and transverse sinuses are more commonly
involved and may result in infarcts (even hemorrhagic ones) in a
nonarterial distribution; the sigmoid and cavernous sinuses are less
commonly involved.
Dural sinus occlusion leads to impaired CSF resorption and elevated
intracranial pressures.
Etiologies in adults: paranasal sinus and mastoid infections, oral
contraceptives, hypercoagulable states, collagen-vascular disorders,
pregnancy, malignancies, surgery, and idiopathic causes (25%); in
children, paranasal sinus and mastoid infections, meningitis, trauma,
and dehydration are common causes.
Presence of diffuse brain edema and hemorrhages carries a poor
prognosis. Obtunded patients and those with seizures have a worse
prognosis.
Hemorrhage occurs due to extension of thrombosis into cortical veins
and increased pressures with venular rupture.
Contrast-enhanced CT and MRI may show a “delta” sign due to
filling defect (clot) surrounded by enhanced blood in the superior
sagittal or transverse sinuses; noncontrast MRI shows T1 or FLAIR
hyperintensity and lack of a flow void in the sinus and dural
enhancement; SWI may show dark “blooming” of the clot; MR or CT
venograms are useful in confirming the diagnosis.
Thrombosis may resolve spontaneously and particularly rapidly in
children.
Hemorrhage does not preclude anticoagulation or thrombolysis.
255
FIGURE 8-104. Axial CT shows hyperdense acute clot in the right
transverse sinus (arrow).
256
FIGURE 8-105. Coronal postcontrast T1, in a different patient,
demonstrates filling defects in the superior sagittal and bilateral transverse
sinuses due to thrombosis.
257
FIGURE 8-106. Sagittal noncontrast T1, in a different patient, shows
bright clot expanding the superior sagittal sinus (arrowheads).
258
filling defect in the superior sagittal sinus (“delta sign”, arrow) with
enhancing subacute venous infarcts (arrowheads).
259
FIGURE 8-108. Coronal postcontrast T1, in a different patient, shows
thrombosis of the inferior sagittal sinus.
260
FIGURE 8-109. TOF MRV, in a different patient, shows extensive
venous collaterals due to chronically occluded venous structures.
SUGGESTED READINGS
Buyck PJ, De Keyzer F, Vanneste D, Wilms G, Thijs V, Demaerel P. CT density
measurement and H:H ratio are useful in diagnosing acute cerebral venous
sinus thrombosis. AJNR Am J Neuroradiol 2013;34:1568–1572.
Meckel S, Reisinger C, Bremerich J, et al. Cerebral venous thrombosis:
diagnostic accuracy of combined, dynamic and static, contrast-enhanced 4D
MR venography. AJNR Am J Neuroradiol 2010;31:527–535.
261
Wallerian Degeneration
Key Facts
Wallerian degeneration (WD) generally occurs after MCA infarction
but may also be seen with hemorrhages, tumors, trauma, surgery, and
primary white matter disease.
It refers to anterograde degeneration of axons and their myelin
sheaths.
Acutely, increased signal on DWI following the corticospinal tracts
(may be wallerian edema as “degeneration” has not yet taken place).
DTI is most sensitive, showing decreased fractional anisotropy (FA)
on the affected side.
With conventional MRI, it is rarely seen during the first month after a
stroke, but 4 weeks after ictus, a band of hypointensity along the
ipsilateral corticospinal tract may be seen on T2WI. About 2 to 3
months after a stroke, the ipsilateral corticospinal tract usually
becomes hyperintense on T2, and there is associated atrophy (which
may be especially obvious in the affected cerebral peduncle).
The medial ipsilateral thalamus may show increased T2 signal
intensity secondary to MCA infarctions.
Patients with WD may have worse clinical outcomes than those
without it.
262
FIGURE 8-110. Axial T2 demonstrates encephalomalacia in the right
MCA territory due to an old infarct.
263
FIGURE 8-111. Axial T2, in the same patient, shows decreased volume in
the right cerebral peduncle (arrow) compatible with WD.
264
FIGURE 8-112. Axial DWI, in a different patient, shows large acute
infarction in the left MCA territory.
265
FIGURE 8-113. Axial DWI, in the same patient, demonstrates bright
DWI signal in the left corticospinal tract at the level of the cerebral
peduncle (arrow) due to wallerian edema.
266
FIGURE 8-114. Axial FLAIR, in a different patient, shows chronic
multiple sclerosis plaque in the left corona radiata.
267
FIGURE 8-115. Axial FLAIR, in the same patient, shows WD (arrow) in
the ipsilateral midbrain.
SUGGESTED READINGS
Mark VW, Taub E, Perkins C, Gauthier LV, Uswatte G, Ogorek J. Poststroke
cerebral peduncular atrophy correlates with a measure of corticospinal tract
injury in the cerebral hemisphere. AJNR Am J Neuroradiol 2008;29:354–358.
Puig J, Pedraza S, Blasco G, et al. Wallerian degeneration in the corticospinal
tract evaluated by diffusion tensor imaging correlates with motor deficit 30
days after middle cerebral artery ischemic stroke. AJNR Am J Neuroradiol
268
2010;31:1324–1330.
Watershed Cerebral Infarctions

Key Facts
Also called “border zone” or “hypotensive” infarctions
Most caused by hypotension but paradoxically some may be embolic;
may also be the result of ICA occlusion, particularly in the setting of
a noncompetent circle of Willis.
Sites: between ACA and MCA territories, between PCA and MCA
territories, parasagittal white matter, deep cerebellum, and corpus
callosum.
May result in laminar necrosis.
Best imaging technique: DWI.
Perfusion studies show < rCBF, < rCBV, > MTT and > TTP in the
affected areas or throughout the affected hemisphere.
Remember that fat emboli may look exactly like deep white matter
watershed infarctions. In addition to history, SWI is helpful in making
this distinction, with fat embolism classically showing diffuse
petechial microhemorrhages.
269
FIGURE 8-116. Axial FLAIR image shows old infarctions in the bilateral
MCA and PCA as well as right MCA and ACA watershed territories.
270
FIGURE 8-117. Axial DWI, in a different patient, demonstrates acute
right parasagittal watershed territory infarctions in a typical linear
distribution, due to occlusion of the right ICA.
271
FIGURE 8-118. Axial DWI, in a different patient, showing left-sided
watershed infarctions.
272
FIGURE 8-119. MR perfusion MTT map, in the same patient as in Figure
8-118, shows a large penumbra with increased transit time.
SUGGESTED READING
Mangla R, Kolar B, Almast J, Ekholm SE. Border zone infarcts: pathophysiologic
and imaging characteristics. Radiographics 2011;31:1201–1214.
273
CHAPTER 9
Nontraumatic
Hemorrhage
Acute Hypertensive Hemorrhages

Key Facts
The most common etiologies for all intracerebral hemorrhage include
hypertension, ruptured aneurysm or vascular malformation, trauma,
venous thrombosis, cerebral amyloid angiopathy (in patients over 60
years of age), collagen vascular disorders, anticoagulation, vasculitis
(including moyamoya), neoplasias (primary and metastases), and
cocaine and methamphetamine use.
Hypertensive bleeds account for 10% of all “strokes” and have a 50%
mortality.
They are more common in males 60 to 80 years old; common
locations include basal ganglia (60% to 70%, particularly the
putamen), thalamus (10% to 20%), pons (5% to 10%), dentate nuclei
(1% to 5%), and hemispheric (1% to 2%).
Hemorrhages due to anticoagulation are large and have a complex
appearance (due to rebleeds). The “spot sign” predicts hematoma
expansion; other risk factors for growth include large initial volume
and use of anticoagulants.
Main differential diagnosis when hemorrhage occurs at an atypical
location or age: rule out underlying lesion (AVM, aneurysm, tumor).
Cerebral amyloid angiopathy tends to be peripheral and “lobar,” as
274
opposed to the usually more central hypertensive bleeds.
FIGURE 9-1. Axial CT shows acute dense hemorrhage in the right basal
ganglia with intraventricular extension and mass effect on the lateral
ventricles.
275
FIGURE 9-2. Axial T2, in a different patient, shows a large hemorrhage
centered in the left thalamus, basal ganglia, and insula, with
intraventricular extension.
276
FIGURE 9-3. Axial SWI, in a different patient, shows the typical
distribution of hypertensive microhemorrhages in the basal ganglia and
thalami.
277
FIGURE 9-4. Axial SWI, in the same patient, shows hemosiderin staining
in the right dentate nucleus from prior hemorrhage.
278
FIGURE 9-5. Axial CT, in a different patient, shows a left medial
cerebellar hemorrhage.
279
FIGURE 9-6. Axial CT, in a different patient, shows a pontine bleed.
Suggested Reading
Fischbein NJ, Wijman CA. Nontraumatic intracranial hemorrhage. Neuroimaging
Clin N Am 2010;20:469–492.
Cerebral Amyloid Angiopathy

Key Facts
280
Disease of older individuals, generally above 70 years of age. Can
occur sporadically or in association with Alzheimer disease, although
some cases in younger patients may be familial.
May present with large “spontaneous” lobar parenchymal bleeds.
These may have fluid levels, generally involve gray and white matter,
are posterior in location, may occur in mirror-like locations, are in
nonarterial distributions, and may be synchronous or metachronous.
Mild leptomeningeal enhancement can be present due to
inflammation.
Hemorrhages may extend to the subarachnoid space or occur
primarily in it (“central sulcus hemorrhage”) given superficial
location; they may infrequently rupture into the ventricles.
Microbleeds are common and usually silent and are located in
subcortical regions or cortex, especially parietal lobes.
Mortality with lobar hemorrhage is 10% to 30%, lower than with
hypertensive bleeds (50%).
Extensive white matter changes and atrophy may be present.
Superficial siderosis in convexities is not uncommon; amyloid is
probably the most common cause of this in older adults.
281
FIGURE 9-7. Axial FLAIR shows peripheral hemorrhage in the right
temporal lobe (arrow) in a patient with cerebral amyloid angiopathy.
282
FIGURE 9-8. Axial SWI, in a different patient, shows innumerable
cortical and subcortical microbleeds in a predominantly posterior and
relatively symmetric distribution.
283
cortical microbleeds with extensive white matter edema due to cerebral
amyloid angiopathy-related inflammation.
284
FIGURE 9-10. Axial SWI minIP, in a different patient, demonstrates
superficial siderosis along the sulci (arrowheads) and multiple cortical and
subcortical microbleeds.
285
FIGURE 9-11. Axial T2, in a different patient, shows a hemorrhage in the
left frontal lobe (arrow). Note white matter disease and subtle blood levels
in the dependent lateral ventricles.
286
FIGURE 9-12. Axial SWI, in the same patient, demonstrates extensive
superficial siderosis bilaterally (arrowheads), cortical/subcortical
microbleeds, and intraventricular blood.
Suggested Readings
Linn J, Herms J, Dichgans M, et al. Subarachnoid hemosiderosis and superficial
cortical hemosiderosis in cerebral amyloid angiopathy. AJNR Am J
Neuroradiol 2008;29:184–186.
Martucci M, Sarria S, Toledo M, et al. Cerebral amyloid angiopathy-related
287
inflammation: imaging findings and clinical outcome. Neuroradiology
2014;56:283–289.
Cerebral Microhemorrhages
Key Facts
Most frequently related to hypertension, cerebral amyloid angiopathy,
and previous radiation therapy, and more common with increasing
age. Hypertensive microbleeds are more often seen in males. Fat
embolism may present with innumerable punctate microhemorrhages.
When related to hypertension, they tend to cluster around deep
cerebral and cerebellar structures, generally found in the presence of
underlying white matter disease.
When related to amyloid, they tend to occur in the cortex or
subcortical white matter. Superficial siderosis may be present, and
there is usually accompanying white matter disease.
High number of microhemorrhages correlates with decreased
cognition.
May predict fatal future hemorrhages and infarctions.
May predict bleeding in anticoagulated patients.
Better seen on SWI than on gradient echo imaging.
288
FIGURE 9-13. Axial SWI shows innumerable microhemorrhages
particularly in and around the basal ganglia in a patient with hypertension.
Some subcortical microhemorrhages are also present.
289
FIGURE 9-14. Axial GRE, in a different patient, shows numerous
microhemorrhages with central predominance related to hypertension.
290
microhemorrhages with a predominantly lobar/peripheral distribution due
to amyloid angiopathy.
291
punctate microhemorrhages in the supratentorial brain, brainstem, and
cerebellum, due to fat embolism.
Suggested Readings
Blitstein MK, Tung GA. MRI of cerebral microhemorrhages. AJR Am J
Roentgenol 2007;189:720–725.
Naka H, Nomura E, Takahashi T, et al. Combinations of the presence or absence
of cerebral microbleeds and advanced white matter hyperintensity as
292
predictors of subsequent stroke types. AJNR Am J Neuroradiol
2006;27:830–835.
Hemorrhage in the Premature Brain

Key Facts
At 32 weeks of gestation, residual germinal matrix is present at the
level of the caudothalamic notch; this tissue is fragile and
hypervascularized and the first site to bleed.
Hemorrhages are probably related to hypoxia–ischemia with
reperfusion and elevated venous pressures.
About 67% of infants born between 28 and 32 weeks develop
germinal matrix hemorrhage.
Grade 1 implies that the bleed is confined to the germinal matrix;
grade 2 implies extension into the adjacent lateral ventricle but
without ventriculomegaly.
Grades 1 and 2 hemorrhages have good overall prognosis.
Grade 3 refers to intraventricular hemorrhage and ventriculomegaly.
Grade 4 refers to hemorrhage in the periventricular white matter.
Grade 3 bleeds are a continuation of grade 1 and 2 bleeds; however,
grade 4 bleeds are probably hemorrhagic infarctions secondary to
compression of deep medullary veins.
Grade 4 hemorrhages have a 90% mortality.
293
FIGURE 9-17. Sagittal oblique US shows a small, grade 1 germinal
matrix hemorrhage (arrowhead) in the caudothalamic notch (c, caudate; t,
thalamus).
294
FIGURE 9-18. Coronal US, in a different patient, demonstrates a right-
sided grade 1 germinal matrix hemorrhage (arrowhead).
295
FIGURE 9-19. Coronal T2, in a different patient, shows localized grade 1
hemorrhage (arrow). Immature brain shows prominent ventricles and
incomplete sulcation.
296
FIGURE 9-20. Axial CT shows a right acute hemorrhage (arrow) in the
region of the caudothalamic notch with ventricular extension but no
hydrocephalus (grade 2).
297
FIGURE 9-21. Coronal US, in a different patient, shows large clots in the
lateral and third ventricles with ventricular dilatation (grade 3).
298
FIGURE 9-22. Coronal US, in a different patient, shows a large grade 4
bleed on the left.
Suggested Reading
Ballabh P. Pathogenesis and prevention of intraventricular hemorrhage. Clin
Perinatol 2014;41:47–67.
Periventricular Leukomalacia (PVL)

Key Facts
Results from hypoxic–ischemic encephalopathy with reperfusion or
299
fetal/maternal infection.
Occurs in 7% to 22% of premature newborns, with those under 32
weeks of gestation at greatest risk.
May occur in utero or during neonatal period and later in life
produces spastic diplegia.
Periventricular leukomalacia (PVL) represents infarction and
coagulation necrosis of the parasagittal watershed zones (located
between centripetal and centrifugal vascular systems) of the cerebral
hemispheres.
Commonly diagnosed initially by sonography as hyperechogenic
regions neighboring the atria of the lateral ventricles; CT and MRI
may show periventricular hemorrhage.
Chronically produces reactive astrocytosis, gliosis, cystic malacia
(20%), and atrophy; findings are more pronounced in the vicinity of
the occipital horns and atria of the lateral ventricles but may extend
anteriorly to the level of the frontal horns.
PVL is seen in 50% of babies with intracranial hemorrhages.
300
FIGURE 9-23. Parasagittal T1 shows multiple subacute hemorrhages in
the periventricular white matter (arrows).
301
FIGURE 9-24. Axial T2, in a different patient, shows diminished white
matter volume posteriorly, dilatation of the atria and occipital horns of the
lateral ventricles, and an “undulating” outer border of the lateral ventricles,
all compatible with the chronic sequelae of PVL.
302
FIGURE 9-25. Oblique sagittal US, in a different patient, shows cystic
leukomalacia with large cavitations in the parieto-occipital region
(arrowheads).
303
FIGURE 9-26. Coronal IR, in a different patient, shows markedly
diminished white matter volume with depth of sulci approximating the
outer wall of the lateral ventricles.
304
FIGURE 9-27. Axial FLAIR, in a different patient, demonstrates
extensive white matter volume loss with bright areas of gliosis/malacia,
and multiple cavitations (arrowheads).
305
FIGURE 9-28. Midsagittal T1, in a different patient, shows atrophy of
posterior corpus callosum in a patient with PVL.
Suggested Readings
Benders MJ, Kersbergen KJ, de Vries LS. Neuroimaging of white matter injury,
intraventricular and cerebellar hemorrhage. Clin Perinatol 2014;41:69–82.
Panigrahy A, Wisnowski JL, Furtado A, Lepore N, Paquette L, Bluml S.
Neuroimaging biomarkers of preterm brain injury: toward developing the
preterm connectome. Pediatr Radiol 2012;42(suppl 1):S33–S61.
306
CHAPTER 10
Aneurysms
Anterior Communicating (AComm)

Artery Aneurysms
Key Facts
Represent 30% to 35% of ruptured intracranial aneurysms (most
common site).
Overall incidence of intracranial aneurysms is 2% to 8% of the
population; risk of bleeding is 1% to 2% per year in previously
nonruptured aneurysms but increases after rupture and with
increasing aneurysm size.
Aneurysms arising from vessels forming the circle of Willis
constitute 90% of all intracranial aneurysms.
Ruptured aneurysms account for 90% of all spontaneous SAH, with a
mortality rate of about 50%.
Causes of convexity SAH: older patients = amyloid, venous
thrombosis; younger patients = PRES, vasculitis, almost never due to
ruptured aneurysm.
More than 90% of aneurysm ruptures occur between the ages of 30
and 70 years.
Aneurysms <5 mm in diameter are unlikely to rupture (critical size: 5
to 7 mm).
Ruptured aneurysms of the AComm artery result in hemorrhage in the
gyri recti, anterior interhemispheric fissure, septum pellucidum, and
frontal horns of lateral ventricles. AComm aneurysms are the most
common to show intra-axial hemorrhage (others: MCA, PComm,
307
basilar tip).
Rupture of any intracranial aneurysm results in hydrocephalus in 10%
of patients.
There is an association between AComm artery aneurysms and
presence of an azygous or fenestrated anterior cerebral artery.
FIGURE 10-1. Axial noncontrast CT shows acute hemorrhage in the left

gyrus rectus with surrounding edema and blood in the occipital horns of
the lateral ventricles. SAH is seen in the bilateral occipital and right
308
opercular regions.
FIGURE 10-2. Axial noncontrast CT in the same patient shows SAH

along the anterior interhemispheric fissure and blood in the septum
pellucidum and lateral ventricles.
309
FIGURE 10-3. In the same patient, coronal 3-D CTA shows a
multilobulated aneurysm (arrow) in the region of the AComm artery.
310
FIGURE 10-4. Coronal oblique 3-D DSA view, in a different patient,
shows an irregular and ruptured AComm aneurysm.
311
FIGURE 10-5. Translucent 3-D DSA view, in a different patient, shows
AComm aneurysm incorporating several arteries in its base.
SUGGESTED READING
Hacein-Bey L, Provenzale JM. Current imaging assessment and treatment of
312
intracranial aneurysms. AJR Am J Roentgenol 2011;196:32–44.
Basilar Artery Tip Aneurysms

Key Facts
Represent 5% to 10% of ruptured intracranial aneurysms; basilar
artery tip is the most common location for aneurysms arising from
posterior circulation vessels but is considered “supratentorial” and
thus constitutes the fourth most common location for supratentorial
aneurysms.
Ruptured aneurysms of the basilar artery tip result in hemorrhage in
the basilar cisterns, posterior third ventricle, and fourth ventricle.
There is an increased incidence of “giant” aneurysms in this location,
and the posterior cerebral artery origins may be involved, which
complicates treatment.
Common etiologies for all intracranial aneurysms include
hemodynamic stress (increased pulsatility and blood flow), apoptosis
(due to metabolic, genetic, and/or inflammatory processes),
atherosclerosis, posttraumatic, infectious, vasculitis-induced, and
metastatic causes. Basilar artery aneurysms may be associated with
anomalous arteries (fenestrations, persistent primitive
communications).
Endovascular treatment is the mainstay of care as surgical
management is challenging and risky. They are aggressively treated
due to a relatively high risk of rupture and poor outcomes.
Of all patients with intracranial aneurysms, 10% have aortic
(abdominal and thoracic) aneurysms (probably an autosomal
dominant disease, older, white, males).
313
FIGURE 10-6. Noncontrast axial CT shows SAH in basilar cisterns
outlining an aneurysm (arrow) of the basilar artery tip.
314
FIGURE 10-7. Corresponding image from CTA confirms the aneurysm.
315
FIGURE 10-8. Frontal view from surface-rendered CTA, in the same
patient, shows the aneurysm.
316
FIGURE 10-9. Lateral DSA view, in a different patient, shows a 9-mm
aneurysm arising from the basilar artery terminus.
317
FIGURE 10-10. Coronal CTA view, in the same patient, shows that the
aneurysm has a wide neck and involves the origins of the posterior
cerebral arteries bilaterally (arrowheads).
318
FIGURE 10-11. Frontal DSA view, in a different patient, shows a giant
basilar tip aneurysm involving the proximal posterior cerebral arteries.
SUGGESTED READING
Marlin ES, Ikeda DS, Shaw A, Powers CJ, Sauvageau E. Endovascular treatment
of basilar aneurysms. Neurosurg Clin N Am 2014;25:485–495.
319
Giant Aneurysms
Key Facts
Measure > 2.5 cm in diameter; account for 5% of all intracranial
aneurysms.
Common types: thin wall (probably small ones that grew) and thick
wall (grow by bleeding into their walls).
Uncommon types: fusiform (postviral, collagen vascular disorders),
serpentine (recanalized thrombosed aneurysms), and dolichoectatic.
75% of patients present with symptoms due to mass effect (seizures,
headaches, visual and focal neurological deficits, and cranial nerve
palsies, especially if located in the cavernous sinus).
More common in females between the fifth and seventh decades of
life.
Most common sites: bifurcation of ICA, intracavernous ICA, MCA
bifurcation, and tip of basilar artery.
When thin-walled ones bleed, the result is SAH; when thick-walled
ones bleed, the result is intramural hematoma and occasionally
localized and adjacent SAH.
Inflammation plays a role in growth of thick-walled ones and may be
an explanation for the surrounding edema.
Spontaneous thrombosis: 13% to 20%. Endovascular coiling may
prevent rupture and decreases their size.
320
FIGURE 10-12. Axial noncontrast CT shows large suprasellar aneurysm
surrounded by SAH.
321
FIGURE 10-13. Axial T2, in a different patient, shows a predominantly
hypointense giant aneurysm with a bright central component arising from
the right A1-A2 ACA segment.
322
FIGURE 10-14. Corresponding axial postcontrast T1 shows enhancement
of the central component (arrowhead, the rest was thrombosed) and
prominent pulsation artifact in the phase-encoding direction (arrows).
323
FIGURE 10-15. Frontal DSA view, in a different patient, shows giant
aneurysm arising from the left supraclinoid ICA containing the typical
“swirling” pattern of intraluminal contrast.
324
FIGURE 10-16. Coronal postcontrast T1 image, in a different patient,
shows enhancing lesion in right cavernous sinus.
325
FIGURE 10-17. Oblique 3-D view from DSA in the same patient as in
Figure 10-16 shows the lesion to be an aneurysm.
SUGGESTED READING
van Rooij WJ, Sluzewski M. Endovascular treatment of large and giant
aneurysms. AJNR Am J Neuroradiol 2009;30:12–18.
Infratentorial Aneurysms
Key Facts
326
Represent 1% to 3% of all intracranial aneurysms.
After rupture: SAH may be isolated to the posterior fossa and/or
fourth ventricle; may also extend to the cerebellum.
Both PICAs need to be studied in all angiograms performed to rule
out an intracranial aneurysm; this can be done by “refluxing” contrast
into the contralateral vertebral artery or by injecting each vertebral
artery individually (see Chapter 4).
Common sites of PICA aneurysms: origin (usually at the level of the
jugular tubercle) and choroidal point (cranial loop).
Most PICA, AICA, and superior cerebellar artery aneurysms are
probably dissecting in nature.
Conventional angiography fails to demonstrate an intracranial
aneurysm in 5% of all patients with spontaneous subarachnoid
hemorrhage.
Calcification has been associated with adverse outcomes.
327
FIGURE 10-18. Frontal oblique MRA shows an aneurysm (arrow) at the
origin of the left PICA.
328
FIGURE 10-19. In a different patient, lateral DSA view shows irregular-
shaped aneurysm (arrow) at the “choroidal” point of the PICA.
329
FIGURE 10-20. Oblique DSA view, in a different patient, shows
aneurysm (arrow) in the region of the PICA origin.
330
FIGURE 10-21. Frontal DSA view, in a different patient, shows aneurysm
(arrow) arising in the right superior cerebellar artery. (Courtesy H.
Alvarez, Chapel Hill, NC.)
331
FIGURE 10-22. Frontal oblique MRA, in a different patient, shows a
small aneurysm arising from the origin of the left superior cerebellar artery
(arrow).
332
FIGURE 10-23. Oblique 3-D DSA view, different patient, shows a
lobulated aneurysm involving the body of the basilar artery.
SUGGESTED READING
Lehto H, Kivisaari R, Niemelä M, et al. Seventy aneurysms of the posterior
inferior cerebellar artery: anatomical features and value of computed
tomography angiography in microneurosurgery. World Neurosurg
333
2014;82:1106–1112.
Middle Cerebral Artery Bifurcation

Aneurysms
Key Facts
Represent 20% to 30% of intracranial aneurysms; may be “mirror-
like.” The bifurcation is by far the most frequent location of MCA
aneurysms.
Rupture results in SAH in sylvian fissures and basilar cisterns. They
frequently result in parenchymal hemorrhage especially in frontal
opercula.
Risk of rebleeding from any ruptured intracranial aneurysm is 20% to
50% during the 2 weeks that follow presentation.
Factors associated with increased risk of intracranial aneurysms
include family history, fibromuscular dysplasia, polycystic kidney
disease, connective tissue disorders, aortic coarctation, and patients
with intracranial AVMs or hypervascular tumors (glioblastoma,
meningiomas). MRA or CTA is beneficial in screening these patients.
MRA detects over 90% of intracranial aneurysms 3 mm or greater in
diameter (MRA at 3T may detect smaller ones); CTA may detect
those down to 1 mm; CTA may show more aneurysms than catheter
angiography, but 3-D rotational catheter angiography still is “gold
standard.”
334
FIGURE 10-24. Axial noncontrast CT shows acute dense SAH (arrow) in
the right sylvian fissure.
335
FIGURE 10-25. MIP axial view from CTA shows an MCA bifurcation
aneurysm (arrow).
336
FIGURE 10-26. Surface-rendered CTA shows the aneurysm.
337
FIGURE 10-27. Axial CTA, in a different patient, shows MCA
aneurysms bilaterally.
338
FIGURE 10-28. Axial T2, in a different patient, shows MCA bifurcation
aneurysms bilaterally (arrows) with different signal intensities on the right
due to partial thrombosis.
339
FIGURE 10-29. Composite frontal DSA image, in the same patient,
shows the MCA aneurysms bilaterally (arrowheads).
SUGGESTED READINGS
Dashti R, Hernesniemi J, Niemelä M, et al. Microneurosurgical management of
middle cerebral artery bifurcation aneurysms. Surg Neurol 2007;67:441–456.
Jabbour PM, Tjoumakaris SI, Rosenwasser RH. Endovascular management of
intracranial aneurysms. Neurosurg Clin N Am 2009;20:383–398.
Multiple Intracranial Aneurysms
340
Key Facts
10% to 15% of intracranial aneurysms are multiple; they can occur in
mirror locations.
Multiple aneurysms occur most often in women and in patients with
family history of aneurysms.
Multiple aneurysms are associated with hypervascular lesions
(AVMs, tumors); may be infectious, due to metastases, collagen-
vascular disorders (particularly Ehler-Danlos), sickle cell disease,
moyamoya, AIDS, cigarette smoking, and posttrauma (generally
pseudoaneurysms).
Helpful signs to determine which aneurysm bled when multiple are
present: largest, irregular, lobulated (excrescence sign), surrounded
by clot or subarachnoid hemorrhage, increased aspect ratio (depth
related to neck), adjacent vasospasm, aneurysm arising in the anterior
communicating artery, and visible contrast extravasation (very rare).
Incidence of multiple aneurysms: 75% of patients have two
aneurysms, 15% of patients have three aneurysms, and 10% of
patients have four or more aneurysms.
341
FIGURE 10-30. Oblique view from DSA shows multiple aneurysms
(arrows).
FIGURE 10-31. Coronal MIP from CTA, in a different patient, shows

bilateral MCA bifurcation aneurysms (arrows) as well as right
parenchymal and extra-axial hematomas.
342
FIGURE 10-32. Volume-rendered CTA, in a different patient, shows
bilateral PComm aneurysms (arrows).
343
FIGURE 10-33. Lateral DSA view, in a different patient, shows two
aneurysms (arrows) in arteries feeding a frontal AVM.
344
FIGURE 10-34. DSA 3-D image, in a different patient, shows aneurysms
arising from the cavernous ICA and ICA terminus.
SUGGESTED READINGS
Backes D, Vergouwen MD, Velthuis BK, et al. Difference in aneurysm
characteristics between ruptured and unruptured aneurysms in patients with
multiple intracranial aneurysms. Stroke 2014;45:1299–1303.
Baumann F, Khan N, Yonekawa Y. Patient and aneurysm characteristics in
multiple intracranial aneurysms. Acta Neurochir Suppl 2008;103:19–28.
345
Posterior Communicating Artery
Aneurysms
Key Facts
Represent 30% to 35% of ruptured intracranial aneurysms.
Present with ipsilateral third cranial nerve palsy and/or SAH.
In ruptured posterior communicating artery aneurysm, SAH tends to
be diffuse but may be concentrated in the basilar cisterns; they may
also bleed intraparenchymally into the mesial temporal lobe region.
May be difficult to see on CTA particularly if small and pointing
inferiorly.
50% of patients with any ruptured intracranial aneurysm die during
the first 30 days that follow the initial hemorrhage.
Almost all intracranial aneurysms are considered to result from
hemodynamic stress, not from a congenital cause (only 2% of
aneurysms are found in children).
Perimesencephalic bleeds may occur without aneurysm rupture and
may be due to tearing of small veins; DSA and CTA are usually
negative.
346
FIGURE 10-35. Top view of 3-D CTA shows bilateral posterior
communicating artery aneurysms (arrows).
347
FIGURE 10-36. Axial CT, in a different patient, shows a hypodense
aneurysm (arrow) with SAH and intraventricular blood. Note parenchymal
hematoma in the right temporal lobe (arrowhead).
348
FIGURE 10-37. Lateral DSA view, in the same patient, demonstrates a
lobulated aneurysm arising from the right posterior communicating artery.
349
FIGURE 10-38. Axial CT, in a different patient, shows a left temporal
lobe hemorrhage due to a ruptured posterior communicating artery
aneurysm.
SUGGESTED READINGS
Greving JP, Wermer MJ, Brown RD Jr, et al. Development of the PHASES score
for prediction of risk of rupture of intracranial aneurysms: a pooled analysis of
six prospective cohort studies. Lancet Neurol 2014;13:59–66.
Wallace AN, Vyhmeister R, Dines JN, et al. Evaluation of an anatomic definition
of non-aneurysmal perimesencephalic subarachnoid hemorrhage. J
Neurointerv Surg 2016;8:378–385. pii: neurintsurg-2015-011680. doi:
10.1136/neurintsurg-2015-011680
350
Pseudoaneurysms
Key Facts
Posttraumatic aneurysms account for <1% of all aneurysms. They
may also be iatrogenic, secondary to tumor invasion, radiation
necrosis, or fungal infection.
They are usually secondary to tears in the parent artery wall, which
may be irregular and/or stenotic; these pseudoaneurysms may have an
irregular shape.
When secondary to penetrating wounds, they are commonly
associated with gunshots.
Nonpenetrating injuries produce aneurysms at the base of the skull or
distal branches of the anterior cerebral artery as they shear against the
undersurface of the falx cerebri.
Common sites ICA: bifurcation, as it enters the base of the skull,
cavernous sinus, and supraclinoid segment.
Common sites vertebral artery: as it enters the foramen
transversarium (generally C6), at C2 and C1, and as it pierces the
dura and enters the foramen magnum.
351
FIGURE 10-39. Oblique contrast-enhanced MRA shows a “spontaneous”
left vertebral pseudoaneurysm (arrow).
352
FIGURE 10-40. Corresponding oblique 3-D DSA reconstruction depicts
the morphology of the pseudoaneurysm.
353
FIGURE 10-41. Oblique DSA shows a posttraumatic left vertebral artery
pseudoaneurysm in a different patient.
354
FIGURE 10-42. Oblique DSA, in a different patient, shows an ICA
pseudoaneurysm (arrow) at the level of the skull base.
355
FIGURE 10-43. Axial CTA, in a different patient, shows a large traumatic
pseudoaneurysm involving the left internal carotid artery (arrow) with a
large mural thrombus (arrowhead).
356
FIGURE 10-44. Corresponding color Doppler ultrasound shows the
typical “yin-yang” sign due to swirling flow within the nonthrombosed
portion of the pseudoaneurysm.
SUGGESTED READINGS
Foreman PM, Griessenauer CJ, Falola M, Harrigan MR. Extracranial traumatic
aneurysms due to blunt cerebrovascular injury. J Neurosurg
2014;120:1437–1445.
Yi AC, Palmer E, Luh GY, Jacobson JP, Smith DC. Endovascular treatment of
carotid and vertebral pseudoaneurysms with covered stents. AJNR Am J
Neuroradiol 2008;29:983–987.
Vasospasm (after SAH)
357
Key Points
Due to release of spasmogenic substances after lysis of blood clots.
Clinically present in about 30% to 40% of patients with aneurysmal
SAH, although up to 70% show angiographic evidence of it.
Unlikely after traumatic SAH.
Almost always present if aneurysm rebleeds.
Major cause of morbidity and mortality after aneurysm rupture due to
delayed cerebral ischemia and multiple brain infarctions.
Begins about 3 to 5 days after SAH, peaks at about 7 to 10 days, and
may last up to 3 weeks.
Traditionally treated with hypertension, hyperhydration, and
hypervolemia (so-called triple H therapy), but other treatments
include vasodilators, mechanical vasodilation, and calcium channel
blockers, which may be given intra-arterially.
May be noninvasively evaluated with Doppler sonography of the
MCA (velocity of >120 cm/s) and CT or (less commonly) MR
angiography. Perfusion CT and MR show areas of low perfusion.
358
FIGURE 10-45. Axial noncontrast CT shows predominantly right-sided
SAH and acute infarctions in both parietal regions. Ventriculostomy is
seen.
359
FIGURE 10-46. Axial noncontrast CT, in a different patient, shows
multiple bilateral acute infarctions and hemorrhages as well as diffuse
brain swelling. Ventriculostomy is seen.
360
FIGURE 10-47. Frontal view right carotid DSA shows severe vasospasm
involving the distal ICA and branches of the middle and anterior cerebral
arteries.
361
FIGURE 10-48. Corresponding Doppler values demonstrate increased
mean velocities in the MCA bilaterally, worse on the right side.
362
FIGURE 10-49. Basal CTA MIP reconstruction in a different patient
shows diffuse vasospasm bilaterally with poor visualization of anterior,
middle, and posterior cerebral artery branches.
363
FIGURE 10-50. Corresponding rCBF map from CT perfusion shows
areas of hypoperfusion bilaterally, worst in the left MCA and bilateral
ACA territories.
SUGGESTED READINGS
Baggott CD, Aagaard-Kienitz B. Cerebral vasospasm. Neurosurg Clin N Am
2014;25:497–528.
Sanelli PC, Kishore S, Gupta A, et al. Delayed cerebral ischemia in aneurysmal
subarachnoid hemorrhage: proposal of an evidence-based combined clinical
and imaging reference standard. AJNR Am J Neuroradiol 2014;35:2209–2214.
364
CHAPTER 11
Vascular
Malformations
Arteriovenous Malformations (AVM)

Key Facts
Congenital in nature, generally present in middle age (65% in patients
> 40 years of age).
Very rare malformation, incidence of 1:100,000 individuals. AVMs
represent 25% of all intracranial vascular malformations.
Symptoms: hemorrhage (more common in children and usually
parenchymal, although primary subarachnoid hemorrhage may
occur), seizures (more common in cortical lesions and superficial
drainage), and headaches.
Risk of bleeding is 2% to 3% per year with 20% to 30% mortality per
episode. Cumulative lifetime risk of bleeding is 70%.
Factors associated with increased risk of bleeding: previous rupture,
deep or periventricular location, intranidal aneurysms, venous
aneurysms or stenoses, deep venous drainage, and single draining
vein.
Location: more than 80% are supratentorial (especially parietal).
AVMs are solitary in 98% of patients, with multiple AVMs (2%) seen
in Rendu-Osler-Weber (who can also present with micro-AVMs),
Wyburn-Mason syndrome, and CAMS (craniofacial arteriovenous
metameric syndrome).
365
Major vascular supply is generally from ICA (pial, 75%), but large
AVMs may recruit external carotid artery (ECA) (dural, 15%) vessels
or both (mixed, 10%) or even contralateral ICA and ECA.
CT shows calcification in 30% of intracranial AVMs; cysts may be
seen especially after stereotactic radiosurgery.
FIGURE 11-1. Axial T2 shows the nidus of a left frontoparietal AVM

(arrow) with arterial feeders from the anterior and left middle cerebral
arteries.
366
FIGURE 11-2. Lateral view DSA reconstruction, in a different patient,
shows an AVM with multiple feeders and large draining veins (arrow).
Note flow-related ACA aneurysm (arrowhead).
367
FIGURE 11-3. Frontal TOF MRA view, in a different patient, only faintly
shows AVM nidus due to fast flow and signal dephasing.
368
FIGURE 11-4. Corresponding angiogram shows feeding arteries, nidus,
and draining vein (arrow) not seen on MRA due to fast and turbulent flow.
369
FIGURE 11-5. 3-D DSA shows frontal AVM with multiple flow-related
aneurysms (arrows) in feeding arteries.
370
FIGURE 11-6. Lateral DSA view, in a different patient, shows the
presence of an AVM and an aneurysm (arrow) in draining vein.
SUGGESTED READINGS
Alexander MD, Cooke DL, Nelson J, et al. Association between venous
angioarchitectural features of sporadic brain arteriovenous malformations and
intracranial hemorrhage. AJNR Am J Neuroradiol 2015;36:949–952.
Alvarez H, Castillo M. Genetic markers and their influence on cerebrovascular
malformations. Neuroimaging Clin N Am 2015;25:69–82.
Capillary Telangiectasias
371
Key Facts
More common in pons but may occur anywhere in the brain; found in
<1% of the population, nearly always asymptomatic and incidentally
found by MRI.
Represent 10% to 20% of cerebral vascular malformations.
Generally measure <2 cm in diameter but occasionally may be
“giant.”
Histologically, they contain dilated capillaries with interspersed
normal brain tissue and have no gliosis, calcification, or hemosiderin.
They may be associated with cavernous and venous malformations
and can show prominent draining veins, particularly when large.
Capillary telangiectasias develop after whole-brain irradiation,
particularly in children. If very large, they have a clinically aggressive
behavior.
Enhance after contrast administration (“brush-like” enhancement), are
of low signal intensity on gradient echo imaging (probably due to
magnetic susceptibility effects from deoxyhemoglobin related to slow
flow), and generally show no abnormality on noncontrast T1/FLAIR
but sometimes may be slightly bright on T2/FLAIR due to gliosis.
They are dark on T2* (with SWI being most sensitive) and DWI
(these findings are better depicted at 3.0T). Capillary telangiectasias
are angiographically occult.
372
FIGURE 11-7. Axial postcontrast T1 shows faint focal enhancement in
the pons (arrow) corresponding to an incidentally found capillary
telangiectasia.
373
FIGURE 11-8. Susceptibility-weighted sequence shows corresponding
low signal.
374
FIGURE 11-9. Axial postcontrast T1, in a different patient, shows a
capillary telangiectasia in the left basal ganglia (arrow).
375
FIGURE 11-10. Corresponding DWI shows the telangiectasia to be of
low signal (arrow).
SUGGESTED READINGS
El-Koussy M, Schroth G, Gralla J, et al. Susceptibility-weighted MR imaging for
diagnosis of capillary telangiectasia of the brain. AJNR Am J Neuroradiol
2012;33:715–720.
Gross BA, Puri AS, Popp AJ, Du R. Cerebral capillary telangiectasias: a meta-
analysis and review of the literature. Neurosurg Rev 2013;36:187–193.
Carotid Artery–Cavernous Sinus Fistulas
376
Key Facts
Direct (high flow) type:
Direct communications between intracavernous ICA and the

cavernous sinus.
Usually seen in young males and constitute nearly 10% of all
intracranial vascular malformations with shunting.
Occur secondary to traumatic tear of the ICA or to rupture of an
intracavernous ICA aneurysm. They are occasionally spontaneous in
patients with fibromuscular dysplasia, Ehlers-Danlos syndrome, and
pseudoxanthoma elasticum.
Usually drain into the superior ophthalmic vein and inferior petrosal
sinus.
Present with pulsatile exophthalmos, bruit, conjunctival chemosis,
and cranial nerve palsies.
Indirect (low flow) type:
Less common than direct type.

Caused by communication of multiple dural branches from ECA
and/or ICA with the cavernous sinus.
Most occur spontaneously in middle-aged females.
May be asymptomatic or present with proptosis, conjunctival
chemosis, and bruit.
377
FIGURE 11-11. Axial CTA shows enlargement of the right superior
ophthalmic vein in a patient with a carotid–cavernous fistula.
378
FIGURE 11-12. Base MRA view, in a different patient, shows prominent
flow-related enhancement of the left greater than right cavernous sinus
(arrow) and bilateral superior ophthalmic veins (arrowheads).
379
FIGURE 11-13. Frontal view DSA, in the same patient, shows
enhancement of the left greater than right cavernous sinus and ophthalmic
veins immediately following left carotid injection.
380
FIGURE 11-14. Lateral DSA view, in a different patient, shows
immediate filling of the cavernous sinus (arrowhead) with arterial blood
and drainage into the superior ophthalmic vein (arrow) and inferior
petrosal sinus.
381
FIGURE 11-15. Lateral DSA view, in a different patient, shows high-flow
fistula with drainage into cortical veins (arrow), inferior petrosal sinus
(arrowhead), and superior ophthalmic vein (wavy arrow).
382
FIGURE 11-16. Lateral DSA view from ECA injection, in a different
patient, shows an indirect cavernous sinus fistula with drainage to the
superior ophthalmic vein (arrow).
SUGGESTED READINGS
Bink A, Goller K, Lüchtenberg M, et al. Long term outcome after coil
embolization of cavernous carotid arteriovenous fistulas. AJNR Am J
Neuroradiol 2010;31:1216–1221.
Zanaty M, Chalouhi N, Tjoumakaris SI, Hasan D, Rosenwasser RH, Jabbour P.
Endovascular treatment of carotid-cavernous fistulas. Neurosurg Clin N Am
2014;25:551–563.
Cavernous Malformations
383
Key Facts
Slow-flow low-pressure malformations typically with no normal
intervening brain parenchyma.
80% are supratentorial and 15% are multiple (often a familial
component is present), and they may occur in the presence of
developmental venous anomalies.
Multiple (10% to 30%) ones may be seen after irradiation or in a
specific familial syndrome (autosomal dominant). Giant ones are
more common in children. Can also rarely arise de novo after brain
biopsy or after treatment of AVM.
Second most common intracranial vascular malformation after
venous ones and most common angiographically occult lesion.
Annual risk of bleeding is <1%; when it occurs, it tends to be self-
limited and clinically not significant; however, risk of bleeding
increases after one hemorrhage. Hemorrhagic ones typically have a
surrounding rim of moderate T1 hyperintensity.
Most common clinical symptom is seizures (50%) but can also
present with progressive neurologic deficits and cranial neuropathies
when in the brainstem. Most are initially asymptomatic.
Many have associated venous malformations (called “transitional”
type); cavernous malformations may form from occlusion of a vein in
a developmental venous anomaly.
Surrounding tissue is gliotic and hemosiderin laden. They are better
visualized with T2* and SWI.
384
FIGURE 11-17. Axial T2 shows the typical “popcorn” or “mulberry”
appearance of a cavernous malformation in the right basal ganglia with
dark hemosiderin deposition.
385
FIGURE 11-18. Corresponding SWI shows numerous additional dark
lesions bilaterally corresponding to multiple cavernous malformations due
to history of radiation.
386
FIGURE 11-19. Axial noncontrast CT, in a different patient, shows a
dense left frontal cavernous malformation (arrow).
387
FIGURE 11-20. Axial T2*, in a different patient with familial cavernous
malformation syndrome shows multiple dark lesions.
388
FIGURE 11-21. Axial noncontrast T1, in a different patient, shows
hemorrhagic cavernous malformation with typical hyperintense rim.
389
FIGURE 11-22. Axial noncontrast T1, in a different patient, shows a giant
cavernous malformation resulting in obstructive hydrocephalus.
SUGGESTED READINGS
Cutsforth-Gregory JK, Lanzino G, Link MJ, Brown RD Jr, Flemming KD.
Characterization of radiation-induced cavernous malformations and
comparison with a nonradiation cavernous malformation cohort. J Neurosurg
390
2015;20:1–9.
Mikati AG, Tan H, Shenkar R, et al. Dynamic permeability and quantitative
susceptibility: related imaging biomarkers in cerebral cavernous
malformations. Stroke 2014;45:598–601.
Developmental Venous Anomalies (DVA)

Key Facts
Most common (60%) cerebral vascular malformation, occurring in
2% of the general population (formerly called venous angioma).
Most are incidentally found and asymptomatic but occasionally may
present with seizures, headaches, and/or focal neurologic deficits.
Hemorrhage is uncommon but may occur particularly in the posterior
fossa; when there is hemorrhage, a coexisting cavernous
malformation (15% to 20%) is usually responsible. Hemorrhagic or
ischemic infarction may also occur secondary to stenosis or
thrombosis of a vein.
Increased risk of concomitant cavernous malformation: DVAs that
are infratentorial, multiple, and have a greater number of medullary
veins.
Isolated DVAs are considered an extreme form of a normal variant
because they drain normal brain and are composed of dilated
medullary veins (“Medusa” head) that form a large channel draining
into cortical veins (65%) or subependymal veins (35%).
>65% are supratentorial, and most occur in the frontal lobes.
Most are solitary; multiple may be seen in the blue rubber bleb nevus
syndrome. May be associated with sinus pericranii, cortical
dysplasias, areas of gliosis, infarction, and facial venolymphatic
malformations.
10% to 50% show T2 FLAIR signal hyperintensity in the parenchyma
drained by the DVA, probably due to venous congestion resulting in
vasogenic edema and/or gliosis.
On perfusion studies, surrounding parenchyma shows increased
rCBV, rCBF, and rMTT. On SWI, they may show surrounding low
signal intensity due to slow blood flow and deoxygenation.
391
FIGURE 11-23. Axial postcontrast T1 shows a right frontal DVA (arrow)
with a small associated cavernous malformation (arrowhead).
392
FIGURE 11-24. Lateral DSA view, in a different patient, shows typical
appearance of “Medusa” head (arrow).
393
FIGURE 11-25. Axial postcontrast T1, in a different patient, shows large
DVA of the right temporal lobe that was accompanied by cortical
dysplasia and seizure.
394
FIGURE 11-26. Axial SWI, in a different patient, shows right parietal
DVA surrounded by halo of low signal due to the presence of
deoxygenated blood.
395
FIGURE 11-27. Axial postcontrast T1, in a different patient, showing
right cerebellar DVA.
396
FIGURE 11-28. Corresponding MR rCBF map shows increased perfusion
in the area of the DVA.
SUGGESTED READINGS
Jung HN, Kim ST, Cha J, et al. Diffusion and perfusion MRI findings of the
signal-intensity abnormalities of brain associated with developmental venous
anomaly. AJNR Am J Neuroradiol 2014;35:1539–1542.
Sharma A, Zipfel GJ, Hildebolt C, Derdeyn CP. Hemodynamic effects of
developmental venous anomalies with and without cavernous malformations.
Dural Arteriovenous Fistulas

Key Facts
Occlusion (thrombosis) of a venous sinus is probably responsible for
397
their formation as it stimulates angiogenesis in an attempt to
recanalize the sinus and supply its walls with blood.
Most occur in the cavernous sinuses, posterior fossa (near transverse
and sigmoid sinuses), and tentorium.
Most are supplied by branches of the ECA, but dural ICA branches
may also be involved.
Most remain asymptomatic; when symptoms are present, bruit
(objective tinnitus), headaches, and bleeds occur (particularly when
drainage occurs via cortical veins). Cortical vein (leptomeningeal)
reflux predicts aggressive behavior (hemorrhage, progressive
neurological deficits) and is an indication for emergent treatment.
This is more common in the anterior cranial fossa and tentorial
incisura.
They are rarely seen by CT or MRI (unless they are very large), and
catheter angiography is the diagnostic method of choice. SWI may
identify enlarged, tortuous, and bizarre-appearing veins related to
shunting and high pressure. MRI may show cerebral edema and/or
macro- or microhemorrhages. ASL may show shunt as area of very
high signal intensity.
398
FIGURE 11-29. Axial “source” image from TOF MRA shows abnormal
blood vessels (arrow) in right transverse sinus.
399
FIGURE 11-30. Nearly frontal MRA view, in the same patient, shows that
the fistula is mainly supplied by an enlarged right occipital artery (arrows).
400
FIGURE 11-31. Axial SWI, in a different patient, shows prominent and
tortuous veins most notably in the left posterior cerebral hemisphere.
401
FIGURE 11-32. Lateral DSA view, in a different patient, shows sigmoid
sinus AVF (arrowhead) fed by branches of the middle meningeal (white
arrow) and occipital (black arrow) arteries.
402
FIGURE 11-33. Lateral DSA view, in a different patient, shows large and
tortuous arteries along the falx cerebri (arrow) arising from the PCAs,
feeding an AVF in the superior sagittal sinus.
403
FIGURE 11-34. Corresponding venous phase demonstrates retrograde
flow into cortical cerebral veins (arrows) and superior sagittal sinus
anterior to the fistula.
SUGGESTED READINGS
Baltsavias G, Parthasarathi V, Aydin E, Al Schameri RA, Roth P, Valavanis A.
Cranial dural arteriovenous shunts. Part 1. Anatomy and embryology of the
bridging and emissary veins. Neurosurg Rev 2015;38:253–264.
Satomi J, Ghaibeh AA, Moriguchi H, Nagahiro S. Predictability of the future
development of aggressive behavior of cranial dural arteriovenous fistulas
based on decision tree analysis. J Neurosurg 2015;123:86–90.
Vein of Galen Malformations

404
Key Facts
Arteriovenous shunting into an embryonic median prosencephalic
vein of Markowski (“vein of Galen” malformation, technically a
misnomer but use is widespread).
Type 1(fistulas):
Choroidal: Multiple feeders from choroidal, thalamostriate, and
pericallosal arteries empty into vein of Galen; usually presents in
neonates with congestive heart failure and/or hydrocephalus
(management of hydrocephalus is controversial; shunting may
aggravate symptoms). Usually show stenosis of draining venous sinus
and persistence of falcine sinus.
Mural: Few feeders from collicular or posterior choroidal arteries
supplying periphery of dilated vein of Galen. This is a true pial
fistula.
Type 2: AVM in the midbrain or thalamus with central drainage into
vein of Galen; usually presents later in infancy with developmental
delay, bruit, seizure, or hemorrhage.
Type 3: Combination of arteriovenous fistula and arteriovenous
malformation.
Pseudomalformation: Refers to dilatation of the true vein of Galen as
a secondary phenomenon due to a vascular lesion (generally an
AVM) proximal to but draining into it.
Intragalenic turbulent flow and distal stenosis probably lead to the
massive dilatation of the outflow tract and produce a vein of Galen
“aneurysm.”
Chronic changes include cerebral atrophy and parenchymal
calcifications (“melting” brain).
405
FIGURE 11-35. Sagittal contrast-enhanced T1 shows a very large
aneurysmal-shaped vein of Galen (arrow) draining into an enlarged
persistent falcine sinus (arrowhead). Note absence of the straight sinus.
406
FIGURE 11-36. Coronal oblique US shows a large aneurysmal vein of
Galen malformation between the posterior lateral ventricles.
407
FIGURE 11-37. Corresponding color Doppler US demonstrates turbulent
flow within the malformation.
408
FIGURE 11-38. Axial T2, in a different child, shows a large vein of Galen
draining into the falcine sinus.
409
FIGURE 11-39. Lateral TOF MRA view in the same child shows this to
be a choroidal type of malformation with multiple feeding arteries.
410
FIGURE 11-40. Axial T2, in a different patient, shows a dilated vein of
Galen (pseudomalformation) due to drainage from a large left temporal
AVM (not shown) via a large perimesencephalic vein (arrow).
SUGGESTED READINGS
Alvarez H, Garcia Monaco R, Rodesch G, Sachet M, Krings T, Lasjaunias P.
Vein of Galen aneurysmal malformations. Neuroimaging Clin N Am
2007;17:189–206.
Chow ML, Cooke DL, Fullerton HJ, et al. Radiological and clinical features of
vein of Galen malformations. J Neurointerv Surg 2014;7:443–448.
411
CHAPTER 12
Extra-Axial Masses
Arachnoid Cyst
Key Facts
Account for 1% of intracranial masses, usually found in children, and
more common in males.
Locations: middle cranial fossa and sylvian fissure (50% to 60%),
suprasellar (10%), quadrigeminal plate cistern (10%),
cerebellopontine angle region (5% to 10%), supravermian cistern
(<5%), cisterna magna (<5%), prepontine cistern, and convexities.
Multiple ones may be seen in glutaric aciduria, tuberous sclerosis, or
mucopolysaccharidoses or be incidental. Other associations: Pallister-
Hall and Aicardi syndromes and neurofibromatosis type I.
They appear as CSF equivalent masses by all imaging modalities;
may have slightly increased signal on DWI and FLAIR due to the
presence of proteins, blood, and turbulence.
The underlying brain may be hypoplastic or compressed.
Arachnoid cysts may produce adjacent bone changes (scalloping) and
grow by pulsations, ball-valve mechanism, and production of CSF.
Only reliable manner to assess communication with remainder of
CSF spaces is via contrast CT cisternography.
Main differential diagnosis: epidermoid, cystic meningioma,
cysticercosis.
412
FIGURE 12-1. Axial CT shows left middle cranial fossa arachnoid cyst
with anterior displacement and thinning (arrow) of the ipsilateral greater
sphenoidal wing and proptosis.
413
FIGURE 12-2. Axial T2, in a different patient, shows an arachnoid cyst in
the suprasellar cistern (arrow) splaying the cerebral peduncles and
displacing the optic chiasm and circle of Willis vessels.
414
FIGURE 12-3. Midsagittal T1 demonstrates cyst in the pineal gland
region with mass effect on the cerebellum/dorsal midbrain and subsequent
hydrocephalus.
415
FIGURE 12-4. Axial CT, in a different patient with known arachnoid
cyst, shows dense contents due to hemorrhage following trauma (arrows).
416
FIGURE 12-5. Axial T2, in a different patient, shows very large left-sided
cyst with brain compression and midline shift.
417
FIGURE 12-6. Axial FLAIR, in a different patient, shows an
intraventricular arachnoid cyst in the atrium and posterior body of the left
lateral ventricle (arrow).
SUGGESTED READINGS
Pradilla G, Jallo G. Arachnoid cysts: case series and review of the literature.
Neurosurg Focus 2007;22:E7.
Westermaier T, Schweitzer T, Ernestus RI. Arachnoid cysts. Adv Exp Med Biol
2012;724:37–50.
Choroid Plexus Tumors

418
Key Facts
Tumors arising from the choroid plexus represent <1% of intracranial
tumors in adults and 2% to 4% of intracranial tumors in children.
>85% are diagnosed by 5 years of age.
Papillomas (WHO grade 1) account for 80% of primary choroid
plexus tumors. In children, they more often involve the atrium of
lateral ventricles (80%, left > right) and are large at diagnosis.
In adults, papillomas are more often found in the fourth ventricle and
are small in size at diagnosis.
Choroid plexus tumors may cause hydrocephalus by overproduction
of CSF, hemorrhage, and desquamation of cells into CSF spaces.
Choroid plexus carcinomas are rare (10% to 20% of all choroid
plexus tumors) and are seen almost exclusively in children; should be
suspected when there is surrounding edema and invasion of the brain
parenchyma.
By CT, 20% of choroid plexus papillomas/carcinomas have
calcifications.
On MR perfusion and DSA, these tumors are extremely vascular. An
enlarged trigonal artery may be seen if the tumor is in the atrium of
the lateral ventricle.
Main differential diagnosis: ependymoma (and subependymoma
when in the fourth ventricle), meningioma, metastasis, astrocytoma.
Associated with Li-Fraumeni and Aicardi syndromes and
hypomelanosis of Ito.
419
FIGURE 12-7. Axial postcontrast CT shows markedly enhancing tumor in
the region of the atrium of the right lateral ventricle.
420
FIGURE 12-8. Axial T2, in a different patient, shows papilloma in the left
lateral ventricle. Note small central flow void due to vascularity as well as
ventricular clot (arrow).
421
FIGURE 12-9. Corresponding coronal postcontrast T1 shows extension of
the tumor into the third ventricle.
422
small papilloma in the atrium of the left lateral ventricle, with typical
“frond-like” appearance.
423
FIGURE 12-11. Midsagittal postcontrast T1, in a different patient, shows
a small and incidentally found tumor (arrow) in the inferior fourth
ventricle.
424
large papilloma centered in the right foramen of Luschka.
SUGGESTED READING
Smith AB, Smirniotopoulos JG, Horkanyne-Szakaly I. From the radiologic
pathology archives: intraventricular neoplasms: radiologic-pathologic
correlation. Radiographics 2013;33:21–43.
Colloid Cyst
Key Facts
Accounts for <1% of intracranial tumors and 10% to 20% of
425
intraventricular tumors.
Derives from embryonic endoderm; usually arise in the
anterosuperior portion of the third ventricle (very rarely in other sites)
and obstruct the foramina of Monro producing hydrocephalus (may
be intermittent or acute with rapid herniation and death).
Symptoms: headache (migraine like), mental status changes, nausea,
and vomiting (particularly severe in the morning).
MRI: most are of high T1 and low T2 signal intensities, probably due
to viscous proteinaceous material. On FLAIR, may be difficult to
distinguish from adjacent CSF! May have a “fried egg” appearance
with a nodule of high or low signal intensity.
CT: most are hyperdense and generally measure 10 to 20 mm in
diameter (may be easier to visualize with CT than with MRI).
Both low T2 signal on MRI and increased density on CT have been
associated with difficult percutaneous aspiration due to high
viscosity. Mammillary body atrophy may be seen after resection,
probably from forniceal injury.
May be giant, and MRS shows a large peak that resembles NAA with
no other viable metabolites.
Main differential diagnosis: none, appearance is typical.
426
FIGURE 12-13. Axial CT shows small hyperdense colloid cyst.
427
FIGURE 12-14. Midsagittal T1, in a different patient, shows the classic
location of a colloid cyst in the roof of the anterior third ventricle abutting
the foramina of Monro (arrow).
428
FIGURE 12-15. Midsagittal T1, in a different patient, shows fried egg
appearance of large colloid cyst with hyperintense nodule inferiorly.
429
FIGURE 12-16. Axial T2, in a different patient, shows fried egg
appearance with hypointense nodule (a.k.a. “black hole” effect).
430
FIGURE 12-17. Axial CT shows a giant, atypical, hyperdense colloid
cyst.
431
FIGURE 12-18. In the same patient, long TE MRS shows a pronounced
NAA-type peak typical of these lesions. (Case courtesy A. Mansour,
Assam, Jordan.)
SUGGESTED READING
Azab WA, Salaheddin W, Alsheikh TM, Nasim K, Nasr MM. Colloid cysts
posterior and anterior to the foramen of Monro: anatomical features and
implications for endoscopic excision. Surg Neurol Int 2014;5:124.
432
Craniopharyngioma
Key Facts
Histologically benign but locally aggressive epithelial tumors, almost
always located in the suprasellar (20%, most common suprasellar
mass in children) or suprasellar/sellar (75%) regions but may be
purely intrasellar (<10%) or even more rarely infrasellar.
Most found between 5 and 14 years of age; second peak in adults
between the fifth and seventh decades of life.
Symptoms: visual abnormalities (compression of the optic chiasm),
endocrine dysfunction (hypothalamus/pituitary compression), and/or
hydrocephalus.
Types: adamantinomatous (cystic and occurring in children) and
papillary (solid and occurring in adults).
85% have cysts (more commonly in the adamantinomatous type),
75% measure between 2 and 6 cm, 90% have calcifications
(adamantinomatous type), and 90% enhance.
MR appearance may be extremely variable, but most show low signal
intensity on T1WI, are bright on FLAIR/T2WI, and show peripheral
enhancement or enhancement of solid portions.
Main differential diagnosis when cystic: arachnoid cysts, Rathke cleft
cyst, epidermoid, degenerated pituitary adenoma; when solid:
astrocytoma, thrombosed aneurysm, germ cell tumors.
433
FIGURE 12-19. Coronal postcontrast T1 shows a mostly cystic tumor
with small nodular enhancement inferiorly.
434
a complex partially cystic large craniopharyngioma.
435
FIGURE 12-21. Axial CT, in a different patient, shows a predominantly
solid, partially cystic craniopharyngioma with multiple calcifications.
436
FIGURE 12-22. Sagittal T2, in a different patient, shows a
craniopharyngioma with hypointensities representing calcifications. Note
obstructive dilatation of the lateral ventricles.
437
FIGURE 12-23. Midsagittal postcontrast T1 shows a predominantly solid,
papillary type craniopharyngioma in an adult patient.
438
FIGURE 12-24. Sagittal CT, in a different patient, shows a calcified
infrasellar craniopharyngioma projecting into the nasopharynx. Note that
the sella turcica is intact.
SUGGESTED READINGS
Larkin SJ, Ansorge O. Pathology and pathogenesis of craniopharyngiomas.
Pituitary 2013;16:9–17.
Lee HJ, Wu CC, Wu HM, et al. Pretreatment diagnosis of suprasellar papillary
craniopharyngioma and germ cell tumors of adult patients. AJNR Am J
Neuroradiol 2015;36:508–517.
Dermoid
Key Facts
439
Probably arises from intracranial inclusion of ectodermal elements
during neural tube closure and may rarely develop after
traumatic/iatrogenic implantation.
Contains skin appendages (sebaceous and sweat glands, hair follicles)
Rare (<1% of all intracranial tumors), present in early-to-middle
adulthood (30 to 50 years) with slight male predominance.
Common sites: lumbar spine (associated with spinal dysraphism),
parasellar region, floor of the anterior cranial fossa, posterior fossa
including the fourth ventricle (may be associated with Goldenhar
syndrome and Klippel-Feil syndrome and other dysraphisms of the
craniocervical junction), and fontanelles.
Almost all intracranial dermoids are midline in location and have the
same imaging characteristics as fat due to the liquid cholesterol that
they contain. They can also have areas of profound T2 hypointensity
and calcifications may be present.
They may produce chemical meningitis or ventriculitis (with
headaches and seizures) and ischemia from vasospasm if they rupture
and spill their contents into the CSF spaces.
Main differential diagnosis: lipoma, teratoma, bright epidermoid,
craniopharyngioma, hemorrhagic arachnoid cyst.
Very rare malignant transformation into squamous cell carcinoma.
440
FIGURE 12-25. Axial CT shows a complex fatty and calcified mass in the
anterior cranial fossa and suprasellar cistern. There are droplets of fat in
the sulci of the left frontal lobe.
441
FIGURE 12-26. Axial CT, in the same patient, shows fat (arrows) in sulci
and in the ventricles and mild hydrocephalus.
442
FIGURE 12-27. Axial T1, in a different patient, shows a dermoid in the
right frontotemporal region (arrow) with a fat-fluid level. Note bright
intraventricular fat due to rupture (arrowhead).
443
FIGURE 12-28. Axial T1 more inferiorly in the same patient shows the
dermoid (arrow) and multiple fat droplets in the sulci and basal cisterns
(arrowheads).
444
FIGURE 12-29. Sagittal T1, in a different patient, shows fat within the
lateral and third ventricles and multiple fat droplets in the sulci, folia, and
interhemispheric fissure.
445
FIGURE 12-30. Axial T1, in a different patient, shows a bright dermoid
in the left prepontine cistern invading Meckel cave (arrow).
SUGGESTED READING
Liu JK, Gottfried ON, Salzman KL, Schmidt RH, Couldwell WT. Ruptured
intracranial dermoid cysts: clinical, radiographic, and surgical features.
Neurosurgery 2008;62:377–384.
Epidermoid
Key Facts
446
Arises from intracranial inclusion of epithelial elements during neural
tube closure and are 10 times more common than intracranial
dermoids. May rarely be acquired after surgery or trauma (more
common in the spine than intracranial).
Does not contain skin appendages.
Tends to present in early adulthood and grow very slowly. Produce
symptoms due to mass effect on neurovascular structures and can
manifest with headaches and cranial neuropathies.
Locations: cerebellopontine angle cistern (50%), sella and parasellar
(10% to 15%), fourth ventricle, and prepontine cistern (all located off
the midline; tend to insinuate rather than displace). Remember that in
the posterior fossa in children, they may be associated with Klippel-
Feil syndrome and even become infected.
Also, occur in the diploic space of the skull (10%), giving rise to a
lytic lesion with scalloped sclerotic borders. Also, involve the
fontanelles in children.
MRI sequences that distinguish epidermoid from arachnoid cyst are
FLAIR and DWI (increased signal in epidermoids), CISS/FIESTA
(visualization of internal structures), and MRS (presence of lipids).
Main differential diagnosis: arachnoid cyst, black dermoid, cystic
tumor, cysticercosis, neurenteric cyst.
Rare malignant degeneration into squamous cell carcinoma (due to
epithelial elements).
447
FIGURE 12-31. Axial postcontrast T1 shows left temporal epidermoid
with mildly heterogeneous low signal.
448
FIGURE 12-32. Corresponding FLAIR shows no CSF suppression within
the lesion and internal heterogeneity with a typical “dirty” appearance.
449
FIGURE 12-33. Corresponding DWI shows the lesion to be very bright.
450
FIGURE 12-34. Coronal CISS, in the same patient, demonstrates the
complex internal features of the mass.
451
FIGURE 12-35. Axial 3-D SPACE, in a different patient, shows an
epidermoid in the posterior fossa encasing and stretching the left IX-X-XI
complex (arrow).
452
FIGURE 12-36. Axial FLAIR, in a different patient, shows an epidermoid
involving the right parietal calvarium.
SUGGESTED READINGS
Nagasawa D, Yew A, Safaee M, et al. Clinical characteristics and diagnostic
imaging of epidermoid tumors. J Clin Neurosci 2011;18:1158–1162.
Santhosh K, Thomas B, Radhakrishnan VV, et al. Diffusion tensor and tensor
metrics imaging in intracranial epidermoid cysts. J Magn Reson Imaging
2009;29:967–970.
Lipoma
453
Key Facts
Arise from erroneous differentiation of cells of the meninx primitiva
(which normally form the subarachnoid space) into fat. They are
congenital lesions rather than true neoplasms.
85% occur in the midline (50% are pericallosal); other locations:
quadrigeminal plate, cerebellopontine angle, suprasellar, and sylvian
cisterns.
While dermoids, which are also T1 bright, tend to occur in the
midline, lipomas happen in infoldings of the subarachnoid space and
are more homogeneous.
Calcification is common in pericallosal or interhemispheric lipomas,
particularly when associated with callosal dysgenesis. Pericallosal
lipomas can be curvilinear, generally with a normal or near-normal
corpus callosum, or tubulonodular (mass-like), which are nearly
always accompanied by callosal dysgenesis.
Main differential diagnosis: dermoid, teratoma.
454
FIGURE 12-37. Axial CT shows thin midline lipoma (arrow) of low
density. A calcification is present anteriorly.
455
FIGURE 12-38. Midsagittal T1, in a different patient, demonstrates a
thick pericallosal lipoma.
456
FIGURE 12-39. Midsagittal T1, in a different patient, shows a small
incidentally found lipoma (arrow) in the peritectal region.
457
FIGURE 12-40. Midsagittal T1 shows a suprasellar lipoma in a different
patient.
458
FIGURE 12-41. Midsagittal T1, in a different patient, shows a large
nodular lipoma with dysgenesis of the corpus callosum.
459
FIGURE 12-42. Axial T1, in a different patient, shows a left IAC lipoma
(arrow).
SUGGESTED READING
Jabot G, Stoquart-Elsankari S, Saliou G, Toussaint P, Deramond H, Lehmann P.
Intracranial lipomas: clinical appearances on neuroimaging and clinical
significance. J Neurol 2009;256:851–855.
Meningioma
Key Facts
Most common extra-axial adult tumor and most common primary
intracranial tumor (15% to 20%) in adults. Very rare in children
unless syndromic.
Occurs mainly in middle-aged women (sex hormones) and patients
460
with neurofibromatosis type II (especially multiple meningiomas).
Radiation-induced meningiomas tend to be more aggressive and have
a higher rate of malignant transformation.
Locations: parasagittal/falcine (50%), sphenoid wing (20%, most
common location for “intra-osseous” ones), floor of the anterior
cranial fossa (10%), parasellar region (10%), tentorium, and
cerebellopontine angle region.
Histological types: typical (WHO grade 1, 90% to 95%), atypical
(WHO grade 2, 3% to 5%), and frankly malignant or anaplastic
(WHO grade 3, 1%).
Classic “hyperostosis” of underlying bone is present in only 5% of
cases and is more common in the skull base; typical meningioma may
erode bone.
On MRI, a “dural tail” suggests a mass is extra-axial and is probably
related to reactive changes rather than tumor extension. By definition,
a true dural tail enhances more than the meningioma and shows
smooth tapering peripherally.
Histology cannot be predicted, but bright meningiomas on T2 and
those with restricted diffusion tend to have more atypical microscopic
features. All have very high perfusion (elevated rCBV), and those
with higher vascularity tend to have lower fractional anisotropy on
DTI.
Brain edema is present in 60% of cases.
On MRS, about 10% to 30% have a peak at 1.5 ppm corresponding to
alanine, which inverts below baseline at TE = 144 ms.
Main differential diagnosis: metastasis, sarcoidosis.
461
FIGURE 12-43. Axial postcontrast T1 shows a left frontal meningioma
with typical “dural tails” (arrows).
462
FIGURE 12-44. Axial T2, in a different patient, shows a large left frontal
meningioma with extensive cerebral edema. Note numerous tiny flow
voids due to hypervascularity.
463
FIGURE 12-45. Coronal CT, in a different patient, shows hyperostosis of
the left anterior clinoid process (arrow). The surrounding meningioma is
not seen due to window settings.
464
FIGURE 12-46. Coronal postcontrast MRI, in a different patient, shows a
partially cystic meningioma in the left frontal region (arrow).
465
FIGURE 12-47. Axial postcontrast MRI, in a different patient, shows an
intraventricular meningioma apposed to the choroid plexus in the right
atrium (arrow).
466
FIGURE 12-48. Axial postcontrast MRI, in a different patient, shows an
intraosseous meningioma involving the right frontal bone and subjacent
dura.
SUGGESTED READINGS
Tang Y, Dundamadappa SK, Thangasamy S, et al. Correlation of apparent
diffusion coefficient with Ki-67 proliferation index in grading meningioma.
467
AJR Am J Roentgenol 2014;202:1303–1308.
Toh CH, Wei KC, Chang CN, et al. Assessment of angiographic vascularity of
meningiomas with dynamic susceptibility contrast-enhanced perfusion-
weighted imaging and diffusion tensor imaging. AJNR Am J Neuroradiol
2014;35:263–269.
Pineal Gland Tumors

Key Facts
Represent 1% to 2% of adult intracranial tumors; 3% to 8% of
intracranial tumors in children.
May be classified according to their origin:
Germ cells:
Germinoma: most common (50%), presents mostly in men
(10:1) during the second to third decades of life. Are
slightly hyperdense on noncontrast CT, low intensity on
ADC/T2/FLAIR (due to high nuclei-to-cytoplasm ratio),
enhance deeply after contrast, high perfusion, 50% show
CSF dissemination.
Teratoma: second most common primary tumor of the
pineal gland, present in neonates.
Others: yolk sac tumor, embryonal cell tumor,
choriocarcinoma (all are also more common in very young
patients).
Pineal cells (<15%):
Pineoblastoma: categorized as a primitive neuroectodermal
tumor, most are found in patients <10 years of age.
Pineocytomas: nonspecific imaging features, most found in
the third to sixth decades of life.
Papillary tumor: may be bright on precontrast T1 images.
Pineal parenchymal tumor of intermediate differentiation:
usually larger and more heterogeneous.
Pineal cell tumors are densely cellular and demonstrate
even lower ADC values than germinomas.
Support cells: astrocytomas and meningiomas.
Others: metastases.
Pineal cysts may have a heterogeneous appearance and at times may
be indistinguishable from true neoplasms; most measure between 10
468
and 15 mm, occur in ~5% of the population, and may enhance on
delay MR scans.
By CT, the pineal gland calcifies after 10 years of age. Germinomas
are classically associated with “engulfed” calcifications, while pineal
tumors may show peripherally dispersed “exploding” calcifications.
FIGURE 12-49. Axial noncontrast CT shows a hyperdense pineal

germinoma with a coarse focus of calcification and hydrocephalus.
469
a large enhancing pineoblastoma with hydrocephalus due to aqueduct
obstruction.
470
FIGURE 12-51. Axial ADC map, in the same patient as Figure 12-50,
shows restricted diffusion in the tumor (arrow).
471
Figure 12-52. Axial CT, in a different adult patient, shows peripherally
displaced (“exploded”) calcifications in a pineocytoma (arrows).
472
FIGURE 12-53. Sagittal postcontrast T1, in a different patient, shows a
large and heterogeneous pineal parenchymal tumor of intermediate
differentiation.
473
FIGURE 12-54. Sagittal T1, in a different patient, shows a papillary
pineal tumor with areas of cystic change.
SUGGESTED READINGS
Dumrongpisutikul N, Intrapiromkul J, Yousem DM. Distinguishing between
germinomas and pineal cell tumors on MR imaging. AJNR Am J Neuroradiol
2012;33:550–555.
Komakula S, Warmuth-Metz M, Hildenbrand P, et al. Pineal parenchymal tumor
of intermediate differentiation: imaging spectrum of an unusual tumor in 11
cases. Neuroradiology 2011;53:577–584.
474
Pituitary Adenoma
Key Facts
Most (75%) microadenomas (<10 mm) are endocrinologically active;
therefore, the diagnosis is a clinical one.
Types: prolactin (27%), growth hormone (13%), and corticotropin
(10%); null cell comprise 26%.
Prolactin-producing microadenomas are more common in females;
growth hormone–producing adenomas are more common in males.
Patients with a serum prolactin level above 200 ng/mL usually have
demonstrable tumor by MRI; serum prolactin level above 1,000
ng/mL suggests cavernous sinus invasion.
Most predictive findings of cavernous sinus invasion (on coronal
MRI): ICA encasement ≥ 67% (240 degrees), obliteration of the
carotid sulcus venous compartment, and/or extension beyond the
lateral intercarotid line.
About 80% to 90% of microadenomas enhance much more slowly
than pituitary tissue and are therefore dark on contrast-enhanced
studies. MRI is slightly superior to CT in the detection of
microadenomas; dynamic MRI detects an additional 10% of
adenomas and is particularly helpful in small ones (Cushing
adenomas).
Most macroadenomas (>10 mm) are hormonally inactive and present
with symptoms related to mass effect (mainly on the optic chiasm,
producing bitemporal hemianopsia, cranial neuropathies, and/or
hypothalamic dysfunction).
Pituitary apoplexy is a clinical syndrome (headache, nausea,
vomiting, photophobia, nuchal rigidity, vision deficits, and altered
consciousness), which may be due to hemorrhage into the gland.
Sudden onset of cortisol deficiency can cause life-threatening
hypotension.
Factors predisposing to pituitary hemorrhage are adenoma, radiation,
bromocriptine therapy, pregnancy, trauma, anticoagulation, lumbar
puncture, and angiography. Most common cause of hemorrhage is
idiopathic.
High FLAIR/T2 signal along optic radiations is generally due to
edema and not tumor extension.
Main differential diagnosis: Rathke cleft cyst, craniopharyngioma,
475
aneurysm, pituitary hyperplasia, metastasis, meningioma.
FIGURE 12-55. Coronal early dynamic postcontrast T1 shows a

microadenoma in the right pituitary gland (arrow).
476
FIGURE 12-56. Coronal postcontrast T1, in a different patient, shows a
macroadenoma with 180-degree ICA encasement and obliteration of the
carotid sulcus venous compartment (arrow).
477
large macroadenoma infiltrating the skull base and extending to the
nasopharynx.
478
FIGURE 12-58. Coronal postcontrast T1, in a different patient, shows a
very large macroadenoma completely encasing the ICAs and optic nerves
(arrowheads).
479
FIGURE 12-59. Sagittal T1 in a different patient with pituitary apoplexy
shows a bright macroadenoma with a fluid/blood level.
480
FIGURE 12-60. Axial FLAIR, in a different patient, shows edema in the
optic radiations (arrows) due to compression by a macroadenoma.
SUGGESTED READINGS
Kinoshita M, Tanaka H, Arita H, et al. Pituitary-targeted dynamic contrast-
enhanced multisection CT for detecting MR imaging-occult functional
pituitary microadenoma. AJNR Am J Neuroradiol 2015;36:904–908.
Yamamoto J, Kakeda S, Shimajiri S, et al. Tumor consistency of pituitary
macroadenomas: predictive analysis on the basis of imaging features with
contrast-enhanced 3D FIESTA at 3T. AJNR Am J Neuroradiol
481
2014;35:297–303.
482
CHAPTER 13
Intra-Axial Tumors
Supratentorial Tumors
Anaplastic Astrocytoma
Key Facts
Accounts for 30% of cerebral gliomas (frontal > parietal > temporal >
occipital) in adults (40 to 60 years of age).
Prognosis is poor; median survival rate is 2 years.
They usually progress from a lower-grade tumor and histologically
contain gemistocytes and protoplasmic elements but no necrosis or
endothelial proliferation.
Higher incidence of TP53 mutations than glioblastomas. Other
mutations include PTEN and EGFR; IDH1 mutation and
methylguanine methyltransferase (MGMT) promoter methylation
have been associated with more favorable outcomes.
Dissemination through the white matter tracts (invasion by DTI with
low fractional anisotropy in abnormal tracts), ependyma, and
subarachnoid space is relatively common.
Imaging studies show them as an inhomogeneous mass, surrounded
by edema, and sometimes peripheral and/or central areas of
enhancement. Contrast enhancement must be considered grade 4
tumor until proven otherwise. Calcification and hemorrhage are rare.
> rCBV by MR and CT perfusion studies.
MRS: low myoinositol, high choline, low NAA and lactate
483
(particularly peripherally reflecting zones of ischemia and
angiogenesis), no lipids (abnormal spectra in edema may occur).
Main differential diagnosis: glioblastoma, lower-grade astrocytoma,
cerebritis, atypical infarction, herpes encephalitis.
FIGURE 13-1. Axial FLAIR shows a large infiltrative anaplastic

astrocytoma in the left temporal lobe in a patient with prior history of a
low-grade tumor.
484
FIGURE 13-2. Corresponding MR rCBV map demonstrates increased
perfusion (arrows).
485
FIGURE 13-3. Axial FLAIR, in a different patient, shows an infiltrating
mass centered in the right insula, basal ganglia, and frontal lobe.
486
FIGURE 13-4. Sagittal postcontrast T1, in the same patient, shows foci of
nodular enhancement.
487
FIGURE 13-5. Axial ADC map in a different patient shows restricted
diffusion (arrow) corresponding to an area of enhancement in an
anaplastic astrocytoma.
488
FIGURE 13-6. Choline to creatine MRS map displays in red areas of
highly elevated choline in the central part of the tumor.
SUGGESTED READING
Lasocki A, Tsui A, Tacey MA, Drummond KJ, Field KM, Gaillard F. MRI
grading versus histology: predicting survival of World Health Organization
grade II-IV astrocytomas. AJNR Am J Neuroradiol 2015;36:77–83.
Ependymomas and Subependymomas

489
Key Facts
Ependymomas: Supratentorial ependymomas are generally tumors of
adults. Locations: 30% to 40% of all ependymomas are
supratentorial. Of these, 50% to 80% are parenchymal (arise from
ependymal cell rests) and are located in the frontoparietal regions,
many times adjacent to ventricles. Nonspecific appearance is
indistinguishable from astrocytoma. They commonly calcify, have
mixed cystic/solid components, and can hemorrhage. May also arise
in the lateral or third ventricles. Main differential diagnosis:
oligodendroglioma, astrocytomas, metastases.
Subependymomas: Rare tumors of adults, more common in males.
They generally occur inside the fourth ventricle followed by the
lateral ventricles, generally in the frontal horns adjacent to the septum
pellucidum. While there is generally none to minimal enhancement,
they can show marked enhancement, particularly those in the fourth
ventricle. They have calcifications or present with hemorrhage, and
microcysts are generally bright on FLAIR images. Main differential
diagnosis: choroid plexus tumors, meningioma, astrocytoma,
neurocytoma.
490
FIGURE 13-7. Axial noncontrast CT shows a calcified partially cystic
mass in the right occipital lobe corresponding to a low-grade
ependymoma.
491
FIGURE 13-8. Axial postcontrast T1, in a different patient, shows an
avidly enhancing mass in the left parietal lobe. This was an anaplastic
ependymoma in an adult.
492
FIGURE 13-9. Axial noncontrast CT, in a different patient, shows a large
hyperdense mass in the lateral ventricles with obstructive hydrocephalus.
493
FIGURE 13-10. Corresponding postcontrast T1 shows areas of
heterogeneous enhancement. This was an anaplastic ependymoma in a
child.
494
FIGURE 13-11. Axial FLAIR, in a different patient, shows a bright mass
in the right frontal horn resulting in obstructive hydrocephalus.
495
FIGURE 13-12. Corresponding axial postcontrast T1 shows no tumor
enhancement. This was a subependymoma.
SUGGESTED READINGS
Bi Z, Ren X, Zhang J, Jia W. Clinical, radiological, and pathological features in
43 cases of intracranial subependymoma. J Neurosurg 2015;122:49–60.
Liu Z, Li J, Liu Z, et al. Supratentorial cortical ependymoma: case series and
review of the literature. Neuropathology 2014;34:243–252.
496
Glioblastoma
Key Facts
Accounts for >50% of cerebral gliomas in adults (the most common
brain tumor in adults, males: 3:2). May arise de novo (older patients)
or from degeneration of low-grade astrocytomas (usually younger
patients).
Numerous mutations have been identified, most frequently: PTEN,
TP53, EGFR, and NF-1 genes.
Prognosis is poor; most patients expire 8 to 12 months after diagnosis.
Methylguanine methyltransferase (MGMT) promoter methylation and
isocitrate dehydrogenase mutations (IDH1 and IDH2) have been
associated with improved outcomes.
Necrosis, neovascularity, and cellular pleomorphism are typical
histological features of GB.
Most GBs arise in white matter of cerebral hemispheres (frontal >
temporal > parietal > corpus callosum) in males >50 years of age.
Imaging studies often reveal a typical nodular rim enhancement;
edema is generally present, hemorrhage may occur, calcifications are
rare, and 10% occur in multiple sites.
They have high rCBV and rCBF in MR and CT perfusion studies, as
well as high microvascular permeability values on MR (e.g., Ktrans).
DWI: low ADC.
DTI: invasion with loss of fractional anisotropy in adjacent white
matter.
MRS: very low myoinositol; high choline; very low NAA, high
lipids, and high lactate (abnormal spectra in edema and even normal-
appearing brain).
497
FIGURE 13-13. Axial postcontrast T1 shows an irregularly enhancing
mass centered in the right insula, basal ganglia, and temporal lobe.
498
FIGURE 13-14. Axial T2, in a different patient, demonstrates a
heterogeneous mass with necrotic changes resulting in obliteration of the
left posterior lateral ventricle.
499
FIGURE 13-15. Corresponding MR rCBV map demonstrates increased
perfusion in the mass.
500
FIGURE 13-16. Axial FLAIR, in a different patient, shows an infiltrative
glioblastoma involving the posterior corpus callosum and periventricular
white matter.
501
FIGURE 13-17. Axial postcontrast MRI shows multiple irregularly
enhancing masses in a patient with multifocal glioblastoma.
502
FIGURE 13-18. Corresponding ADC map shows low signal in the solid
enhancing components of the larger mass (arrow).
SUGGESTED READINGS
Çoban G, Mohan S, Kural F, Wang S, O'Rourke DM, Poptani H. Prognostic value
of dynamic susceptibility contrast-enhanced and diffusion-weighted MR
imaging in patients with glioblastomas. AJNR Am J Neuroradiol
2015;36:1247–1252.
Yun TJ, Park CK, Kim TM, et al. Glioblastoma treated with concurrent radiation
therapy and temozolomide chemotherapy: differentiation of true progression
503
from pseudoprogression with quantitative dynamic contrast-enhanced MR
imaging. Radiology 2015;274:830–840.
Gliomatosis Cerebri
Key Facts
Rare disorder found predominantly in older males.
Diffuse neoplastic glial cell infiltration of two or more cerebral lobes,
often bilateral, and sometimes involving the posterior fossa. Most are
of astrocytic origin, rarely oligodendroglial or mixed.
Poor prognosis with 75% mortality by 3 years. There may be
discrepancy between the extent of disease on imaging and clinical
symptoms.
May contain areas of WHO grades 2 and 3, rarely of WHO grade 4.
Begins in white matter with widening of tracts and then extends
diffusely with relative preservation of brain architecture.
Contrast enhancement may be minimal or absent.
MRS shows moderately high myoinositol, high choline (occasionally
choline levels may be normal), low NAA.
MR and CT perfusion shows low rCBV.
Main differential diagnosis: lymphoma, demyelinating disease, other
astrocytoma, encephalitis, vasculitis, PML.
504
FIGURE 13-19. Axial postcontrast T1 shows low intensity in right
temporo-occipital regions and in splenium which is also thick. Note absent
enhancement.
505
FIGURE 13-20. Corresponding FLAIR image shows extension of bright
tumor to a better degree.
506
FIGURE 13-21. Axial T2, in a different patient, shows high signal in left
temporo-occipital region with infiltration of basal ganglia. Long TE MRS
(inset) shows high choline (arrow) and low NAA.
507
FIGURE 13-22. Axial FLAIR, in a different patient, shows tumor in left
frontal and temporal regions with diffuse infiltration of gray matter
structures.
508
FIGURE 13-23. Axial T2, in a different patient, shows infiltrative tumor
involving the corpus callosum and the left parietal, occipital, and temporal
lobes.
509
FIGURE 13-24. Postcontrast T1, in the same patient, shows minimal
patchy enhancement in the corpus callosum and left parietal lobe.
SUGGESTED READING
Rudà R, Bertero L, Sanson M. Gliomatosis cerebri: a review. Curr Treat Options
Neurol 2014;16:273.
Astrocytoma, Low Grade
510
Key Facts
Accounts for 10% to 30% of cerebral gliomas in adults.
Histologically, most low-grade astrocytomas are fibrillary (diffuse),
and over 80% eventually degenerate to anaplastic astrocytoma and
GB (particularly those with TP53 mutation and the gemistocytic
type).
Usually occurs in the cerebral hemispheres at age 20 to 45 years.
Median survival rate varies between 4 and 10 years.
10% to 20% of low-grade astrocytomas show calcification by CT; all
are hyperintense on MR T2/FLAIR; contrast enhancement may be
absent or mild; edema and hemorrhage are rare.
They show no increased rCBV on MR and/or CT perfusion studies.
MRS: high myoinositol, moderately elevated choline, low NAA, no
lipids/lactate (spectra are normal in edema).
DTI: no invasion of neighboring white matter tracts.
Main differential diagnosis: other astrocytoma, cerebritis, vasculitis,
sequelae of seizures, and ischemia.
511
FIGURE 13-25. Axial FLAIR shows an expansile diffuse astrocytoma
centered in the left frontoparietal region and insula.
512
FIGURE 13-26. Corresponding postcontrast T1 demonstrates that the
lesion does not enhance.
513
FIGURE 13-27. MRI rCVB map in the same patient shows low perfusion
in the tumor.
514
FIGURE 13-28. ADC map, in a different patient, shows no restricted
diffusion in a low-grade astrocytoma (arrow).
515
FIGURE 13-29. Short TE MRS shows high myoinositol (Myo) in a
different patient with low-grade astrocytoma.
516
FIGURE 13-30. Long TE MRS shows high choline (arrow) and low NAA
(at 2.0 ppm).
SUGGESTED READING
Roy B, Gupta RK, Maudsley AA, et al. Utility of multiparametric 3-T MRI for
glioma characterization. Neuroradiology 2013;55:603–613.
517
Lymphoma
Key Facts
Of primary brain lymphomas: >90% are non-Hodgkin and B-cell
type.
Accounts for 1% to 5% of primary brain tumors in adults (>60 years
of age) but occur in 6% of AIDS patients (younger).
8% to 44% of the lesions are multiple, most commonly mass-like.
In immunocompetent patients, primary lymphoma presents as deep
gray and/or white matter lesions (most common) that are slightly
hyperdense on noncontrast CT, have little mass effect or edema, and
show prominent homogeneous enhancement. Enhancement along
perivascular spaces is suggestive of primary lymphoma.
Lymphoma associated with Epstein-Barr virus may present with
atypical features such as necrosis, even in non-AIDS patients.
Primary brain lymphomas may be slightly bright on precontrast T1,
dark on T2/FLAIR, and show restricted diffusion, contrast
enhancement, high choline and lipids/lactate on MRS, and lower
rCBV than other tumors such as metastases and glioblastomas.
Lymphoma may cross corpus callosum.
In AIDS patients, primary lymphoma presents as single or multiple
ring-enhancing lesions with necrosis and edema (often
indistinguishable from toxoplasmosis, although lymphoma tends to
abut ependymal surfaces). Hemorrhage is very rare but may occur
and seems to be more common in AIDS patients.
Leptomeningeal or dural involvement is more common in secondary
lymphoma.
Main differential diagnosis: toxoplasmosis, astrocytomas, metastases,
sarcoidosis.
518
FIGURE 13-31. Axial noncontrast CT shows a hyperdense large B-cell
lymphoma (arrow) in the splenium of the corpus callosum with
surrounding edema.
519
FIGURE 13-32. Axial postcontrast T1 shows nodular periventricular
masses in a different patient with large B-cell lymphoma.
520
FIGURE 13-33. Axial DWI, in the same patient, shows increased signal
within the mass related to restricted diffusion.
521
leptomeningeal lymphoma on cerebellar surface and in the skull.
522
FIGURE 13-35. Sagittal postcontrast T1, in a different patient, shows
linear perivascular enhancement around the corpus callosum (arrows).
523
FIGURE 13-36. Axial postcontrast T1 shows a necrotic lymphoma in a
non-HIV patient who tested positive for Epstein-Barr virus.
SUGGESTED READINGS
Haldorsen IS, Espeland A, Larsson EM. Central nervous system lymphoma:
characteristic findings on traditional and advanced imaging. AJNR Am J
Neuroradiol 2011;32:984–992.
Toh CH, Wei KC, Chang CN, Ng SH, Wong HF. Differentiation of primary
central nervous system lymphomas and glioblastomas: comparisons of
diagnostic performance of dynamic susceptibility contrast-enhanced perfusion
MR imaging without and with contrast-leakage correction. AJNR Am J
Neuroradiol 2013;34:1145–1149.
524
Metastases
Key Facts
25% of cancer patients develop brain metastases, 10% to 35% of
brain tumors are metastases. Most intracranial metastases are
parenchymal.
Most common primary tumors in adults to cause bone or epidural
metastases: breast, prostate, lung, kidney, and multiple myeloma; in
children: neuroblastoma and Langerhans cell histiocytosis.
Most common primary tumors in adults to cause dural or
leptomeningeal metastases: breast, small cell carcinoma, melanoma,
lymphoma, and leukemia; in children consider PNETs.
Most common tumor in adults to cause parenchymal metastases: lung,
breast, melanoma, kidney, and GI tract; unknown primaries account
for 10% to 15% of brain metastases.
Most parenchymal metastases occur at gray–white junctions and
terminal “watershed” areas; 80% are supratentorial, and 60% to 80%
are multiple.
Average survival for patients with brain metastases is 3 to 12 months.
Brain metastases with increased T1 signal intensity before contrast
administration: melanoma (due to melanin and/or hemorrhage),
kidney, lung, choriocarcinoma, and bowel (due to mucin).
MRS: low myoinositol; high choline; low to absent NAA, lipids, and
lactate (surrounding tissues have normal spectra).
High rCBV and rCBF in MR and CT perfusion.
Densely cellular metastases show restricted diffusion (only in solid,
nonnecrotic components).
DTI: displace but do not invade neighboring white matter tracts.
Main differential diagnosis for intra-axial lesions: abscess, multifocal
primary tumor, tuberculomas, cysticercosis, tumefactive
demyelination.
525
FIGURE 13-37. Axial postcontrast T1 shows numerous enhancing
metastases, the largest in the left occipital lobe with surrounding edema.
526
FIGURE 13-38. Axial noncontrast T1 in a melanoma patient shows bright
metastases (arrows).
527
FIGURE 13-39. Axial postcontrast T1, in a different patient, shows
diffuse leptomeningeal metastases coating the supratentorial brain,
brainstem, and cerebellum.
528
FIGURE 13-40. Axial postcontrast T1 shows bilateral metastases from
medulloblastoma. Note cystic changes and a tiny amount of layering blood
on the right (arrow).
529
FIGURE 13-41. Axial postcontrast T1, in a different patient with prostate
cancer, shows a large dural-based metastasis in the left frontal region. Note
left frontal lobe edema.
530
FIGURE 13-42. Axial noncontrast T1 in a different patient with mucinous
breast carcinoma shows a left calvarial metastasis with locules of layering
blood.
SUGGESTED READING
Wang S, Kim S, Chawla S, et al. Differentiation between glioblastomas, solitary
brain metastases, and primary cerebral lymphomas using diffusion tensor and
dynamic susceptibility contrast-enhanced MR imaging. AJNR Am J
Neuroradiol 2011;32:507–514.
531
Neuronal Tumors
Key Facts
Gangliogliomas (mostly WHO grades 1 and 2 but variable):
<1% of intracranial tumors in adults and 4% of all intracranial
tumors in children.
80% occur in patients <30 years of age, slight male
predominance.
Locations: temporal and parietal lobes and cerebellum.
Calcifications in 30%; cysts in 30% to 50%; surrounding edema
is uncommon.
Variable enhancement; often present as a cyst with an enhancing
nodule.
May cause scalloping of inner table of the skull.
Chronic seizures are most common symptom.
Main differential diagnosis: astrocytoma, DNET,
oligodendroglioma. Desmoplastic infantile gangliogliomas look
similar but tend to be larger and present in patients <2 years old.
Dysembryoplastic neuroepithelial tumor (DNET, WHO grade 1):
Rare, occur mostly in children and young adults, result in
seizures, affect cortex, scallop inner skull table, are accompanied
by cortical dysplasia, may have a microcystic appearance.
Typically do not enhance, but 20% may show faint focal or ring
enhancement.
Main differential diagnosis: low-grade astrocytoma or
oligodendroglioma, ganglioglioma, cortical dysplasia.
Central neurocytoma (WHO grade 2):
Rare, “bubbly” mass attached to septum pellucidum in one
frontal horn of lateral ventricle, calcification in >50%, moderate-
to-marked enhancement. Can rarely be extraventricular.
Main differential diagnosis: subependymoma, choroid plexus
tumor, metastasis, astrocytoma, meningioma.
532
FIGURE 13-43. Coronal T2 shows ganglioglioma involving the white
matter and cortex in left temporal lobe and resulting in scalloping (arrows)
of the inner skull table.
533
FIGURE 13-44. Axial postcontrast T1 in a child shows large enhancing
mass (ganglioglioma) surrounded by cysts with little mass effect or edema.
The differential diagnosis included a desmoplastic infantile ganglioglioma.
534
FIGURE 13-45. Axial FLAIR, in a different patient, shows a DNET in the
right frontal lobe (arrow).
535
FIGURE 13-46. Coronal T2, in a different patient, shows “cystic” DNET
in right amygdala region.
536
FIGURE 13-47. Axial postcontrast T1, in a different patient, demonstrates
a central neurocytoma along the septum pellucidum. Note “bubbly”
appearance and heterogeneous enhancement.
537
FIGURE 13-48. Coronal postcontrast T1, in a different patient,
demonstrates a very large cystic mass with an enhancing solid component.
This was a desmoplastic infantile ganglioglioma.
SUGGESTED READINGS
Shin JH, Lee HK, Khang SK, et al. Neuronal tumors of the central nervous
system: radiologic findings and pathologic correlation. Radiographics
2002;22:1177–1189.
Zhang JG, Hu WZ, Zhao RJ, Kong LF. Dysembryoplastic neuroepithelial tumor:
a clinical, neuroradiological, and pathological study of 15 cases. J Child
Neurol 2014;29:1441–1447.
538
Oligodendroglioma
Key Facts
Accounts for 5% to 20% of cerebral gliomas in adults, generally
found during the fourth to sixth decades of life, more commonly in
men.
Five-year survival is 75% for pure oligodendrogliomas.
50% are histologically “mixed” and contain neoplastic astrocytes
(called an “oligoastrocytoma,” which is identical to
oligodendroglioma on imaging studies); growth is very slow.
>85% are supratentorial.
They involve the cortex and subcortical white matter in the
frontotemporal regions (also in temporal and occipital lobes and
corpus callosum)
Most common symptom: seizures (due to early cortical involvement).
Most common intracranial tumor to calcify (70%); may cause
scalloping of inner table of the skull (17%); cysts are present in 20%;
hemorrhage occurs in 20%, and contrast enhancement is seen in 50%.
They have high (paradoxical) rCBV due to a dense capillary network;
thus, it does not reflect higher histological grade but correlates with
deletions in chromosomes 1p and 19q, which portray a better
prognosis. Calcifications also appear to be more common in tumors
with 1p/19q codeletion (regarded as the “genetic signature” of
oligodendrogliomas).
MRS in low-grade oligodendroglioma: moderate-to-high myoinositol,
moderate-to-high choline, low NAA, no lipids/lactate. When high
grade, there are elevated choline and lactate/lipids. DWI: no
restriction of diffusion when low grade; high-grade tumors have low
ADC values.
Main differential diagnosis: ganglioglioma, astrocytoma, DNET,
PXA, AVM.
539
FIGURE 13-49. Axial FLAIR shows an infiltrative mass in the right
frontal lobe. Note the prominent gyral expansion.
540
infiltrative mass with patchy enhancement in the left frontotemporal region
and basal ganglia.
541
FIGURE 13-51. Axial noncontrast CT, in a different patient, demonstrates
the gyriform calcifications sometimes described in oligodendrogliomas.
542
FIGURE 13-52. Axial MR perfusion rCBV map, in the same patient,
shows the mass to be hyperperfused.
543
FIGURE 13-53. Corresponding axial postcontrast T1 shows no contrast
enhancement, despite the presence of increased perfusion.
544
FIGURE 13-54. Axial FLAIR, in a different patient, shows a large
anaplastic oligodendroglioma in the left frontal lobe with surrounding
edema.
SUGGESTED READINGS
Chawla S, Krejza J, Vossough A, et al. Differentiation between
oligodendroglioma genotypes using dynamic susceptibility contrast perfusion-
weighted imaging and proton MR spectroscopy. AJNR Am J Neuroradiol
2013;34:1542–1549.
Khalid L, Carone M, Dumrongpisutikul N, et al. Imaging characteristics of
545
oligodendrogliomas that predict grade. AJNR Am J Neuroradiol
2012;33:852–857.
Infratentorial Tumors
Brainstem Astrocytoma
Key Facts
Represents 25% of posterior fossa tumors.
Pilocytic tumors (55%) are WHO grade 1; diffuse tumors (45%) are
WHO grades 2 to 4. Most tumors in the pons are high-grade
(anaplastic astrocytomas or glioblastomas) and those in the tectum are
almost always low grade.
Occurs mainly in children (M > F); most are diagnosed in the first
decade of life and over 75% during the first two decades of life.
Locations: pons (most common), midbrain (with or without thalamic
extension), or medulla; 50% involve both the pons and medulla at
time of diagnosis, and 60% of all brainstem astrocytomas have
exophytic components.
Dorsal exophytic astrocytomas protrude into the fourth ventricle, are
of lower histological grades, and have better prognosis.
Hydrocephalus is a late complication, except in tectal tumors. May be
seen in NF-1 patients, particularly in tectum (these tend to have a
better prognosis along with pilocytic tumors).
Enhancement is variable and may be diffuse, nodular, or ring-like;
calcifications and hemorrhage are rare.
MRS: high myoinositol (on short TE studies), moderate-to-high
choline (depends on grade), low NAA, no lipids/lactate in lower
grades but present in higher grades.
No increased perfusion or restricted diffusion in lower grades.
Main differential diagnosis: viral pontomesencephalitis.
546
FIGURE 13-55. Axial FLAIR shows a bright infiltrative and expansile
mass in the pons.
547
FIGURE 13-56. Corresponding MR rCBV map shows no increased
perfusion in this low-grade tumor.
548
FIGURE 13-57. Axial T2, in a different patient, shows a large infiltrative
tumor in the pons effacing the fourth ventricle.
549
FIGURE 13-58. Coronal postcontrast T1, in the same patient, shows areas
of nodular enhancement. This was a glioblastoma.
550
FIGURE 13-59. Midsagittal noncontrast T1, in a different patient, shows
typical appearance of dorsal exophytic brainstem glioma with mass
protruding into the fourth ventricle.
551
FIGURE 13-60. MRS, long TE, shows high choline, low NAA, and
presence of lactate in the same patient as Figure 13-59.
SUGGESTED READINGS
Lober RM, Cho YJ, Tang Y, et al. Diffusion-weighted MRI derived apparent
diffusion coefficient identifies prognostically distinct subgroups of pediatric
diffuse intrinsic pontine glioma. J Neurooncol 2014;117:175–182.
Sedlacik J, Winchell A, Kocak M, Loeffler RB, Broniscer A, Hillenbrand CM.
MR imaging assessment of tumor perfusion and 3D segmented volume at
baseline, during treatment, and at tumor progression in children with newly
diagnosed diffuse intrinsic pontine glioma. AJNR Am J Neuroradiol
2013;34:1450–1455.
552
Ependymoma and Subependymoma
Key Facts
Ependymomas account for 2% to 6% of intracranial gliomas, most
are found in first (peak: 5 years) and second decades of life but also
occur in adults (peak: 40 years).
Overall 5-year survival rate: 50%.
Common locations:
Children: 60% in the fourth ventricle (third most common
pediatric brain tumor).
Approximately one third are supratentorial; about half of which
are extraventricular.
Although benign, nearly always fatal if they recur.
CT shows calcification in 50%, cysts/edema in 50%, hemorrhage not
uncommon. Nearly all fourth ventricular ependymomas enhance.
They tend to extrude through the fourth ventricular outflow foramina
(“toothpaste” or “plastic” appearance). Spinal seeding occurs in 10%
of cases.
Subependymomas are variants of ependymoma and are more
common in adults, most are subclinical, and occur in the fourth
ventricle followed by the lateral ventricles.
MRS: nonspecific, variable levels of myoinositol, high choline, low
NAA, lactate may present.
Malignant and anaplastic ependymomas may show high perfusion.
ADC values of ependymomas are nonspecific but usually higher than
medulloblastomas and lower than juvenile pilocytic astrocytomas.
Main differential diagnosis: astrocytoma, medulloblastoma,
ganglioglioma.
553
FIGURE 13-61. Axial noncontrast CT shows an iso- to hyperdense mass
with cystic change in the fourth ventricle.
554
FIGURE 13-62. Corresponding noncontrast sagittal T1 shows a large
ventricular ependymoma in this pediatric patient.
555
FIGURE 13-63. Sagittal T2, in a different patient, demonstrates a
heterogeneous mass with cystic changes in the fourth ventricle.
556
FIGURE 13-64. Corresponding axial postcontrast T2 shows avid
enhancement of the mass and extension through the fourth ventricular
outflow foramina.
557
FIGURE 13-65. ADC map, in a different patient, shows intermediate to
bright signal in a fourth ventricular ependymoma.
558
FIGURE 13-66. Axial FLAIR image, in a different patient, shows a bright
fourth ventricular subependymoma.
SUGGESTED READING
Poretti A, Meoded A, Huisman TA. Neuroimaging of pediatric posterior fossa
tumors including review of the literature. J Magn Reson Imaging
2012;35:32–47.
Hemangioblastoma
559
Key Facts
Accounts for <2% of all intracranial tumors.
Locations: cerebellar hemispheres (80%), vermis, medulla, and spinal
cord (10% to 15%). Supratentorial tumors are rare.
Most common primary tumor of cerebellum in patients aged 30 to 80
years. Most found during the third to fourth decades of life.
10% to 20% of patients have von Hippel-Lindau disease.
Sporadic hemangioblastomas are usually solitary. Multiple tumors are
present in 5% to 20% of patients and are more frequently seen in von
Hippel-Lindau disease, where they are generally diagnosed earlier.
About 60% of hemangioblastomas are cystic masses with peripheral
enhancing nodule and 40% consist of a densely enhancing solid
tumor. They are only symptomatic when cystic. Erythropoietin
abnormalities are very rare and mostly seen with very large ones.
Enhancing tumor nodule nearly always abuts pia or ependyma.
Solid hemangioblastomas are more commonly found in patients with
von Hippel-Lindau syndrome.
In the absence of von Hippel-Lindau syndrome, prognosis is good
after total resection.
Clues on MRI that may help in the diagnosis of hemangioblastoma:
flow voids in nodule, nodule always abuts pia or ependyma, very high
perfusion in nodule. The cyst wall usually does not enhance.
Main differential diagnosis: metastasis, ganglioglioma, atypical
meningioma, astrocytoma.
560
FIGURE 13-67. Axial postcontrast T1 shows enhancing tumor nodule
with an associated cyst.
561
FIGURE 13-68. Coronal postcontrast T1, in the same patient, shows the
full extent of the cyst and the pial-based tumor nodule.
562
FIGURE 13-69. Axial T2, in a different patient, shows cystic tumor with
laterally located nodule that is isointense to the brain.
563
FIGURE 13-70. Sagittal postcontast T1, in a different patient, shows a
solid, avidly enhancing mass in the fourth ventricle.
564
FIGURE 13-71. Corresponding MR rCBV map demonstrates very high
perfusion in the tumor (arrow).
565
FIGURE 13-72. Lateral view DSA, in the same patient, shows this lesion
to be highly vascular.
SUGGESTED READING
Bamps S, Calenbergh FV, Vleeschouwer SD, et al. What the neurosurgeon should
know about hemangioblastoma, both sporadic and in Von Hippel-Lindau
disease: a literature review. Surg Neurol Int 2013;4:145.
Dysplastic Cerebellar Gangliocytoma

(Lhermitte-Duclos Disease)
Key Facts
566
Rare lesion, combination of hamartoma and neoplasia (WHO grade
1).
Nearly always in association with Cowden syndrome and loss of
PTEN gene function. No malignant transformation has been reported;
however, there is an increased risk for malignancy, particularly
breast, thyroid, and endometrial cancer.
Other associations: megalencephaly, polydactyly, skull
malformations, and macroglossia.
Commonly found in the fourth decade of life.
CT and MRI: “striated” or “corduroy” pattern is classic, mass in one
cerebellar hemisphere with mass effect, may grow very slowly,
contrast enhancement is very rare, minimally restricted diffusion may
be seen, may have high perfusion, SWI may show large veins in
tumor.
MRS: high myoinositol, low choline, normal creatinine, low NAA, no
lipids/lactate.
Main differential diagnosis: none, appearance is typical.
May present with symptoms of elevated intracranial pressure.
Treatment consists of ventricular decompression and surgical
resection.
FIGURE 13-73. Axial postcontrast T1 shows nonenhancing mass with the
567
typical “corduroy” striated pattern of Lhermitte-Duclos disease.
FIGURE 13-74. Axial ADC map, in a different patient, shows minimally

restricted diffusion in a right cerebellar tumor.
568
nonenhancing posterior fossa mass with compression of the brainstem and
aqueduct.
569
FIGURE 13-76. Corresponding axial T2 demonstrates a “gyriform” or
“tigroid” pattern with widened cerebellar folia.
570
FIGURE 13-77. Axial T2, in a different patient, shows a mass in the right
cerebellum with a striated pattern.
571
FIGURE 13-78. Superimposed MR rCBV map demonstrates
hyperperfusion of the mass.
SUGGESTED READING
Klisch J, Juengling F, Spreer J, et al. Lhermitte-Duclos disease: assessment with
MR imaging, positron emission tomography, single-photon emission CT, and
MR spectroscopy. AJNR Am J Neuroradiol 2001;22:824–830.
Medulloblastoma
Key Facts
572
Accounts for 30% to 40% of posterior fossa tumors and 10% to 20%
of brain tumors in children (most common malignant pediatric brain
tumor).
Five-year survival rate is 50% with combination chemo/radiation
therapy.
Genetically, heterogeneous with four distinct molecular subgroups:
sonic hedgehog (SHH), wingless (WNT), group 3, and group 4. Best
and worst outcomes with WNT and group 3, respectively, regardless
of histology.
Most (>50%) are found in <5 years of life; a second peak occurs in
third decade of life and accounts for <30% of all medulloblastomas.
Most common locations in children: cerebellar vermis (75%); in
adults: cerebellar hemispheres (lateral desmoplastic
medulloblastoma). May also occur in cerebellar
peduncle/cerebellopontine angle region.
CT: frequently midline cerebellar tumor, hyperdense before contrast,
usually no calcium, homogeneous enhancement, hydrocephalus;
occasional tumor cysts, calcifications, hemorrhage, and/or absence of
enhancement.
MRI: tumor is isointense on T1, dark on T2, restricted ADC, contrast
enhancement (90%).
MRS: high choline; low-to-absent NAA, lipids, and lactate;
occasional taurine peak.
About 50% develop diffuse subarachnoid metastases; therefore, MRI
of the spine with contrast is recommended in all patients (remember
to do it before surgery as some postoperative changes, especially
irritation from blood, may enhance and be indistinguishable from
metastases).
Main differential diagnosis: atypical teratoid–rhabdoid tumor (similar
location but more common in patients <3 years of age), astrocytoma,
ependymoma.
573
FIGURE 13-79. Axial noncontrast CT shows a hyperdense midline
cerebellar mass (arrow).
574
FIGURE 13-80. Corresponding axial T2 demonstrates a mass that is
predominantly isointense to gray matter. Note cystic changes and a trace
amount of layering blood.
575
FIGURE 13-81. Corresponding postcontrast T1 shows enhancement of
the solid component.
576
FIGURE 13-82. ADC map in a different patient with a large nodular
tumor shows low signal related to restricted diffusion (arrows). This was a
desmoplastic variant.
577
FIGURE 13-83. Axial T2, in a different adult patient, shows laterally
located mass in the left cerebellum.
578
FIGURE 13-84. MRS, long TE, shows very high choline when compared
to creatinine, low NAA, and an inverted lactate peak.
SUGGESTED READINGS
Perreault S, Ramaswamy V, Achrol AS, et al. MRI surrogates for molecular
subgroups of medulloblastoma. AJNR Am J Neuroradiol 2014;35:1263–1269.
Yeom KW, Mobley BC, Lober RM, Andre JB, Partap S, Vogel H, et al.
Distinctive MRI features of pediatric medulloblastoma subtypes. AJR Am J
Roentgenol 2013;200:895–903.
579
Pilocytic Astrocytoma
Key Facts
Most common cerebellar tumor in children (5 to 15 years of age) after
medulloblastoma.
Accounts for 5% to 10% of all gliomas and for 30% of all pediatric
brain tumors.
BRAF-KIAA fusion gene mutation predicts better clinical outcomes
and is present in the vast majority of sporadic pilocytic astrocytomas.
Overall excellent prognosis with 95% survival at 10 years.
Most arise in vermis or hemispheres, third ventricular region, or optic
chiasm (especially in neurofibromatosis type 1 [NF-1] patients, seen
in 15% of NF-1 patients); they are rare in the cerebral hemispheres
(supratentorial tumors are more common in older children and
adults).
Appear as a well-demarcated cyst (50% to 80%) with mural nodule(s)
that enhance; absence of edema is characteristic, calcification is
present in 10%; occasionally, they are a solid enhancing tumor, and
they can rarely demonstrate an infiltrative pattern.
MRS: high myoinositol, high choline, low NAA, high lactate (reflects
glycolytic tumor nature). ADC maps show no restricted diffusion, and
perfusion studies generally show low perfusion (occasionally it may
be elevated).
580
FIGURE 13-85. Axial postcontrast T1 shows a cystic mass in the vermis
with a large enhancing mural nodule (arrow).
581
FIGURE 13-86. Corresponding ADC map shows no restricted diffusion
(arrow).
582
tumor in which the cyst wall enhances.
583
FIGURE 13-88. Axial T2, in a different patient, demonstrates a
predominantly solid cerebellar tumor displacing the brainstem and effacing
the fourth ventricle.
584
FIGURE 13-89. Axial MR perfusion rCBV map, in a different patient,
shows no perfusion in midline tumor.
585
FIGURE 13-90. Axial MR perfusion rCBV map, in a different patient,
shows high perfusion in midline tumor nodule (arrow).
SUGGESTED READINGS
de Fatima Vasco Aragao M, Law M, Batista de Almeida D, Fatterpekar G, et al.
Comparison of perfusion, diffusion, and MR spectroscopy between low-grade
enhancing pilocytic astrocytomas and high-grade astrocytomas. AJNR Am J
Neuroradiol 2014;35:1495–1502.
Rumboldt Z, Camacho DL, Lake D, Welsh CT, Castillo M. Apparent diffusion
coefficients for differentiation of cerebellar tumors in children. AJNR Am J
Neuroradiol 2006;27:1362–1369.
586
CHAPTER 14
Infections and
Inflammations
Acute Disseminated Encephalomyelitis

(ADEM)
Key Facts
Immune-mediated response occurring 1 to 3 weeks after nonspecific
viral or bacterial illness (75% of cases; may be subclinical) or less
commonly vaccination; it is a diagnosis of exclusion; occurs mostly
in children (5 to 10 years of age) but may affect any age group. May
represent allergic reaction to autoantigens due to shared antigenic
determinants with an infectious agent.
Typically monophasic, but some patients may be at increased risk of
recurrence (attacks separated by 3 months) or development of
multiple sclerosis.
Symptoms: seizures, headache, fever, encephalomyelopathy, optic
neuritis, cranial neuropathy, and cerebellar (e.g., ataxia) and
brainstem (e.g., lower cranial nerve palsies) symptoms.
Lesions in ADEM are characterized by perivenous inflammation and
demyelination.
MRI: bilateral, asymmetric, and well-defined hyperintensities in
white matter with no mass effect and enhancement in 25% of
patients; involvement of the centrum semiovale is typical.
Deep gray matter involvement (particularly in the thalami) may be
587
present in up to 60% of patients and may help to distinguish ADEM
from the initial presentation of multiple sclerosis (gray matter
involvement is less common in multiple sclerosis).
Acute variant with blood is termed “acute hemorrhagic
encephalomyelitis” and is rare.
Prognosis is usually good, although 10% to 20% of the cases may be
fatal or result in permanent neurological sequelae.
Main differential diagnosis: viral encephalitis, PRES, demyelinating
disease, vasculitis.
FIGURE 14-1. Axial T2 shows bright signal in the pons and adjacent
middle cerebellar peduncles.
588
FIGURE 14-2. Sagittal T2 in a young child (different patient)
demonstrates an expansile lesion involving a long segment of the cervical
spinal cord.
589
FIGURE 14-3. Axial FLAIR, in a different patient, shows numerous
lesions in the subcortical white matter and centrum semiovale.
590
FIGURE 14-4. Axial FLAIR, in the same patient, shows additional
lesions in the temporal, parietal, and occipital lobes.
591
FIGURE 14-5. Axial FLAIR in a different patient shows extensive,
bilateral, and asymmetric lesions involving the deep gray matter,
subcortical white matter, corpus callosum, and insula.
592
FIGURE 14-6. Axial postcontrast T1 in a different patient demonstrates
numerous enhancing lesions consistent with active demyelination, some
with an incomplete ring.
SUGGESTED READING
Bester M, Petracca M, Inglese M. Neuroimaging of multiple sclerosis, acute
disseminated encephalomyelitis, and other demyelinating diseases. Semin
Roentgenol 2014;49:76–85.
593
Cerebral Pyogenic Abscess
Key Facts
Uncommon, generally seen in males between ages 10 and 30 years
particularly those with AIDS (however, 25% occur in children <15
years old).
Mortality is 20% despite antibiotics. 90% of all cerebral abscesses are
bacterial in nature.
Sources: sinusitis, otitis media, mastoiditis, meningitis (particularly in
children), penetrating head injury, and hematogenous spread from
remote source (more commonly multiple, occasionally seen in
patients with cyanotic cardiac disease and pulmonary AVMs).
Locations: temporal, frontal, and parietal lobes; 85% are
supratentorial.
Early cerebritis occurs during the initial 5 days; late cerebritis (with
central necrosis) occurs from 4 to 11 days; early capsule formation
(incomplete abscess) occurs from 10 to 18 days; mature abscess is
seen from days 14 to 19, and rim enhancement (in intact abscesses)
may persist for up to 8 months.
MRI features include a smooth capsule of low T2/FLAIR and slightly
bright precontrast T1 signal intensity, greater thickness of the side of
the capsule neighboring gray matter, and surrounding vasogenic
edema.
Abscesses show a “dual rim” sign with two concentric rims on SWI
(not present in glioblastomas): a hypointense outer capsule (probably
due to free radicals) and a hyperintense inner rim (relative to the core,
possibly due to granulation tissue).
DWI: restricted diffusion in cavity is typical of bacterial abscesses but
may be less prominent in those due to TB, toxoplasmosis, or fungi.
DWI may be useful in posttherapy follow-up of abscesses; persistent
high DWI may indicate treatment failure.
Perfusion studies show low blood flow with the capsule usually
having lower rCBV relative to white matter (as opposed to
glioblastomas and metastases).
MRS shows low choline, creatine, and NAA; high lipids/lactate; high
amino acids (succinate, acetate, alanine, glycine).
Main differential diagnosis: primary and secondary tumors.
594
FIGURE 14-7. Axial T2 shows a left temporal abscess with a thick
hypointense capsule.
595
FIGURE 14-8. Corresponding DWI shows very bright signal in the pus-
filled cavity.
596
FIGURE 14-9. Coronal postcontrast T1, in the same patient, shows an
enhancing capsule that is thinnest on the ventricular side.
597
FIGURE 14-10. Corresponding MR rCBV map shows low perfusion in
the lesion.
598
FIGURE 14-11. Axial SWI, in a different patient, shows the “dual rim”
sign with concentric hypo- and hyperintense rims surrounding the core.
599
FIGURE 14-12. Axial ADC map, in a different patient, shows left
temporal abscess to have very restricted diffusion centrally and
surrounding edema. (Case courtesy of D. Camacho, Milwaukee,
Wisconsin.) MRS show lipids/lactate and succinate/acetate.
SUGGESTED READINGS
Cartes-Zumelzu FW, Stavrou I, Castillo M, Eisenhuber E, Knosp E, Thurnher
MM. Diffusion-weighted imaging in the assessment of brain abscesses
therapy. AJNR Am J Neuroradiol 2004;25:1310–1317.
Pal D, Bhattacharyya A, Husain M, Prasad KN, Pandey CM, Gupta RK. In vivo
600
proton MR spectroscopy evaluation of pyogenic brain abscesses: a report of
194 cases. AJNR Am J Neuroradiol 2010;31:360–366.
Creutzfeldt-Jakob Disease (CJD)

Key Facts
Rapidly progressive spongiform encephalopathy caused by prions (a
form of transmissible, infectious protein); CJD constitutes 90% of all
sporadic prion diseases.
Typical clinical findings: rapid progression of dementia, ataxia,
myoclonus, and mutism; EEG typically shows periodic high-voltage
sharp waves.
Affects older patients (median age 65 years), although the new
variant form (vCJD) usually affects much younger individuals.
MRI shows high signal in the cortex (particularly parietal, temporal,
and occipital regions) and basal ganglia (better seen on DWI and
FLAIR) without enhancement. Bilateral symmetrical involvement of
posterior and dorsomedial thalami (pulvinar and “hockey stick” signs,
respectively) is typical of vCJD but can also be seen in the sporadic
form (particularly the MV2 molecular subtype). The cerebellum may
be also involved, and there may be white matter changes detected on
DTI (decreased fractional anisotropy). Affected areas are
hypometabolic on FDG–PET. CT usually shows only atrophy.
DWI is most sensitive for early diagnosis and may show changes
before characteristic EEG and clinical findings.
Main differential diagnosis: anoxia, hepatic encephalopathy,
hypoglycemia, mitochondrial diseases.
601
FIGURE 14-13. Axial FLAIR shows bilateral symmetrical high signal in
the basal ganglia and thalami.
602
FIGURE 14-14. Corresponding DWI shows high signal in the same
regions.
603
FIGURE 14-15. Axial DWI, in a different patient, shows extensive areas
of high signal in the frontal and parietal cortices bilaterally.
604
FIGURE 14-16. Axial DWI in a different patient shows high signal in the
corpus striatum bilaterally and right hemispheric cortex.
605
FIGURE 14-17. Axial DWI, in a different patient, shows increased signal
in the basal ganglia and cerebral cortex, left worse than right.
606
FIGURE 14-18. FDG–PET in the same patient demonstrates
corresponding dark areas of hypometabolism.
SUGGESTED READINGS
Letourneau-Guillon L, Wada R, Kucharczyk W. Imaging of prion diseases. J
607
Magn Reson Imaging 2012;35:998–1012.
Zerr I, Kallenberg K, Summers DM, et al. Updated clinical diagnostic criteria for
sporadic Creutzfeldt-Jakob disease. Brain 2009;132:2659–2668.
Cryptococcus
Key Facts
Most common fungal infection of the CNS in general population, and
the third most common CNS infection in AIDS patients after HIV
encephalitis and toxoplasma (2% to 5% of all AIDS patients).
Cryptococcosis produces a meningoencephalitis with mucoid
exudate, causing a widening of subarachnoid and perivascular spaces.
Sensitivity of CT is low, but close to 80% of MR studies may show
cryptococcal-related lesions, most commonly dilated perivascular
spaces.
Infection is usually hematogenous from the lungs and favors the
meninges. Extension along perivascular spaces may give origin to
cystic basal ganglia lesions (“gelatinous pseudocysts”); <50% of
these cysts enhance after MR contrast is administered.
Cryptococcus may result in choroid plexitis (nocardia and
tuberculosis can also do this); involvement of choroid plexus at
ventricular atrium by cryptococcomas is typical. In other parts of the
brain, cryptococcomas appear as solid or ring-enhancing lesions.
Other findings: communicating hydrocephalus, miliary-enhancing
nodules, nodular leptomeningitis, cerebellar involvement (medial
aspects), and cryptococcoma (which may be indistinguishable from
any other abscess when ring enhancing).
Main differential diagnosis: sarcoidosis, vasculitis, tuberculosis,
toxoplasmosis.
608
FIGURE 14-19. Axial postcontrast T1 shows multiple enhancing lesions
in the basal ganglia and leptomeningeal enhancement worst in the right
posterior hemisphere.
609
FIGURE 14-20. Axial FLAIR, in a different patient, shows multiple
bright lesions in the basal ganglia bilaterally.
610
enhancing lesions in the basal ganglia.
611
FIGURE 14-22. Axial T2, in a different patient, demonstrates multiple
gelatinous pseudocysts in the basal ganglia.
612
FIGURE 14-23. Axial postcontrast T1 shows gelatinous pseudocysts in
the medial cerebellar hemispheres (arrows).
613
ring-enhancing cryptococcoma (arrow) in the left frontal lobe.
SUGGESTED READINGS
Gottumukkala RV, Romero JM, Riascos RF, Rojas R, Glikstein RS. Imaging of
the brain in patients with human immunodeficiency virus infection. Top Magn
Reson Imaging 2014;23:275–291.
Smith AB, Smirniotopoulos JG, Rushing EJ. From the archives of the AFIP:
central nervous system infections associated with human immunodeficiency
virus infection: radiologic-pathologic correlation. Radiographics
2008;28:2033–2058.
614
Cysticercosis
Key Facts
Most common parasitic infection in immunocompetent patients
(incidence is not increased in AIDS). Acquired by ingesting
fruits/vegetables contaminated with Taenia solium eggs; ingesting
larvae (such as in undercooked pork) results in intestinal taeniasis.
Most common worldwide cause of acquired seizures with peak
incidence between 25 and 35 years of age. Symptoms: seizures,
headaches, and other manifestations of elevated intracranial pressure
due to CSF obstruction.
Hematogenous spread of larvae produces lesions at gray–white
junctions of the cerebral hemispheres.
Almost all CNS cysticercosis involves the brain; spinal cord
involvement is seen in 1% of patients. Intraventricular lesions are the
second most common site (20% to 50%). Subarachnoid space lesions
(generally of the racemose type) are the third most common site
(<10%). Best way to identify cysts in CSF spaces: CISS/FIESTA
images.
Common imaging findings:
Vesicular stage: cyst-like lesion with mural nodule (viable larva
with full bladder and scolex; intact membrane generally with no
contrast enhancement).
Colloidal vesicular stage: cyst dies and produces inflammatory
reaction (incomplete ring-enhancing lesion with edema).
Granular nodular stage: dead organism produces classic ring-
enhancing lesion with thick capsule.
Nodular calcified stage: final stage in which the lesion calcifies
(rarely these lesions will show contrast enhancement on MRI).
Occasionally, multiple lesions are in the colloidal stage and produce
an encephalitis-like picture.
Main differential diagnosis when intra-axial and acute: metastases,
abscesses, tuberculomas; when extra-axial: arachnoid cysts,
epidermoid.
615
FIGURE 14-25. Axial postcontrast CT shows a cystic lesion in the left
frontal lobe with marginal enhancement and scolex centrally.
616
multiple cystic lesions with peripheral enhancement some of them
containing a scolex.
617
racemose type cysticercosis in left Sylvian cistern. Enhancement is
atypical in this type of cysticercosis and probably related to meningitis.
618
FIGURE 14-28. Axial CISS image in a different patient shows multiple
cysts in the lateral ventricles.
619
FIGURE 14-29. Axial CT, in a different patient, shows innumerable
calcified parenchymal lesions in the final stage of the disease.
620
FIGURE 14-30. Sagittal T2 shows an intramedullary lesion with a scolex
(arrow) and surrounding edema in a different patient with cysticercosis.
SUGGESTED READINGS
Abdel Razek AA, Watcharakorn A, Castillo M. Parasitic diseases of the central
nervous system. Neuroimaging Clin N Am 2011;21:815–841.
Castillo M. Imaging of neurocysticercosis. Semin Roentgenol 2005;39:465–473.
Human Immunodeficiency Virus Infection

621
Key Facts
In children, maternal transmission accounts for the majority of cases;
2% of all AIDS patients are children, most children die during the
first year of life, and the brain shows basal ganglia calcifications
(appear generally after 12 months of age), atrophy, and microcephaly.
In adults, HIV produces a subacute encephalitis characterized by
demyelination, gliosis, and multinucleated giant cells; it constitutes
initial presentation in 10% of AIDS patients and eventually develops
in up to 60% of them leading to the HIV-associated neurocognitive
disorder.
In adults, MR shows confluent, nonenhancing, ill-defined, and
relatively symmetric areas of high signal intensity on T2/FLAIR,
especially in the white matter of the frontal and parietal lobes (may
involve corpus callosum); there is diffuse atrophy (particularly
cortical and basal ganglia), which correlates with the degree of
cognitive impairment; occasionally, HIV results in an aseptic
meningitis and produces meningeal enhancement. In 10% of patients,
posterior fossa involvement occurs, particularly in middle cerebellar
peduncles. Occasionally, gray matter involvement is present. DTI
may show increased mean diffusivity and decreased fractional
anisotropy soon after seroconversion. Functional MRI has shown
greater frontal and parietal activation, presumably due to recruitment
of additional brain regions. PET shows cortical hypometabolism as
the disease progresses.
MRS may initially show elevation of choline and myoinositol and
low NAA; with chronicity, all metabolites are low.
HIV-associated vasculopathy can be seen in intra- or extracranial
vessels, with or without aneurysm formation.
The abnormal signal intensity in the white matter of adults may
improve or even resolve after treatment.
Main differential diagnosis in adults: PML.
622
FIGURE 14-31. Axial FLAIR shows high signal in white matter of both
hemispheres, cortical atrophy, and ventricular dilation.
623
FIGURE 14-32. Coronal FLAIR in a different, 48-year-old patient,
demonstrates marked cortical and deep gray matter atrophy and increased
bihemispheric signal.
624
FIGURE 14-33. Corresponding axial T2 shows extensive confluent
increased white matter signal intensity bilaterally.
625
FIGURE 14-34. Axial CT shows calcification of the basal ganglia in a
baby at 1 year of age with congenital AIDS.
626
FIGURE 14-35. Axial postcontrast CT, in a different patient, shows
dilated vasculopathy of AIDS. These same findings can be seen with
infections of the arterial wall by other pathogens. (Courtesy H. Alvarez,
Chapel Hill, NC.)
SUGGESTED READING
Ances BM, Hammoud DA. Neuroimaging of HIV-associated neurocognitive
627
disorders (HAND). Curr Opin HIV AIDS 2014;9:545–551.
Meningitis, Complications
Key Facts
Suspect in young child with meningitis and progressively enlarging
head; most common complication is hydrocephalus.
Sterile subdural effusions are more likely to be a complication of
Haemophilus influenzae meningitis and tend to be large, bilateral, and
frontoparietal.
About 2% of subdural effusions become infected (empyemas).
Most effusions resolve spontaneously (large ones may require
drainage).
Both effusions and empyema show membrane enhancement.
Empyemas occur in 15% of patients with meningitis; may also be
secondary to sinusitis, postsurgical infection, or infection of an
epidural hematoma.
Subdural (as opposed to epidural) empyemas tend to incite more
severe cerebral edema due to lack of an intervening dura. Epidural
ones are less common and more frequently due to sinusitis or
otomastoiditis.
Complications from empyemas and/or meningitis include venous
thrombosis, infarctions, cerebritis, ventriculitis, vasculopathies, and
abscesses.
Empyemas may be identified by high signal on DWI (most reliable
imaging technique); this high signal is due to restricted diffusion
secondary to complex environment of pus (inflammatory cells,
products of cell death, bacteria).
628
FIGURE 14-36. Axial postcontrast T1 shows right frontal (arrowhead)
and parafalcine subdural empyemas and an epidural empyema (arrow)
crossing the midline.
629
FIGURE 14-37. Corresponding axial FLAIR demonstrates cerebral edema
adjacent to the right frontal subdural collection.
630
FIGURE 14-38. Axial DWI, in a different patient, shows bright signal
within a left hemispheric subdural empyema.
631
FIGURE 14-39. Axial postcontrast T1 in a different patient with
ventriculitis shows ventriculomegaly, ependymal enhancement, and
layering debris/pus (arrowheads).
632
extensive enhancement in otomastoiditis and thrombophlebitis of the
adjacent sigmoid sinus (arrow).
633
FIGURE 14-41. DWI in a different patient with meningitis and bifrontal
subdural collections shows multiple infarctions.
SUGGESTED READINGS
Acosta JH, Rantes CI, Arbelaez A, Restrepo F, Castillo M. Noncongenital central
nervous system infections in children: radiology review. Top Magn Reson
Imaging 2014;23:153–164.
Mohan S, Jain KK, Arabi M, et al. Imaging of meningitis and ventriculitis.
634
Meningitis (Uncomplicated)
Key Facts
Common causative organisms include Escherichia coli (E. coli) and
streptococcus group B (newborns), Haemophilus (H. influenzae)
(children <7 years), Neisseria (N. meningitidis) (older children and
adolescents), and Streptococcus (S. pneumoniae) (adults). Listeria
mainly affects elderly individuals and those with impaired cellular
immunity.
Mortality (even with treatment) of meningitis is as high as 30% and
10% of patients have persistent neurological deficits.
Viral agents (“lymphocytic” meningitis) include enteroviruses,
mumps virus, Epstein-Barr virus, and arbovirus. Viral meningitis in
adults is rare.
Chronic meningitis is generally due to Mycobacterium tuberculosis or
fungi.
Diagnosis of meningitis is based on clinical assessment and CSF
analysis, imaging is reserved for complications.
Mechanism of spread: hematogenous from paranasal sinus or mastoid
infections (emissary veins are valveless), otitis media, penetrating
head injury, and prior surgery.
MRI is more sensitive than CT and shows leptomeningeal and/or
ependymal enhancement (remember that dural enhancement at 3.0 T
may be normal); FLAIR images show high signal in CSF (other
causes of high CSF signal on FLAIR include high protein
concentration [blood, tumor], susceptibility artifacts, oxygen
administration [high FiO2, generally above 50%], some sedatives,
and prior gadolinium administration in renal failure). Postcontrast
FLAIR may be more sensitive than postcontrast T1 in the detection of
leptomeningeal enhancement.
Main differential diagnosis for leptomeningeal enhancement:
metastases, sarcoidosis.
635
FIGURE 14-42. Axial FLAIR shows bright signal intensity within cortical
sulci (compare with a few normal sulci in the frontal lobes [arrow] and
with normal CSF signal within the ventricles).
636
leptomeningeal enhancement in cortical sulci.
637
FIGURE 14-44. Axial postcontrast T1, in a different patient, demonstrates
leptomeningeal enhancement bilaterally and also “coating” of the
brainstem.
638
FIGURE 14-45. Corresponding sagittal postcontrast T1 shows extensive
leptomeningeal enhancement along cerebral sulci, cerebellar folia,
brainstem, and tectum.
SUGGESTED READINGS
Castillo M. Imaging of meningitis. Semin Roentgenol 2005;39:458–464.
Mohan S, Jain KK, Arabi M, Shah GV. Imaging of meningitis and ventriculitis.
639
Multiple Sclerosis
Key Facts
Most common type of demyelinating disease (1:1,000 individuals);
seen more often in women (60% of patients) between the ages of 20
and 40 years.
Multiple sclerosis is a clinical diagnosis; sensitivity of MR (>85%)
surpasses that of all noninvasive clinical tests; FLAIR is very
sensitive in cerebral hemispheres (particularly in regions close to
cerebrospinal fluid) but is less useful for lesions in the brainstem and
cerebellum which can be better seen on T2. Sagittal FLAIR
demonstrates involvement of the callosal–septal interface, which is
typical. “Black holes” on T1 correspond to areas of severe and
irreversible neuronal loss. Some lesions have mild high signal on
DWI and restricted ADC. DTI shows loss of fractional anisotropy in
lesions and surrounding normal-appearing brain.
Deep gray nuclei, dentate nucleus, and cortex may appear dark on T2
due to increased cerebral iron accumulation; iron deposits may be
seen with iron-sensitive techniques such as SWI and quantitative
susceptibility mapping.
Locations: periventricular (>80%) and juxtacortical white matter,
corpus callosum (especially its undersurface: “callosal–septal
interface,” 50% to 85%), the visual pathways (optic neuritis),
posterior fossa (10%), and brainstem (seen more commonly in
younger patients).
Occasionally, gray matter may be involved (remember that
myelinated fibers may travel through gray matter), better seen at 3T.
Enhancement generally lasts 4 to 8 weeks (but may persist for up to 5
months) implies active demyelination and breakdown of the blood–
brain barrier.
Lesions may be solitary and simulate a neoplasm (called
“tumefactive” lesions). Balo sclerosis is a type of tumefactive lesion
characterized by rings of concentric involvement.
Types: chronic relapsing (70%), chronic progressive (20% to 25%),
and acute fulminant (<%5).
Main differential diagnosis: vasculitis, Lyme disease, Susac
syndrome (callosal lesions are central and spare the callosal-septal
interface), ADEM (lesions tend to be larger, involve the basal ganglia
640
earlier, and patients have symptoms of encephalitis).
FIGURE 14-46. Axial FLAIR shows numerous periventricular matter

lesions, some of which are ovoid and perpendicular to the lateral
ventricles.
641
FIGURE 14-47. Midsagittal FLAIR, in a different patient, shows bright
lesions (arrows) in posterior “callosal–septal” interface region.
642
enhancing periventricular plaques.
643
FIGURE 14-49. Axial postcontrast T1, in a different patient with
tumefactive MS shows large left frontal hypointense lesion with
incomplete rim of enhancement (arrows) and containing a vein centrally
(arrowhead).
644
FIGURE 14-50. Parasagittal FLAIR, in a different patient with Balo
concentric sclerosis, shows large lesion with rings of demyelination and
remyelination.
645
FIGURE 14-51. Coronal fat-saturated T2 image in a different patient
shows left optic neuritis with increased signal within the nerve (arrow).
SUGGESTED READINGS
Filippi M, Rocca MA. MR imaging of multiple sclerosis. Radiology
2011;259:659–681.
Rocca MA, Messina R, Filippi M. Multiple sclerosis imaging: recent advances. J
Neurol 2013;260:929–935.
Neuromyelitis Optica
646
Key Facts
Relapsing inflammatory and necrotizing process (aka: Devic disease)
previously believed to be a form of multiple sclerosis.
Sites of involvement: periventricular regions (particularly around
third and fourth ventricles, cerebral aqueduct, and dorsal midbrain;
frequently in areas of high aquaporin-4 expression), optic nerves
(characteristically long segment, bilateral, and involving the chiasm),
corticospinal tracts, and spinal cord (long lesions, three to four
segments, usually central). May also involve the corpus callosum in a
“marbled” pattern. Periventricular lesions tend to follow the
ependyma (as opposed to the perpendicular “Dawson fingers” of
multiple sclerosis).
Most lesions show some contrast enhancement, which is usually ill
defined with a “cloud-like” appearance. Linear “pencil-thin”
enhancement along the ependyma is thought to be characteristic.
DTI may show abnormal parameters on normal-appearing white
matter.
MRS shows low NAA, low myoinositol, and sometimes high choline.
Diagnosis is confirmed by high IgG (highly specific antibodies
against aquaporin-4 membrane receptors, which are one of the most
important water channels in humans).
Main differential diagnosis of spinal lesions: tumor, MS, ischemia,
idiopathic transverse myelitis, AVF; in the brain, distribution of
lesions is typical.
647
FIGURE 14-52. Midsagittal postcontrast T1 shows multiple
periventricular enhancing areas and ill-defined enhancement in the upper
cervical cord.
648
FIGURE 14-53. Axial FLAIR image, in a different patient, shows
bilateral signal abnormality centered along the anterior aspect of the third
ventricle.
649
FIGURE 14-54. Corresponding postcontrast T1 shows peripheral ring
enhancement that crosses anatomic boundaries.
650
FIGURE 14-55. Axial postcontrast T1 in a different patient shows
posterior optic nerve enhancement (arrows).
651
FIGURE 14-56. Sagittal T2, in a different patient, shows a hyperintense
longitudinal lesion involving the upper cervical cord (arrows).
652
FIGURE 14-57. Sagittal T2, in a different patient, demonstrates a lesion
preferentially involving the central gray matter of the thoracic cord.
SUGGESTED READINGS
Barnett Y, Sutton IJ, Ghadiri M, Masters L, Zivadinov R, Barnett MH.
Conventional and advanced imaging in neuromyelitis optica. AJNR Am J
Neuroradiol 2014;35:1458–1466.
Kim HJ, Paul F, Lana-Peixoto MA, et al. MRI characteristics of neuromyelitis
optica spectrum disorder: an international update. Neurology
2015;84:1165–1173.
653
Progressive Multifocal
Leukoencephalopathy (PML)
Key Facts
PML is usually due to reactivation of the JC polyomavirus and is seen
in 1% to 4% of patients with AIDS, most commonly in those with
CD4 counts <200; other patients at risk are those with organ
transplants, Hodgkin lymphoma, chronic lymphocytic leukemia,
congenital immunodeficiencies, lupus erythematosus, sarcoidosis,
amyloidosis, scleroderma, and receiving steroids and certain
immunomodulatory drugs (e.g., natalizumab, rituximab, and others).
PML destroys oligodendrocytes leading to demyelination.
Imaging studies show multifocal peripheral white matter
abnormalities (usually occipitoparietal with or without callosal
involvement and less likely frontal), which may be symmetrical, have
little or no mass effect, and typically show no enhancement.
Natalizumab-related PML is more often monofocal and favors the
frontal lobes.
Subcortical lesions tend to involve the U fibers resulting in a
“scalloped” appearance. New and larger lesions may show a leading
edge of increased DWI and intermediate to low ADC signal.
Perfusion studies show low blood flow.
Immune reconstitution inflammatory syndrome (IRIS) should be
suspected if there is mass effect, contrast enhancement, and edema,
particularly in the setting of paradoxical clinical worsening after
initiation of highly active antiretroviral therapy.
MRS shows high choline, low NAA, and lactate initially and then is
followed by pan metabolite decrease and lipids.
Up to 50% may have involvement of gray matter structures
(especially basal ganglia and thalamus).
Some lesions may improve after treatment.
Main differential diagnosis: HIV.
654
FIGURE 14-58. Axial FLAIR in a poorly compliant HIV patient shows
asymmetric confluent hyperintensity in the posterior white matter with
callosal and U fiber involvement.
655
FIGURE 14-59. Axial DWI, in the same patient, shows a leading edge of
increased signal in a right parietal lesion.
656
FIGURE 14-60. Axial postcontrast T1 in the same patient 2 weeks after
initiation of antiretroviral therapy shows development of IRIS with
enhancement and gyral edema.
657
FIGURE 14-61. Axial FLAIR in a different patient with multiple sclerosis
on monoclonal antibodies demonstrates multifocal white matter signal
abnormalities bilaterally.
658
FIGURE 14-62. Axial arterial spin labeling perfusion images, in a
different patient, show low CBF in affected white matter.
659
FIGURE 14-63. MRS, long TE, in a different patient, shows decreased
levels of choline and NAA and lipids.
SUGGESTED READINGS
Bag AK, Curé JK, Chapman PR, Roberson GH, Shah R. JC virus infection of the
brain. AJNR Am J Neuroradiol 2010;31:1564–1576.
Berger JR, Aksamit AJ, Clifford DB, et al. PML diagnostic criteria: consensus
statement from the AAN Neuroinfectious Disease Section. Neurology
2013;80:1430–1438.
Rasmussen Encephalitis
Key Facts
660
A form of chronic focal encephalitis that is progressive and unilateral.
Uncertain etiology, probably viral initially and then autoimmune.
Important cause of progressive relentless seizures (particularly
“epilepsia partialis continua”) and hemiparesis.
Usually begins at 6 to 10 years of age.
Treatment may require hemispherectomy.
MRI shows high T2/FLAIR signal intensity in the cortex (initially
affecting insula and perisylvian regions) and progressive unilateral
volume loss that may involve caudate head and putamen; cortical
enhancement is rare.
PET shows unilateral decreased metabolism.
MRS shows low choline, creatine, and NAA but high lipids/lactate
and glutamine/glutamate; MRS is more sensitive than MRI and may
identify bilateral abnormalities precluding surgery.
Main differential diagnosis acutely: ischemia, mitochondrial diseases,
seizure-induced changes; chronic: sequelae of ischemia, Sturge-
Weber syndrome.
661
FIGURE 14-64. Coronal T2 shows loss of volume throughout left
hemisphere and increased signal intensity in the temporal cortex.
662
FIGURE 14-65. Coronal view from PET, in the same patients, shows left
hemispheric hypometabolism.
663
FIGURE 14-66. Axial T2, in a different patient during early period shows
swollen left insular cortex.
664
FIGURE 14-67. Axial FLAIR, in a different patient, shows mild swelling
of left insular and perisylvian cortex. Note ipsilateral caudate head and
putaminal atrophy and mild ex vacuo ventricular dilatation.
665
FIGURE 14-68. Axial T2, in a different patient, shows chronic changes
with atrophy of the left cerebral hemisphere, caudate head, and putamen.
666
FIGURE 14-69. Axial postcontrast T1, in a different patient, shows loss
of volume and some cortical enhancement in the left hemisphere.
SUGGESTED READINGS
Bien CG, Granata T, Antozzi C, et al. Pathogenesis, diagnosis and treatment of
Rasmussen encephalitis: a European consensus statement. Brain 2005;128(pt
3):454–471.
Pradeep K, Sinha S, Saini J, et al. Evolution of MRI changes in Rasmussen's
encephalitis. Acta Neurol Scand 2014;130:253–259.
667
Sarcoidosis
Key Facts
Occurs most commonly in African Americans, females, between 20
and 40 years.
CNS involvement occurs in 5% of patients with systemic disease but
is found in up to 14% of autopsies in patients with systemic
sarcoidosis.
Symptoms: cranial neuropathies (II, VII, VIII), aseptic meningitis,
hydrocephalus, parenchymal lesions (most spread via perivascular
spaces), and occasional vasculitis. Neurologic symptoms are the first
manifestation of sarcoidosis in 60% to 70% of cases.
Infiltration of the pituitary gland, optic chiasm, and hypothalamus is
typical; the spinal cord (1%, particularly the cauda equina) may also
be involved.
Cerebral lesions may have an appearance similar to multiple sclerosis.
Most patients respond to steroid treatment, but CNS sarcoidosis
accounts for significant morbidity and mortality in patients with
systemic sarcoidosis.
Dural involvement (most common imaging finding) may simulate
meningioma(s) (particularly in an “en-plaque” fashion). Sarcoid
lesions may be dark on T2, as can be seen with other granulomatous
diseases.
Main differential diagnosis: tumor, meningioma, idiopathic
pachymeningitis, chronic inflammatory demyelinating polyneuritis
(when cranial nerves are affected); when white matter is involved:
MS, Lyme, vasculitis.
668
FIGURE 14-70. Axial postcontrast T1 shows thick and diffuse dural
enhancement.
669
FIGURE 14-71. Axial T2 image, in a different patient, shows a dural
lesion (arrow) along the anterior falx with very low signal intensity.
670
FIGURE 14-72. Sagittal postcontrast T1 in a different patient
demonstrates an avidly enhancing lesion involving the hypothalamus and
supraoptic recess (arrow).
671
extensive leptomeningeal disease in the suprasellar cisterns and
hydrocephalus with aqueductal dilatation.
672
FIGURE 14-74. Sagittal postcontrast T1, in the same patient, shows
extension of leptomeningeal disease to the spinal cord and dilatation of the
fourth ventricle.
673
FIGURE 14-75. Axial postcontrast T1 in a different patient demonstrates
an avidly enhancing sarcoid granuloma in the cervicomedullary junction.
SUGGESTED READINGS
Lury KM, Smith JK, Matheus MG, Castillo M. Neurosarcoidosis—review of
imaging findings. Semin Roentgenol 2005;39:495–504.
Shah R, Roberson GH, Cure JK. Correlation of MR imaging findings and clinical
manifestations in neurosarcoidosis. AJNR Am J Neuroradiol
2009;30:953–961.
Tuberculosis
674
Key Facts
Mostly caused by M. tuberculosis; atypical organisms are rare except
in immunodepressed patients; 30% of patients are HIV positive
(particularly intravenous drug users), where mortality is much higher
(up to 70%).
Because of hematogenous dissemination, lesions usually occur at the
gray–white junctions of cerebral hemispheres, basal ganglia, or
cerebellum (especially in children) and may also be dural and/or
involve adjacent bones.
Most common manifestations: meningitis (particularly at the basilar
cisterns in non-HIV patients) and hydrocephalus; meningitis may be
focal (especially in suprasellar and insular regions).
Acutely, a cerebritis may be seen, which then progresses into ring-
enhancing lesions; lesions (tuberculomas) occur in 25% of cases and
are generally small, solitary (multiple lesions seen in <30% of cases,
some may be clustered), and surrounded by edema; calcification is
detectable by CT in 1% to 6% of lesions (especially old lesions);
early-stage tuberculomas and rim of lesions show low signal intensity
on T2WI/FLAIR. MRS shows low metabolites but high lipids/lactate
and no amino acids (which are seen in bacterial abscesses);
occasionally, choline may be high reflecting active inflammation.
Tuberculosis may result in a vasculitis and cerebral infarctions.
Main differential diagnosis: viral/bacterial meningitis,
pyogenic/fungal abscess, tumor, sarcoidosis.
675
FIGURE 14-76. Axial postcontrast T1 shows thick basilar enhancement
with nodular conglomerates in the suprasellar cistern in a non-HIV patient.
676
miliary tuberculosis with numerous lesions predominantly at the gray–
white matter junctions.
677
FIGURE 14-78. Corresponding axial T2 demonstrates many of these
lesions to be of low signal intensity.
678
FIGURE 14-79. Axial postcontrast T1 in a different patient shows large
frontal tuberculomas with parenchymal and meningeal components.
679
FIGURE 14-80. Parasagittal postcontrast T1, in a different patient, shows
occipital ring-enhancing tuberculoma.
680
FIGURE 14-81. MRS, long TE, in the same patient as in Figure 14-80,
shows elevated choline and low NAA but no amino acids as are expected
in pyogenic abscesses.
SUGGESTED READINGS
Arbelaez A, Medina E, Restrepo F, Castillo M. Cerebral tuberculosis. Semin
Roentgen 2005;39:474–481.
Patkar D, Narang J, Yanamandala R, Lawande M, Shah GV. Central nervous
system tuberculosis: pathophysiology and imaging findings. Neuroimaging
Clin N Am 2012;22:677–705.
681
Toxoplasmosis
Key Facts
Most common (20% to 40%) opportunistic infection in AIDS
patients. Almost always due to reactivation or spread of previous
infection.
Produces focal lesions or disseminated encephalitis.
Locations: basal ganglia and gray–white junctions of the cerebral
hemispheres, but it may occur anywhere.
Imaging shows ring-enhancing lesions (1 to 3 cm) with marked
surrounding edema, almost always hypodense/hypointense before
contrast administration, occasionally hemorrhagic, and solitary in
about 15% of cases. Eccentric target-like nodular enhancement (in
30% and thought to be highly suggestive, due to infolding of inflamed
vessels) or concentric alternating T2/FLAIR bright-dark rings (due to
layers of hemorrhage, necrosis, and edema) may occur. DWI shows
less restriction in toxoplasmosis than in lymphoma. Perfusion shows
lower rCBV and rCBF in toxoplasmosis than in lymphoma. MRS
shows normal to mildly elevated choline, low creatine and NAA, and
presence of lipids/lactate, or large lipid peaks with suppression of
other metabolites. PET shows decreased metabolism in toxoplasmosis
when compared to lymphoma.
After 2 to 4 weeks of antitoxoplasma treatment, some healing should
be present by imaging (if not, consider lymphoma); by 3 to 6 weeks
of treatment, the lesions should resolve. Healed lesions show as focal
areas of malacia, which may calcify.
Main differential diagnosis in immune-suppressed patients:
lymphoma, tuberculosis, pyogenic abscess, metastases, glioblastoma.
682
FIGURE 14-82. Axial postcontrast T1 shows a lesion in the left
cerebellum displaying the “eccentric target” sign (arrow).
683
FIGURE 14-83. Axial T2, in a different patient, demonstrates a lesion in
the right basal ganglia with alternating signal intensities and surrounding
edema.
684
FIGURE 14-84. Axial T2, in a different patient, shows a toxoplasma
lesion at the gray–white matter junction in the right frontal lobe.
685
FIGURE 14-85. ADC map, in a different patient, shows no restricted
diffusion in a left basal ganglia lesion.
686
FIGURE 14-86. Corresponding perfusion map shows no increase in blood
volume.
687
FIGURE 14-87. MRS, long TE, in a different patient, shows only mildly
elevated choline (arrows) in the lesion and low NAA.
SUGGESTED READINGS
Camacho DL, Smith JK, Castillo M. Differentiation of toxoplasmosis and
lymphoma in AIDS patients by using apparent diffusion coefficients. AJNR
Am J Neuroradiol 2003;24:633–637.
Shih RY, Koeller KK. Bacterial, fungal, and parasitic infections of the central
nervous system: radiologic-pathologic correlation and historical perspectives.
Radiographics 2015;35:1141–1169.
688
Viral Encephalitis
Key Facts
Herpes type I: Occurs more commonly in adults from primary
infection or reactivation (dormant virus in trigeminal ganglion or
lower cranial nerves), accounts for 20% of all viral and 90% of herpes
virus encephalitides, mortality is 50% to 70%, and produces
necrotizing encephalitis in the insula and orbital surface of frontal
lobes (may be bilateral), typically sparing the basal ganglia; the
brainstem is occasionally involved; hemorrhagic transformation is
common. Best imaging techniques are FLAIR and DWI (which may
be more sensitive and demonstrate extent of disease better).
Herpes type II: Results from direct inoculation during vaginal
delivery (especially in premature babies); produces a diffuse
meningoencephalitis, which may involve the cerebellum, and the end
result is cystic malacia and atrophy.
Cytomegalovirus (CMV): Although rare, it is the most common
transplacental encephalitis; most patients remain asymptomatic but
may have microcephaly (50% to 75%), mental retardation, deafness,
seizures, and intracranial calcifications (70%) including
periventricular regions and along perforating arteries; it also affects
the germinal matrix resulting in neuronal migration anomalies and
produces a chorioretinitis and microphthalmia (more common with
CMV than with toxoplasmosis); its incidence in AIDS patients
declined significantly after the advent of highly active antiretroviral
therapy. In adults, the most common findings are focal areas of
cerebritis accompanied by overlying meningeal enhancement.
Ventriculitis, myelitis, and radiculitis may also be seen.
689
FIGURE 14-88. Axial CT shows swelling in the mesial right temporal
lobe with low density and effacement of the temporal horn (arrow).
690
FIGURE 14-89. Corresponding axial FLAIR better demonstrates the
extent of inflammatory change involving also the adjacent right frontal
lobe.
691
FIGURE 14-90. Coronal postcontrast T1 in the same patient shows
primarily cortical enhancement in most of the temporal lobe (arrowheads)
and insula (arrow).
692
FIGURE 14-91. Axial DWI, in a different patient, shows high signal in
left temporal lobe, bilateral insulae, frontal lobes (arrowheads), and left
hippocampal tail/posterior cingulate gyrus (arrow).
693
FIGURE 14-92. Axial CT, patient with congenital CMV shows
periventricular calcifications, hydrocephalus and thickened cortex.
694
FIGURE 14-93. Sagittal oblique sonogram shows branching pattern of
calcification involving the lenticulostriate arteries.
SUGGESTED READINGS
Bulakbasi N, Kocaoglu M. Central nervous system infections of herpesvirus
family. Neuroimaging Clin N Am 2008;18:53–84.
Castillo M, Thurnher M. Imaging viral and prion infections. Semin Roentgenol
2005;39:482–494.
695
CHAPTER 15
Leukodystrophies
Key Facts
Most common peroxisomal disorder characterized by very long chain
fatty acids that are not metabolized and are elevated in serum.
The neonatal type is rare and due to multiple enzyme deficiencies; it
involves the white matter diffusely.
The X-linked type is more common (>80%) and is due to mutation in
the ABCD1 gene leading to decreased lignoceroyl-CoA ligase
activity and accumulation of very long chain fatty acids; it presents in
males 4 to 8 years of age (hearing and visual defects, loss of
developmental milestones); neurologic symptoms precede adrenal
insufficiency in a majority of cases; a vegetative state or death usually
occurs 2 years after onset of symptoms.
In 80% of patients, imaging shows bilateral and symmetrical
demyelination in occipitoparietal regions with an enhancing margin at
the front; auditory pathways and the splenium of corpus callosum
may be involved.
Early in the disorder, only the corticospinal tracts or the lateral
lemnisci may be affected.
Adrenomyeloneuropathy is a subtype of the X-linked disease that
may affect the cord (dorsal and lateral columns) and is seen in older
males.
MRS shows high choline and low NAA at enhancing “front,” low
choline, creatine and NAA and high myoinositol at areas of chronic
696
involvement.
DTI may show to better extent the degree of white matter
involvement.
Main differential diagnosis: none, findings are typical.
FIGURE 15-1. Axial FLAIR image shows symmetrical high signal in

posterior white matter and splenium.
697
FIGURE 15-2. Post-contrast axial T1, in the same patient, shows
enhancement in margins (arrows) of white matter lesions.
698
FIGURE 15-3. Axial FLAIR in a different patient with chronic
adrenoleukodystrophy demonstrates prominent volume loss and gliosis in
the occipitoparietal regions.
699
FIGURE 15-4. Corresponding axial T2 demonstrates chronic changes
with posterior encephalomalacia and gliosis.
700
FIGURE 15-5. Coronal post-contrast T1 in a different patient shows
temporoparietal white matter abnormalities with a leading edge of
enhancement.
701
FIGURE 15-6. Corresponding MR spectroscopy demonstrates elevated
choline and undetectable NAA.
SUGGESTED READINGS
Miller WP, Mantovani LF, Muzic J, et al. Intensity of MRI gadolinium
enhancement in cerebral adrenoleukodystrophy: a biomarker for inflammation
and predictor of outcome following transplantation in higher risk patients.
van der Voorn JP, Pouwels PJ, Powers JM, et al. Correlating quantitative MR
imaging with histopathology in X-linked adrenoleukodystrophy. AJNR Am J
Neuroradiol 2011;32:481–489.
Alexander Disease
Key Facts
Alexander disease is due to mutations in the GFAP gene
(chromosome 17). It generally presents in infancy, adolescence, and
rarely in adults.
702
Histologic landmark: increased astrocytic eosinophilic Rosenthal
fibers, which lead to increased size and weight of brain (patients have
macrocephaly); however, these fibers are also found in astrocytomas,
and inflammatory and vascular disorders.
Early on, demyelination occurs in frontal lobes and then extends to
involve the entire cerebral hemispheres; the cavum septi pellucidi and
cavum vergae may be widened.
Contrast enhancement may be present (particularly in the basal
ganglia and the periventricular regions), and its significance is
uncertain (it is probably related to active demyelination).
Periventricular rim of low T2 and high T1 is typical. MRS may show
low NAA and high myoinositol and lactate.
In children, death occurs 2 to 3 years after diagnosis.
Adult pattern has less supratentorial involvement showing high T2
signal in medulla (corticospinal tracts and medial lemnisci),
cerebellar dentate nuclei, and cervical spinal cord, all of which may
enhance.
Main differential diagnosis: none, findings are typical but when
diffuse include Canavan and its differential diagnosis.
703
FIGURE 15-7. Axial T2 shows extensive white matter signal
hyperintensity in the frontal lobes.
704
FIGURE 15-8. Axial T2 in the same patient shows involvement of the
basal ganglia and external capsules bilaterally.
705
FIGURE 15-9. Axial T2 in an adult shows abnormal signal in medulla in
the region of white matter tracts.
706
FIGURE 15-10. Midsagittal post-contrast T1 shows enhancement in
medulla.
SUGGESTED READINGS
Poloni CB, Ferey S, Haenggeli CA, et al. Alexander disease: early presence of
707
cerebral MRI criteria. Eur J Paediatr Neurol 2009;13:556–558.
Vazquez E, Macaya A, Mayolas N, et al. Neonatal Alexander disease: MR
imaging prenatal diagnosis. AJNR Am J Neuroradiol 2008;29:1973–1975.
Canavan Disease
Key Facts
Autosomal recessive disorder characterized by mutations in the
ASPA gene (chromosome 17) leading to deficiency of N-
acetylaspartylase and accumulation of NAA. Begins in first year of
life.
Brain is enlarged and macroscopically appears spongy (hence,
“spongiform leukodystrophy”); occasionally, cysts are large enough
to be visualized by imaging studies.
Demyelination involves all white matter including subcortical U-
fibers during the first 6 months of life. Involvement of globi pallidi
(with sparing of corpus striatum) and thalami is also typical. Brain
stem and dentate nuclei are occasionally affected.
It usually has a rapidly fatal course.
MRS: large NAA peak and high myoinositol.
Main differential diagnosis: Alexander disease, merosin-deficient
muscular dystrophy, vanishing white matter disease, megalencephalic
leukoencephalopathy with subcortical cysts, mucopolysaccharidoses.
708
FIGURE 15-11. Axial T2 shows diffusely bright white matter and
increased signal in globi pallidi and lateral thalami.
709
FIGURE 15-12. Axial T2, in the same patient, shows diffuse white matter
involvement and large lateral ventricles.
710
FIGURE 15-13. Axial T2, in the same patient, shows sparing of cortex
and white matter abnormalities.
711
FIGURE 15-14. Long TE MRS, in the same patient, shows very high
NAA peak (arrow).
SUGGESTED READINGS
Cheon JE, Kim IO, Hwang YS, et al. Leukodystrophy in children: a pictorial
review of MR imaging features. Radiographics 2002;22:461–476.
Yang E, Prabhu SP. Imaging manifestations of the leukodystrophies, inherited
disorders of white matter. Radiol Clin North Am 2014;52:279–319.
712
Krabbe Disease
Key Facts
Autosomal recessive lysosomal disorder (also called “globoid cell
leukodystrophy”) caused by mutations in the GALC gene
(chromosome 14) characterized by a deficiency of galactocerebroside
beta-galactosidase.
Brain may be initially enlarged but later becomes small and atrophic.
Most commonly diagnosed between the 3rd to 6th months of life;
children may have dysmorphic facies and large ears. Ten percent
present later in life (including adulthood).
Noncontrast CT: increased density in basal ganglia (particularly the
thalami) and corona radiata.
MRI: nonspecific white matter hyperintensities, especially in the
periventricular regions and relatively hypointense thalami on
T2/FLAIR images. Cerebellar white matter, dentate nuclei, and
corticospinal tracts may also be affected. Abnormalities may be
confined to the parietooccipital white matter and corticospinal tracts
in the later forms of the disease.
MRS: mild-to-moderate high choline, low NAA, and high
myoinositol.
May result in hypertrophy of the optic nerves and nerve roots in the
cauda equina.
It is rapidly progressive and fatal (1 to 2 years after diagnosis).
713
FIGURE 15-15. Axial T2 shows symmetrical abnormal high signal in
periventricular white matter.
714
FIGURE 15-16. Axial T2, in the same patient, shows involvement of
medial regions in both cerebellar hemispheres.
715
FIGURE 15-17. MRS, long TE, in the same patient, shows high choline
(arrow) and low NAA (arrowhead).
716
FIGURE 15-18. Axial FLAIR in a different patient shows enlargement of
the optic nerves bilaterally (arrow).
SUGGESTED READINGS
Escolar ML, Poe MD, Smith JK, et al. Diffusion tensor imaging detects
717
abnormalities in the corticospinal tracts of neonates with infantile Krabbe
disease. AJNR Am J Neuroradiol 2009;30:1017–1021.
Yang E, Prabhu SP. Imaging manifestations of the leukodystrophies, inherited
disorders of white matter. Radiol Clin North Am 2014;52:279–319.
Metachromatic Leukodystrophy
Key Facts
Lysosomal disorder characterized by a deficiency of arylsulfatase A
(rarely of saposin B), results in the accumulation of sulfatides and is
toxic to white matter.
Most common inherited leukodystrophy (1:100,000 newborns).
Most patients present between 1 and 2 years of age; juvenile and adult
forms of the disease also exist (up to 40% of cases); most common
type of adult-onset leukodystrophy.
Imaging studies show diffuse white matter disease; MRI shows
sparing of subcortical U-fibers and areas of increased T2/FLAIR
signal intensity in cerebellum. Sparing of perivascular white matter
may give rise to “tigroid” appearance. Low T2 signal intensity may
be seen in the thalami in severe cases.
MRS shows moderately high choline, low NAA, high myoinositol.
DWI may show restricted diffusion in areas of abnormal high
T2/FLAIR signal.
End-stage disease is indistinguishable from other leukodystrophies by
imaging studies.
Death occurs 1 to 4 years after onset of symptoms.
718
FIGURE 15-19. Axial T2 shows extensive white matter involvement but
sparing of subcortical U-fibers.
719
FIGURE 15-20. Axial T2, in a different patient, shows typical tigroid
appearance of white matter, which contains residual myelinated regions.
720
FIGURE 15-21. Axial noncontrast CT, in a different patient, shows
extensive white matter hypodensity bilaterally.
721
FIGURE 15-22. Axial T2, in a different patient, shows increased signal in
the cerebellum and brachium pontis.
SUGGESTED READINGS
Eichler F, Grodd W, Grant E, et al. Metachromatic leukodystrophy: a scoring
system for brain MR imaging observations. AJNR Am J Neuroradiol
2009;30:1893–1897.
Martin A, Sevin C, Lazarus C, et al. Toward a better understanding of brain
lesions during metachromatic leukodystrophy evolution. AJNR Am J
Neuroradiol 2012;33:1731–1739.
722
Pelizaeus-Merzbacher Disease
Key Facts
X-linked recessive disorder characterized by lack of myelin-specific
lipids that impair function of oligodendrocytes leading to
hypomyelination.
The disease presents in males with nystagmus, hypotonia, seizures,
and ataxia; however, carrier females can show mild-to-moderate
manifestations.
Pathologically, perivascular myelin is preserved but surrounded by
extensive abnormal myelin giving a “tigroid” appearance.
Two types exist: the connatal form, which is rapidly fatal, and the
classic form, which presents in young males and has a protracted
course.
Lack of mature myelin on MRI shows diffuse high signal intensity on
T2 sequences, at times the abnormal signal intensity has a patchy
appearance reflecting the “tigroid” dysmyelination; the posterior
fossa and brain stem may be normal.
Basal ganglia may have low signal intensity on T2, presumably
because of increased iron deposition.
MRS: mildly elevated choline and creatine and low NAA.
Main differential diagnosis: trichothiodystrophy.
723
FIGURE 15-23. Axial T1 shows complete absence of the normal
brightness expected in the white matter of this 2-year-old boy.
724
FIGURE 15-24. Axial T2, in a different patient, shows diffusely bright
white matter. The internal capsules are less severely affected, but their
signal intensity is not completely normal either.
725
FIGURE 15-25. Axial T2, in a different patient, shows diffuse high signal
in white matter.
726
FIGURE 15-26. Coronal T2, in the same patient as Figure 15-25, shows
diffuse high signal in the white matter of cerebrum and cerebellum. (Case
courtesy A. Pajon, Medellin, Colombia.)
SUGGESTED READINGS
Krishna SH, McKinney AM, Lucato LT. Congenital genetic inborn errors of
metabolism presenting as an adult or persisting into adulthood: neuroimaging
727
in the more common or recognizable disorders. Semin Ultrasound CT MR
2014;35:160–191.
Laukka JJ, Stanley JA, Garbern JY, et al. Neuroradiologic correlates of clinical
disability and progression in the X-linked leukodystrophy Pelizaeus-
Merzbacher disease. J Neurol Sci 2013;335:75–81.
728
CHAPTER 16
Metabolic Disorders
Amino Acid Disorders

Key Facts
In this rare group of disorders, amino acid pathways are deficient, and
proteolipids, which are essential for formation of myelin, are
abnormal.
Phenylketonuria is caused by defective phenylalanine hydroxylase,
producing increased phenylalanine, which inhibits a proteolipid,
leading to dysmyelination; patients are normal at birth, and MRI
shows nonspecific, predominantly supratentorial white matter
changes, which may be more obvious on DWI.
Maple syrup urine disease is characterized by a failure to metabolize
branched-chain amino acids; it presents in the neonatal period and is
rapidly fatal; white matter, basal ganglia and particularly brainstem,
internal capsules, and perirolandic cortex are involved and show
restricted ADC acutely. MRS may show a broad methyl peak at 0.9
ppm, probably from branched chain amino acids and α-keto acids.
Homocystinuria is due to an error in methionine metabolism, which
leads to abnormal collagen and elastin formation and presents as
multiple arterial and venous occlusions leading to strokes, and a high
incidence of ectopia lentis.
Glutaric aciduria type 1 typically results in dilatation of sylvian
fissures (not true arachnoid cysts) and high T2/FLAIR signal in basal
ganglia, hemispheric white matter, substantia nigra, and cerebellar
dentate nuclei.
729
Other rare aminoacidopathies include methylmalonic acidemia
(symmetrical involvement of globus pallidi), nonketotic
hyperglycinemia (abnormal signal in tracts that are myelinated at
birth), and oculocerebrorenal (Lowe’s) syndrome.
FIGURE 16-1. Axial DWI shows high signal in periventricular regions,

along optic radiations and in corpus callosum in an adult with
phenylketonuria.
730
FIGURE 16-2. Axial T2, in a different patient, with glutaric aciduria type
1 shows wide sylvian fissures and abnormal high signal in basal ganglia
bilaterally.
731
FIGURE 16-3. Axial DWI, in a different patient, shows high signal in
midbrain and corticospinal tracts in a patient with maple syrup urine
disease. (Case courtesy A. Vidal, Chile.)
732
FIGURE 16-4. Axial DWI in the same patient showing high signal in the
posterior corticospinal tracts (all abnormalities had restricted ADC values).
SUGGESTED READINGS
Cakmakci H, Pekcevik Y, Yis U, et al. Diagnostic value of proton MR
spectroscopy and diffusion-weighted MR imaging in childhood inherited
neurometabolic brain diseases and review of the literature. Eur J Radiol
2010;74:e161–171.
Manara R, Burlina AP, Citton V, et al. Brain MRI diffusion-weighted imaging in
733
patients with classical phenylketonuria. Neuroradiology 2009;51:803–812.
Cerebral Calcifications
Key Facts
Most cerebral calcifications are found in the basal ganglia, are
idiopathic, and are of no clinical significance.
Idiopathic basal ganglia calcifications occur after 10 years of age and
tend to be symmetrical.
Hypoparathyroidism, pseudohypoparathyroidism, and
hyperparathyroidism can all produce intracranial calcifications (basal
ganglia, cerebellum, thalami, white matter).
Other causes for symmetrical basal ganglia calcifications are Fahr
disease (familial idiopathic cerebral ferrocalcinosis),
postinflammatory, postanoxia, postradiation and chemotherapy
(methotrexate), AIDS in newborns, Cockayne disease, and Aicardi-
Goutières syndrome.
Remember to look for calcifications elsewhere (scalp, parotid glands,
meninges, muscles) that imply a systemic disorder of calcium
metabolism. Progressive dural calcifications may be seen in
nephrogenic systemic sclerosis.
734
FIGURE 16-5. Axial CT shows calcifications in basal ganglia, thalami,
and subcortical white matter (especially in frontal lobes).
735
FIGURE 16-6. Axial CT, in the same patient, shows calcifications in
cerebellum. This patient had hyperparathyroidism.
736
FIGURE 16-7. Axial noncontrast CT in a different patient with Fahr
disease shows prominent calcifications in the basal ganglia, white matter,
and cortex.
737
FIGURE 16-8. Axial CT in a different patient with history of anoxia
shows calcifications in the thalami and low density in the lentiform nuclei
due to prior injury.
SUGGESTED READINGS
Livingston JH, Stivaros S, Warren D, et al. Intracranial calcification in childhood:
a review of aetiologies and recognizable phenotypes. Dev Med Child Neurol
2014;56:612–626.
738
Whitehead MT, Oh C, Raju A, et al. Physiologic pineal region, choroid plexus,
and dural calcifications in the first decade of life. AJNR Am J Neuroradiol
2015;36:575–580.
Mitochondrial Disorders
Key Facts
Disorders due to abnormally functioning mitochondria lead to a
defective oxidative respiratory cycle, which produces accumulation of
lactic acid.
Involve the CNS and smooth muscle tissue.
MELAS is characterized by mitochondrial myopathy,
encephalopathy, lactic acidosis, and strokes and produces large
cerebral infarctions involving both white (parieto occipital) and gray
(deep nuclei) matter.
MERRF is characterized by myoclonic epilepsy with ragged red
fibers; MRI findings are similar to those of MELAS.
Leigh disease is an X-linked, subacute necrotizing
encephalomyelopathy characterized by deficiencies in pyruvate
dehydrogenase and cytochrome c oxidase; MRI shows preferential
and symmetrical involvement of deep gray matter nuclei (basal
ganglia, thalami, periaqueductal gray matter, and brainstem) in these
patients (rarely the cortex or white matter may also be affected).
Menke Kinky Hair syndrome is characterized by abnormally large
and dysfunctional mitochondria, severe copper deficiency, cerebral
atrophy, large and tortuous intracranial arteries, abnormal high
T2/FLAIR signal in white matter, and sometimes high T1 signal in
basal ganglia.
MRS may show lactate in all of these disorders.
With involvement of lentiform nuclei bilaterally remember to include
glutaric aciduria type 2 in the differential diagnosis.
739
FIGURE 16-9. Axial T2 shows bright and swollen posterior left lentiform
nucleus in a patient with MELAS.
740
FIGURE 16-10. Corresponding axial DWI shows high signal in left
lentiform nucleus and also anterior to it. Long TE MRS (inset) shows
inverted lactate peak (arrow) and low NAA.
741
FIGURE 16-11. Axial T2 in a different patient with MELAS shows high
signal in basal ganglia, predominantly laterally and atrophic and bright
occipital white matter.
742
FIGURE 16-12. Axial T2, in a different MELAS patient, shows high
signal in globi pallidi and thalami.
743
FIGURE 16-13. MRS, long TE, from the same patient shown in Figure
16-12, shows inverted lactate peak.
744
FIGURE 16-14. Coronal T2 in a patient with Leigh disease shows
symmetrically increased signal in basal ganglia, brain stem, and white
matter bilaterally.
SUGGESTED READINGS
Finsterer J, Zarrouk Mahjoub S. Leukoencephalopathies in mitochondrial
disorders: clinical and MRI findings. J Neuroimaging 2012;22:e1–e11.
Ishak GE, Poliakov AV, Poliachik SL, et al. Tract-based spatial statistical
analysis of diffusion tensor imaging in pediatric patients with mitochondrial
disease: widespread reduction in fractional anisotropy of white matter tracts.
745
Key Facts
Inherited lysosomal storage disorders characterized by deficiencies in
metabolism of heparan, dermatan, and keratan sulfate.
Disorders include Hurler (IH), Hunter (II), Sanfilippo (III A-D),
Morquio (IV A-D), Maroteaux-Lamy (VI), Scheie (IS), and Sly
syndrome.
All mucopolysaccharidoses are autosomal recessive except Hunter
which is X-linked.
All mucopolysaccharidoses involve the CNS and musculoskeletal
system.
Findings are usually nonspecific and range from mild-to-severe white
matter abnormalities, communicating hydrocephalus, initial
macrocephaly (all are microcephalic chronically), cerebral atrophy,
dilatation of perivascular spaces, thick skull, thick dura, arachnoid
cysts, mega cisterna magna, macrocerebellum (types I and II)
abnormal odontoid, and thick posterior longitudinal ligament in spine
(particularly cervical region).
MRS: mildly high choline, low NAA, and peak at 3.7 ppm, which
may be related to sugars that are a part of mucopolysaccharides.
Main differential diagnosis: tuberous sclerosis, Canavan, Lowe
disease.
746
FIGURE 16-15. Axial FLAIR image in a patient with Hurler disease
shows increased signal throughout white matter and dilated perivascular
spaces.
747
FIGURE 16-16. Parasagittal T1, in the same patient, shows dilated
perivascular spaces in corpus callosum and ventricular dilatation.
748
FIGURE 16-17. Sagittal T2 in a different patient with Hunter syndrome
shows increased signal in the white matter and numerous dilated
perivascular spaces.
749
FIGURE 16-18. Coronal T2, in a different patient with Hunter syndrome,
shows increased white matter signal, dilated perivascular spaces in
subcortical white matter and basal ganglia, hydrocephalus, thick skull, and
mega cisterna magna.
SUGGESTED READINGS
750
Matheus MG, Castillo M, Smith JK, et al. Brain MRI findings in patients with
mucopolysaccharidosis types I and II and mild clinical presentation.
Neuroradiology 2004;46:666–672.
Zafeiriou DI, Batzios SP. Brain and spinal MR imaging findings in
mucopolysaccharidoses: a review. AJNR Am J Neuroradiol 2013;34:5–13.
Wernicke Encephalopathy
Key Facts
Acute, ataxia, oculomotor dysfunction, confusion (nearly 50% not
related to alcohol but seen in cancer and bariatric surgery patients,
diets).
MRI shows bilateral and symmetric high T2/FLAIR signal in medial
thalamic, tectal, and periaqueductal gray matter and contrast
enhancement of mammillary bodies (more frequent in alcoholics).
Uncommon findings include symmetric signal hyperintensity in the
cerebellum including hemispheres, dentate nuclei, and vermis.
Fornix, caudate nuclei heads, red nuclei, and corpus callosum may
also be rarely involved. High DWI with low ADC signal may be
present due to cytotoxic edema.
MRS shows low metabolites, but high lactate is occasionally present.
In the nonalcoholic variant of the disease, the findings are more
severe and may involve the cerebellar and cerebral cortex.
Differential diagnosis: neuromyelitis optica, mitochondrial disease.
Korsakoff psychosis has no imaging findings.
751
FIGURE 16-19. Axial FLAIR shows symmetric high signal in the
thalami.
752
FIGURE 16-20. Axial FLAIR in the same patient shows increased signal
involving the periaqueductal gray (arrow).
753
FIGURE 16-21. Axial FLAIR, in a different patient, shows high signal in
the floor of the fourth ventricle.
754
FIGURE 16-22. Postcontrast T1, in a different patient, shows avid
enhancement of the mammillary bodies (arrow) and periaqueductal
gray/tectum (arrowhead).
755
cortex of frontal lobes.
756
FIGURE 16-24. Axial T2 in a different patient shows high signal in
cerebellar cortex.
SUGGESTED READINGS
Manzo G, De Gennaro A, Cozzolino A, et al. MR imaging findings in alcoholic
and nonalcoholic acute Wernicke's encephalopathy: a review. Biomed Res Int
2014;2014:503596.
Zuccoli G, Santa Cruz D, Bertolini M, et al. MR imaging findings in 56 patients
with Wernicke encephalopathy: nonalcoholics may differ from alcoholics.
Wilson Disease (Hepatolenticular
757
Degeneration)
Key Facts
Autosomal recessive disorder characterized by deficient
ceruloplasmin due to mutations in the ATP7B gene, which leads to
the accumulation of copper in liver, brain, corneas (Kayser-Fleischer
rings—present in nearly all patients with neurologic disease—and
sunflower cataracts), bones, and kidneys.
Most cases are diagnosed in late adolescence. Hepatic disease nearly
always precedes neurological symptoms.
Findings on imaging studies are symmetrical and reflect neuronal
loss, spongy degeneration, and cavitation and affect predominantly
the basal ganglia (particularly the putamen) and the midbrain. All
patients with classic disease and symptoms show brain abnormalities.
MRI: high T2/FLAIR signal in basal ganglia (particularly lateral
putamen) and claustra, high signal in midbrain (particularly tectum)
with preservation of low T2 signal in red nuclei and substantia nigra
is typical (“Panda” face sign). DWI may show early restriction of
diffusion in these areas.
MRS: all metabolites are low in affected regions.
Main differential diagnosis: mitochondrial diseases, CO poisoning
and anoxia, CJD, glutaric aciduria, viral encephalitis.
758
FIGURE 16-25. Axial T2 shows high signal in basal ganglia (particularly
in lateral putamen) and in subinsular regions.
759
FIGURE 16-26. Axial FLAIR, in a different patient, shows high putamen
and thalamic signal intensity.
760
FIGURE 16-27. Axial T2, in the same patient, shows high signal in
midbrain.
761
FIGURE 16-28. Axial FLAIR, in the same patient, shows high signal
outlining the red nuclei and substantia nigra giving rise to the Panda face
sign.
SUGGESTED READINGS
Kim TJ, Kim IO, Kim WS, et al. MR imaging of the brain in Wilson disease of
childhood: findings before and after treatment with clinical correlation. AJNR
762
Prashanth LK, Sinha S, Taly AB, et al. Do MRI features distinguish Wilson’s
disease from other early onset extrapyramidal disorders? An analysis of 100
cases. Mov Disord 2010;25:672–678.
763
CHAPTER 17
Degenerative and
Iatrogenic Disorders
Alzheimer Disease
Key Facts
Most common degenerative brain disorder and most common cause
of dementia (sixth cause of death in the United States), found more
often in individuals >50 years of age.
Symptoms: forgetfulness, language difficulties, conceptual loss,
orientation abnormalities, and apraxia.
Characterized by the presence of neurofibrillary tangles and amyloid
that result in the death of neurons leading to dementia.
MRI: diffuse atrophy, marked atrophy of the hippocampi, areas of
increased T2/FLAIR signal intensity in the white matter (more than
expected for age), increased T2/FLAIR signal intensity in the cortex
of the temporal lobes, dilated perivascular spaces, and increased
deposition of iron in parietal regions and basal ganglia. Increased
incidence of lobar microbleeds, which are best seen on SWI
(probably related to amyloid).
PET and SPECT, respectively, show frontoparietal hypometabolism
and hypoperfusion. PET amyloid binding agents (11C-PiB and
others) can show plaque deposition and distribution in challenging
cases.
MRS: elevated levels of myoinositol and low NAA throughout the
764
brain.
FIGURE 17-1. Axial T2 shows significant hippocampal atrophy.
765
FIGURE 17-2. Axial T2, in the same patient, shows significant dilatation
of precentral and parietal sulci.
766
FIGURE 17-3. Coronal T2, in a different patient, shows dilated
perivascular spaces bilaterally.
767
FIGURE 17-4. FDG-PET in a different patient demonstrates
hypometabolism of the parietal lobes bilaterally with preservation of the
occipital lobes.
SUGGESTED READINGS
Chiang GC, Cruz Hernandez JC, Kantarci K, et al. Cerebral microbleeds, CSF p-
Tau, and cognitive decline: significance of anatomic distribution. AJNR Am J
Neuroradiol 2015;36:1635–1641.
Walhovd KB, Fjell AM, Brewer J, et al. Combining MR imaging, positron-
emission tomography, and CSF biomarkers in the diagnosis and prognosis of
768
Alzheimer disease. AJNR Am J Neuroradiol 2010;31:347–354.
Amyotrophic Lateral Sclerosis

Key Facts
Most common degenerative motor neuron disease (although very
rare) occurring in patients older than 50 years of age, usually males.
Ninety percent of cases are sporadic.
Symptoms: muscle atrophy, hand and arm weakness, leg spasticity,
and hyperreflexia. A less common bulbar form may affect chewing
and swallowing due to brain stem involvement.
Most patients die within 6 years of the onset of the disease.
Histologically, there is degeneration of neurons in the central gray
matter and in the ventral gray matter horns of the spinal cord, leads to
Wallerian degeneration of the corticospinal tracts and of the cauda
equina.
Imaging shows atrophy of the frontal lobes including widening of the
central sulcus, increased deposition of iron (low T2 signal intensity,
may be normal with aging, better seen using SWI) in the motor strip,
and increased signal intensity in the posterior limbs of the internal
capsules (more obvious on FLAIR). DTI may show decreased FA and
increased MD in white matter.
MRS: high choline and myoinositol in precentral gyrus, low NAA;
increased glutamate/glutamine in medulla.
Despite abnormal anterior horns in spinal cord, spine MRI studies are
generally normal.
Early on it may be unilateral.
Main differential diagnosis: Wallerian degeneration.
769
FIGURE 17-5. Axial FLAIR shows symmetrical high signal in both
corticospinal tracts.
770
the posterior limbs of the internal capsules bilaterally (arrows).
771
FIGURE 17-7. Coronal T2, in the same patient, shows high signal
extending along the corticospinal tracts (arrows).
772
FIGURE 17-8. Axial T2 in a different patient demonstrates increased
signal involving the corticospinal tracts in the pons (arrow).
SUGGESTED READINGS
Agosta F, Chio A, Cosottini M, et al. The present and the future of neuroimaging
in amyotrophic lateral sclerosis. AJNR Am J Neuroradiol 2010;31:1769–1777.
Chio A, Pagani M, Agosta F, et al. Neuroimaging in amyotrophic lateral sclerosis:
insights into structural and functional changes. Lancet Neurol
2014;13:1228–1240.
773
Carbon Monoxide and Methanol
Intoxication
Key Facts
Carbon monoxide intoxication:
Results in necrosis of the globi pallidi (but also affects the
subcortical white matter, hippocampi, and cortex) and diffuse
brain swelling. Acutely, these regions may show restricted
diffusion on DWI.
Symptoms may be acute (headache, dizziness, alteration of
consciousness, impaired vision, seizures, coma, and death) or
chronic (mental deterioration, gait abnormalities, fecal
incontinence, and mutism).
Patients generally die from cardiac arrhythmias.
Methanol intoxication:
Results in necrosis of the retina and optic disc, cerebral edema,
hemorrhagic necrosis of the lateral portion of the putamen and
gray/white frontal matters.
Clinically, methanol intoxication produces blindness, headaches,
nausea, vomiting, dyspnea, and abdominal pain.
Main differential diagnosis: diffuse anoxia, Wilson disease, CJD,
mitochondrial diseases, and viral encephalitis.
774
FIGURE 17-9. Axial CT in a patient presenting with carbon monoxide
intoxication shows hypodensity in the globi pallidi bilaterally (arrow).
775
FIGURE 17-10. Axial DWI in a different patient shows bright signal in
the globi pallidi secondary to carbon monoxide intoxication.
776
FIGURE 17-11. Axial T2 after methanol ingestion shows swollen and
bright basal ganglia and increased signal in most of the white matter.
777
FIGURE 17-12. Axial CT, in the same patient, shows that the basal
ganglia are very swollen and of low density. White matter abnormalities
are also seen as hypodense zones.
SUGGESTED READINGS
Beppu T. The role of MR imaging in assessment of brain damage from carbon
monoxide poisoning: a review of the literature. AJNR Am J Neuroradiol
2014;35:625–631.
Blanco M, Casado R, Vazquez F, et al. CT and MR imaging findings in methanol
intoxication. AJNR Am J Neuroradiol 2006;27:452–454.
778
Hippocampal Sclerosis
Key Facts
Most common cause of medically intractable complex partial
seizures, seen in 60% to 80% of patients with complex partial
seizures. Most common predisposing factor: febrile seizures in
infancy.
Hippocampal sclerosis is pathologically characterized by neuronal
cell loss (30% to 50%) in the hilar region and cornu ammonis (CA)
fields 1, 3, and 4 of hippocampal gyrus, with relative sparing of field
2.
Loss of volume is more common (>80%) than high signal intensity on
T2WI/FLAIR, which is seen in 10% to 70% of cases.
Ancillary findings: atrophy of ipsilateral column of fornix and
mamillary body, loss of undulations of pes hippocampus, ipsilateral
temporal lobe volume loss and blurring of gray–white matter junction
and dilatation of temporal horn of lateral ventricle.
Bilateral in 10% to 15% of patients.
MRS: low NAA and mildly elevated myoinositol. Occasionally,
choline may be also minimally elevated. Acutely, lactate may be
seen.
FDG-PET may show focal hypometabolism.
70% to 90% of patients will experience resolution/improvement of
seizures after temporal lobe resection.
Main differential diagnosis (during or immediately after seizures
when hippocampus may be swollen, needs imaging two to three
months later to document resolution): tumor (astrocytoma), limbic
encephalitis.
779
FIGURE 17-13. Coronal FLAIR shows atrophy and increased signal in
the right hippocampus (arrow) and atrophy of the right fornix
(arrowhead).
780
FIGURE 17-14. Coronal T2, in the same patient, also shows atrophy of
the right mammillary body (arrow).
781
FIGURE 17-15. Axial FLAIR, in the same patient, shows atrophy and
increased signal in the right hippocampus (arrow).
782
FIGURE 17-16. Corresponding FDG-PET demonstrates interictal
hypometabolism in the right hippocampus.
783
FIGURE 17-17. Coronal FLAIR in a different patient presenting acutely
with seizures demonstrates swelling and increased signal in the left
hippocampus (arrow).
784
FIGURE 17-18. Follow-up coronal T2 3 months later in the same patient
demonstrates marked volume loss in the left hippocampus.
SUGGESTED READING
Van Paesschen W. Qualitative and quantitative imaging of the hippocampus in
mesial temporal lobe epilepsy with hippocampal sclerosis. Neuroimaging Clin
N Am 2004;14:373–400, vii.
Huntington Disease (Chorea)
785
Key Facts
Rare, progressive autosomal dominant (complete penetrance) disorder
occurring during the fourth to fifth decades of life, caused by
trinucleotide (CAG) repeat expansion in the huntingtin gene
(chromosome 4).
Symptoms: choreoathetosis (due to loss of GABAergic neurons),
rigidity, dementia, and emotional lability. Manifestations are probably
due to toxicity of the mutant huntingtin protein. Chorea is absent in
juvenile Huntington disease (10% of cases, occurring before 20 years
of age), which may present with seizures and parkinsonism.
MRI: atrophy of caudate nuclei (particularly their heads) and
putamen; diffuse atrophy is also present. Occasionally, the basal
ganglia show high T2/FLAIR signal. Degree of basal ganglia atrophy
correlates with psychomotor dysfunction.
Increase iron deposition in basal ganglia may also be seen as low
signal on T2/FLAIR.
MRS: low NAA and moderately high choline (due to gliosis) and
rarely, lactate.
786
FIGURE 17-19. Axial CT shows ballooning of the frontal horns of the
lateral ventricles due to atrophy of the heads of the caudate nuclei.
787
FIGURE 17-20. Axial T2 shows atrophy of caudate nuclei heads and
prominent sulci in both frontal lobes.
788
FIGURE 17-21. Coronal T1 shows straightening of lateral walls of the
frontal horns due to atrophy of the caudate nuclei heads.
789
FIGURE 17-22. Axial FLAIR in a different patient demonstrates atrophy
of the putamen and caudate nuclei heads bilaterally.
790
both putamina and residual caudate nuclei heads.
SUGGESTED READINGS
Hobbs NZ, Barnes J, Frost C, et al. Onset and progression of pathologic atrophy
in Huntington disease: a longitudinal MR imaging study. AJNR Am J
Neuroradiol 2010;31:1036–1041.
Mahalingam S, Levy LM. Genetics of Huntington disease. AJNR Am J
Neuroradiol 2014;35:1070–1072.
791
Nonketotic Hyperglycemia
Key Facts
Disease of adults (but may be seen at any age), particularly diabetics
who present with sudden onset of extrapyramidal symptoms that may
be unilateral (with contralateral hemiballismus and hemichorea) or
occasionally bilateral.
Glucose serum levels are generally above 300 mg/dL (which also
explains confusion that is common in these patients) or HbA1c higher
than 13%.
CT: increased density in striatum (caudate nucleus head + putamen),
not due to overt hemorrhage (cannot exclude microbleeds), no
contrast enhancement.
MRI: increased T1 signal in striatum (particularly the posterior
putamen), generally no corresponding T2 abnormality or only mildly
increased signal, DWI may show restriction of diffusion in this
region. Reversible subcortical white matter T2 hypointensity may be
seen (possibly related to free radical accumulation from seizures).
MRS: low NAA, high glutamine/glutamate, lactate, lipids.
Cause of finding is uncertain but may be due to infarction,
microbleeds, manganese deposition, or presence of reactive
gemistocytes.
Findings may resolve completely.
Main differential diagnosis: calcifications (hydrated calcium),
hemorrhage.
792
FIGURE 17-24. Axial noncontrast CT shows high density in the right
lentiform nucleus (arrow).
793
FIGURE 17-25. Axial noncontrast CT in a different patient shows high
density involving the left lentiform nucleus and caudate nucleus head.
794
FIGURE 17-26. Corresponding noncontrast T1 shows increased signal in
the same structures.
795
FIGURE 17-27. Long TE MRS in a different patient shows low NAA
(arrow) and high glutamine/ glutamate (arrowhead).
SUGGESTED READINGS
Bathla G, Policeni B, Agarwal A. Neuroimaging in patients with abnormal blood
glucose levels. AJNR Am J Neuroradiol 2014;35:833–840.
Wintermark M, Fischbein NJ, Mukherjee P, et al. Unilateral putaminal CT, MR,
and diffusion abnormalities secondary to nonketotic hyperglycemia in the
setting of acute neurologic symptoms mimicking stroke. AJNR Am J
Neuroradiol 2004;25:975–976.
796
Intracranial Hypotension
Key Facts
Characterized by chronic headaches, particularly in women during
third to fourth decades of life.
Due to chronic leak of CSF leading to low intracranial pressure.
Etiologies: idiopathic, prior surgery and trauma, prior lumbar
puncture, spontaneous dural tear (such as a nerve root “cyst,” more
frequently in patients with connective tissue disorders), dehydration.
MRI: “sagging” midbrain (decreased size of suprasellar,
interpeduncular and prepontine cisterns; decreased mamillopontine
distance and pontomesencephalic angle), cerebellar tonsillar and
rarely uncal herniation, small lateral ventricles, chronic subdural
collections (may be complicated by subdural hematomas); after
contrast administration significant enhancement in thickened dura
(probably most common cause of diffuse dural enhancement), venous
sinuses, dural surfaces (falx, tentorium, clivus), engorged pituitary
gland, spinal epidural space (can rarely result in myelopathy). Dural
venous sinuses are engorged with a round (as opposed to triangular)
shape. Some patients may show T2 FLAIR hyperintensity in basal
ganglia or brain stem.
May need to do MRI with T2 images of entire spine to look for leaks
and/or nerve root diverticula.
May need myelogram w/radionuclide to find site of CSF leak.
Complications: venous thrombosis and infarctions particularly of
cerebellum and medulla.
797
FIGURE 17-28. Midsagittal postcontrast T1 shows brain stem sagging
with small basilar cisterns and fourth ventricle, flattening of the pons,
engorged pituitary gland, and engorged dural venous sinuses with a
rounded appearance.
798
FIGURE 17-29. Coronal postcontrast T1, in the same patient, shows
collapse of the left lateral ventricle and uncal herniation bilaterally
(arrows).
799
FIGURE 17-30. Midsagittal postcontrast T1 1 week after treatment shows
return to a normal appearance with resolution of sagging and venous
engorgement.
800
diffuse dural thickening and enhancement as well as a rounded appearance
of the venous sinuses.
801
FIGURE 17-32. Axial T2, in a different patient, shows bilateral small
subdural fluid collections.
802
FIGURE 17-33. Axial FLAIR, in a different patient, shows bilateral
inferior cerebellar infarctions as complications of intracranial hypotension.
SUGGESTED READINGS
Schievink WI, Maya MM, Louy C, et al. Diagnostic criteria for spontaneous
spinal CSF leaks and intracranial hypotension. AJNR Am J Neuroradiol
2008;29:853–856.
Urbach H. Intracranial hypotension: clinical presentation, imaging findings, and
imaging-guided therapy. Curr Opin Neurol 2014;27:414–424.
803
Liver Insufficiency and Total Parenteral
Nutrition
Key Facts
Both may cause increased signal intensity in the basal ganglia on T1
while they appear normal on T2WI and have no corresponding
abnormalities on CT. Acute encephalopathy can present with diffuse
cortical edema with or without restricted diffusion.
Increased T1 signal intensity is believed to be related to deposition of
paramagnetic cations particularly manganese (probably from
increased blood flow to deep gray matter in liver disease).
Basal ganglia may become normal again at about 3 months after liver
transplantation or discontinuation of total parenteral nutrition.
Liver insufficiency may also cause nonspecific white matter changes
(that also resolve when function recovers).
Normal basal ganglia calcifications are occasionally bright on T1 due
to presence of hydrated calcium and are not related to liver failure or
parenteral nutrition.
T1 with magnetization transfer normally produces a slight increase in
signal intensity of the basal ganglia (due to a drop of signal intensity
of surrounding tissues).
In liver disease with hyperammonemia, the gray matter is diffusely
swollen and may have restricted diffusion.
Main differential diagnosis (when cortex is diffusely involved):
anoxia, CJD.
804
FIGURE 17-34. Axial noncontrast T1 shows increased signal intensity in
the basal ganglia bilaterally.
805
FIGURE 17-35. In a different patient, midsagittal T1 shows increased
signal intensity in dorsal brainstem, midbrain, and adenohypophysis.
806
FIGURE 17-36. Axial FLAIR in a different patient with acute
encephalopathy demonstrates increased signal in the cerebral cortex and
thalami bilaterally.
807
FIGURE 17-37. Corresponding DWI shows increased signal due to
cytotoxic edema.
SUGGESTED READINGS
Rovira A, Alonso J, Cordoba J. MR imaging findings in hepatic encephalopathy.
Zhang XD, Zhang LJ, Wu SY, et al. Multimodality magnetic resonance imaging
in hepatic encephalopathy: an update. World J Gastroenterol
2014;20:11262–11272.
808
Multisystem Atrophy
Key Facts
Relentless progressive neurodegenerative disorder with pyramidal,
extrapyramidal, and autonomic symptoms.
It is a sporadic adult-onset progressive neurodegenerative disorder,
characterized by degeneration of basal ganglia and
olivopontocerebellar system.
Three clinically overlapping subtypes: olivopontocerebellar atrophy
(now termed MSA-C, with predominant cerebellar ataxia),
striatonigral degeneration (MSA-P, with predominant parkinsonism),
and Shy-Drager syndrome (autonomic dysfunction that can be present
in both MSA-P and MSA-C).
More common in patients older than 60 years.
MRI shows severe atrophy of brainstem/cerebellum, decreased
thickness of middle cerebellar peduncles (≤8 mm on sagittal images),
high T2/FLAIR signal in pons (at base may have a “hot cross bun”
appearance, relatively specific but only present in about 50% of
cases), increased iron deposition in basal ganglia (dark T2),
sometimes putaminal T1 hyperintensity, and generalized brain
atrophy.
MRS shows markedly low NAA.
809
FIGURE 17-38. Midsagittal T1 shows severe atrophy of brainstem and
cerebellum but relatively normal-appearing cerebrum.
810
FIGURE 17-39. Axial T2, in the same patient, shows the atrophy of the
midbrain.
811
FIGURE 17-40. Midsagittal T1 in a different patient shows a flattened
appearance of the pons and medulla and cerebellar volume loss.
812
FIGURE 17-41. Axial FLAIR in the same patient shows cruciform
hyperintensity in the pons (the “hot cross bun” sign) due to degeneration of
transverse pontocerebellar fibers.
813
FIGURE 17-42. Sagittal T1 in a different patient shows decreased width
of the middle cerebellar peduncle (arrow, in this case measuring 7 mm).
SUGGESTED READING
Matsusue E, Fujii S, Kanasaki Y, et al. Cerebellar lesions in multiple system
atrophy: postmortem MR imaging-pathologic correlations. AJNR Am J
Neuroradiol 2009;30:1725–1730.
Osmotic Demyelination Syndrome

814
Key Facts
Most commonly secondary to rapid correction of hyponatremia (<115
mmol/L) in chronic alcoholics but may also be seen with advanced
liver disease, many causes of malnutrition, burns, and paraneoplastic
syndromes.
Most common site is pons (50%) followed by extrapontine sites
(50%): midbrain, thalami, basal ganglia, and white matter including
the corpus callosum.
Symptoms include lethargy, swallowing problems, and progressive
quadraparesis. Mortality is high.
Imaging findings usually lag behind clinical symptoms and may
resolve slowly. Affected areas show symmetric high T2/FLAIR and
DWI signal intensity (may be earliest technique to demonstrate
abnormalities).
Marchiafava Bignami is a type of osmotic myelinolysis affecting the
corpus callosum (particularly centrally) and the white matter
(especially frontal lobes); cortex may be occasionally affected.
Main differential diagnosis (for pontine lesion): infarct,
demyelinating disease, tumor.
815
FIGURE 17-43. Axial T2 image shows central pontine bright lesion
typically sparing corticospinal tracts. Note the rim of normal-appearing
tissue surrounding the lesion. (Case courtesy A. Vidal, Chile.)
816
peripherally enhancing triangular lesion in the central pons.
817
FIGURE 17-45. Axial FLAIR, in the same patient, shows symmetric high
signal intensity in the basal ganglia.
818
FIGURE 17-46. Axial T2, in a different patient, shows presumed bright
areas of extrapontine myelinolysis in mid-thalami.
819
FIGURE 17-47. Axial CT in a different patient with chronic alcoholism
shows the sequela of remote pontine myelinolysis.
820
FIGURE 17-48. Midsagittal T2, in a different patient, shows high signal
lesion in the posterior corpus callosum in Marchiafava Bignami.
SUGGESTED READINGS
Alleman AM. Osmotic demyelination syndrome: central pontine myelinolysis and
extrapontine myelinolysis. Semin Ultrasound CT MR 2014;35:153–159.
Arbelaez A, Pajon A, Castillo M. Acute Marchiafava-Bignami disease: MR
findings in two patients. AJNR Am J Neuroradiol 2003;24:1955–1957.
Pantothenate Kinase Deficiency

(Hallervorden-Spatz Syndrome)
Key Facts
821
Rare autosomal recessive disorder, 50% familial (mutation in PANK2
gene: 20p13), deficiency of pantothenate kinase leading to
accumulation of cysteine, free radicals, and iron deposition.
Clinically evident during the first two decades of life and
characterized by progressive dystonia, oromandibular abnormalities,
mental deterioration, pyramidal signs, and retinal degeneration.
Types: classic or early onset (rapidly progressive) and atypical or late
(slowly progressive), latter type is rare.
Pathologic features: vacuolization and deposition of iron in globi
pallidi and degeneration of the pars reticulata of the substantia nigra
with surrounding gliosis.
MRI (T2/FLAIR) shows typical “eye-of-the-tiger” abnormality
involving the pallidum, which reflects increased iron deposition,
demyelination, and reactive gliosis. All patients with classic type
show MR abnormalities. Iron deposition is best seen on SWI and with
higher field magnets.
MRS: low NAA and high myoinositol.
Main differential diagnosis: anoxia, CO intoxication, sequelae of
kernicterus.
822
FIGURE 17-49. Axial CT shows calcification in medial globi pallidi.
823
FIGURE 17-50. Axial FLAIR, in a different patient, shows high signal
(gliosis) surrounded by low signal (iron deposition) in globi pallidus (“eye
of the tiger” sign).
824
FIGURE 17-51. Axial T2, in a different patient, shows bright internal
globi pallidi and surrounding low signal from iron deposition.
825
FIGURE 17-52. Axial FLAIR, in a different patient, demonstrates bright
signal in the globi pallidi but little iron accumulation (probably an earlier
stage).
SUGGESTED READINGS
Awasthi R, Gupta RK, Trivedi R, et al. Diffusion tensor MR imaging in children
with pantothenate kinase-associated neurodegeneration with brain iron
accumulation and their siblings. AJNR Am J Neuroradiol 2010;31:442–447.
Kruer MC, Boddaert N, Schneider SA, et al. Neuroimaging features of
neurodegeneration with brain iron accumulation. AJNR Am J Neuroradiol
826
2012;33:407–414.
Pseudotumor Cerebri
Key Facts
Most common in obese women of childbearing age; precise etiology
is unknown.
Symptoms are those of increased intracranial pressure (similar to
those caused by a true tumor), most commonly headache and visual
deficits.
Brain imaging is usually normal although minor changes in
subarachnoid space volumes (increased and decreased) and
ventricular size (small or large) have been reported. There is a 10% to
20% incidence of Chiari I–type imaging findings with tonsillar
ectopia, although a causal relationship has not been established.
May be accompanied by an empty or partially empty sella turcica and
enlarged Meckel caves.
Orbits may show increased fluid around optic nerves, flattening of the
posterior globes, and/or protrusion of the optic nerve heads into the
vitrei.
There may be stenosis or thrombosis of the transverse sinuses.
827
FIGURE 17-53. Axial T2 shows papilledema with protrusion of the optic
nerve heads and distention of the optic nerve sheaths.
828
FIGURE 17-54. Midsagittal T1 in a different patient shows nearly empty
sella.
829
FIGURE 17-55. Axial T2, in a different patient, shows marked
enlargement of the CSF-filled Meckel caves.
830
FIGURE 17-56. Postcontrast MRV, in a different patient, demonstrates
stenoses involving the transverse sinuses bilaterally (arrows).
831
FIGURE 17-57. Axial T2, in a different patient after right optic nerve
sheath fenestration for treatment of pseudotumor cerebri, shows fluid-
filled pouch posterior to globe.
SUGGESTED READING
Degnan AJ, Levy LM. Pseudotumor cerebri: brief review of clinical syndrome
and imaging findings. AJNR Am J Neuroradiol 2011;32:1986–1993.
Radiation Injury
Key Facts
Acute type (<3 months) manifests during treatment and is probably
832
related to edema, responds to steroids, and resolves. May happen
earlier after stereotactic radiosurgery.
Late type (1 to 10 years, doses >50 Gy) may be associated with white
matter demyelination, cavernous malformations and capillary
telangiectasias, meningiomas, sarcomas, gliomas, mineralizing
angiopathy, and vasculitis; is irreversible, progressive, and
occasionally fatal.
“Pseudoprogression” in gliomas is most commonly seen with
concomitant radiation/temozolomide and is usually asymptomatic.
Histologically, radiation injury is related to thickening of endothelium
of small- and medium-sized vessels leading to cerebral infarcts.
Focal late radiation necrosis presents as seizures and is
indistinguishable from residual or recurrent tumor by imaging (70%
occur during the first 2 years).
MRS: no viable metabolites, only lipids and lactate.
Perfusion MRI: very low rCBV and rCBF.
DTI: complete loss of fractional anisotropy.
SWI may show small micro lesions whose etiology is uncertain
and probably asymptomic.
Necrotizing leukoencephalopathy is rare and results from a
combination of the treatment with radiation and chemotherapy
(mainly methotrexate).
833
FIGURE 17-58. Axial FLAIR in a patient 2 years after whole brain
radiation demonstrates extensive confluent white matter hyperintensity and
volume loss.
834
irregular enhancing lesion in the left temporal lobe (arrow) with a so-
called “cut green pepper” appearance.
835
FIGURE 17-60. Corresponding rCBV MR maps show lack of perfusion
in this lesion that proved to be radiation necrosis on biopsy.
836
enhancing lesion in callosal splenium indistinguishable from high-grade
glioma.
837
FIGURE 17-62. Long TE MRS shows basically no viable metabolites
only presence of lipids/lactate corresponding to necrosis.
838
FIGURE 17-63. Axial SWI in a different patient several years after
whole-brain radiation shows numerous dark foci corresponding to
cavernous malformations.
SUGGESTED READINGS
Shah R, Vattoth S, Jacob R, et al. Radiation necrosis in the brain: imaging
features and differentiation from tumor recurrence. Radiographics
2012;32:1343–1359.
Varon D, Simons M, Chiang F, et al. Brain radiation-related black dots on
susceptibility-weighted imaging. Neuroradiol J 2014;27:445–451.
839
CHAPTER 18
Congenital
Malformations
Agenesis of Corpus Callosum

Key Facts
Corpus callosum forms from anterior to posterior (except for the
rostrum which forms last) and myelinates from posterior to anterior
generally by 10 months of age.
Complete agenesis is accompanied by absent cingulate gyrus and
sulcus, high riding third ventricle, bundles of Probst (thought to
represent white matter that would have crossed the midline), dorsal or
occasionally rostral interhemispheric arachnoid cysts (one third of
patients), and colpocephaly (dilatation of atria and occipital horns of
lateral ventricles). White matter volume is commonly reduced, and
the anterior commissure may be abnormal in size.
Agenesis may be accompanied by isolated dilatation of the temporal
horns of the lateral ventricles (does not imply early hydrocephalus)
and associated with abnormally shaped hippocampi due to incomplete
rotation.
Dysgenesis may represent a partially absent callosum (commonly the
posterior body/splenium or rostrum) or hypoplasia.
Associated anomalies: Chiari II, neuronal migration disorders,
Dandy-Walker complex, holoprosencephaly, interhemispheric lipoma
(3%, particularly tubulonodular type), migration anomalies, azygous
840
anterior cerebral artery, and abnormalities of optic chiasm and
pituitary gland.
Most patients have mental retardation and seizures but occasionally
may be normal.
FIGURE 18-1. Midsagittal T1 shows partial callosal agenesis with

residual genu (arrow) and radial arrangement of cerebral gyri.
841
FIGURE 18-2. Coronal T1, in the same patient, shows upturned
configuration of the lateral ventricular bodies, subependymal heterotopia
(arrows), and prominent temporal horns with accompanying hippocampal
malrotation.
842
FIGURE 18-3. Axial FLAIR in a different patient demonstrates a parallel
configuration of the lateral ventricles and a septated anterior
interhemispheric cyst (arrow).
843
FIGURE 18-4. Midsagittal T1, in a different patient, shows callosal
agenesis and rostral midline tubulonodular lipoma.
844
FIGURE 18-5. Sagittal T1 in a different patient shows a very small corpus
callosum.
845
FIGURE 18-6. Sagittal T1, in a different patient, demonstrates a diffusely
thinned and dysplastic corpus callosum and absence of the cingulate gyrus.
SUGGESTED READING
Hetts SW, Sherr EH, Chao S, et al. Anomalies of the corpus callosum: an MR
analysis of the phenotypic spectrum of associated malformations. AJR Am J
Roentgenol 2006;187:1343–1348.
Agyria/Pachygyria Complex and Band
846
Heterotopia
Key Facts
Agyria (lissencephaly) refers to a “smooth” brain with no sulcations
(the appearance of the brain is similar to that of a 17-week-old fetus).
May involve brain totally (agyria) or partially (agyria-pachygyria
complex in which some cortical sulci are present).
Lissencephaly type 1 (classic): 40% of all lissencephaly disorders
arise from mutations in LIS1 (chromosome 17, parietooccipital
predominant, may be seen in Miller-Dieker syndrome) and DCX
(“doublecortin,” X-linked, frontal predominant in males and band
heterotopia in females); these have a “four-layer” cortex.
Lissencephaly type 2 (cobblestone): caused by mutations in multiple
genes; due to neuronal overmigration; lumpy–bumpy cortex; may be
seen in Walker Warburg syndrome, Fukuyama congenital muscular
dystrophy, and muscle–eye–brain disease.
Variant lissencephaly: “three-layer” cortex in ARX mutation with
normal cerebellum and hypoplastic pons or “two-layer” lissencephaly
with diffuse brain involvement (no gradient) and marked cerebellar
hypoplasia.
Imaging reveals thick gray matter that contains insufficient neurons;
shallow sylvian fissures (so-called figure-of-eight brain
configuration); lack of sulci, and lateral ventricles are colpocephalic
(dilatation of atria and occipital horns). Laminar or band heterotopia
(double cortex) is seen as a band of gray matter between subcortical
and periventricular white matter. Associated with forebrain
commissural anomalies, particularly corpus callosum.
Patients are hypotonic at birth but develop spasticity and seizures.
847
FIGURE 18-7. Axial T1 image shows complete absence of cortical sulci,
thick gray matter, and large ventricles compatible with lissencephaly
(noncobblestone type).
848
FIGURE 18-8. Axial T1 image, in a different patient, shows thin
pachygyric cortex and band of heterotopic gray matter within the white
matter (double cortex sign). Lateral ventricles are enlarged.
849
FIGURE 18-9. Axial T2 image, in a different patient, shows diffuse band
heterotopia. (Courtesy Catalina Vargas, Bogota, Colombia.)
850
FIGURE 18-10. Axial T1 inversion recovery image in a different patient
demonstrates lissencephaly with a cobblestone pattern.
851
FIGURE 18-11. Axial T2 in a different patient shows frontotemporal
pachygyria and parietooccipital agyria.
852
FIGURE 18-12. Axial T2, in a different patient, shows parietooccipital
predominant lissencephaly.
SUGGESTED READINGS
Abdel Razek AA, Kandell AY, Elsorogy LG, et al. Disorders of cortical
formation: MR imaging features. AJNR Am J Neuroradiol 2009;30:4–11.
Kara S, Jissendi-Tchofo P, Barkovich AJ. Developmental differences of the major
forebrain commissures in lissencephalies. AJNR Am J Neuroradiol
2010;31:1602–1607.
853
Anterior Nasal Masses
Key Facts
The incidence of sincipital encephaloceles in the United States is low
(1:20,000 to 40,000 live births); they are more common in Asia and
Latin America.
More common in boys and always accompanied by hypertelorism.
Encephalocele locations: nasofrontal (40% to 60%), nasoethmoidal
(30%), and nasolateral (also called nasoorbital).
Most sincipital encephaloceles contain nonfunctioning gliotic brain
and are accompanied by complex intracranial malformations
(particularly the larger ones).
Differential diagnosis includes nasal gliomas (brain heterotopias) and
nasal dermoids.
Nasal gliomas are isolated (nonneoplastic) masses of brain trapped in
the prenasal space, nasal dorsum, or glabella; they can have a fibrous
stalk, may grow slightly, enhance after contrast, and often have signal
intensities different than that of brain due to gliosis.
Nasal dermoids may form in the prenasal space or outside of it
anywhere from the tip of the nose to the nasal bones; they have
varying signal intensities and may enhance particularly if infected;
occasionally, they have a fatty nature; they tend to communicate with
the foramen cecum in front of a wide or bifid crista galli.
Dermoids may rarely show restricted diffusion, although this is a
more consistent feature of epidermoids that are more often
paramidline in location and are devoid of dermal appendages.
854
FIGURE 18-13. Midsagittal T1 shows a nasofrontal encephalocele.
855
FIGURE 18-14. Midsagittal T1, in a different patient, shows an anterior
nasoethmoidal encephalocele and an anterior ethmoidal bone defect
(arrow).
856
FIGURE 18-15. Sagittal T2 in a different patient shows a nasal glioma
with mildly heterogeneous signal. This lesion enhanced with contrast (not
shown).
857
nonenhancing cystic lesion that is slightly off midline.
858
FIGURE 18-17. Corresponding DWI image shows bright signal
consistent with restricted diffusion. This was a biopsy proven epidermoid.
859
FIGURE 18-18. Axial CT, in a different patient, shows fatty dermoid
(arrow) in left anterior nasal cavity.
SUGGESTED READINGS
Hedlund G. Congenital frontonasal masses: developmental anatomy,
malformations, and MR imaging. Pediatr Radiol 2006;36:647–662.
Kadom N, Sze RW. Radiological reasoning: pediatric midline nasofrontal mass.
860
AJR Am J Roentgenol 2010;194:WS10–WS13.
Aqueductal Stenosis
Key Facts
Most common cause of congenital hydrocephalus (20% of all
hydrocephalus cases); generally presents in infancy but may manifest
at any time.
This spectrum of disorders includes congenital narrowing (septum or
membrane, forking, gliosis, webs, or stenosis), postinflammatory
changes (postmeningitis, posthemorrhage), and tumors (especially
those arising in the tectum and pineal gland).
Causes deformity of the tectum, which appears thick but not bulbous
(if so, consider tumor, which is almost always bright on T2/FLAIR
images). High-resolution T2 images (CISS or FIESTA) are useful in
identifying the aqueductal abnormality and patency of third
ventriculostomies, flow-sensitive images confirm diagnosis.
May be associated with Chiari type I and II, Dandy Walker, Bickers-
Adams-Edwards syndrome (“X-linked hydrocephalus”), and other
congenital malformations.
Endocrine dysfunction occurs in 15% to 20% of patients and is
probably secondary to compression of hypothalamus-pituitary axis
due to enlarged third ventricular recesses.
861
FIGURE 18-19. Midsagittal T1 image shows distal narrowing (arrow) of
aqueduct.
862
FIGURE 18-20. Midsagittal flow sensitive study, in a different patient,
shows CSF flow in black that is present only in superior aspect of
aqueduct and stops suddenly (arrow).
863
FIGURE 18-21. Midsagittal high resolution CISS image shows an
aqueductal web (arrow) and persistent hydrocephalus despite the presence
of a third ventriculostomy. Note dark CSF jet through the ventriculostomy
defect (arrowhead).
864
FIGURE 18-22. Midsagittal T2 in utero image shows aqueductal stenosis
(arrow) and massive hydrocephalus.
SUGGESTED READING
Stoquart-El Sankari S, Lehmann P, Gondry-Jouet C, et al. Phase-contrast MR
865
imaging support for the diagnosis of aqueductal stenosis. AJNR Am J
Neuroradiol 2009;30:209–214.
Chiari Malformation Type I

Key Facts
Found more often in adults (incidentally by MRI) than in children.
Etiology: probably related to mesenchymal malformation leading to a
small posterior fossa and steep tapering of the upper spinal canal.
Defined as displacement of cerebellar tonsils below (>6 mm) foramen
magnum; 5% to 30% of patients with this degree of displacement are
symptomatic, and patients with displacement >12 mm are always
symptomatic.
Displacement of tonsils between 3 and 6 mm is indeterminate; <3 mm
is normal. Cerebellar tonsils need to be “pointed” to make diagnosis
in mild cases.
CSF flow studies show lack of flow posteriorly or around foramen
magnum. Movement of tonsils on CSF flow studies is also abnormal.
Symptoms: Valsalva-induced headache (hydrocephalus in 25% of
cases), neck pain, nystagmus, lower cranial nerve palsies, basilar
invagination (25%), odontoid deformities, scoliosis, spinal cord cysts
(20% to 40%), Klippel-Feil syndrome, and atlanto-occipital
assimilation.
Spinal cord cysts are more common in the cervical region.
Treatment generally involves resection of the hypoplastic posterior
arch of C1, dural resection with duroplasty and recreation of cisterna
magna, some surgeons resect the cerebellar tonsils.
866
FIGURE 18-23. Midsagittal T1 image shows marked inferior
displacement of triangular-shaped cerebellar tonsils.
867
FIGURE 18-24. CSF flow study shows significant inferior motion of
tonsils (arrow) and abnormal flow anterior to cord.
868
FIGURE 18-25. CSF flow study, in a different patient, shows normal
cephalad flow anteriorly in white (arrow) but absent flow posteriorly.
869
FIGURE 18-26. Midsagittal noncontrast T1 shows inferior herniation of
pointed cerebellar tonsils and holocord cyst (arrows).
SUGGESTED READING
McVige JW, Leonardo J. Imaging of Chiari type I malformation and
870
syringohydromyelia. Neurol Clin 2014;32:95–126.
Chiari Malformation Type II

Key Facts
Always associated with an open myelomeningocele (chronic CSF
leakage in utero may lead to collapse of the developing brain
producing Chiari II changes).
Skull and dura anomalies include lacunar skull (resolves
spontaneously by 6 to 12 months), small posterior fossa, scalloped
petrous ridges and clivus, large foramen magnum, insufficient
tentorial incisura, and hypoplastic or fenestrated falx.
Brain anomalies: cerebellar atrophy, inferiorly displaced vermis into
foramen magnum, superior cerebellar herniation through tentorial
incisura, beaked tectum, callosal dysgenesis, enlarged massa
intermedia, interdigitation of cortical sulci and stenogyria, and
anomalies of neuronal migration.
Brain anomalies may be subtle after in utero treatment of spine
dysraphism.
About 90% of patients have hydrocephalus and colpocephaly
(dilatation of atria and occipital horns of lateral ventricles).
Spinal cord cysts seen in up to 90% of patients.
Segmentation anomalies of upper cervical spine are seen in 10% of
patients.
871
FIGURE 18-27. Midsagittal T1 image shows dysgenetic corpus callosum
(black arrowhead, posterior body/splenium are absent), “beaked” tectum
(white arrowhead), large massa intermedia (M), absent aqueduct, absence
of normal sulcation of cerebellar vermis, small fourth ventricle and
inferiorly herniated vermis (arrow).
872
FIGURE 18-28. Axial T2, in a different patient, shows upward herniation
of a “heart-shaped” cerebellum through a dysplastic tentorial incisura
(arrows).
873
FIGURE 18-29. Axial CT, in a different patient, shows incomplete mid-
falx with “interdigitation” of cortical sulci.
874
FIGURE 18-30. Axial T2, in a different patient, shows mild sulcal
interdigitation and posteromedial stenogyria (narrow and “packed” gyri).
Suggested Reading
Miller E, Widjaja E, Blaser S, et al. The old and the new: supratentorial MR
findings in Chiari II malformation. Childs Nerv Syst 2008;24:563–575.
Cortical Dysplasias
875
Key Facts
Focal cortical dysplasia and polymicrogyria are postmigrational
developmental disorders with heterogeneous etiologies that involve
prenatal/perinatal injury (such as ischemia or congenital infection,
particularly cytomegalovirus) producing laminar necrosis (involving
layer 5 of the cortex). Most occur in the distribution of the posterior
branches of a middle cerebral artery (so-called perisylvian syndrome).
Genetic mutations may also play a role in some cases.
Gray matter is thick and has a lumpy surface by direct visual
inspection, but by MRI, it generally appears smooth and
indistinguishable from agyria.
Seizures are present in >80% of patients, and there may be
hippocampal sclerosis.
In some patients, MRI reveals anomalous venous drainage (persistent
fetal leptomeningeal vessels) of the dysplasia and/or a deep cleft
continuous with the sylvian fissure.
In 25% of patients, MRI shows high T2/FLAIR white matter signal
intensity extending from the cortex to the ventricle (transmantle sign)
probably reflecting hypomyelination and presence of balloon cells.
Conversely, underlying white matter may appear “hyper-myelinated”
and change over time.
Rarely, these abnormalities will show calcifications on CT.
876
FIGURE 18-31. Axial T2 image shows focal cortical dysplasia in the right
frontal lobe (arrow).
877
FIGURE 18-32. Axial T2 image in a different patient shows focal cortical
dysplasia with thick gyri (arrowhead) and white matter signal
hyperintensity extending toward the lateral ventricle (arrow).
878
FIGURE 18-33. Coronal T2 image, in a different patient, shows dark
signal underlying an area of cortical dysplasia due to white matter
hypermyelination (arrow).
879
FIGURE 18-34. Axial T2 image in a different patient shows posterior
continuation of the sylvian fissures with polymicrogyria bilaterally.
SUGGESTED READINGS
Leach JL, Greiner HM, Miles L, et al. Imaging spectrum of cortical dysplasia in
children. Semin Roentgenol 2014;49:99–111.
Mellerio C, Labeyrie MA, Chassoux F, et al. Optimizing MR imaging detection
of type 2 focal cortical dysplasia: best criteria for clinical practice. AJNR Am J
Neuroradiol 2012;33:1932–1938.
880
Craniosynostoses
Key Facts
Clinical presentation: deformed head and signs of increased
intracranial pressure, incidence is 1:2,000.
Approximately 10% are familial or syndromic (e.g., Crouzon, Apert,
Pfeiffer syndromes, among others) and may be associated with
mutations in FGFR1, FGFR2, FGFR3, TWIST1, and other genes.
Coronal synostosis most common in girls, sagittal in boys.
Classification: simple (one suture or part of it), compound (more than
suture, may be syndromic), bony or cartilaginous (only bony type is
seen by CT).
Most common sites: sagittal (55%), coronal (25%), metopic (15%),
lambdoid (5%).
Most sutures remain “open” until 18 to 24 months of age (metopic
closes sooner, probably around 6 to 9 months).
Posterior fontanelle closes by 2 months, anterior by 7 to 9 months.
Sutures grow perpendicular to their long axis, remember that closure
of even a small portion of a suture behaves like synostosis.
881
FIGURE 18-35. Lateral CT 3-D reformation in a patient with sagittal
suture synostosis shows anterior posterior skull elongation (dolico- or
scaphocephaly).
882
FIGURE 18-36. Corresponding top view 3-D CT shows bony bridge with
fusion of the sagittal suture (arrow) and also fusion of the metopic suture
(arrowhead).
883
FIGURE 18-37. Frontal view 3-D CT in a different patient shows
unilateral synostosis with fusion of the right coronal suture and resultant
“harlequin eye” deformity.
884
FIGURE 18-38. Posterior 3-D view, in a different patient, shows closed
right lambdoid suture with skull flattening.
885
FIGURE 18-39. Oblique 3-D view, in a different patient, shows bony
bridge in closed metopic suture (“keel-shaped” head pointed anteriorly or
trigonocephalic).
886
FIGURE 18-40. Oblique 3-D view in patient with Crouzon syndrome
shows multiple synostoses and turricephaly.
SUGGESTED READING
Glass RB, Fernbach SK, Norton KI, et al. The infant skull: a vault of information.
Dandy-Walker Complex
887
Key Facts
Represents a spectrum of malformations, varying from the mega
cisterna magna to the Dandy-Walker syndrome; occurs in 1:25,000
live births.
Dandy-Walker syndrome includes obstruction of outlet foramina of
fourth ventricle, which becomes massively dilated, large posterior
fossa, high insertion of venous torcular, hydrocephalus (80%),
inferior vermian hypoplasia, corpus callosum agenesis (30%) and
lipomas, neuronal migration anomalies (10%), and cephaloceles
(15%).
Dandy-Walker variant includes mild inferior vermian hypoplasia
without torcular elevation, enlarged vallecula that communicates with
a mildly enlarged and key hole–shaped fourth ventricle and
hydrocephalus (30%).
Mega cisterna magna includes an intact vermis, normal fourth
ventricle, occasional expansion of posterior fossa, and scalloping of
inner table of occipital bone.
Main differential diagnoses: Blake’s pouch and arachnoid cysts in
which there is no vermian hypoplasia.
888
FIGURE 18-41. Midsagittal T1 image shows a large posterior fossa cyst
that is continuous with a large fourth ventricle. The vermis (black arrow)
is hypoplastic and upwardly rotated, the venous torcular (white arrow) is
superiorly located, there is only a small portion of the corpus callosum
(arrowhead) present and there is marked hydrocephalus.
889
FIGURE 18-42. Axial T2 in a different patient shows a large posterior
fossa cyst-like structure communicating with the fourth ventricle and
marked hydrocephalus.
890
FIGURE 18-43. Midsagittal T1 image shows a large fourth ventricle
communicating with a large cisterna magna and mild hypoplasia of
inferior vermis which is rotated superiorly.
891
FIGURE 18-44. Axial T2 image, in the same patient, shows “keyhole”
appearance formed by large fourth ventricle and large vallecular space
communicating with cisterna magna.
SUGGESTED READING
Correa GG, Amaral LF, Vedolin LM. Neuroimaging of Dandy–Walker
malformation: new concepts. Top Magn Reson Imaging 2011;22:303–312.
Focal Gray Matter Heterotopias

892
Key Facts
Heterotopias represent rests of neurons along migration pathways in
white matter (anywhere from ventricular walls to subcortical regions).
Some periventricular forms may be X-linked (filamin 1 gene).
Most common clinical presentation is seizures (up to 10% of seizure
patients have a neuronal migration disorder).
Posterior periventricular ones (particularly when bilateral) may be
more frequently associated with cerebral cortical and mid or hind
brain malformations, decreased white matter volume, and callosal
abnormalities.
Most heterotopias are of the nodular type, generally seen as foci of
gray matter along the ependymal surface of the lateral ventricles (as
opposed to the subependymal nodules of tuberous sclerosis
heterotopias do not calcify).
Heterotopias may also be subcortical and mass-like and may involve
the entire thickness of white matter and be “trans-mantle.”
Heterotopias do not enhance and follow gray matter signal intensity
in all sequences.
Five to twenty-five percent of subsequent siblings will have a
neuronal migration disorder.
893
FIGURE 18-45. Axial noncontrast CT demonstrates bilateral
periventricular nodular heterotopias that are isodense to gray matter
(arrows).
894
FIGURE 18-46. Coronal noncontrast T1 in a different patient
demonstrates nodular heterotopias to be isointense to gray matter.
895
FIGURE 18-47. Axial T2, in a different patient, shows bilateral nodular
heterotopias (arrows).
896
FIGURE 18-48. Axial T2, in a different patient, shows bilateral nodular
heterotopias (arrows) associated with polymicrogyria (arrowheads).
SUGGESTED READINGS
Abdel Razek AA, Kandell AY, Elsorogy LG, et al. Disorders of cortical
formation: MR imaging features. AJNR Am J Neuroradiol 2009;30:4–11.
Mandelstam SA, Leventer RJ, Sandow A, et al. Bilateral posterior periventricular
nodular heterotopia: a recognizable cortical malformation with a spectrum of
associated brain abnormalities. AJNR Am J Neuroradiol 2013;34:432–438.
897
Holoprosencephalies
Key Facts
Group of anomalies in which there may be midline cleavage of the
face and a failure of diverticulation of the brain.
Incidence: 1:16,000 live births.
Alobar and semilobar types have hypotelorism and facial midline
clefts.
Alobar: most severe type, monoventricle; absent septum pellucidum,
falx cerebri, and interhemispheric fissure; interhemispheric cyst;
thalami are fused, and facial anomalies are common.
Semilobar: intermediate type, monoventricle but with rudimentary
occipital and temporal horns, absent or hypoplastic septum
pellucidum; falx cerebri and interhemispheric fissure may be present
posteriorly; thalami are fused, and facial anomalies are usually mild
or absent.
Lobar: mild type, lateral ventricles are almost normal, but frontal
horns “point” inferiorly; septum pellucidum is absent; falx cerebri,
interhemispheric fissure, and thalami may be separated; may be
indistinguishable from septooptic dysplasia. An azygous anterior
cerebral artery is commonly present.
Middle interhemispheric variant (syntelencephaly): very rare type
characterized by near-vertical orientation of the sylvian fissure with
continuation across the midline, fused posterior frontal/anterior
parietal lobes, and absence of the body of corpus callosum. Frontal
and occipital poles are well separated.
898
FIGURE 18-49. Axial CT in lobar holoprosencephaly shows nearly
normally formed lateral ventricles, absent septum pellucidum, fused massa
intermedia and solitary anterior cerebral artery (arrow).
899
FIGURE 18-50. Midsagittal T1, in the same patient, shows absence of
callosal genu.
900
FIGURE 18-51. Axial T2 in semilobar holoprosencephaly shows absent
anterior lateral ventricles and midline continuation of gray/white matter as
well as a solitary anterior cerebral artery.
901
FIGURE 18-52. Axial T1 shows large monoventricle communicating with
posterior cysts and a flat-shaped rostrally located brain with midline fusion
in a patient with alobar holoprosencephaly.
902
FIGURE 18-53. Axial T2 in a different patient with the middle
interhemispheric variant shows near-vertical continuation of the sylvian
fissure across the midline.
903
FIGURE 18-54. Sagittal T1 in the same patient shows absent callosal
body with formation of the genu and a small splenium (arrows).
SUGGESTED READINGS
Dill P, Poretti A, Boltshauser E, et al. Fetal magnetic resonance imaging in
midline malformations of the central nervous system and review of the
literature. J Neuroradiol 2009;36:138–146.
Hahn JS, Barnes PD. Neuroimaging advances in holoprosencephaly: refining the
spectrum of the midline malformation. Am J Med Genet C Semin Med Genet
2010;154C:120–132.
904
Joubert Syndrome
Key Facts
Refers to anatomic abnormalities that encompass a set of clinical
symptoms including ataxia, hyperpnea, apnea, seizures, mental
retardation, oculomotor apraxia, polydactyly, colobomas, and
hypotonia.
Typically autosomal recessive with many different genes involved
(rarely X-linked).
Major abnormalities include a hypoplastic or aplastic cerebellar
vermis, abnormal tracts in brainstem, and lack of decussation of the
superior cerebellar peduncles.
The superior cerebellar peduncles become thin and parallel to each
other giving the superior brainstem the so-called “molar tooth”
appearance on axial imaging.
905
FIGURE 18-55. Midsagittal T1 shows small brainstem, absent inferior
vermis, abnormally shaped fourth ventricle, and dysgenetic corpus
callosum.
906
FIGURE 18-56. Axial T1 in a different patient shows the typical molar
tooth configuration of the midbrain.
907
FIGURE 18-57. Axial DTI FA map in a different patient (left) shows
green color in the superior cerebellar peduncles due to their horizontal
orientation (arrow) and lack of the expected decussation “red dot” (seen
on a normal patient for comparison, arrowhead, right).
908
FIGURE 18-58. Axial T1, in a different patient, shows abnormally shaped
fourth ventricle (inferior to midbrain).
SUGGESTED READINGS
Poretti A, Boltshauser E, Huisman TA. Pre- and postnatal neuroimaging of
congenital cerebellar abnormalities. Cerebellum 2016;15:5–9.
Poretti A, Huisman TA, Scheer I, et al. Robert syndrome and related disorders:
spectrum of neuroimaging findings in 75 patients. AJNR Am J Neuroradiol
2011;32:1459–1463.
909
Posterior (Occipital and/or Parietal)
Encephaloceles
Key Facts
Rare malformations: 1–3:100,000 live births.
In the United States, most encephaloceles are occipital (80%) and
parietal (10%), in Asia most are sincipital (frontoethmoidal); rare
types include atretic parietal, sphenoidal (associated to
neurofibromatosis type I), and nasopharyngeal.
Herniated brain is usually nonfunctioning due to necrosis, gliosis,
fibrosis, and anomalies of neuronal migration.
Encephalocele may contain pons, midbrain, and aberrant but
important venous structures.
Atretic parietal meningocele refers to a form “fruste” with only a
small amount of meninges, gliotic brain, and CSF in the midline
parietal region that may cross the superior sagittal sinus and be
associated with other venous anomalies (commonly a persistent
falcine sinus) as well as malformations of cortical development and
Walker-Warburg syndrome.
Spinal cord cysts may be present.
Chiari III is very rare and is the combination of the intracranial
features of Chiari II with a low occipital and high cervical
encephalocele.
Occipital/parietal encephaloceles may be associated with the Dandy
Walker malformations. Occipital ones may be seen in Meckel-Gruber
syndrome.
910
FIGURE 18-59. Midsagittal T1 shows a large parietal meningocele.
911
FIGURE 18-60. Parasagittal T1 in a different patient shows a large
occipital meningoencephalocele.
912
FIGURE 18-61. Midsagittal T1 shows an atretic parietal cephalocele
(arrow) with a persistent falcine sinus (arrowhead).
913
FIGURE 18-62. Axial T2 in the same patient shows what is probably
gliotic tissue within the cephalocele.
914
FIGURE 18-63. Oblique TOF MR venogram in a different patient shows
fenestration (arrow) of the superior sagittal sinus at the site of an atretic
cephalocele.
915
FIGURE 18-64. Midsagittal CT venogram shows persistent parietal sinus
(arrow) in a patient with an atretic parietal meningocele.
SUGGESTED READINGS
Agrawal A, Mittal A, Kohali GB, et al. Chiari III malformation. Pediatr
Neurosurg 2011;47:309–310.
Morioka T, Hashiguchi K, Samura K, et al. Detailed anatomy of intracranial
venous anomalies associated with atretic parietal cephaloceles revealed by
high-resolution 3D-CISS and high-field T2-weighted reversed MR images.
Childs Nerv Syst 2009;25:309–315.
916
Schizencephalies
Key Facts
Transcerebral cleft (extending from cortex to ventricle) lined by
abnormal (generally polymicrogyric) gray matter; sometimes an
anomaly of venous drainage is also present and closely associated
with the dysplastic gray matter. Some clefts have an overlying CSF-
filled cyst that may grow.
Types: open lip, which contains CSF within the cleft, and closed lip,
in which the walls of the cleft are in apposition with each other.
Bilateral open lip clefts are not uncommon and may be considered a
variant of hydranencephaly. Most are in frontal regions.
Fifty percent of patients have other zones of neuronal migration
anomalies. Hippocampi are “malrotated” in 50%.
Septum pellucidum is absent in 90% of cases. May be associated with
septooptic dysplasia.
Symptoms (usually seizures and hemiparesis) are proportional to the
size of the clefts and more common with the open lip type and
bilateral lesions.
917
FIGURE 18-65. Axial T2 shows CSF-filled cleft (open lip) in left
frontotemporal region extending from external subarachnoid space to
lateral ventricle. Note absent septum pellucidum, small left hemisphere,
and remodeling of inner skull table on left presumably due to CSF
pulsations. Dysplastic gray matter lines the cleft.
918
FIGURE 18-66. Axial CT shows faint cleft (arrowhead) lined by gray
matter. The right hemisphere is smaller than the left one, the lateral
ventricles are prominent, and the septum pellucidum is absent.
919
FIGURE 18-67. Axial T2 in a different patient shows bilateral closed-lip
clefts lined by gray matter (arrows).
920
FIGURE 18-68. Axial T2, in a different patient, shows large open lip
schizencephaly bilaterally with a typical “bat wing” appearance.
SUGGESTED READING
Nabavizadeh SA, Zarnow D, Bilaniuk LT, et al. Correlation of prenatal and
postnatal MRI findings in schizencephaly. AJNR Am J Neuroradiol
2014;35:1418–1424.
Septooptic Dysplasia
921
Key Facts
Considered as a mild form of lobar holoprosencephaly.
Characterized by absent septum pellucidum and hypoplastic anterior
optic pathways (chiasm and nerves), flat roof of lateral ventricles,
inferiorly pointed frontal horns, and continuation of the callosum and
fornices.
Associated with maternal diabetes, quinidine ingestion, antiseizure
medications, drug abuse, cytomegalovirus infection, and congenital
brain malformations (Chiari II and aqueductal stenosis).
Fifty percent of patients with septooptic dysplasia have a
schizencephaly and classically present with seizures; these patients
have a remnant of septum pellucidum and an almost normal visual
apparatus; this type may not be a true form of septooptic dysplasia but
rather an interruption of the visual tract by the cerebral cleft. Some
call this group of anomalies “septooptic dysplasia plus.”
Fifty percent of patients with septooptic dysplasia do not have
schizencephaly and about 60% present with endocrine dysfunction
secondary to hypoplastic pituitary gland (posterior lobe may be
translocated). Nearly 80% have pituitary dysfunction. Olfactory bulbs
may be hypoplastic.
922
FIGURE 18-69. Coronal T1 shows flat roof of lateral ventricles, the
septum pellucidum is absent, frontal horns point down, the pituitary stalk
is absent, and the posterior lobe is translocated to the hypothalamus
(arrow).
923
FIGURE 18-70. Axial T2, in a different patient, shows marked hypoplasia
of the optic nerves (arrows).
924
FIGURE 18-71. Midsagittal T1, in a different patient, shows continuation
of a dysplastic corpus callosum with the fornices.
925
FIGURE 18-72. Axial T2 in a different patient shows absent septum
pellucidum and schizencephaly with a gray matter–lined cleft (arrow).
SUGGESTED READING
Cemeroglu AP, Coulas T, Kleis L. Spectrum of clinical presentations and
endocrinological findings of patients with septo-optic dysplasia: a
retrospective study. J Pediatr Endocrinol Metab 2015;28:1057–1063.
926
CHAPTER 19
Neurocutaneous
Syndromes
NF-1
Key Facts
Autosomal dominant (chromosome 17) or sporadic (50%) disorder,
10 times more common than NF-2; prominent cutaneous lesions,
plexiform neurofibromas, and bilateral optic nerve gliomas
(hallmarks of the disease) are noted.
Other intracranial abnormalities include sphenoid wing dysplasia
leading to pulsatile exophthalmos, buphthalmos, cranial nerve
neurofibromas, vascular stenoses, and aneurysms.
Foci of myelin vacuolization are present in 80% of patients; they are
bright on T2/FLAIR, do not enhance or have significant mass effect,
are not premalignant, and usually regress in adulthood.
About 15% to 40% of patients with NF-1 have optic pathway
gliomas, and most (>80%) are slow growing (some even regress and
could be considered “hamartomas”). Chiasmatic and postchiasmatic
involvement is relatively rare.
Plexiform neurofibromas have a 5% to 10% risk of malignant
transformation (rapid increase in size, continued pain, and areas of
induration should raise suspicion).
About 10% of NF-1 patients have gliomas that involve mainly the
brainstem and tectum (producing hydrocephalus); most are low grade.
927
Spine abnormalities are present in 60% of patients and include
kyphoscoliosis, lateral thoracic meningoceles (characteristic of NF-1),
and vertebral scalloping due to dural ectasia, arachnoid cysts, or
rarely neurofibromas. Intramedullary gliomas may be seen.
FIGURE 19-1. Axial T2 shows enlargement of the optic nerves bilaterally

in a patient with optic gliomas.
928
FIGURE 19-2. Axial T2 in a different patient shows plexiform
neurofibromata in the right cavernous sinus, right orbit, and right temporal
subcutaneous tissues.
929
FIGURE 19-3. Axial CT, in a different patient, shows marked dysplasia
of the right sphenoid wing with mass effect on the right orbit and
proptosis.
930
FIGURE 19-4. Axial FLAIR, in a different patient, shows foci of myelin
vacuolization (UBOs) in the basal ganglia and thalami.
931
FIGURE 19-5. Axial T2 in a different patient shows multiple spinal and
paraspinal neurofibromata with mass effect on the cord. Note dark
fibrocollagenous cores (arrow, the so-called “target sign”).
932
FIGURE 19-6. Axial T2, in a different patient, shows a large lateral
thoracic meningocele.
SUGGESTED READINGS
Borofsky S, Levy LM. Neurofibromatosis: types 1 and 2. AJNR Am J
Neuroradiol 2013;34:2250–2251.
Rodriguez D, Young Poussaint T. Neuroimaging findings in neurofibromatosis
type 1 and 2. Neuroimaging Clin N Am 2004;14:149–170.
933
NF-2
Key Facts
Autosomal dominant (chromosome 22q12) disorder is 10 times less
common than NF-1; cutaneous lesions are less common (<50%).
Presents in third to fourth decades of life, all patients develop CNS
tumors.
Bilateral vestibular schwannomas are the hallmark sign (95% of
patients); the trigeminal and oculomotor nerves are the second and
third most commonly involved cranial nerves (a tumor arising in a
purely motor cranial nerve should prompt a search for NF-2).
Brain lesions arise from the coverings of the brain, that is, Schwann,
meningeal, and ependymal cells (schwannomas, meningiomas, and
ependymomas, respectively).
Meningiomas are multiple in 50% of patients, may be plaque-like,
and may occur in atypical locations.
Meningioma in a child should raise suspicion of NF-2.
Meningiomas in NF-2 patients present more commonly in the lateral
ventricles than do sporadic meningiomas (16% vs. 5%).
Ependymomas usually occur in the brainstem and spinal cord. Other
spinal tumors include schwannomas and rarely meningiomas, which
are almost always intracranial in NF-2.
934
FIGURE 19-7. Axial postcontrast T1 shows bilateral vestibular
schwannomas, a left trigeminal schwannoma (arrow), and a small right
facial schwannoma (arrowhead).
935
multiple meningiomas including one within the atrium of the left lateral
ventricle and along the upper cerebellar tentorium.
936
FIGURE 19-9. Axial postcontrast T1 in a different patient shows a
schwannoma in the left parapharyngeal space (arrow).
937
bilateral trigeminal nerve schwannomas.
938
multiple tiny schwannomas in the cauda equina nerve roots and a small
meningioma (arrow).
939
FIGURE 19-12. Sagittal postcontrast T1 in a different patient shows
numerous enhancing intramedullary masses compatible with
ependymomas.
SUGGESTED READINGS
940
Borofsky S, Levy LM. Neurofibromatosis: types 1 and 2. AJNR Am J
Neuroradiol 2013;34:2250–2251.
Rodriguez D, Young Poussaint T. Neuroimaging findings in neurofibromatosis
type 1 and 2. Neuroimaging Clin N Am 2004;14:149–170.
PHACE(S) Syndrome
Key Facts
Comprised by posterior fossa malformations (Dandy-Walker,
hemisphere hypoplasia ipsilateral to hemangioma), segmental
hemangiomas (often large), arterial anomalies (circle of Willis and
aorta), cardiac defects, eye abnormalities, and sternal defects or
supraumbilical raphe.
Much more common in girls than in boys with an 8 to 9:1 ratio.
Abnormalities in circle of Willis include hypoplasia, agenesis,
dolichoectasia, persistence of embryonic vessels, progressive
stenoses, occlusions, moyamoya-like collaterals, and aneurysms.
PHACE syndrome is present in 2% of patients with facial
hemangiomas and up to 20% of those with segmental facial
hemangiomas.
941
FIGURE 19-13. Axial T2 shows bilateral large hemangiomas in parotid
spaces.
942
FIGURE 19-14. MRA in same patient shows multiple anomalies in the
circle of Willis.
943
FIGURE 19-15. Axial T2, in a different patient, shows a skin
hemangioma in right face and scalp.
944
FIGURE 19-16. Sagittal T1 in a different patient shows Dandy-Walker
malformation and thickening of the upper lip due to a facial hemangioma.
945
FIGURE 19-17. Axial postcontrast T1 in the same patient shows a
hemifacial hemangioma in the right periorbital region.
946
FIGURE 19-18. Axial MRA in the same patient shows a diminutive right
internal carotid artery (arrow).
SUGGESTED READINGS
Hess CP, Fullerton HJ, Metry DW, et al. Cervical and intracranial arterial
anomalies in 70 patients with PHACE syndrome. AJNR Am J Neuroradiol
2010;31:1980–1986.
Oza VS, Wang E, Berenstein A, et al. PHACES association: a neuroradiologic
review of 17 patients. AJNR Am J Neuroradiol 2008;29:807–813.
947
Sturge-Weber Syndrome
Key Facts
Congenital vascular disorder of neural crest cell migration and
differentiation where there is inadequate control of capillary
formation. It involves the face, eyes, brain, and meninges.
Occurs sporadically without hereditary transmission.
Although patients are normal at birth, 90% develop seizures,
dementia, hemiplegia, and visual defects during their lifetime.
Port-wine nevi are located in the trigeminal nerve distribution
affecting V1 and less commonly V2 (virtually never V3 in isolation).
Ipsilateral to the face abnormality, there is a pial angioma that is
generally occipitoparietal in distribution, bilateral in 20%, and rarely
infratentorial. Subtle angiomatosis may be detected with SWI or
postcontrast FLAIR.
Cortical veins do not develop in the area of the pial angioma, and
there is recruitment of deep medullary veins and choroid plexus for
venous drainage; blood stasis leads to secondary dystrophic
calcification of underlying cortex (ischemia?), hypermyelination of
underlying white matter, and eventual atrophy. Calcifications are
uncommon in infants.
Initially, perfusion shows high rCBV in the region of the pial
angioma that decreases as ischemia ensues.
Remember that a normal MRI in an infant <1 year of age does not
rule out Sturge-Weber, and these patients need follow-up if there is
clinical suspicion for such.
948
FIGURE 19-19. Axial T2 shows mild right cerebral volume loss and
small venous flow voids in the deep white matter and periventricular
regions (arrows).
949
FIGURE 19-20. Corresponding axial SWI minIPs better demonstrate deep
medullary and ventricular/choroidal venous collaterals.
950
extensive leptomeningeal enhancement and engorgement of the right
choroid plexus.
951
FIGURE 19-22. Axial postcontrast CT in a different patient with bilateral
angiomas demonstrates prominent occipitoparietal calcifications and
engorged choroid plexuses.
952
FIGURE 19-23. Axial T2 in a different patient with severe Sturge-Weber
shows marked left cerebral atrophy and compensatory expansion of the
calvarium and frontal sinus (Dyke-Davidoff-Masson).
953
FIGURE 19-24. Coronal postcontrast T1 in the same patient shows both
left cerebral and cerebellar involvement.
SUGGESTED READINGS
Adams ME, Aylett SE, Squier W, et al. A spectrum of unusual neuroimaging
findings in patients with suspected Sturge-Weber syndrome. AJNR Am J
Neuroradiol 2009;30:276–281.
Miao Y, Juhasz C, Wu J, et al. Clinical correlates of white matter blood flow
perfusion changes in Sturge-Weber syndrome: a dynamic MR perfusion-
weighted imaging study. AJNR Am J Neuroradiol 2011;32:1280–1285.
954
Schwannomatosis (NF-3)
Key Facts
Clinically and genetically distinct from NF-1 and NF-2 and much less
common, with an annual incidence of 0.58 cases per 1,000,000
individuals.
Median age of diagnosis is 40 years; patients present with chronic
pain.
Fifteen to 25% of cases are familial, and the rest are sporadic;
mutations occur in the SMARCB1 and LZTR1 genes (chromosome
22 but separate from the NF-2 gene).
Characterized by multiple peripheral (legs or arms) and spinal
schwannomas without bilateral vestibular schwannomas (the presence
of which is diagnostic for NF-2 and rules out schwannomatosis).
Intracranial schwannomas are not common; patients may rarely
develop unilateral vestibular schwannomas, and other cranial nerves
may also be affected.
As opposed to NF-2, intracranial meningiomas are rare and usually
solitary.
Greater than 70% of patients have spinal schwannomas, half of which
occur in the lumbar spine (commonly along the cauda equina),
followed by thoracic and cervical spine. No increased risk for
intramedullary ependymomas has been identified.
There may be an increased risk for atypical teratoid rhabdoid tumors.
955
FIGURE 19-25. Coronal postcontrast T1 shows numerous subcutaneous
schwannomas in the head, more numerous on the left side.
956
FIGURE 19-26. Coronal postcontrast T1, in the same patient, also shows
schwannomas in the bilateral oculomotor (arrow) and right trigeminal
nerves (arrowhead), as well as left masticator space and masseter muscle.
957
bilateral schwannomas with mass effect on the thecal sac.
958
FIGURE 19-28. Sagittal T2 in the same patient demonstrates
schwannomas involving the cervical and upper thoracic spine with
foraminal expansion.
SUGGESTED READINGS
959
Li P, Zhao F, Zhang J, et al. Clinical features of spinal schwannomas in 65
patients with schwannomatosis compared with 831 with solitary
schwannomas and 102 with neurofibromatosis type 2: a retrospective study at
a single institution. J Neurosurg Spine 2016;24:145–154.
Merker VL, Esparza S, Smith MJ, et al. Clinical features of schwannomatosis: a
retrospective analysis of 87 patients. Oncologist 2012;17:1317–1322.
Tuberous Sclerosis
Key Facts
Eighty percent of cases occur de novo, and the rest are autosomal
dominant due to mutations in the TSC1 (hamartin) or TSC2 (tuberin)
genes, occurs in 1:10,000 live births.
Classic triad (one third of cases): facial angiofibromas, seizures (80%
to 100%), and mental retardation.
Ninety-five percent of patients show cortical/subcortical tubers and
subependymal nodules (both hamartomas). Twenty-four percent have
cerebellar tubers.
Hamartomas are composed of disordered glial tissues, heterotopic
neurons, giant cells, and calcifications.
On T2/FLAIR images, cortical tubers are wedge shaped, may have
slight mass effect, 10% enhance, and by CT 50% are calcified.
Subependymal nodules usually calcify except in early life.
Cortical tubers are often accompanied by T2-bright radial migration
lines extending from them to the ventricle. White matter cysts may
also be seen due to “cystoid degeneration.”
Epileptogenic cortical hamartomas may show increased perfusion.
Subependymal giant cell astrocytomas (SEGAs) are seen in 5% to
20% of patients and derive from subependymal nodules from which
they are indistinguishable except for their progressive growth.
SEGAs can occur anywhere within the ventricles but are more
common at the foramina of Monro. They are histologically benign but
can occasionally invade the brain.
960
FIGURE 19-29. Axial CT shows multiple calcified subependymal
nodules and calcified subcortical hamartomas posteriorly.
961
enhancement of subependymal nodules bilaterally (arrowhead) and
enhancement of a cortical tuber (arrow).
962
FIGURE 19-31. Axial FLAIR, in a different patient, shows numerous
bright and slightly expansile cortical tubers bilaterally.
963
FIGURE 19-32. Axial FLAIR, in a different patient, shows cortical tubers
with a radial white matter migration line (arrowhead) and a subependymal
giant cell astrocytoma (arrow).
964
FIGURE 19-33. Axial T1 in a baby shows linear bright areas (arrows) of
hypermyelinated white matter extending from ventricles to cortex.
965
FIGURE 19-34. Axial postcontrast T1 in a different patient shows an
avidly enhancing subependymal giant cell astrocytoma in the region of the
left foramen of Monro with mild ventriculomegaly.
SUGGESTED READINGS
Northrup H, Krueger DA; International Tuberous Sclerosis Complex Consensus
Group. Tuberous sclerosis complex diagnostic criteria update:
recommendations of the 2012 International Tuberous Sclerosis Complex
Consensus Conference. Pediatr Neurol 2013;49:243–254.
Wong AM, Wang HS, Schwartz ES, et al. Cerebral diffusion tensor MR
966
tractography in tuberous sclerosis complex: correlation with neurologic
severity and tract-based spatial statistical analysis. AJNR Am J Neuroradiol
2013;34:1829–1835.
von Hippel-Lindau Disease

Key Facts
Autosomal dominant disorder linked to defect in chromosome 3
(defective tumor suppressor gene), occurs in 1:35,000 to 40,000 live
births.
Patients with CNS lesions become symptomatic between 20 and 50
years of age.
Hemangioblastomas (multiple ones in 10% of patients) are the
hallmark of this disorder and are seen in up to 50% of patients.
Sixty-five percent of hemangioblastomas are located in cerebellum,
15% in spinal cord, and 20% in brainstem.
Twenty to 40% of hemangioblastomas are solid, and the majority are
cystic (60% to 80%) with a mural nodule, which tends to be subpial
in location. They are highly vascular, and prominent flow voids may
be a clue to the diagnosis. The cyst wall does not enhance.
Retinal angiomas are present in half of patients and are small; by
imaging the most common eye finding is retinal detachment.
Endolymphatic sac tumors occur in 4% of patients and may be the
first manifestation of this disorder; endolymphatic sac tumors in
children should raise suspicion of von Hippel-Lindau disease.
967
FIGURE 19-35. Axial postcontrast T1 shows an avidly enhancing, solid
hemangioblastoma in the upper cerebellum (arrow).
968
FIGURE 19-36. Axial postcontrast T1, in the same patient, shows a pial-
based hemangioblastoma in the upper cervical spine (arrow).
969
FIGURE 19-37. Postcontrast T1 in a different patient shows a cystic
hemangioblastoma with an enhancing mural nodule that abuts the pial
surface.
970
FIGURE 19-38. Corresponding MR perfusion rCBV map shows high
perfusion in tumor nodule (arrow).
971
FIGURE 19-39. Axial T2 shows an endolymphatic sac tumor along the
left petrous ridge with cystic changes and heterogeneous signal due to
prior hemorrhages.
972
FIGURE 19-40. Corresponding postcontrast T1 demonstrates
heterogeneous enhancement of the tumor.
SUGGESTED READINGS
Nandigam K, Mechtler LL, Smirniotopoulos JG. Neuroimaging of
neurocutaneous diseases. Neurol Clin 2014;32:159–192.
Slater A, Moore NR, Huson SM. The natural history of cerebellar
hemangioblastomas in von Hippel-Lindau disease. AJNR Am J Neuroradiol
2003;24:1570–1574.
973
SECTION B SPINAL
IMAGING
974
CHAPTER 20
Brachial Plexus
Inflammatory and Infectious Plexitis

Key Facts
Viral and idiopathic plexitis:
These are predominantly sensory and have an insidious onset.

Most patients are between 30 and 70 years of age (uncommon in the
very young).
Most resolve spontaneously in 8 to 12 weeks after their onset.
Other causes include: drug reaction (allergic), post viral
(autoimmune), due to a vasculitis and a heredofamilial type.
Main differential diagnosis: perineural tumor spread, post radiation
changes, stretching injury, hypertrophic polyneuropathy, lymphoma,
chronic inflammatory demyelinating polyneuropathy, thoracic outlet
syndrome.
Radiation-induced plexitis:
Generally occurs with doses >6,000 cGy

In the acute type, symptoms tend to be permanent (probably due to
vascular injury and nerve ischemia)
In the subacute type (onset generally at about 6 months after
treatment), symptoms are transient and usually reversible.
Most radiation-induced plexopathies are predominantly sensory.
Diffuse thickening and enhancement of the brachial plexus may be
indistinguishable from metastases and follow up is needed to rule out
975
progression, viral plexitis and chronic inflammatory demyelinating
polyneuropathy may have a similar appearance.
FIGURE 20-1. Coronal fat suppressed T2 shows thickening and increased

signal in the roots and trunks of the left brachial plexus due to a herpes
zoster infection.
976
FIGURE 20-2. Coronal fat suppressed T2, in a different patient, show
diffuse thickening of the right plexus. The cause was never found but
symptoms resolved with only supportive treatment.
977
FIGURE 20-3. Coronal fat suppressed T2 in a different patient with
diffuse (presumably drug-induced) plexopathy shows diffuse thickening
and increased signal in the brachial plexus bilaterally.
978
FIGURE 20-4. Coronal post contrast T1 shows increased signal intensity
from the left infraclavicular brachial plexus (arrow) in a patient post
ipsilateral mastectomy and radiation therapy.
FIGURE 20-5. Corresponding coronal fat suppressed T2 shows increased

signal (arrow) in the abnormal brachial plexus.
SUGGESTED READINGS
Chhabra A, Thawait GK, Soldatos T, et al. High-resolution 3T MR neurography
of the brachial plexus and its branches, with emphasis on 3D imaging. AJNR
Tharin BD, Kini JA, York GE, et al. Brachial plexopathy: a review of traumatic
and nontraumatic causes. AJR Am J Roentgenol 2014;202:W67–W75.
Traumatic Brachial Plexus Injuries

Key Facts
979
Vascular injuries:
Injuries to the subclavian artery may result in pseudoaneurysms and a

compressive mixed sensory/motor neuropathy. The brachial plexus
may also be damaged during placement of subclavian catheters.
Hematomas are generally post traumatic and most are not associated
with significant vascular injuries.
While well-encapsulated hematomas that result in a plexopathy may
be surgically drained, most are diffuse and not amenable to drainage
(in such patients the plexopathy is generally due to traction injuries or
post-traumatic thoracic outlet syndrome).
Avulsion and stretch injuries:
Cervical nerve root avulsions are generally caused by traction injuries

of the upper extremities (in the lower spine, they are related to spinal
or pelvic fractures).
With complete avulsion, the nerve roots retract leaving behind fluid-
filled pseudomeningoceles.
Nerve avulsions at “root entry zones” may result in adjacent
subarachnoid hematomas and edema/hemorrhage in the spinal cord.
Enhancement of intradural nerve roots indicates functional avulsion
despite morphologic continuity. Enhancement of paraspinal muscles
(due to denervation) may be an indirect sign of avulsion injury.
Stretching of the brachial plexus may sometimes lead to neuroma
formation.
980
FIGURE 20-6. Coronal non contrast T1 shows large rounded and
“laminated” appearance of a right subclavian artery pseudoaneurysm.
981
FIGURE 20-7. Sagittal T1, in the same patient, clearly shows the
concentric layers of varying signal intensities that are typical of giant
aneurysms. The plexus is compressed and not identifiable.
982
FIGURE 20-8. Coronal T2, in a different patient, shows a fluid-filled
pseudomeningocele (arrow) from avulsion of the left C7 nerve root.
983
FIGURE 20-9. Axial T2, in a different patient, shows dark blood-filled
pseudomeningocele (arrow) with inward displacement of thecal sac and
subarachnoid blood.
984
FIGURE 20-10. Coronal T2 in a different patient shows thickening and
increased signal throughout the right brachial plexus (arrows) due to
stretching injury.
985
FIGURE 20-11. Coronal T1, in a different patient with poorly healed
clavicle fracture, shows large callus (arrow) which resulted in a
compressive plexopathy.
SUGGESTED READINGS
Castillo M. Imaging the anatomy of the brachial plexus: review and self-
assessment module. AJR Am J Roentgenol 2005;185:S196–S204.
Yoshikawa T, Hayashi N, Yamamoto S, et al. Brachial plexus injury: clinical
manifestations, conventional imaging findings, and the latest imaging
techniques. Radiographics 2006;26(suppl 1):S133–S143.
Brachial Plexus Tumors
986
Key Facts
Fatty tumors:
Brachial plexus lipomas are not uncommon and are generally

asymptomatic but may result in a compressive plexopathy when very
large. Liposarcomas are rare.
Lipomas do not enhance and are nearly homogeneous (but may have
inner strands). Liposarcomas show inhomogeneous contrast
enhancement and have an aggressive appearance. Invasion of the
plexus may render the patient inoperable.
Metastases:
Most brachial plexus metastases are due to carcinomas of the breast

and lung and lymphoma. They may present as a focal mass or
infiltrate the plexus diffusely.
Pancoast tumors:
Arise at the apical pleuropulmonary groove and are most commonly

squamous, adeno- or large cell carcinomas of the lung. Prognosis is
poor when the brachial plexus is involved (stage T4).
In 25% of patients the tumor will extend to the roots of the brachial
plexus and even intraspinally resulting in cord compression.
Nerve sheath tumors:
Schwannomas are the most common primary tumor of the brachial

plexus and more commonly occur in the roots and trunks. Extension
into and expansion of the corresponding neural foramen is typical.
Enhancement pattern is non-specific.
Neurofibromas are the hallmark of NF-1 and may occasionally be
sporadic. They may attain a large size, involve the entire plexus, and
may be bilateral. Enhancement is variable but a significant change in
size and enhancement may indicate malignant degeneration.
987
FIGURE 20-12. Corresponding fat suppressed post contrast image shows
peripheral and nodular enhancement in a liposarcoma. Note that the
brachial plexus (arrow) enhances suggesting invasion.
988
FIGURE 20-13. Parasagittal T1, in a different patient, shows well-defined
fatty benign lipoma (arrow).
989
FIGURE 20-14. Coronal fat suppressed T2, in a different patient, shows
massive thickening of the right plexus (arrow) due to breast cancer
infiltration.
990
FIGURE 20-15. Coronal non contrast T1 shows normal right interscalene
fat triangle (arrow). On the left side, a large Pancoast tumor has invaded
and obliterated this fat which is where the brachial plexus is located.
991
FIGURE 20-16. Coronal T2 shows a schwannoma with cystic changes in
the region of the right roots/trunks extending to a neural foramen (arrow).
992
FIGURE 20-17. Coronal fat suppressed T2 shows very large
neurofibromas in the right plexus and subtle involvement of the left
plexus.
SUGGESTED READING
Lutz AM, Gold G, Beaulieu C. MR imaging of the brachial plexus. Neuroimaging
Clin N Am 2014;24:91–108.
993
CHAPTER 21
Congenital
Malformations
Caudal Agenesis (Regression) Syndrome

Key Facts
Rare and complex anomaly of impaired gastrulation that includes
absent lower vertebrae, anal atresia, malformed genitalia, renal
abnormalities, and, rarely, fusion of the lower extremities
(sirenomelia, probably due to vascular steal through the vitelline
artery with hypoperfusion of caudal structures).
Sixteen percent of patients with this anomaly are the offspring of
diabetic mothers.
Most patients with caudal regression anomalies have a neurogenic
bladder, motor weakness, and feet deformities.
Types:
Type 1: The sacrum is completely or partially absent, and the
distal conus (wedge shaped) and cauda equina are not
completely formed and terminate at a high level.
Type 2: Less severe form where the spinal cord is dysplastic and
tethered at the level of the agenesis (with or without lipoma and
rarely a teratoma). Both types may have associated cord cysts.
Associated abnormalities are diastematomyelia, intraspinal lipomas,
dermoids, and dermal sinuses. May be seen as part of the VACTERL
association or Currarino syndrome.
994
FIGURE 21-1. Lateral view radiograph shows a dysplastic S1 vertebra
and absence of the sacrum below this level (arrow).
995
FIGURE 21-2. Corresponding sagittal T2 shows a truncated spinal cord
with hydromyelia and absence of most of the sacrum in a patient with type
1 syndrome.
996
FIGURE 21-3. Frontal radiograph in a different patient shows complete
absence of the sacrum.
997
FIGURE 21-4. Coronal T2 in a different patient shows a truncated cord
terminating at a high level (arrow ) in a patient with scoliosis.
998
FIGURE 21-5. Midsagittal T2, in a different patient with type 2
syndrome, shows cord terminating in a cyst at agenesis level.
999
FIGURE 21-6. Midsagittal T1, in a different patient with type 2
syndrome, shows absent distal sacrum, small intraspinal lipoma, and a
dysplastic cord extending to level of agenesis. A cavity enlarges the cord
at L2-L4.
SUGGESTED READING
1000
Nievelstein RA, Valk J, Smit LM, Vermeij-Keers C. MR of the caudal regression
syndrome: embryologic implications. AJNR Am J Neuroradiol 1994;15:1021.
Diastematomyelia (Split-Cord
Malformation)
Key Facts
There are two types of sagittal splitting (diastematomyelia) of the
spinal cord:
Type 1 (external, 50%): caused by persistence of the accessory
neurenteric canal resulting in a bony, fibrous, and/or fatty band,
which usually originates from posterior elements and courses
ventrally to join the vertebral body. Two dural tubes are present.
Symptomatic due to tethering by band.
Type 2 (internal, 50%): milder form with single dural tube and
no mesenchymal band, only splitting of cord. Generally
asymptomatic.
Diastematomyelia is seen in 5% of scoliosis cases and in ~30% of
patients with myelomeningoceles and is more common in females.
Fifty percent of these lesions occur in the thoracic spine and 50% in
the lumbar region.
Most hemicords reunite into a single one inferior to the splitting.
Diastematomyelias may be associated with syringomyelia in one or
both hemicords or above or below level of splitting, and the type 2
generally becomes symptomatic only when a syrinx is present.
Vertebral body abnormalities (generally of segmentation) and
overlying skin lesions (hairy patch and hemangiomas are typical in
the type 1) are seen in the majority of patients with diastematomyelia.
1001
FIGURE 21-7. Axial T2 in a patient with type 1 disease shows a thin
osseous bar splitting the spinal cord into two hemicords, each with a set of
anterior and posterior nerve roots.
1002
FIGURE 21-8. Sagittal T2 in a different patient shows an osseous bar
(arrow) with a low position of the spinal cord, a syrinx, and a lipoma at the
level of the split (arrowhead).
1003
FIGURE 21-9. Axial CT, in a different patient, shows a bar that
terminates anteriorly in a pseudoarticulation (arrow), which will make its
removal easier.
1004
FIGURE 21-10. Coronal CT, in the same patient, shows the bar (arrow)
and an intraspinal lipoma (arrowhead).
1005
FIGURE 21-11. Axial T2 in a different patient with type 2 disease shows
that the right hemicord contains a cyst.
1006
FIGURE 21-12. Axial T2, in a different patient with type 2 disease, shows
that the left hemicord has an associated lipoma (arrow) posteriorly.
SUGGESTED READING
Rufener SL, Ibrahim M, Raybaud CA, et al. Congenital spine and spinal cord
1007
malformations—pictorial review. AJR Am J Roentgenol 2010;194:S26–S37.
Dermoid and Epidermoid

Key Facts
These masses account for 3% to 17% of all spinal tumors in children,
and 20% to 30% are associated with a dermal sinus tract. The
majority are developmental, but they may occasionally be iatrogenic.
In the spine, dermoids are more common than epidermoids.
Histologically, an epidermoid has a fibrous capsule and squamous
epithelium, and a dermoid contains skin appendages (hair follicles
and sebaceous and sweat glands).
Chemical meningitis occurs secondary to their rupture. They may
become infected through a dermal sinus tract and show contrast
enhancement; infection may be subclinical and contrast may be
indicated in all.
Most common location for both lesions is the lumbosacral spine
(region of filum terminale and conus medullaris).
Epidermoids contain liquid cholesterol and may be difficult to
visualize by MRI; dermoids contain solid cholesterol and have signal
intensities similar to fat but may have cystic components or be
completely cystic and indistinguishable from epidermoids.
Main differential diagnosis: teratoma, arachnoid cyst, and neurenteric
cyst.
1008
FIGURE 21-13. Midsagittal T2 shows an epidermoid (arrows) at the L5
level that is nearly isointense to CSF.
1009
FIGURE 21-14. Corresponding postcontrast T1 shows that the mass
approximates CSF intensity and does not enhance.
1010
a complex, partially cystic, and fatty dermoid in the filum terminale and
conus medullaris.
1011
FIGURE 21-16. Sagittal T2 in a different patient shows a large,
heterogeneous dermoid with a small amount of layering blood (arrow).
1012
FIGURE 21-17. Midsagittal T2, in a different patient with fever, shows a
complex and large dermoid with a dermal sinus tract.
1013
FIGURE 21-18. Corresponding postcontrast T1 shows abnormal, diffuse,
and marked enhancement of the lesion.
SUGGESTED READING
Thompson DN. Spinal inclusion cysts. Childs Nerv Syst 2013;29:1647–1655.
1014
Lipomas
Key Facts
A filar lipoma is the most common intraspinal lipoma, occurs in 1%
to 6% of the population, and is an incidental and asymptomatic
finding in 95% of patients.
With a filar lipoma, the filum terminale is thickened (generally, its
normal size should not exceed that of neighboring nerve roots) and
has fat within it.
In the presence of a filar lipoma, if the conus medullaris is normal in
position, then the findings are probably not clinically significant;
however, if the conus medullaris is abnormally low, consider the
diagnosis of tight filum terminale syndrome where patients most
commonly present with urological or bowel complains, lower
extremity pain, and back pain.
Intradural lipomas are rare and are mostly found in the cervical region
in children and thoracic region in adults but may occur anywhere in
the spine; most become symptomatic by 5 years of age and result in
paresis, spasticity, sensory loss, weakness, and bowel/bladder
dysfunction.
Main differential diagnosis: dermoid/epidermoid and teratoma.
1015
FIGURE 21-19. Sagittal T1 shows a filar lipoma (arrow) with a normal
termination of the conus medullaris.
1016
FIGURE 21-20. Midsagittal T1, in a different patient, shows a bright
intraspinal lipoma inseparable from the dorsal surface of a mildly low-
positioned conus medullaris. The bones are intact.
1017
FIGURE 21-21. Corresponding fat-suppressed T2 shows loss of signal
from the lipoma.
1018
FIGURE 21-22. Sagittal CT in a different patient shows a large cervical
lipoma with fat density encroaching on the posterior fossa. There has been
prior occipitocervical decompression.
1019
FIGURE 21-23. Corresponding sagittal noncontrast T1 shows the lipoma
to have the same signal as that of the subcutaneous fat.
1020
FIGURE 21-24. Sagittal noncontrast T1 in a different patient shows a
large thoracic lipoma with evidence of prior surgery.
SUGGESTED READINGS
Cools MJ, Al-Holou WN, Stetler WR Jr, et al. Filum terminale lipomas: imaging
1021
prevalence, natural history, and conus position. J Neurosurg Pediatr
2014;13:559–567.
Unsinn KM, Geley T, Freund MC, Gassner I. US of the spinal cord in newborns:
spectrum of normal findings, variants, congenital anomalies, and acquired
diseases. RadioGraphics 2000;20:923–938.
Lipomyelomeningocele and Lipomyelocele

Key Facts
Lipomyelomeningocele and lipomyelocele arise from premature
disjunction of the neural tube from the cutaneous ectoderm resulting
in a midline bone spinal defect covered by skin that may have a
dermal sinus, hairy patch, or hemangioma above the intergluteal fold.
They account of 16% for closed spinal dysraphisms.
In a lipomyelocele, meninges and neural placode are located at the
level of a spina bifida but covered by a lipoma and skin.
In a lipomyelomeningocele, the meninges and neural placode
protrude through a bone opening but are covered by a lipoma and
skin; this lipoma is in direct contact with the surface of the neural
placode, and the patients have a prominent mass in the low back.
They have a better prognosis than do open dysraphisms and are not
associated with Chiari II because there is no CSF leak.
Both lesions may be detected at birth or be mostly clinically silent
and not found until adulthood.
1022
FIGURE 21-25. Midsagittal T1 shows a lumbosacral fatty mass with
continuation of subcutaneous fat into the spinal canal via a spina bifida.
The lipoma is intimately associated with the dysplastic and inferiorly
positioned cord.
1023
FIGURE 21-26. Axial T2, in the same patient, shows intraspinal
extension (star) of subcutaneous lipoma.
1024
FIGURE 21-27. Axial T2 cephalad to Figure 21-26 shows a placode
(arrow) and the closely associated lipoma (wavy arrow) posteriorly.
1025
FIGURE 21-28. Midsagittal T1, in a different patient, shows cord (wavy
arrow) extending into sac (C) as well as a syrinx (arrow).
1026
FIGURE 21-29. Midsagittal ultrasound, in a different patient, shows an
abnormally wide lumbosacral canal, low-lying and tethered cord, and
herniation of neural elements and sac into the posterior soft tissues.
1027
FIGURE 21-30. Corresponding axial ultrasound demonstrates a large
posterior lipoma (arrows) with a portion of the sac.
SUGGESTED READING
Rufener S, Ibrahim M, Parmar HA. Imaging of congenital spine and spinal cord
malformations. Neuroimaging Clin N Am 2011;21:659–676.
Myelomeningocele and Myelocele

Key Facts
Myelomeningocele and myelocele are disorders of primary
neurulation that result in midline bone defects through which
meninges and/or neural placode (unfolded spinal cord) are visible
(open dysraphism); most are lumbar in location and discovered by in
utero sonography or MRI.
In a myelomeningocele (99% of open dysraphisms and 0.1% of live
1028
births), the neural placode and meninges protrude above the skin; it is
almost always seen in the context of a Chiari II malformation and is
generally sporadic but may affect subsequent siblings.
In a myelocele (less common), the neural placode is flush with the
skin.
Nerves along the ventral surface of the neural placode are frequently
stretched, and there may be variable degrees of hydromyelia in the
cord above.
Both entities are untethered and closed at birth, and imaging is done
for recurrent symptoms.
For practical purposes after repair of one of these defects, all spinal
cords are retethered (due to scar), and imaging studies are mainly
used to exclude other abnormalities, such as syringomyelia (30% to
75%), diastematomyelia (30%), lipomas, dermoids, and epidermoids.
1029
FIGURE 21-31. Midsagittal T1 shows open defect in lumbosacral region
with a CSF-filled sac also containing nerve roots that terminate in placode
(arrow). The cord is dysplastic and extends to the level of dysraphism.
1030
FIGURE 21-32. Corresponding T2 shows similar findings.
1031
FIGURE 21-33. Sagittal in utero T2 shows large CSF-filled sac (arrows)
containing nerve roots superiorly. The sac herniates through a spina bifida.
Hydrocephalus and polyhydramnios are present.
1032
FIGURE 21-34. Axial in utero T2 at the level of the fetal pelvis shows a
myelomeningocele (arrow).
1033
Figure 21-35. Corresponding sagittal T2 demonstrates the
myelomeningocele (arrow) and Chiari II malformation with a small
posterior fossa and descent of the brainstem and cerebellar vermis.
SUGGESTED READING
1034
Huisman TA, Rossi A, Tortori-Donati P. MR imaging of neonatal spinal
dysraphia: what to consider? Magn Reson Imaging Clin N Am 2012;20:45–61.
1035
CHAPTER 22
Degenerative Spine
Annular Fissures
Key Facts
Most are found in the lumbar spine in middle-aged and older adults
but may occur at any age.
They are seen as small linear areas of high T2 signal in the posterior
band of annulus fibrosus.Fissures are classified as concentric (if
parallel to the peripheral contour of the disc), radial (if they extend
radially from the nucleus pulposus to or through the annulus, may be
horizontal, vertical, or oblique), and transverse (horizontally oriented,
may be limited from the peripheral annulus).
Annular fissures may enhance after contrast administration
(representing ingrowth of granulation tissue).
They may be asymptomatic or produce symptoms by virtue of release
of inflammatory substances that irritate nerve endings.
It is better to avoid using the term “tear” as it may have legal
implications.
1036
FIGURE 22-1. Sagittal T2 shows a small bright transverse posterior
annular fissure (arrow) at L4-L5.
1037
FIGURE 22-2. Sagittal postcontrast T1, in a different patient, shows
enhancing transverse annular fissure (arrow) at L5-S1.
1038
FIGURE 22-3. Sagittal T2 in a different patient shows a concentric
annular fissure at L4-L5 with loss of disc height and signal due to
degeneration.
SUGGESTED READING
Brinjikji W, Luetmer PH, Comstock B, et al. Systematic literature review of
imaging features of spinal degeneration in asymptomatic populations. AJNR
Facet Cysts
1039
Key Facts
They are of the following types (may not be possible or practical to
differentiate among them; all require similar treatment):
1. True synovial cysts: contain fluid, communicate with joint space,
and are lined by synovium.2. Pseudosynovial cysts: contain fluid,
communicate with joint space, and are lined by fibrous capsule.3.
Ganglion cysts: contain fluid, do not communicate with joint space,
and are lined by fibrous capsule.
Are associated with a degenerated facet joint (may be unilateral and
rarely bilateral especially when spondylolysis is present).
Filled with fluid, gelatin-like substance, blood, or air (which accounts
for varying signal intensities). T2 hyperintense cysts (more fluid-like)
may be easier to treat percutaneously.
Margins may be calcified or erode adjacent bone, and the walls may
enhance.
Most occur at the L3-L5 levels (75% at L4), but occasionally, they
may be found at multiple levels. They are rare in the cervical spine
where they most often arise at the C1-C2 level and extremely
uncommon in the thoracic spine.
There may be adjacent facet joint effusions secondary to degenerative
changes.
Main differential diagnosis: migrated disc fragment, nerve sheath
tumor, and thick ligamentum flavum.
1040
FIGURE 22-4. Sagittal postcontrast T1 shows a synovial cyst at L4-L5
with capsular enhancement (arrow).
1041
FIGURE 22-5. Midsagittal T2, in a different patient, shows L3-L4
synovial cyst with blood level.
1042
FIGURE 22-6. Axial CT, in a different patient, shows right-sided synovial
cyst containing gas.
1043
FIGURE 22-7. Midsagittal T2, in a different patient, shows synovial cyst
(arrow) posterior to the dens with mass effect on the cervicomedullary
junction.
1044
FIGURE 22-8. Axial T2 in a different patient shows a synovial cyst
(arrow) exerting mass effect on the traversing nerves within the left canal
and subarticular zone.
1045
FIGURE 22-9. Midsagittal T2, in a different patient, shows a synovial
cyst projecting posteriorly from the L4-L5 facet joint (arrow ). Note facet
joint effusion at this level.
SUGGESTED READINGS
1046
Cambron SC, McIntyre JJ, Guerin SJ, Li Z, Pastel DA. Lumbar facet joint
synovial cysts: does T2 signal intensity predict outcomes after percutaneous
rupture? AJNR Am J Neuroradiol 2013;34:1661–1664.
Khalatbari K, Ansari H. MRI of degenerative cysts of the lumbar spine. Clin
Radiol 2008;63:322–328.
Herniated Disc
Key Facts
Degenerative lumbar disc disease is seen in 28% of population by age
18 and 85% to 95% after age 50. In the cervical spine, changes are
present in 25% of population <40 years of age and in 60% of
population >60 years of age. Low T2 signal intensity of discs
probably reflects loss of proteoglycans.
Most lumbar herniated discs occur at L4-L5 and L5-S1 and are
slightly eccentric due to median ligament. Most cervical herniations
are central and occur at C5-C6 and C6-C7.
Disc herniations are classified as protrusions (the base of the
herniated disc is larger than the amount of disc material extending
beyond the disc space) or extrusions (distance of the disc material
extending beyond the disc space is greater than the base of the
herniation in any one plane).
A sequestered disc is an extrusion that has lost continuity with the
parent disc and can become migrated if displaced away from its
parent site.
Herniated disc implies a tear in the annulus fibrosus (small linear area
of high T2 signal) with posterior protrusion/extrusion of nucleus
pulposus, which may be contained by an intact posterior longitudinal
ligament (subligamentous) or be accompanied by a tear of that
ligament. Extrusions can separate from the parent disc and become
“sequestered” and migrate. Occasionally, discs can erode the dura and
become intrathecal.
In the cervical spine, GRE sequences are useful to differentiate disc
(bright) versus osteophyte (dark). Postcontrast sequences, although
not routinely indicated for degenerative spines, can best delineate the
neural foramina due to enhancement of the prominent cervical venous
plexus.
In the lumbar spine, central and slightly eccentric herniated discs
1047
affect the roots exiting at the level immediately below.
Ninety percent of individuals with disc herniations show clinical
improvement after 8 to 10 weeks of symptomatic treatment, 50%
show significant reduction in size of herniation by MRI, and 8% to
10% will get worse.
Residual or recurrent discs are a common cause of failed back
syndrome; most important imaging aspect is to differentiate these
from scar, which enhances markedly, while herniated disc does not
(MRI >90% accurate, although discs may enhance on delayed
imaging due to ingrowth of granulation tissue).
FIGURE 22-10. Midsagittal T2 shows a subligamentous disc herniation at

L5-S1.
1048
FIGURE 22-11. Axial T2, in the same patient, shows a right central and
subarticular disc protrusion resulting in compression of the right S1 nerve
root (arrow).
1049
FIGURE 22-12. Midsagittal T2 image, in a different patient, shows a disc
extrusion at L5-S1. Note torn inferior annulus and posterior longitudinal
ligament and that disc fragment is brighter than the parent one indicating
edema and thus acuteness.
1050
FIGURE 22-13. Axial GRE image in a different patient shows a bright
right central, subarticular, and foraminal disc protrusion.
1051
FIGURE 22-14. Midsagittal T2, in a different patient, showing
sequestered disc fragment in ventral epidural space posterior to S1.
1052
FIGURE 22-15. Axial postcontrast T1 in a different patient with prior
surgery shows peripheral enhancement (arrow) corresponding to
granulation tissue surrounding a large nonenhancing recurrent disc
fragment (star).
SUGGESTED READINGS
Bittane RM, de Moura AB, Lien RJ. The postoperative spine: what the spine
surgeon needs to know. Neuroimaging Clin N Am 2014;24:295–303.
Fardon DF, Williams AL, Dohring EJ, Murtagh FR, Gabriel Rothman SL, Sze
GK. Lumbar disc nomenclature: version 2.0: recommendations of the
combined task forces of the North American Spine Society, the American
Society of Spine Radiology and the American Society of Neuroradiology.
Spine J 2014;14:2525–2545.
Ossified Posterior Longitudinal Ligament

(OPLL)
1053
Key Facts
This entity is more common in Japan, where it affects 2% to 4% of
the population (most frequently males) generally between 60 and 70
years of age.
OPLL represents heterotopic bone formation; the etiology has not
been entirely elucidated, but there are suspected genetic factors and
repeated mechanical stress may play a role.
It is associated with diffuse idiopathic skeletal hyperostosis (DISH) or
ankylosing spondylitis.
OPLL can be continuous or segmental and may produce spinal cord
compression (in general, cervical spinal canal stenosis occurs with an
anteroposterior diameter of <12 mm) and result in a myelopathy.
It affects the C4-C6 levels most commonly.
The incidence of spinal cord injury following trauma (even minor
one) is increased in patients with this disease.
Main differential diagnosis: calcified disc herniations, en plaque
meningioma, dural calcification in hypercalcemia (particularly related
to hemodialysis), and epidural hematoma.
1054
FIGURE 22-16. Midsagittal T1 shows a thick posterior longitudinal
ligament that is somewhat bright probably related to ossification with
presence of fatty marrow.
1055
FIGURE 22-17. Axial GRE, in the same patient, shows thick ligament
(arrow) resulting in marked stenosis and cord compression.
1056
FIGURE 22-18. Midsagittal CT reformation, in a different patient, shows
continuous OPLL.
1057
FIGURE 22-19. Axial CT, in the same patient shown in Figure 22-18,
shows the marked ossification of the PLL.
1058
FIGURE 22-20. Midsagittal CT reformation in a different patient shows
segmental posterior longitudinal ligament ossification and findings of
DISH.
1059
FIGURE 22-21. Midsagittal T2 in a different patient shows continuous
OPLL with mass effect on the spinal cord (arrowheads).
SUGGESTED READING
Matsunaga S, Sakou T. Ossification of the posterior longitudinal ligament of the
1060
cervical spine: etiology and natural history. Spine (Phila Pa 1976)
2012;37:E309–E314.
Paget Disease
Key Facts
Disorder of uncertain etiology characterized by excessive bone
remodeling and overgrowth.
It affects 3% of the population >40 years of age, although its
prevalence seems to be declining over the years.
Paget disease may be solitary or involve multiple bones.
Multiple sites include mainly the spine (75%), skull (65%), and pelvis
(40%).
Most common symptoms include pain, motor weakness,
incontinence, compressive neuropathies (due to osseous expansion),
and cord compression (from superimposed fractures).
In the initial osteolytic phase, MRI shows fibrous conversion of
marrow, which contains multiple enlarged vessels; these changes
disappear in the mixed phase, and finally, in the osteosclerotic phase,
the bone marrow returns to normal, and bone cortices are thickened.
New onset of pain in a bone involved by Paget disease should raise
the possibility of sarcomatous degeneration.
Main differential diagnoses: metastasis, multiple hemangiomas,
postradiation changes, and lymphangiomatosis.
1061
FIGURE 22-22. Lateral radiograph shows enlargement and sclerosis of
L2 (arrow).
1062
FIGURE 22-23. Midsagittal T1, in the same patient, shows patchy signal
and enlargement of L2 (arrow) and of L1 (arrowhead).
1063
Paget involvement of C2 including its posterior elements.
1064
FIGURE 22-25. Corresponding T1 shows an enlarged and low-signal C2.
1065
FIGURE 22-26. Axial CT in a different patient shows coarsened
trabeculae and mild cortical thickening of the L3 vertebra.
1066
FIGURE 22-27. Corresponding sagittal CT shows expansion and
“squaring” of the vertebral body (arrow). Note loss of cortical definition in
the posterior elements (arrowhead).
SUGGESTED READINGS
1067
Smith SE, Murphey MD, Motamedi K, Mulligan ME, Resnik CS, Gannon FH.
From the archives of the AFIP. radiologic spectrum of Paget disease of bone
and its complications with pathologic correlation. Radiographics
2002;22:1191–1216.
Theodorou DJ, Theodorou SJ, Kakitsubata Y. Imaging of Paget disease of bone
and its musculoskeletal complications: review. AJR Am J Roentgenol
2011;196:S64–S75.
Schmorl Nodes
Key Facts
Represent herniation of nucleus pulposus into endplate (which may
be “soft” or not).
Most are found in superior endplate and clinically asymptomatic or
present with self-limiting back pain. They are seen in 30% to 40% of
thoracolumbar spine MRI studies and are multiple in 14% of cases.
Most are found between T7 and L2.
They may form after an endplate fracture.
They occur with equal frequency in the young and elderly.
Schmorl nodes may show contrast enhancement acutely.
Occasionally, they may be large and “cyst-like.” They can be
considered “giant” when occupying at least one half of the vertebral
body height; these are more commonly painful.
Main differential diagnosis: endplate fracture, discitis, and metastasis.
1068
FIGURE 22-28. Midsagittal T2 shows central discontinuity of the inferior
endplate of L3 with high signal in that vertebral body.
1069
FIGURE 22-29. Corresponding postcontrast T1 shows a “giant” Schmorl
node with peripheral enhancement.
1070
FIGURE 22-30. Midsagittal noncontrast T1 shows nonspecific area of
low signal in inferior aspect of L3 (indistinguishable from tumor) in a
patient with acute back pain.
1071
FIGURE 22-31. Corresponding postcontrast T1 shows typical enhancing
Schmorl node.
1072
FIGURE 22-32. Midsagittal T2, in a different patient, shows a giant
“cystic” L4 Schmorl node.
SUGGESTED READING
Pfirrmann CW, Resnick D. Schmorl nodes of the thoracic and lumbar spine:
1073
radiographic-pathologic study of prevalence, characterization, and correlation
with degenerative changes of 1,650 spinal levels in 100 cadavers. Radiology
2001;219:368–374.
Spondylolysis and Spondylolisthesis,

Lumbar Spine
Key Facts
Spondylolysis is a cleft (with or without fibrous union) of the pars
interarticularis of a vertebra; most common levels are L5 and L4;
most are bilateral; they may be congenital or posttraumatic in nature,
are found in 1% to 5% of population, and are better seen on oblique
plain films, axial CT, and far lateral sagittal MRI. If unilateral, the
opposite side may show significant sclerosis. L5 hypoplasia may
predict the presence of bilateral spondylolysis.
Spondylolisthesis refers to displacement (posterior or anterior) of a
vertebra with respect to adjacent ones; it may be secondary to
ligamentous laxity associated with degenerative changes (particularly
facet hypertrophy) or to spondylolysis (especially in young persons)
or be a sequela of trauma; L4 through S1 are the most common
levels; on midsagittal MRI, the anteroposterior diameter of the spinal
canal is widened in spondylolisthesis secondary to spondylolysis but
not in cases due to degenerative facet disease.
1074
FIGURE 22-33. Sagittal CT in a young patient demonstrates a cleft in an
L5 pars interarticularis (arrow).
1075
FIGURE 22-34. Axial CT in the same patient shows pars interarticularis
defects bilaterally (arrow).
1076
grade 1 anterior spondylolisthesis of L4 on L5 and widened canal at this
level.
1077
FIGURE 22-36. Axial CT in a baby with bilateral L5 pars defects.
1078
FIGURE 22-37. Axial CT, in a different patient, shows left-sided pars
defect with contralateral sclerosis due to stress.
1079
FIGURE 22-38. Sagittal T2, in a different patient, shows a cleft in an L5
pars interarticularis (arrow).
SUGGESTED READINGS
Park JS, Moon SK, Jin W, Ryu KN. Unilateral lumbar spondylolysis on
1080
radiography and MRI: emphasis on morphologic differences according to
involved segment. AJR Am J Roentgenol 2010;194:207–215.
Viana SL, Viana MA, de Alencar EL. Atypical, unusual, and misleading imaging
presentations of spondylolysis. Skeletal Radiol 2015;44:1253–1262.
1081
CHAPTER 23
Infection Inflammation
Ankylosing Spondylitis (Seronegative

Spondyloarthropathy)
Key Facts
This disease occurs predominantly in young men and is associated
with histocompatibility antigen B27.
Ankylosing spondylitis is found in 1.4% of the population
(particularly white Europeans or individuals of European descent).
Sacroiliitis is often the first manifestation followed by ankylosis of
the lumbar and thoracic spine and ligamentous laxity (atlantoaxial
subluxation), fractures, and epidural hematoma.
Complications: fractures (“banana” type, often with minimal trauma),
atlantoaxial subluxation, spontaneous epidural hematoma, erosive
arachnoiditis with cauda equina syndrome, spinal cord infarctions,
and destructive noninfectious process (amyloidosis).
Main differential diagnoses: DISH, juvenile rheumatoid arthritis,
infectious sacroiliitis, and retinoid-induced changes.
1082
FIGURE 23-1. Lateral cervical spine radiograph shows fusion of vertebral
bodies by anterior syndesmophytes. The facet joints are also diffusely
fused.
1083
FIGURE 23-2. Midsagittal T2, in the same patient, shows vertebral body
fusion and partial disc reabsorption. Note the MRI correlate of the
Romanus lesion or “shiny corner” (arrow).
1084
FIGURE 23-3. Axial T2, in the same patient, shows erosive changes in
bone and nerve root clumping secondary to arachnoiditis.
1085
atlantoaxial dislocation and fracture with severe displacement of C5
through C7 involving the disc spaces.
1086
FIGURE 23-5. Sagittal CT in a different patient shows fracture through
the L4-L5 intervertebral disc.
1087
FIGURE 23-6. Coronal CT in a different patient shows fusion of the
vertebral bodies with fracture across the T6-T7 disc.
SUGGESTED READING
Wang YF, Teng MM, Chang CY, Wu HT, Wang ST. Imaging manifestations of
1088
spinal fractures in ankylosing spondylitis. AJNR Am J Neuroradiol
2005;26:2067–2076.
Arachnoiditis
Key Facts
Arachnoiditis affects all three meningeal layers; therefore,
“arachnoiditis” is a misnomer.
Occurs in 6% to 16% of postsurgical backs but may also be seen after
infections, intrathecal steroids or anesthesia, trauma, subarachnoid
hemorrhage, and postmyelography. Rare cases of familial spinal
arachnoiditis have been described.
Most common symptoms: chronic pain radiating to legs, hypesthesia,
paraparesis, and occasionally paraplegia.
Types of arachnoiditis by myelography: (1) featureless thecal sac
appearance (not visible on MRI); (2) multiple localized filling
defects, nerve roots may be clumped or adherent to the walls of thecal
sac (visible on MRI); and (3) soft tissue mass (fibrosing or ossifying
types) filling the spinal canal (lack of enhancement on MRI suggests
arachnoiditis rather than tumor).
Chronic arachnoiditis may result in secondary syringomyelia.
Main differential diagnoses: postradiation, metastases, and
carcinomatosis.
1089
FIGURE 23-7. Axial T2 shows peripheral displacement of nerve roots,
which are adherent to the thecal sac.
1090
FIGURE 23-8. Sagittal STIR in the same patient shows an “empty sac”
appearance below the level of L3.
1091
FIGURE 23-9. Axial T2, in a different patient, shows clumped group of
nerve roots in the center of the thecal sac.
1092
FIGURE 23-10. Midsagittal T2, in the same patient, shows clumping of
all cauda equina nerve roots.
1093
FIGURE 23-11. Midsagittal postcontrast fat–suppressed T1, in a different
patient, shows thick and enhancing nerve roots. The patchy aspect of the
bone marrow was idiopathic.
SUGGESTED READING
1094
Fitt GJ, Stevens JM. Postoperative arachnoiditis diagnosed by high resolution fast
spin-echo MRI of the lumbar spine. Neuroradiology 1995;37:139–145.
Guillain-Barré Syndrome
Key Facts
Guillain-Barré is an inflammatory disorder affecting the peripheral
nervous system but also the anterior gray matter horns in the spinal
cord and rarely the brainstem and cranial nerves too. West Nile virus
can result in an identical appearance.
In 65% of patients, it is preceded by a respiratory or gastrointestinal
tract infection (most commonly Campylobacter) 1 to 6 weeks before
the onset of neurological symptoms (progressive ascending paralysis
for 2 to 30 days and then plateau with improvement occurring in the
next 2 weeks).
Most patients are under 18 years of age and >65% recover
completely.
It is pathologically characterized by demyelination and inflammation.
Main differential diagnoses: infectious (including viral) and
carcinomatous meningitis, postradiation, chronic inflammatory
demyelinating polyneuropathy, vasculitis, and arachnoiditis.
1095
FIGURE 23-12. Midsagittal postcontrast T1 shows enhancement of
multiple nerve roots.
1096
FIGURE 23-13. Axial postcontrast T1, in the same patient, shows
enhancement of anterior nerve roots in cauda equina.
1097
enhancement of anterior roots (arrows) in cervical region.
1098
preferential enhancement of dorsal nerve roots.
1099
thickening and enhancement of both ventral and dorsal nerve roots.
SUGGESTED READING
Yikilmaz A, Doganay S, Gumus H, et al. Magnetic resonance imaging of
childhood Guillain-Barre syndrome. Childs Nerv Syst 2010;26:1103–1108.
Hypertrophic Polyneuropathies
Key Facts
1100
In this group of disorders, histologically, there is demyelination and
inflammation, which subside and lead to remyelination producing the
so-called “onion” bulb appearance of nerves.
Common disorders included in this category are chronic
inflammatory demyelinating polyneuropathy (probably the most
common, immune-mediated and related to Guillain-Barré syndrome,
more common in males with a mean age of 50 years), Charcot-Marie-
Tooth disease (also relatively common, inherited, and occurring
mostly in young females), and Dejerine-Sottas disease (more severe
subtype of Charcot-Marie-Tooth disease with an infantile onset).
Clinical symptoms include atrophy of the feet, legs, arms, and trunk,
ataxia, and weakness.
Main differential diagnoses: viral neuritis, perineural tumor spread,
and plexiform neurofibromas.
1101
FIGURE 23-17. Sagittal postmyelogram CT reformation shows very thick
lumbosacral nerve roots (arrows) in a patient with Charcot-Marie-Tooth
disease.
1102
FIGURE 23-18. Axial postmyelogram CT shows very thick nerve roots in
the same patient.
1103
FIGURE 23-19. Coronal fat–suppressed T2 in a different patient with the
same disease shows diffuse thickening of the left brachial plexus with
increased signal.
1104
FIGURE 23-20. Coronal T2 in a different patient with chronic
inflammatory demyelinating polyneuropathy demonstrates diffuse
thickening of cervical nerve roots (arrows).
1105
FIGURE 23-21. Axial T2 in a different patient with the same disease as in
Figure 23-4 shows thickening of cervical nerve roots with a nodular
appearance (arrows).
1106
thickening and increased enhancement of the V3 branches of the
trigeminal nerves bilaterally (arrows).
SUGGESTED READINGS
Chhabra A, Thakkar RS, Andreisek G, et al. Anatomic MR imaging and
functional diffusion tensor imaging of peripheral nerve tumors and tumorlike
conditions. AJNR Am J Neuroradiol 2013;34:802–807.
Tanaka K, Mori N, Yokota Y, Suenaga T. MRI of the cervical nerve roots in the
diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy: a
single-institution, retrospective case–control study. BMJ Open
2013;3:e003443.
1107
Rheumatoid Arthritis
Key Facts
This disease is found mostly in females age 25 to 55 years.
It affects the cervical region in 50% to 90% of patients and leads to
C1-C2 instability in 25% of them (this involvement carries a poor
prognosis as it may result in spinal cord compression and sudden
death).
Pannus surrounding and eroding the dens is seen in 14% to 35% of
patients. Pannus in rheumatoid arthritis does not usually calcify; if
calcifications are present, consider calcium pyrophosphate (or
hydroxyapatite) deposition disease (CPPD) and gout.
Other symptoms are basilar invagination, paresthesias, paresis, and
muscle atrophy.
Main differential diagnoses: CPPD, ankylosing spondylitis, gout,
infection, and hemodialysis (amyloid) arthropathy.
1108
FIGURE 23-23. Axial CT shows prominent pannus (arrow) surrounding
the dens with deformity and erosive changes also involving the anterior C1
arch.
1109
FIGURE 23-24. Midsagittal T2, in a different patient, shows pannus
surrounding a deformed dens with mass effect on the cervicomedullary
junction.
1110
FIGURE 23-25. Corresponding axial GRE image shows high-signal
pannus with erosion along the right aspect of the dens and disruption of the
transverse ligament.
1111
FIGURE 23-26. Midsagittal T1 in a different patient shows increased
atlantodental space due to posterior dens subluxation. Pannus is seen
anterior to the dens.
1112
marked pannus in dens region.
1113
FIGURE 23-28. Corresponding postcontrast T1 shows peripheral pannus
enhancement and lack of dens visualization that has been completely
eroded.
SUGGESTED READING
1114
Jurik AG. Imaging the spine in arthritis—a pictorial review. Insights Imaging
2011;2:177–191.
Spondylitis, Discitis, and Epidural Abscess,

Pyogenic
Key Facts
Most spinal infections are caused by Staphylococcus aureus (60%,
even in patients with AIDS), Enterobacter spp. (30%), Escherichia
coli, Pseudomonas, and Klebsiella organisms.
Most discitis and osteomyelitis arise from hematogenous
dissemination (e.g., drug users and immunosuppressed patients),
ascending route (genitourinary tract manipulations), and direct
inoculation (traumatic injuries and postsurgical).
In adults, the infection begins in the vertebral endplates, while in
children, the infection begins in the disc.
Discitis and osteomyelitis are usually seen in men between the sixth
and seventh decades of life; the presenting symptoms are nonspecific
(e.g., fever, pain, and elevated erythrocyte sedimentation rate).
Main differential diagnoses: degenerative changes, Schmorl node,
trauma, hemodialysis (amyloid) arthropathy, tuberculosis, brucellosis,
and fungus.
Epidural abscess can occur due to extension of discitis/osteomyelitis
(80%) or direct hematogenous spread; it is seen in middle-aged men
and most commonly affects the lumbar and thoracic spine.
An epidural abscess has a nonenhancing fluid component (pus) and
peripheral enhancement; a diffusely enhancing extradural collection
represents a phlegmon.
Epidural abscesses can in turn lead to osteomyelitis or paraspinal
abscesses (frequently in the psoas muscle).
1115
FIGURE 23-29. Sagittal T2 shows bright L1-L2 disc with erosion of
endplates and edema in bone marrow.
1116
FIGURE 23-30. Corresponding postcontrast T1 shows disc enhancement
and phlegmon in epidural space compressing the cauda equina.
1117
FIGURE 23-31. Sagittal noncontrast CT in a different patient shows
extensive destruction of the L4-L5 vertebral bodies and endplates related
to discitis and osteomyelitis.
1118
FIGURE 23-32. Parasagittal postcontrast fat–suppressed T1, in a different
patient, shows multiple loculated epidural abscesses.
1119
FIGURE 23-33. Axial postcontrast T1 in a different patient shows ventral
epidural abscess (arrow) compressing the thecal sac and loculated
paraspinal abscesses (arrowheads).
1120
diffusely thick and enhancing epidural tissues without fluid collections,
thus a phlegmon.
SUGGESTED READINGS
Esendagli-Yilmaz G, Uluoglu O. Pathologic basis of pyogenic, nonpyogenic, and
other spondylitis and discitis. Neuroimaging Clin N Am 2015;25:159–161.
Tali ET, Oner AY, Koc AM. Pyogenic spinal infections. Neuroimaging Clin N
Am 2015;25:193–208.
1121
Tuberculosis
Key Facts
The incidence of all tuberculosis is increasing due to immigration and
AIDS.
Spinal tubercular involvement is more common in males, aged 4 to 50
years.
Most common locations are thoracic, lumbar, cervical, and sacral
regions.
Infection begins in the anteroinferior vertebral body and extends
under ligaments skipping the discs; skip lesions are not uncommon
and are probably related to spread along the valveless Batson venous
plexus.
Occasionally, tuberculosis involves the posterior elements in
combination with vertebral body involvement or isolated.
At diagnosis, more than one level is involved in nearly 50% of
patients.
It is accompanied by significant soft tissue abnormalities such as
paraspinal abscess (“cold”) in about 65% of patients, calcification of
which is virtually diagnostic of tuberculosis.
Gibbus deformity (short-segment kyphosis) is not an uncommon
sequela of spinal tuberculosis (particularly thoracic).
Main differential diagnoses: brucellosis, fungus, hemodialysis
(amyloid) arthropathy, and neurogenic arthropathy.
1122
FIGURE 23-35. Postcontrast T1 shows enhancement of the vertebral
bodies and an anterior paraspinal soft tissue phlegmon but no disc
enhancement.
1123
and extensive cold abscesses in paraspinal regions and ventral epidural
phlegmon.
1124
involvement mostly in ventral epidural space with erosion of posterior
superior aspect of L1.
1125
FIGURE 23-38. Midsagittal T2, in a different patient, shows Gibbus
deformity due to destruction of a lower thoracic vertebra and extensive
subligamentous disease (arrows).
1126
FIGURE 23-39. Sagittal T2 in a different patient shows liquefaction of the
L3-L4 disc, destruction of the adjacent endplates, posterior epidural
abscess, and compression of the cauda equina.
1127
FIGURE 23-40. Axial T2, in the same patient, shows abscesses in the
psoas and erector spinae muscles.
SUGGESTED READING
Kilborn T, Janse van Rensburg P, Candy S. Pediatric and adult spinal
tuberculosis: imaging and pathophysiology. Neuroimaging Clin N Am
2015;25:209–231.
1128
CHAPTER 24
Spine Tumors and
Tumor-like Conditions
Aneurysmal Bone Cyst (ABC)

Key Facts
ABCs comprise <2% of primary spinal bone tumors and are
histologically benign but locally aggressive. They usually occur in
patients younger than 30 years of age and are more common in
females.
About 20% of all ABCs occur in the spine (most frequently lumbar),
and the neural arch is more commonly (60%) involved than the
vertebral body (40%); ABCs may cross to adjacent vertebrae.
It is believed that about a third of them arise from other underlying
lesions such as chondroblastoma, giant cell tumor, osteoblastoma, and
fibro-osseous lesions.
Generally, they are discovered in adolescents and present with pain,
paraplegia, mass, radicular symptoms, and scoliosis or kyphosis.
They are expansile masses with a thin rim of bone, although there
may be cortical breach with extension into the soft tissues or spinal
canal narrowing; MRI and CT show a complex internal appearance
due to repeated intratumoral hemorrhages, blood–fluid levels are
nearly pathognomonic (but patients need to rest on their back for at
least 20 to 30 minutes before scanning for them to become obvious),
and there may be enhancement of the septations. These lesions are
1129
hypervascular and may need preoperative embolization.
Main differential diagnoses: osteoblastoma, telangiectatic
osteosarcoma, metastasis, giant cell tumor, and plasmacytoma.
FIGURE 24-1. Axial CT shows an expansile lesion involving the

posterior elements and pedicle of T10. Note thin bone shell around lesion.
1130
FIGURE 24-2. Axial T2 in a different patient shows an ABC with fluid
levels (arrow) resulting in marked spinal canal narrowing.
1131
FIGURE 24-3. Sagittal fat-saturated postcontrast T1 in the same patient
shows enhancement of the septations and encroachment of the lesion on
the adjacent neural foramina.
1132
FIGURE 24-4. Axial CT in a different patient shows an ABC in the
sacrum with only a very faint sclerotic rim (arrows).
1133
FIGURE 24-5. Corresponding axial fat-saturated T2 shows the
characteristic cystic spaces with numerous small fluid levels (arrow).
1134
FIGURE 24-6. Axial fat-saturated postcontrast T1 in the same patient
shows septal enhancement.
SUGGESTED READING
Ropper AE, Cahill KS, Hanna JW, McCarthy EF, Gokaslan ZL, Chi JH. Primary
vertebral tumors: a review of epidemiologic, histological, and imaging
findings. Part I: benign tumors. Neurosurgery 2011;69:1171–1180.
Spinal Cord Astrocytoma

Key Facts
Of spinal cord astrocytomas, >75% (in both children and adults) are
low grade histologically, and they are found more commonly in
males.
1135
They are the second most common primary spinal cord tumor after
ependymoma.
About half of them are of the pilocytic type (avidly enhancing and
relatively well circumscribed), and the remainder are infiltrative.
Most high-grade spinal astrocytomas are anaplastic, and
glioblastomas are rare.
Most spinal cord astrocytomas involve the cervicothoracic region.
Most spinal cord astrocytomas are discovered during the third to
fourth decades of life.
Early symptoms are nonspecific or nonlocalizing and include pain
and scoliosis.
Spinal cord astrocytomas are slow growing and result in bone
changes in 50% of cases.
Associated cysts are common (40%), and holocord involvement is not
rare. Hemorrhage is seen less frequently than with ependymomas.
About 90% of spinal cord astrocytomas enhance on postcontrast MRI.
Main differential diagnoses: ganglioglioma, ependymoma,
lymphoma, and transverse myelitis.
1136
FIGURE 24-7. Midsagittal postcontrast T1 shows expansion of
cervicothoracic spinal cord with patchy zones of enhancement.
1137
FIGURE 24-8. Corresponding T2 shows high-signal and cyst-like areas in
the expanded cord.
1138
FIGURE 24-9. Midsagittal T2, in a different patient, shows an infiltrative
expansile mass in the upper cervical cord.
1139
FIGURE 24-10. Midsagittal T2, in a different patient, shows a lesion in
the upper thoracic and lower cervical cord with extensive cystic changes
and a dark focus of hemorrhage.
1140
FIGURE 24-11. Corresponding postcontrast T1 shows the enhancing
component of the tumor.
1141
FIGURE 24-12. Axial postcontrast T1, in the same patient, shows that the
tumor involves nearly the entire spinal cord cross-sectional area.
SUGGESTED READINGS
Kim DH, Kim JH, Choi SH, et al. Differentiation between intramedullary spinal
ependymoma and astrocytoma: comparative MRI analysis. Clin Radiol
2014;69:29–35.
Seo HS, Kim JH, Lee DH, et al. Nonenhancing intramedullary astrocytomas and
other MR imaging features: a retrospective study and systematic review.
Chordoma
1142
Key Facts
Chordomas arise from notochordal remnants at the sacrum and
coccyx (50%) (most common primary sacral tumor), clivus (35%),
and cervical and thoracic regions (15%).
They are slow growing but locally aggressive, and metastases are
seen in 10% to 40% of patients.
Chordomas are generally found in males between the fifth and
seventh decades of life, although vertebral body chordomas occur
earlier in life.
They are usually lytic and destructive lesions more commonly located
at the midline presenting with pain, rectal dysfunction, mass, and
urinary incontinence.
CT shows that 30% to 70% contain amorphous calcifications, and
more than half may have a fibrous pseudocapsule.
Chordomas are usually lobulated and typically show bright T2 signal
with dark intervening septations and enhancement in a “honeycomb”
pattern (due to mucinous and gelatinous contents) although they may
also enhance diffusely. The chondroid type may show relatively low
signal intensity due to cartilaginous elements.
Main differential diagnoses: chondrosarcoma, giant cell tumor, and
metastasis.
1143
FIGURE 24-13. Axial CT shows a destructive mass in the sacrum
extending to the pelvis.
1144
FIGURE 24-14. Sagittal T2, in a different patient, shows a multilobulated
mass (arrow) in the distal sacrum.
1145
FIGURE 24-15. Axial fat-saturated T2 in a different patient shows a very
bright lobulated mass in a lumbar vertebra encroaching on the ventral
epidural space.
1146
FIGURE 24-16. Axial T2, in a different patient, demonstrates a large
clival chordoma with high T2 signal and dark internal septations. There is
elevation of the prevertebral muscles and mass effect on the cord and
brainstem.
1147
FIGURE 24-17. Corresponding axial postcontrast T1 shows
heterogeneous enhancement with some areas showing a “honeycomb”
pattern (arrows).
1148
FIGURE 24-18. Midsagittal postcontrast CISS, in the same patient, shows
disruption of the dura (arrows) with subarachnoid tumor extension.
SUGGESTED READING
Sciubba DM, Cheng JJ, Petteys RJ, Weber KL, Frassica DA, Gokaslan ZL.
Chordoma of the sacrum and vertebral bodies. J Am Acad Orthop Surg
2009;17:708–717.
Spinal Cord Cysts, Nonneoplastic

Key Facts
Hydromyelia refers to ependymal-lined dilatation of the central canal,
while syringomyelia refers to a spinal cord cavity lined by gliotic
tissue occurring separate from the central canal; however, it is not
clinically important to distinguish them apart.
1149
Most hydromyelias are congenital (associated with the Chiari I and II
malformations and basilar invagination), posttraumatic, or secondary
to tumors.
Hydromyelia occurs at any location, but congenital cysts tend to be
more common in cervical and upper thoracic regions.
Spinal cord cysts may occur above or below the level of prior trauma.
Most common symptoms include pain and temperature alterations,
weakness followed by atrophy, motor disturbances, spastic
paraparesis, and neurotrophic joints (especially the shoulder and
elbow).
A persistent terminal ventricle (“fifth” ventricle) is due to incomplete
involution of central cord cavitation during development; it is found
in 1% of newborns particularly premature ones; it is generally
asymptomatic and regresses during the first week of life but may
grow and produce symptoms of hydromyelia.
Main differential diagnoses: hemangioblastoma, ependymoma, and
astrocytoma.
1150
FIGURE 24-19. Midsagittal T2 shows a septated spinal cord cyst in a
patient with Chiari I malformation.
1151
FIGURE 24-20. Midsagittal T2 in a different patient with Chiari I
malformation shows some dephasing of intracavitary fluid signal (dark
areas in thoracic region) due to turbulence. Turbulence accompanied by
progressive or new symptoms may indicate growth of hydromyelia.
1152
FIGURE 24-21. Midsagittal sonogram in a baby shows a fluid–filled
terminal ventricle (arrow) and a thick lipomatous filum terminale
(arrowhead).
1153
FIGURE 24-22. Midsagittal T2 in a different adult patient shows a
persistent terminal ventricle (arrow).
SUGGESTED READING
Do-Dai DD, Brooks MK, Goldkamp A, Erbay S, Bhadelia RA. Magnetic
1154
resonance imaging of intramedullary spinal cord lesions: a pictorial review.
Curr Probl Diagn Radiol 2010;39:160–185.
Eosinophilic Granuloma
Key Facts
Eosinophilic granuloma is the localized and most common (70%)
form of histiocytosis of Langerhans (histiocytosis X).
It is characterized by infiltration of tissues with myeloid dendritic
cells, although the pathogenesis is unclear. It is rare and occurs most
commonly in children between 3 and 5 years of age.
Common sites of involvement are skull, mandible, spine, ribs, femur,
and humerus, and the disease is most commonly monostotic.
In the spine, eosinophilic granuloma predominantly affects the
vertebral body, and mechanical stress results in a “vertebra plana”
(sometimes, there may be restitution of the vertebral height after
healing); the discs are preserved, T2 signal tends to be normal or near
normal, and there is very little soft tissue mass associated with it.
Main differential diagnoses: metastasis (neuroblastoma),
telangiectatic osteosarcoma, and trauma.
1155
FIGURE 24-23. Lateral radiograph shows collapsed C5 resulting in
vertebra plana.
1156
FIGURE 24-24. Corresponding midsagittal T2 shows preserved signal
from involved vertebra and lack of associated soft tissue mass.
1157
FIGURE 24-25. Midsagittal CT reformation of cervicothoracic spine, in a
different patient, shows multiple lytic lesions in vertebrae.
1158
FIGURE 24-26. Axial CT, in the same patient, shows lesions of
permeative aspect.
1159
FIGURE 24-27. Midsagittal postcontrast T1 in a different patient shows
enhancement of a C7 lesion (arrow) with a small amount of epidural tissue
narrowing the spinal canal (arrowhead ).
1160
FIGURE 24-28. Coronal postcontrast T1 in a different patient shows an
enhancing lesion replacing the left lateral mass of C1 (arrow) and encasing
the vertebral artery.
SUGGESTED READING
1161
Khung S, Budzik JF, Amzallag-Bellenger E, et al. Skeletal involvement in
Langerhans cell histiocytosis. Insights Imaging 2013;4:569–579.
Spinal Cord Ependymoma

Key Facts
Most common (65%) primary tumor of the spinal cord, increased
incidence in patients with NF2.
Spinal ependymomas occur predominantly in the lower spinal cord,
conus medullaris, and filum terminale (at this location, it is typically
of the myxopapillary subtype).
They are predominantly found during the fifth to sixth decades of life
(patients are usually older than those with astrocytomas) with a slight
female predominance.
Present with only back pain, but occasionally patients have leg
weakness and sphincter dysfunction. Occasionally present with
superficial siderosis and even acute hemorrhage.
Spinal ependymomas are slow-growing tumors that may produce
bone scalloping; widening of the spinal canal is seen in 30% of cases.
About 64% of spinal cord ependymomas have evidence of prior
hemorrhage by MRI; they tend to be centrally located, and most show
well-defined margins (gross total resection is usually easier than
astrocytomas, although occasionally ependymomas may invade the
conus medullaris); T2 signal is variable and may be bright or dark if
calcifications and/or hemorrhage is present, and all enhance after
contrast administration.
Intratumoral cysts are more common than with astrocytomas.
Main differential diagnoses: intramedullary lesions, astrocytoma,
ganglioglioma, lymphoma, metastasis, hemangioblastoma, and
transverse myelitis; conal/filum lesions, nerve sheath tumors,
meningioma, astrocytoma, and paraganglioma.
1162
FIGURE 24-29. Midsagittal postcontrast T1 shows enhancing mass in
upper cervical cord and medulla. The spinal canal is slightly expanded.
1163
FIGURE 24-30. Midsagittal T2 in a different patient shows a cervical
ependymoma with a cystic component (arrow) and hypointensity due to
hemorrhage posteriorly (arrowhead).
1164
FIGURE 24-31. Corresponding midsagittal postcontrast T1 shows
enhancement of the solid component (arrow) and linear intrinsic
hyperintensity posteriorly in the area of hemorrhage.
1165
FIGURE 24-32. Midsagittal T2, in a different patient, shows classic
location and morphology of a myxopapillary ependymoma resulting in
scalloping of a few vertebral bodies.
1166
FIGURE 24-33. Midsagittal T2 in a different patient shows a smaller
myxopapillary ependymoma (arrow) with linear hypointensity in the
cauda equina (arrowhead) due to prior hemorrhage/ siderosis.
1167
FIGURE 24-34. Midsagittal postcontrast T1, in the same patient, shows
the mass to enhance very avidly.
SUGGESTED READING
Tarapore PE, Modera P, Naujokas A, et al. Pathology of spinal ependymomas: an
1168
institutional experience over 25 years in 134 patients. Neurosurgery
2013;73:247–255.
Vertebral Body Hemangioma

Key Facts
It is the most common primary tumor of the vertebrae.
They are found in 11% of the population; 70% are solitary, and 30%
are multiple; most common locations are the thoracic and lumbar
spine and slightly more common in females.
Vertebral body hemangiomas grow slowly and almost never produce
symptoms, but occasionally, they may lead to spinal cord
compression, fractures, or epidural hematomas.
Most of these benign tumors show high signal intensity on T1, may
remain of slightly high signal intensity on T2, and show contrast
enhancement.
Occasional aggressive hemangiomas show lower signal intensity on
both T1 (less fat) and T2 and are indistinguishable from metastases.
On CT and plain films, thick bone trabeculae (“corduroy pattern” on
sagittal reformation and “salt-and-pepper” or “polka-dot” pattern on
axial images) are typical.
Main differential diagnoses: metastasis, Paget disease, and
postradiation changes.
1169
FIGURE 24-35. Midsagittal noncontrast T1 shows a bright L3 vertebral
body with dark vertical linear striations.
1170
FIGURE 24-36. Sagittal CT reformation, in a different patient, shows
typical striate pattern in an upper thoracic vertebral body.
1171
FIGURE 24-37. Axial CT in a different patient shows the typical “polka-
dot” appearance of the thickened trabeculae (arrow).
1172
FIGURE 24-38. Axial CT in a different patient shows a large
hemangioma with thick trabeculae that has encroached on the spinal canal.
1173
FIGURE 24-39. Sagittal CT reformation, in the same patient, shows
involvement of the posterior elements.
1174
FIGURE 24-40. Axial T1 in a different patient with an “aggressive”
hemangioma shows the trabeculae as tiny black dots and involvement of
the epidural space with a “curtain sign” due to the presence of the midline
septum.
SUGGESTED READING
Thakur NA, Daniels AH, Schiller J, et al. Benign tumors of the spine. J Am Acad
Orthop Surg 2012;20:715–724.
Osteoid Osteoma
1175
Key Facts
They comprise 10% of all primary bone tumors, and 10% of them are
found in the spine.
Most are found in males, age 6 to 17 years.
Most common locations are the lumbar (60%), cervical (27%),
thoracic (12%), and sacral (1%) regions.
They are solitary lesions, and most arise in the pedicles, laminae,
facet joints, and spinous processes.
Most result in pain (worse at night and typically relieved by aspirin),
radiculopathies, gait abnormalities, painful scoliosis (as opposed to
idiopathic juvenile scoliosis, which is painless), painful torticollis,
and muscle atrophy.
When there is scoliosis, the lesion is always on the concave side of
the apex.
May produce inflammatory changes in surrounding soft tissues
including nerve roots.
Main differential diagnoses: osteoblastoma, stress fracture,
metastasis, and chronic osteomyelitis.
1176
FIGURE 24-41. Frontal radiograph shows lumbar dextroscoliosis and a
dense and expanded L4 pedicle (arrow).
1177
FIGURE 24-42. Axial CT, in the same patient, shows sclerosis
surrounding the lucent lesions, which contains centrally a dense nidus.
1178
FIGURE 24-43. Frontal bone scan study, in a different patient, shows
focal increased uptake (arrow) in T1.
1179
FIGURE 24-44. Axial CT, in the same patient, shows a left-sided pedicle
lucency with a faintly dense intralesional nidus.
1180
FIGURE 24-45. Sagittal CT in a different patient shows a lucent lesion
(arrow) at C3 with a central sclerotic nidus.
1181
FIGURE 24-46. Corresponding sagittal T2 shows the dark sclerotic focus
(arrow) and surrounding bone marrow edema.
SUGGESTED READING
Kan P, Schmidt MH. Osteoid osteoma and osteoblastoma of the spine. Neurosurg
1182
Clin N Am 2008;19:65–70.
Perineural (Tarlov) Cysts

Key Facts
Perineural cysts arise at junction of the dorsal nerve roots and nerve
ganglia between the endoneurium and perineurium.
They are most common in the sacrum (S2 and S3) but may be found
at any level in the spine (less common in thoracic and rare in cervical
regions).
The cysts may be solitary or multiple.
They may communicate freely with the subarachnoid space (and
therefore opacify immediately during myelography), have partial
communication (therefore fill slowly after myelography), or be
noncommunicating and expand owing to a ball–valve mechanism that
traps CSF (“valved cysts,” more likely to be symptomatic).
Approximately 1% is symptomatic. If expansile, perineural cysts may
produce pain as nerve roots traveling along the margins of the cyst
become compressed or due to irritation of periosteal pain fibers.
Spontaneous rupture may be a cause of headaches and intracranial
hypotension.
1183
FIGURE 24-47. Midsagittal T2 shows cysts projecting into the sacral
canal and a large one eroding S1.
1184
FIGURE 24-48. Axial T2, in a different patient, shows a nerve root sleeve
cyst involving the left S2 nerve root. Note nerve root (arrow) compressed
by cyst.
1185
FIGURE 24-49. Axial T1, in a different patient, shows multiple sleeve
cysts with a large one eroding the sacrum.
1186
FIGURE 24-50. Axial T2, in a different patient, shows foraminal and
extraforaminal cysts causing bone expansion with presumed nerve root
(arrow) in its center.
SUGGESTED READING
Lucantoni C, Than KD, Wang AC, et al. Tarlov cysts: a controversial lesion of
the sacral spine. Neurosurg Focus 2011;31:E14.
Spinal Schwannoma and Meningioma

Key Facts
1187
Schwannoma is the most common spine tumor; meningioma is
second (25%).
Both tumors are nearly always histologically benign and found in
middle-aged (40 to 60 years) women.
Schwannomas are found in the cervical region; meningiomas occur in
the thoracic (80%), cervical (15%), and lumbar (5%) regions.
Most schwannomas are both extradural and intradural (“dumbbell”
shaped), but 15% can be entirely extradural. Most meningiomas are
entirely intradural and lateral to the spinal cord.
By CT, 10% of meningiomas show calcifications.
Neurofibromas and less commonly schwannomas may show a
“target” appearance on T2, in neurofibromas due to a
fibrocollagenous core and in Schwannomas possibly due to the
relative distribution of Antoni A and Antoni B tissue. Schwannomas
may be occasionally cystic or hemorrhagic.
Spinal neurofibromas are more common in patients with NF1,
schwannomas occur in NF2 and NF3 (mixed), and spinal
schwannomatosis occur sporadically.
Malignant degeneration of neurofibromas occurs in 4% to 11% of
patients with NF1; malignant degeneration of schwannomas is
extremely rare and may have an aggressive appearance with bone
erosion. Meningiomas may also be malignant.
1188
FIGURE 24-51. Axial CT shows enlargement of the left neural foramen
(star) secondary to a nerve root schwannoma.
1189
FIGURE 24-52. Axial postcontrast T1 in a different patient shows an
enhancing intradural and extradural cervical schwannoma with a cystic
component (arrow).
1190
FIGURE 24-53. Coronal T2, in a different patient, shows a large thoracic
schwannoma (arrow) expanding the neural foramen (arrowhead) with a
predominant extradural component and cystic change.
1191
FIGURE 24-54. Midsagittal postcontrast T1 shows a primarily cystic
lumbar Schwannoma (arrow).
1192
a thoracic meningioma with compression of the spinal cord.
1193
a meningioma at the craniocervical junction with a dural tail that enhances
more avidly than the tumor and tapers peripherally (arrow).
SUGGESTED READING
Beall DP, Googe DJ, Emery RL, et al. Extramedullary intradural spinal tumors: a
pictorial review. Curr Probl Diagn Radiol 2007;36:185–198.
1194
Spinal Cord and Leptomeningeal
Metastases
Key Facts
These types of metastases occur in <1% of cancer patients, especially
those with primary tumors in the lung (>50%) and breast or those
with lymphoma, melanoma, colorectal carcinoma, head and neck
carcinoma, and leukemia.
The thoracic region is most commonly involved by both types of
metastases.
Spinal cord metastases from primary CNS tumors (mainly
medulloblastoma) spread via CSF pathways.
Tumors outside the CNS that may produce spinal cord metastases via
hematogenous spread include breast carcinoma, lung carcinoma,
melanoma, gastrointestinal tract carcinomas, and lymphoma, but they
can also be seen in up to 50% of patients with lymphoblastic and
myelogenous leukemias.
Patients usually have widespread disease, but spinal metastases may
occur without a known primary and may be asymptomatic.
Hemorrhage and necrosis are rare.
Tumors of the CNS that most commonly spread via CSF include
medulloblastoma, ependymoma, and glioma.
Faint enhancement along the ventral surface of the conus medullaris
may be normal vascular enhancement and should not be confused
with metastases; enhancement may also occur 4 to 6 weeks after
spinal or posterior fossa surgery.
Main differential diagnoses: for spinal cord lesions—primary tumors
and demyelinating disease; for leptomeningeal lesions—tuberculosis,
chemical meningitis, postsubarachnoid hemorrhage changes,
hypertrophic polyneuropathies, and Guillain-Barré syndrome.
1195
FIGURE 24-57. Midsagittal T2 in a patient with lung cancer shows
numerous metastatic nodules along the roots of the cauda equina.
1196
extensive nodular leptomeningeal enhancement along the cord and conus
(arrows). The primary esophageal cancer is seen anteriorly (star).
1197
FIGURE 24-59. Axial postcontrast T1, in a different patient, shows thick-
enhancing nerve roots.
1198
FIGURE 24-60. Midsagittal T2 in a patient with melanoma shows a dark
metastasis (arrow) with edema in the cervical cord.
1199
FIGURE 24-61. Corresponding midsagittal postcontrast T1 shows avid
enhancement of the mass.
1200
FIGURE 24-62. Midsagittal postcontrast T1 in a different patient with
ovarian cancer shows an enhancing intramedullary metastasis (arrow).
SUGGESTED READING
Diehn FE, Rykken JB, Wald JT, et al. Intramedullary spinal cord metastases:
1201
prognostic value of MRI and clinical features from a 13-year institutional case
series. AJNR Am J Neuroradiol 2015;36:587–593.
Subacute Combined Degeneration

Key Facts
Due to deficiency of vitamin B12, which leads to demyelination and
vacuolation of nerve fibers.
This affects the dorsal columns with or without involvement of the
lateral columns of the spinal cord, and high T2 signal in these regions
is typical (although sensitivity of MRI appears to be low); contrast
enhancement is rare; noncontrast T1 images tend to be normal. The
cervical and upper thoracic cord is most severely affected.
The white matter in the posterior regions of the centrum semiovale
may show high T2 signal, and microstructural abnormalities may be
seen on DTI.
More common in males at any age; macrocytic anemia is present.
The cord signal abnormalities may resolve after treatment, but there
may be atrophy in severe and long-standing disease.
Main differential diagnoses: infarction, demyelinating disease, HIV
vacuolar myelopathy (may be also due to B12 deficiency), nitrous
oxide toxicity (interrupts metabolism of B12 temporarily), Wilson
disease or copper deficiency, decompression (Caisson) disease, and
neurosyphilis (tabes dorsalis).
1202
FIGURE 24-63. Midsagittal T2 image shows linear area of high signal
intensity in the posterior spinal cord from C2 to C5 (arrow).
1203
FIGURE 24-64. Corresponding STIR image shows the abnormality in its
full extent.
1204
FIGURE 24-65. Axial T2 image shows high signal in the dorsal columns
(white arrow) and dorsolateral spinal cord (black arrow).
1205
FIGURE 24-66. Axial gradient-echo image in a different patient with
celiac disease shows high signal confined to the dorsal columns.
1206
enhancement of the dorsal columns (arrow).
1207
FIGURE 24-68. Corresponding postcontrast midsagittal T1 shows that the
abnormality involves the entire cervical spine and extends to the thoracic
spine (arrows).
SUGGESTED READING
1208
Sun HY, Lee JW, Park KS, Wi JY, Kang HS. Spine MR imaging features of
subacute combined degeneration patients. Eur Spine J 2014;23:1052–1058.
Vertebral Metastases
Key Facts
Vertebral metastases occur in 5% to 10% of all cancer patients,
especially those with primary tumors in the breast, prostate, uterus,
lung, myeloma, and lymphoma.
Most vertebral metastases have a combined
osseous/epidural/intradural location.
Most vertebral metastases occur in the thoracic spine.
Vertebral metastases are multiple in 90% of patients.
Most common symptoms from metastases include pain, weakness,
autonomic dysfunction, and sensory loss. Neurologic deficits are
related to the degree of epidural disease; metastatic spinal cord
compression in some patients may be subclinical.
Most vertebral body metastases may be screened with noncontrast
sagittal T1 where they will appear dark due to replacement of marrow
fat; evaluation of epidural/intradural extension needs contrast
administration; following contrast administration, many vertebral
metastases become isointense to normal marrow and difficult to
visualize (fat suppression may be needed in this situation). Tumor is
bright on DWI (shows low ADC) due to hypercellularity.
1209
FIGURE 24-69. Midsagittal noncontrast T1 shows multifocal metastases
that appear dark against the normal marrow fat in a patient with ovarian
cancer.
1210
FIGURE 24-70. Midsagittal fat-saturated postcontrast T1 shows vertebral
metastases from lung cancer spanning multiple levels with a “vertebra
plana” (arrow) and epidural extension into the spinal canal.
1211
FIGURE 24-71. Corresponding axial T2 shows compression of the cord
(arrow) with complete obliteration of the surrounding CSF space, as well
as mediastinal masses.
1212
FIGURE 24-72. Midsagittal T2 fat-suppressed image shows bright tumor
deposits with posterior epidural involvement and cord compression at C6.
1213
FIGURE 24-73. Axial postcontrast T1, in a different patient, shows tumor
in the epidural space and a “curtain” sign.
1214
FIGURE 24-74. Axial CT, in the same patient, shows the mostly lytic
nature of these metastases from a thyroid carcinoma.
SUGGESTED READING
Switlyk MD, Hole KH, Skjeldal S, et al. MRI and neurological findings in
1215
patients with spinal metastases. Acta Radiol 2012;53:1164–1172.
1216
CHAPTER 25
Trauma
Occipitoatlantal Separation
Key Facts
Commonly a fatal injury caused by rupture of craniocervical
ligaments (tectorial membrane, cruciate, apical, and alar ligaments)
due to rapid deceleration with hyperflexion or hyperextension of the
head.
It is two to three times more common in children due to large size of
the head, small size of the occipital condyles, and nearly horizontal
plane of the atlanto-occipital joint.
To assess the space between the base of skull and C1, the best and
easiest measurement is the “basion–dental interval” (inferior tip of
clivus to top of odontoid process), and normally, this space should not
exceed 12 mm in children (even if the ossiculum terminale is not yet
ossified) or adults.
MRI shows injuries to the brainstem in a significant number of these
patients. If the tectorial membrane is torn, injury is nearly always
fatal.
1217
FIGURE 25-1. Lateral radiograph shows increased basion–dental distance
(arrow).
1218
FIGURE 25-2. Midline CT reformation, in a different patient, shows
markedly increased basion–dental distance.
1219
FIGURE 25-3. Parasagittal CT reformation, in the same patient, shows
separation of occipital condyle from lateral mass of C1.
1220
FIGURE 25-4. Midsagittal T2, in a different patient, shows OA separation
with intact tectorial membrane, a normal-appearing spinal cord, and a large
prevertebral hematoma.
1221
FIGURE 25-5. Midsagittal T2, in a different patient, shows OA separation
with torn tectorial membrane and spinal cord edema.
1222
FIGURE 25-6. Axial CT, in a different patient, shows absence of normal
bone structures at the level of OA separation and significant swelling of
soft tissues.
SUGGESTED READINGS
Bertozzi JC, Rojas CA, Martinez CR. Evaluation of the pediatric craniocervical
junction on MDCT. AJR Am J Roentgenol 2009;192:26–31.
Dreizin D, Letzing M, Sliker CW, et al. Multidetector CT of blunt cervical spine
trauma in adults. Radiographics 2014;34:1842–1865.
1223
Benign Compression Fractures
Key Facts
Benign compression fractures are caused by flexion and axial loading.
They may involve the cervical, thoracic, or lumbar spine but occur
most commonly at T7-T8 and T12-L1.
These injuries represent the most common osteoporotic compression
fractures and occur more often in older females.
They may be unstable, and depending on the degree of flexion, there
may be tearing of interspinous, capsular, or posterior longitudinal
ligaments; the disc may rupture, and anterior subluxation of the
involved vertebra may occur.
CT shows bone fragments that may be displaced into the spinal canal
and unsuspected fractures of posterior elements (bursting of a
vertebral body may occur).
The most severe flexion–compression fracture in the cervical region
is the “teardrop” type, which is often accompanied by posterior
displacement of the vertebral body, damaging the spinal cord.
If the compression involves only the anterior third of a vertebra, the
ligaments are generally intact, and the injury is considered stable.
MRI signs supporting benignity in compression fracture: lack of
accompanying soft tissue masses, preservation of a nonconvex
posterior cortical margin and “wedge” shape, preservation of normal
bone marrow signal intensity, bone fragment in canal, gas or fluid in
vertebral body, dark horizontal T2 line of compacted bone at fracture
site, non-to-mild contrast enhancement. On DWI, benign fractures
have low signal.
1224
FIGURE 25-7. Midsagittal T1 shows compression fracture of superior
endplate of T12 with preservation of normal signal intensity in the
remaining vertebral body.
1225
FIGURE 25-8. Corresponding T2 shows dark line from compaction of
fractured superior endplate (arrow), preservation of posterior cortical
margin, normal signal from most of the marrow in the affected vertebral
body, and absent soft tissue mass.
1226
FIGURE 25-9. Midsagittal CT in a different patient demonstrates C4
hyperflexion injury with small fracture fragment (arrow), in a patient with
ruptured interspinous ligament on MRI (not shown).
1227
FIGURE 25-10. Midsagittal fat-suppressed postcontrast T1 image, in a
different patient, shows linear enhancement of a superior L1 endplate
fracture suggesting a benign underlying etiology.
1228
gas in a benign fracture.
1229
FIGURE 25-12. Midsagittal DWI, in a different patient, shows low signal
in an acute benign fracture (arrow).
SUGGESTED READING
Dohm M, Black CM, Dacre A, et al. A randomized trial comparing balloon
1230
kyphoplasty and vertebroplasty for vertebral compression fractures due to
osteoporosis. AJNR Am J Neuroradiol 2014;35:2227–2236.
Pathologic (Malignant) Compression

Fractures
Key Facts
These injuries are more common in elderly adults, particularly those
with cancer and spine metastases.
Signs that suggest underlying malignancy in a compression fracture:
soft tissue mass especially in ventral epidural space, absent well-
defined bone margins, convex posterior vertebral margin, enhancing
soft tissues, lesions in other vertebrae, and restricted diffusion.
Pathologic fractures may show higher perfusion values on MRI
compared to benign fractures.
Remember that it is necessary to image the entire spine to exclude
other lesions, which may need to be treated simultaneously.
1231
FIGURE 25-13. Sagittal fat-saturated postcontrast T1 shows an avidly
enhancing vertebral metastasis (arrow) with mild fracture of the superior
endplate of L2.
1232
FIGURE 25-14. Midsagittal T1 shows a compression fracture of T10 with
rounded posterior margin projecting into the canal and compressing the
spinal cord. Note low signal in the affected vertebra and in T12.
1233
FIGURE 25-15. Axial postcontrast T1 in a different patient shows tumor
extending into the ventral epidural space resulting in a “curtain sign”
(arrow) due to attachment of the midline septum and displacement of the
lateral membranes.
1234
FIGURE 25-16. Sagittal noncontrast T1 in a different patient shows
multiple low-signal vertebral metastases with marked compression fracture
of T12 (arrow) and retropulsion.
1235
FIGURE 25-17. Corresponding sagittal fat-saturated T2 better
demonstrates compression and displacement of the spinal cord with mildly
increased signal in the conus medullaris.
1236
FIGURE 25-18. Axial noncontrast T1 in the same patient shows tumor
encroaching into the ventral epidural space (arrow).
SUGGESTED READINGS
Arevalo-Perez J, Peck KK, Lyo JK, et al. Differentiating benign from malignant
vertebral fractures using T1-weighted dynamic contrast-enhanced MRI. J
Magn Reson Imaging 2015;42:1039–1047.
Sung JK, Jee WH, Jung JY, et al. Differentiation of acute osteoporotic and
malignant compression fractures of the spine: use of additive qualitative and
quantitative axial diffusion-weighted MR imaging to conventional MR
1237
imaging at 3.0 T. Radiology 2014;271:488–498.
Chance-Type Fractures
Key Facts
These injuries are produced by hyperflexion and distraction
associated with falls from high altitudes or vehicular accidents while
wearing only a lap seat belt.
In Chance-type fractures, there is involvement of all three columns
with disruption of the posterior ligament complex and shearing of the
pedicles and vertebral body; occasionally a Chance fracture occurs
through the disc, and up to 25% may be purely ligamentous.
Severe disruption of facet joints leads to complete dislocation and
produces the “naked facet” sign on CT studies.
MRI commonly shows edema and/or hematoma in the spinal cord.
A large proportion of patients with Chance fractures have injuries to
abdominal organs (up to 80% in some series).
1238
FIGURE 25-19. Midsagittal CT shows a Chance-type fracture involving
the L3 vertebra (arrow) and the L2 spinous process. There is also a sacral
fracture (arrowhead).
1239
FIGURE 25-20. Parasagittal CT in a different patient shows a horizontal
fracture through a midthoracic vertebra extending to the pedicle and
articular pillar.
1240
FIGURE 25-21. Parasagittal CT reformation shows a horizontal fracture
(arrows) crossing the posterior elements and vertebral body.
1241
FIGURE 25-22. Midsagittal T2, in a different patient, shows a Chance-
type fracture involving the disc space at T12-L1, compression of L1, and a
disc herniation. The fracture extends to the posterior elements and there is
near-shearing of the conus medullaris. The distal thoracic spinal cord
contains edema and hematoma.
1242
FIGURE 25-23. Midsagittal T2, in a different patient, shows compression
fractures of T11 and T12 with posterior ligamentous injuries and cord
edema. Regional bone marrow edema is also present at T10 and L3.
1243
FIGURE 25-24. Midsagittal T2, in a different patient, shows extensive
ligamentous disruption as well as disruption of the dura (arrow) with intra-
and extradural hematomas. Note layering blood in the thecal sac
(arrowhead).
SUGGESTED READINGS
Looby S, Flanders A. Spine trauma. Radiol Clin North Am 2011;49:129–163.
Wintermark M, Mouhsine E, Theumann N, et al. Thoracolumbar spine fractures
in patients who have sustained severe trauma: depiction with multi-detector
row CT. Radiology 2003;227:681–689.
1244
Facet Dislocation
Key Facts
This type of injury is produced by a combination of flexion and
rotation, which leads to tearing of the articular capsule and sliding of
the superior facet forward on the inferior facet.
In unilateral facet dislocation, the superior vertebral body rotates and
slides anteriorly (<50%) on the vertebra below. Bilateral facet
dislocation requires more severe forces and results in >50% anterior
displacement of the superior vertebra.
Unilateral lesions may be stable if the posterior longitudinal ligament
is preserved by MRI.
May result in injury to the vertebral arteries (particularly bilateral
dislocations and in patients with ascending progression of
neurological symptoms).
In bilateral dislocations, there is significant canal stenosis at the level
of injury, which may be accentuated by herniated disks and/or
epidural hematoma; the cord is generally compressed and may
contain hemorrhages.
Dislocations need to be distinguished from the rare congenital
absence of a cervical pedicle, which can simulate a “perched” facet.
1245
FIGURE 25-25. Parasagittal CT reformation shows anterior dislocation of
the facet of C5 (star) on that of C6.
1246
FIGURE 25-26. Axial CT shows the “reverse hamburger bun” sign of the
dislocated right facets.
1247
FIGURE 25-27. Midsagittal T2, in a different patient, shows anterior
displacement of C5 and focal cord edema at that level.
1248
FIGURE 25-28. Axial CT, in a different patient, shows typical appearance
of bilateral facet dislocations.
1249
FIGURE 25-29. Midsagittal T2, in a different patient, shows C4-C5
anterior dislocation with associated cord edema.
1250
FIGURE 25-30. Midsagittal T1, in a different patient, shows C7-T1
dislocation with inferiorly extending posterior epidural hematoma (arrow).
SUGGESTED READING
Daffner SD, Daffner RH. Computed tomography diagnosis of facet dislocations:
1251
the “hamburger bun” and “reverse hamburger bun” signs. J Emerg Med
2002;23:387–394.
Hangman Fracture
Key Facts
This fracture of both C2 pedicles is secondary to hyperextension,
compression, and distraction, which may produce instant death
secondary to spinal cord transection.
Avulsion of anterior aspect of endplates of C2 and C3 may be
present.
Pedicle fractures are usually bilateral, but asymmetrical fractures may
extend to lamina, facets, or vertebral body.
Fracture is unstable if there is >3 mm between fragments, >15 degree
angulation, subluxation of C2 on C3, or concomitant C1 fractures.
Grade 1: minimal distraction; only anterior longitudinal ligament is
torn. Grade 2: moderate distraction and angulation; anterior and
posterior longitudinal ligaments are torn and there is disk herniation.
Grade 3: significant distraction, tearing of most of the ligamentous
complex, epidural and spinal cord hematomas, vertebral artery
injuries, and disc herniation.
1252
FIGURE 25-31. Axial CT shows fractures of the C1 pedicles with a
displaced fragment on the left.
1253
FIGURE 25-32. Parasagittal CT in the same patient shows the fracture
(arrow) in one of the pedicles.
1254
FIGURE 25-33. Corresponding midsagittal CT demonstrates anterior
subluxation of C2 with small avulsed fragments posteriorly.
1255
FIGURE 25-34. Midsagittal CT in a different patient shows anterior
subluxation of C2 and fracture of the posterior elements.
1256
FIGURE 25-35. Midsagittal fat-saturated T2, in the same patient, shows
disruption of the anterior and posterior longitudinal ligaments and cord
compression with edema.
1257
FIGURE 25-36. Midsagittal T2 in a child with anterior C2 tilting, a
disrupted posterior longitudinal ligament, and cord edema.
SUGGESTED READINGS
Darras K, Andrews GT, McLaughlin PD, et al. Pearls for interpreting computed
tomography of the cervical spine in trauma. Radiol Clin North Am
2015;53:657–674, vii.
Samaha C, Lazennec JY, Laporte C, Saillant G. Hangman’s fracture: the
relationship between asymmetry and instability. J Bone Joint Surg Br
2000;82:1046–1052.
1258
Jefferson Fracture
Key Facts
Most common fracture of C1 in adults (very rare in infants and young
children), caused by axial compression.
Results in bilateral fractures at the junctions of the anterior and
posterior arches with the lateral masses (the weakest points of the C1
vertebra) but may be unilateral if the head is tilted upon impact.
Generally, there is no spinal cord damage because the canal diameter
is not significantly compromised.
Vertebral arteries may be damaged.
Fracture is unstable if the combined offset of the lateral masses of C1
measures >7 mm or if the atlantodental distance is >3 mm (both
imply torn transverse ligament).
MRI helps to establish status of transverse ligament; if avulsed at
bony insertion, patients do not need immediate surgery as healing
occurs in >60%; if substance of ligament is torn, surgical fixation is
needed.
1259
FIGURE 25-37. Frontal open mouth radiograph shows lateral offset of the
C1 lateral masses (arrows).
1260
FIGURE 25-38. Corresponding axial CT shows fractures at the junctions
of the anterior and posterior arches with the lateral masses of C1.
1261
FIGURE 25-39. Coronal CT in a different patient shows lateral offset of
the C1 lateral masses (arrows) on C2.
1262
FIGURE 25-40. Axial CT, in a different patient, shows left-sided
fractures of C1.
1263
FIGURE 25-41. Axial MR T2, in the same patient, shows that the
transverse ligament is avulsed at both of its insertions.
SUGGESTED READINGS
Looby S, Flanders A. Spine trauma. Radiol Clin North Am 2011;49:129–163.
Lustrin ES, Karakas SP, Ortiz AO, et al. Pediatric cervical spine: normal
anatomy, variants, and trauma. Radiographics 2003;23:539–560.
Odontoid Fractures
1264
Key Facts
They are the most common fractures of C2.
Type 1 occurs through the tip of the dens, is the least common type
and stable (if the C2-C3 disc and longitudinal ligaments are intact),
and may be confused with an os odontoideum or persistent ossiculum
terminale. The last two should be well corticated; a V-shaped
cartilaginous cleft may be seen between the ossiculum terminale and
the remaining dens on coronal images.
Type 2 is the most common type, involves the base of the dens, and is
unstable; it may be missed on axial CT; therefore, plain radiographs
and sagittal or coronal CT reformations are mandatory; in adults, this
fracture disrupts the blood supply, and there is a high incidence of
nonunion.
Type 3 extends from the base of the dens to the body of C2, occurs
below the level of the transverse band of the cruciform ligament, and
is unstable; if it extends into the articular facets, the prognosis
worsens.
Os odontoideum refers to the lack of assimilation of an occipital
vertebra or a hypertrophied ossiculum terminale; it may be
accompanied by hypoplasia of the remaining dens and may have a
hyperplastic anterior C1 arch and C1-C2 instability.
Vertical fractures are a newly recognized and rare type of injury to
the dens.
1265
FIGURE 25-42. Midsagittal CT reformation shows a type 2 dens fracture
and anterior displacement and angulation of the odontoid process.
1266
FIGURE 25-43. Coronal CT in a different patient shows a type 3 dens
fracture extending to the left articular facet.
1267
a nondisplaced type 2 dens fracture.
1268
FIGURE 25-45. Midsagittal T2 in a child shows type 2 fracture with
anterior displacement of dens and torn tectorial membrane.
1269
FIGURE 25-46. Coronal CT, in a different patient, shows a round and
well-corticated os odontoideum and incomplete segmentation of the C2
and C3 vertebrae.
1270
FIGURE 25-47. Corresponding sagittal CT shows hypertrophy of the
anterior C1 arch (arrow).
SUGGESTED READING
O’Brien WT Sr, Shen P, Lee P. The dens: normal development, developmental
variants and anomalies, and traumatic injuries. J Clin Imaging Sci 2015;5:38.
Spinal Cord Injury

Key Facts
Most commonly affects young men involved in motor vehicle
accidents followed by falls and penetrating injury. A second peak
occurs in the elderly with degenerative spinal canal stenosis.
Bone injury does not accurately correlate with neurologic findings in
>65% of spinal trauma cases, especially in children and older adults
1271
with extensive degenerative changes or ossification of the posterior
longitudinal ligament.
Hemorrhagic contusions are seen as central hypointensity
(deoxyhemoglobin) surrounded by edema on T2WI and have a poor
prognosis with little chance of recovery; prognosis is worse with
extensive hematomas.
Nonhemorrhagic contusions produce edema, have normal T1WI and
hyperintensities on T2WI, and may also have poor prognosis if
extensive.
Patients with significant DJD or OPLL are predisposed to cord
contusion and may clinically present with central cord syndrome.
Spinal cord transection may be anatomical (actual gap) or functional
(severe compression with irreversible neurological symptoms); both
are generally seen with severe fracture/dislocations, but in children,
bone trauma may be minimal and rarely absent. Epidural hematomas
and subarachnoid fluid collections may also be present.
1272
FIGURE 25-48. Midsagittal T2 shows bright edema in a slightly
expanded cord at C4-C7 and degenerative changes at the same levels
resulting in canal stenosis.
1273
FIGURE 25-49. Midsagittal T2, in a different patient with significant DJD
from C4-C6, shows cord edema after mild trauma.
1274
FIGURE 25-50. Sagittal T2 shows area of cord edema at C3 level that
also contains some patchy hypointense regions (arrow).
1275
FIGURE 25-51. Axial T2*, in the same patient, shows presence of dark
areas (arrow) related to acute intramedullary hematoma
(deoxyhemoglobin).
1276
FIGURE 25-52. Midsagittal T2, in a different patient, shows complete
T12-L1 dislocation with anatomic cord transection; note superior cord
stump (arrow).
1277
FIGURE 25-53. Midsagittal T2, in a different patient, shows midthoracic
dislocation with cord compression and edema. Despite the absence of
anatomic transection, the patient was completely paraplegic and did not
recover; thus, this can be considered a functional transection.
SUGGESTED READINGS
De Smet E, Vanhoenacker FM, Parizel PM. Traumatic myelopathy: current
concepts in imaging. Semin Musculoskelet Radiol 2014;18:318–331.
Dreizin D, Kim W, Kim JS, et al. Will the real SCIWORA please stand up?
Exploring clinicoradiologic mismatch in closed spinal cord injuries. AJR Am J
1278
Roentgenol 2015;205:853–860.
Vertebral Artery Injury

Key Facts
Can occur without or with a fracture (particularly at C1-C3 and those
extending to foramen transversarium).
Types: occlusion and dissections with mural thrombus or
pseudoaneurysm.
CTA shows all types of dissections equally to MRI and MRA.
All injuries can be uni- or bilateral.
May result in posterior fossa infarctions, which may present at the
time of injury or in a delayed fashion.
Most dissection involving a previously normal underlying artery will
heal spontaneously within 6 months.
1279
FIGURE 25-54. Axial T2 shows crescentic mural thrombus in wall of left
vertebral artery (arrow).
1280
FIGURE 25-55. Contrast-enhanced MRA, in the same patient, shows
focal narrowing (arrow) from dissection.
1281
FIGURE 25-56. Parasagittal CT, in a different patient, shows a mildly
displaced fracture involving the right foramen transversarium of C2.
1282
FIGURE 25-57. Axial time-of-flight MRA shows absence of flow-related
signal in the right vertebral artery (arrow) due to traumatic dissection.
1283
FIGURE 25-58. Contrast-enhanced MRA, in the same patient, shows
abrupt occlusion of the right vertebral artery (arrow) with some flow
distally due to cross-filling from the contralateral side.
1284
FIGURE 25-59. Contrast-enhanced MRA, in a different patient, shows
vertebral artery pseudoaneurysm (arrow).
SUGGESTED READINGS
Lum C, Chakraborty S, Schlossmacher M, et al. Vertebral artery dissection with a
normal-appearing lumen at multisection CT angiography: the importance of
identifying wall hematoma. AJNR Am J Neuroradiol 2009;30:787–792.
Rodallec MH, Marteau V, Gerber S, et al. Craniocervical arterial dissection:
spectrum of imaging findings and differential diagnosis. Radiographics
2008;28:1711–1728.
1285
CHAPTER 26
Vascular Disorders
Spinal Cord Infarction

Key Facts
Spinal cord infarction is rare (at least partially due to its extensive
vascular collateral network) but has a poor prognosis.
Etiologies for arterial infarctions include atherosclerosis,
hypertension, diabetes, severe hypotension/hypoperfusion,
thoracoabdominal aortic aneurysms, sickle cell anemia, spinal trauma,
caisson disease, cardiovascular malformations (particularly in
children), cocaine, and arteritis.
Arterial spinal cord infarctions have an acute onset, and most are
accompanied by back pain; venous infarcts may have a more
protracted course and may be seen with AVFs.
Most infarcts affect the anterior spinal artery territory; infarctions
affecting the posterior spinal artery territories are very rare.
Most infarctions occur in the lower thoracic cord and conus
medullaris; the second most common site is probably the
cervicothoracic region.
Most common causes for venous infarctions include dural
arteriovenous fistulas, hypercoagulable states, and fibrocartilaginous
emboli.
Venous infarctions have an insidious onset and should be
differentiated from subacute necrotizing myelopathy.
Main differential diagnosis: transverse myelitis, dural AV fistula,
demyelinating disease, postradiation changes, and vasculitis.
1286
DWI is very helpful in making the diagnosis (high DWI signal,
restricted ADC). As is the case in the brain, contrast enhancement
may be present in the subacute stage and some infarcts may be
complicated by hemorrhage.
FIGURE 26-1. Midsagittal T2 shows alternating bands of high and

normal signal intensity in the distal cord and conus medullaris due to acute
infarction. This appearance is due to partial averaging of high-signal
edema in anterior gray matter horns, commissure, and posterior gray
matter horns.
1287
FIGURE 26-2. Axial T2, in the same patient, shows bright and enlarged
gray matter horns (arrows).
1288
FIGURE 26-3. Midsagittal STIR, in a different patient, shows increased
signal anteriorly in the cervical spinal cord (arrows).
1289
FIGURE 26-4. Axial T2, in the same patient, shows increased signal
involving the anterior gray matter horns and resulting in the so-called
“snake-eye” appearance.
1290
FIGURE 26-5. Axial DWI, in the same patient, shows increased signal
due to acute infarction.
1291
enhancement of anterior gray matter horns (arrows) in subacute phase of
infarction.
SUGGESTED READINGS
Vargas MI, Gariani J, Sztajzel R, et al. Spinal cord ischemia: practical imaging
tips, pearls, and pitfalls. AJNR Am J Neuroradiol 2015;36:825–830.
Weidauer S, Nichtweiss M, Hattingen E, et al. Spinal cord ischemia: aetiology,
clinical syndromes and imaging features. Neuroradiology 2015;57:241–257.
1292
Spinal Epidural and Subdural Hematomas
Key Facts
Spinal epidural hematomas are more common than subdural ones;
they may be mixed epidural/subdural and difficult to isolate to one
compartment.
Ventrally, epidural hematomas may be constrained at the disc levels
by the attachment of the posterior longitudinal ligament and by the
midline septum, although these tethers may be disrupted in trauma.
Predisposing factors for both types of hematomas are coagulopathy,
trauma, prior spinal puncture, spinal vascular malformations,
hypertension, pregnancy, infection, advanced age, spinal surgery,
forceful sneezing, and lupus erythematosus (but nearly 50% of all
spinal epidural hematomas are spontaneous).
Most common locations for both types of hematomas are thoracic,
lumbar, and cervical regions; most idiopathic epidural hematomas
occur in the dorsolateral canal in the cervicothoracic junction.
Most occur in males aged 40 to 60 years.
Most result in symptoms due to compression of the spinal cord or
cauda equina (usually with acute axial pain and progressive
neurologic deficits, which may mimic a disc herniation) and need to
be decompressed in <24 hours or irreversible neural damage occurs.
Main differential diagnosis for epidural hematomas: lipomatosis,
abscess, phlegmon, angiolipoma, and tumor (particularly lymphoma);
main differential diagnosis for subdural hematomas: epidural
hematoma and abscess.
1293
FIGURE 26-7. Midsagittal T1 shows a ventral collection with mass effect
on the thecal sac (arrows) following a lumbar puncture in a coagulopathic
patient.
1294
FIGURE 26-8. Corresponding axial T2 shows circumferential
compression of the thecal sac (arrow) due to a bright subdural hematoma
(arrowhead). Note epidural space peripheral to this (wavy arrow)
demarcated by the midline septum and lateral membranes.
1295
FIGURE 26-9. Midsagittal T1, in a different patient, shows a subacute
subdural hematoma diffusely affecting the extra-axial spaces in the lumbar
spine of a coagulopathic child after minor trauma.
1296
FIGURE 26-10. Axial T1, in the same patient, shows preservation of the
peripherally located bright epidural fat and the dark linear dura
surrounding a subdural hematoma. The hematoma compresses the thecal
sac, which is anchored by meningeal ligaments resulting in the “Mercedes
Benz” sign that is typical for spinal subdural hematomas.
1297
FIGURE 26-11. Axial T1 in a different patient shows an epidural
hematoma (arrow) replacing the normal dorsal epidural fat.
1298
FIGURE 26-12. Sagittal T2, in the same patient, shows an extensive
dorsal epidural hematoma (arrows) with mass effect on the upper thoracic
thecal sac.
SUGGESTED READING
1299
Al-Mutair A, Bednar DA. Spinal epidural hematoma. J Am Acad Orthop Surg
2010;18:494–502.
Spinal Arteriovenous Fistula

Key Facts
Spinal arteriovenous fistulas usually present in patients older than 50
to 60 years of age and are more common in men.
These lesions are abnormal communications between artery
(generally a radicular one) and neighboring vein leading to
recruitment of cord veins for drainage.
Potentially treatable; delayed diagnosis may result in permanent
disability.
Symptoms are insidious, characterized by a progressive myelopathy
(Foix-Alajouanine syndrome), probably related to venous
hypertension and congestion from increased arterial flow.
Lesions are usually solitary but may be multiple (1%).
MRI shows longitudinally extensive T2 hyperintensity within the
cord (highly sensitive for the presence of a fistula but nonspecific);
dilated blood vessels may be seen as intradural flow voids in the
majority of patients; pinpointing the exact site of the fistula
necessitates catheter angiography and may sometimes be identified by
MRA (preferably time resolved) or CTA.
Spinal cord may show contrast enhancement at the level of edema;
these patients generally have a slow and minimal recovery post fistula
occlusion.
Spinal AVFs in young children are typical of Rendu-Osler-Weber
syndrome.
Main differential diagnosis: transverse myelitis, demyelinating
disease, vasculitis, tumor, cord infection (schistosomiasis where
endemic), and subacute combined degeneration.
1300
FIGURE 26-13. Midsagittal T2 shows longitudinally extensive signal
abnormality in the thoracic cord with small intradural flow voids
posteriorly (arrows).
1301
FIGURE 26-14. Corresponding postcontrast midsagittal T1 demonstrates
mild ill-defined intramedullary enhancement (arrows) and tortuous pial
vessels posteriorly.
1302
subtle enhancement of the conus and pial surface of the distal cord.
1303
FIGURE 26-16. Corresponding fat-saturated midsagittal T2 demonstrates
longitudinally extensive intramedullary signal abnormality and a
heterogeneous appearance of the dorsal intradural space.
1304
FIGURE 26-17. Frontal view DSA, in the same patient, shows a dural
arteriovenous fistula arising from a left L1 injection (left) with
opacification of tortuous spinal draining veins (arrow, right).
1305
FIGURE 26-18. Frontal MR myelogram view, in a different patient,
shows large vein in cord surface.
SUGGESTED READINGS
Amarouche M, Hart JL, Siddiqui A, et al. Time-resolved contrast-enhanced MR
1306
angiography of spinal vascular malformations. AJNR Am J Neuroradiol
2015;36:417–422.
Oda S, Utsunomiya D, Hirai T, et al. Comparison of dynamic contrast-enhanced
3T MR and 64-row multidetector CT angiography for the localization of
spinal dural arteriovenous fistulas. AJNR Am J Neuroradiol 2014;35:407–412.
Spinal Cord Arteriovenous Malformations

Key Facts
Spinal cord AVMs may be of the following types:
Small mass-like vascular nidus (glomus) supplied by multiple
feeders from anterior and/or posterior spinal arteries and
draining into enlarged veins
Large mass-like vascular nidus with extramedullary extension
supplied by a myriad of arterial feeders (juvenile type)
Large malformation involving the spinal cord and adjacent
vertebrae and soft tissues (metameric type)
Common locations: cervical, lower thoracic, and upper lumbar spine;
most are found in middle-aged men.
Common symptoms: paresis, sensory alterations, autonomic
dysfunction, and impotence due to hemorrhage, venous hypertension,
compression myelopathy, or vascular steal. Spinal subarachnoid
hemorrhage may present with sudden “stabbing” back pain (“coup de
poignard of Michon”).
Main differential diagnosis: hypervascular tumor (hemangioblastoma,
metastases) and cavernous hemangioma.
1307
FIGURE 26-19. Midsagittal T2 shows multiple dilated blood vessels in
and outside of the distal spinal cord. Fast flow in these vessels results in
their signal void.
1308
FIGURE 26-20. Coronal T2, in the same patient, shows the large
inferiorly located vascular nidus with venous “aneurysms.”
1309
FIGURE 26-21. Midsagittal T2 image, in a different patient, shows
diffuse cord edema and a focal area of dark signal at C2.
1310
FIGURE 26-22. Frontal DSA view, in the same patient as in Figure 26-
21, shows AVM fed by a left vertebral artery branch.
1311
FIGURE 26-23. Midsagittal T2 in a child shows abnormal signal in
conus, engorged vessels in the posterior spinal canal, and serpiginous
appearance of nerve roots.
1312
FIGURE 26-24. Frontal DSA view, in the same patient as in Figure 26-
23, shows the AVM nidus (arrow) fed by a spinal artery arising from a
lumbar one.
SUGGESTED READINGS
1313
Chaudhary N, Pandey AS, Gemmete JJ. Endovascular treatment of adult spinal
arteriovenous lesions. Neuroimaging Clin N Am 2013;23:729–747.
Condette-Auliac S, Boulin A, Roccatagliata L, et al. MRI and MRA of spinal
cord arteriovenous shunts. J Magn Reson Imaging 2014;40:1253–1266.
Spinal Cord Cavernous Malformation

Key Facts
Histologically, cavernous malformations are composed of dilated
vascular sinusoids devoid of smooth muscle and elastic fibers; there
are no normal intervening neural tissues and abundant hemosiderin is
present.
Cavernous malformations constitute 5% to 10% of spinal vascular
malformations.
Most spinal cord malformations are located in the thoracic spine
followed by the cervical spine. Very rarely, they may be
extramedullary or even extradural.
The majority are sporadic, but they may also be seen as part of an
autosomal-dominant familial syndrome or following radiation
therapy, particularly when multiple.
If strictly intramedullary, these malformations are usually solitary.
Approximately 20% of patients have a coexistent intracranial
cavernous malformation.
Common clinical symptoms include progressive paraparesis, sensory
alterations, pain, and occasionally subarachnoid hemorrhage or
hematomyelia. Annual risk of hemorrhage is about 2%.
Main differential diagnosis: AVM, hemorrhagic tumor (primary or
metastases), and hemorrhagic contusion.
1314
FIGURE 26-25. Axial T2 shows a cavernous malformation in the cervical
spine with a dark peripheral rim of hemosiderin
1315
FIGURE 26-26. Corresponding midsagittal T2 shows the mildly expansile
malformation (arrow) with heterogeneous central signal.
1316
FIGURE 26-27. Midsagittal T2 in a different patient demonstrates a small
cavernous malformation (arrow) in the lower thoracic spine.
1317
FIGURE 26-28. Corresponding midsagittal T1 shows a perilesional T1
hyperintense rim (arrow) likely related to recent hemorrhage.
1318
FIGURE 26-29. Midsagittal T2, in a different patient, shows a cavernous
malformation in the medulla with hemorrhage into the spinal cord.
1319
FIGURE 26-30. Corresponding T1 image clearly shows the typical
features of the cavernous malformation and high signal in hematomyelia.
SUGGESTED READING
Badhiwala JH, Farrokhyar F, Alhazzani W, et al. Surgical outcomes and natural
1320
history of intramedullary spinal cord cavernous malformations: a single-center
series and meta-analysis of individual patient data: clinic article. J Neurosurg
Spine 2014;21:662–676.
1321
SECTION C EAR,
NOSE, AND THROAT
IMAGING
1322
CHAPTER 27
Neck Masses
Branchial Cleft Cysts

Key Facts
Branchial cleft cysts represent incomplete proliferation, migration, or
obliteration of one of the four branchial clefts. Remember that these
cysts are very unusual in adults and that a cystic neck mass in an adult
should be considered a metastasis until proven otherwise.
First branchial cleft cysts (8%) occur in the vicinity of the external
auditory canal (may drain into it) or in the anterior triangle of the
neck inferior to the mandible. They may course within or adjacent to
the parotid gland and be closely related to the facial nerve. Main
differential diagnosis: lymphatic malformation, parotid cyst
(lymphoepithelial), and lymph node.
Second branchial cleft cysts (>90%) occur in the anterior triangle at
midneck, cross the common carotid artery bifurcation, and end at the
palatine tonsil. Main differential diagnosis: lymphatic malformation,
necrotic node, and cystic schwannoma.
Third branchial cleft cysts are uncommon (3%), arise from the
pyriform sinus, and are located anterior to sternocleidomastoid
muscle in the lower neck or posterior triangle in the upper neck. Main
differential diagnosis: second or fourth branchial cleft cysts,
lymphatic malformation, thyroglossal duct cysts, necrotic node, and
external laryngocele.
Fourth branchial cleft cysts are very rare; they arise from the
pyriform sinus and may extend caudally behind the aortic arch or
1323
cephalad posterior to the carotid artery; most are left-sided. Main
differential diagnosis: thyroglossal duct cyst, thymic cyst, third
branchial cyst, lymphatic malformation, thyroid and parathyroid cyst,
and thyroid abscess. Remember that both third and fourth branchial
anomalies can present with recurrent thyroiditis or thyroid abscess
which usually occur on the left side.
FIGURE 27-1. Parasagittal CT shows an elongated type 1 cyst in the

parotid gland (arrow) extending superiorly to the floor of the external
auditory canal.
1324
FIGURE 27-2. Coronal CT reformat, in a different patient, shows a type 1
cyst in the right auricle (arrow) with mass effect on the external auditory
canal.
1325
FIGURE 27-3. Axial postcontrast CT, in a different patient, shows a type
2 cyst (arrow) anterior to the left sternocleidomastoid muscle and lateral to
the carotid space.
1326
FIGURE 27-4. Axial postcontrast CT, in a different patient, shows an
infected type 4 cyst along the left aspect of the thyroid gland (arrow).
1327
FIGURE 27-5. Coronal postcontrast CT, in a different patient, shows
formation of a large abscess along the left thyroid gland related to a type 4
cyst (arrow).
1328
FIGURE 27-6. Coronal postcontrast CT, in a different patient, shows a
type 3 cyst (arrow) in the deep neck.
SUGGESTED READINGS
Ibrahim M, Hammoud K, Maheshwari M, et al. Congenital cystic lesions of the
head and neck. Neuroimaging Clin N Am 2011;21:621–639.
Prosser JD, Myer CM III. Branchial cleft anomalies and thymic cysts.
1329
Otolaryngol Clin North Am 2015;48:1–14.
Buccal Space Masses

Key Facts
This space is located posterolateral to the lower maxilla between the
buccinator muscle and overlying skin and fascia. It contains mostly
fat but also carries the facial artery and vein, distal parotid duct,
lymphatic channels, and branches of the mandibular and facial
nerves.
The processes affecting this space are similar to those involving the
masticator space and in the majority of cases represent extension from
masticator space, maxilla, alveolar ridge, and maxillary sinus
pathology into the buccal (buccinator) space.
FIGURE 27-7. Axial CT shows a pleomorphic adenoma (arrows) in the

right buccal space.
1330
FIGURE 27-8. Axial CT, in a different patient, shows an abscess (arrow)
in the left buccal space. Note overlying stranding of fat due to
inflammation.
1331
FIGURE 27-9. Coronal CT in the same patient shows the abscess (arrow)
that is an extension of an ipsilateral maxillary sinus infection through a
bone defect in the anterior wall of this sinus.
1332
FIGURE 27-10. Coronal fat-suppressed postcontrast T1 in patient with an
osteosarcoma shows an enhancing mass arising in the left maxilla
extending laterally into the buccal space.
1333
FIGURE 27-11. Axial fat-suppressed postcontrast T1 in a patient with a
reparative granuloma arising in the left maxilla and presenting as a buccal
space mass.
1334
FIGURE 27-12. Axial CT in a patient with a large carcinoma of the left
maxillary sinus showing extension into the buccal space (arrow).
SUGGESTED READING
Kim HC, Han MH, Moon MH, et al. CT and MR imaging of the buccal space:
normal anatomy and abnormalities. Korean J Radiol 2005;6:22–30.
1335
Carotid Space Masses
Key Facts
The carotid space (also called the “poststyloid” parapharyngeal space)
normally contains the internal carotid artery and its sheath; internal
jugular vein; cranial nerves 9, 10, 11, and 12; nodes; and sympathetic
plexus.
Common lesions in this space: carotid body tumor (paraganglioma
arising at the common carotid artery bifurcation, 10% are multiple,
10% are malignant and occur in the fifth decade of life), glomus
vagale, schwannoma (usually from the vagus nerve), nodal metastases
(from airway squamous cell carcinoma or lymphoma),
pseudoaneurysms, and extracranial meningiomas.
Most lesions arising in this space present as indolent masses in the
lateral neck.
Vascular abnormalities such as ectatic arteries, pseudoaneurysms, and
jugular vein thrombosis may also affect this space. Remember that
the carotid arteries may be medially displaced and become
“retropharyngeal” in location.
Remember that the jugular veins are normally asymmetric (generally
the right one being larger than the left).
1336
FIGURE 27-13. Axial T2 shows a bright right carotid schwannoma
(arrow) displacing the carotid artery vessels anterolaterally.
1337
FIGURE 27-14. Lateral DSA, in a different patient, shows a
hypervascular tumor separating the external from the internal carotid
artery typical of a carotid body tumor.
1338
FIGURE 27-15. Parasagittal T2 shows a small carotid body tumor
(arrow) splaying the internal and external carotid arteries.
1339
FIGURE 27-16. Coronal T2 in a different patient shows a large mass with
internal flow voids in the right neck (arrow).
1340
FIGURE 27-17. Axial postcontrast T1, in the same patient, shows that the
mass enhances avidly and displaces the carotid vessels anteriorly (arrow).
The jugular vein is displaced posteriorly and not seen. This was a glomus
vagale tumor.
1341
FIGURE 27-18. Axial noncontrast T1, in the same patient, shows bright
and dark foci within the tumor due to small hemorrhages and vascular flow
voids (“salt and pepper”).
SUGGESTED READINGS
Kuwada C, Mannion K, Aulino JM, et al. Imaging of the carotid space.
Varoquaux A, Fakhry N, Gabriel S, et al. Retrostyloid parapharyngeal space
tumors: a clinician and imaging perspective. Eur J Radiol 2013;82:773–782.
Fissural Cysts
1342
Key Facts
They represent cystic developmental masses arising from epithelium
presumably trapped during closure of the frontal nasal and maxillary
(palatine) fusion lines.
They may enlarge or become symptomatic due to
inflammation/infection, and occasionally they become large expansile
lesions.
Most are found in individuals <30 years of age.
May occur in the following locations:
Globulomaxillary: between lateral incisor and canine
Median mandibular: located between the central incisors
Nasolabial: between nasal ala and upper lip
Nasoalveolar: between upper alveolus and anterior nasal
aperture
Nasopalatine: at nasopalatine foramen
Medial palatal: midline hard palate
1343
FIGURE 27-19. Axial CT shows lucent defect in the anterior right maxilla
in the incisor–canine region.
1344
FIGURE 27-20. Corresponding bone window settings show smooth
margins of this large globulomaxillary cyst.
1345
FIGURE 27-21. Axial T2, in a different patient, shows bright right-sided
globulomaxillary cyst.
1346
FIGURE 27-22. Axial T2, in a different patient, shows bright median
mandibular cyst.
SUGGESTED READING
Pierse JE, Stern A. Benign cysts and tumors of the paranasal sinuses. Oral
Maxillofac Surg Clin North Am 2012;24:249–264.
Hemangiomas
1347
Key Facts
Hemangiomas are slow-flow vascular neoplastic masses characterized
by cavernous blood-containing spaces and represent a hamartomatous
neoplastic proliferation of vascular cells.
They are the most common airway and parotid masses in children and
also occur anywhere in the neck (e.g., muscles, temporal bone, or
paranasal cavities) and orbits.
Infantile-type hemangiomas are the most common; they are absent at
birth and become manifest in the first few months of life, and half of
them involute by age 5.
Congenital-type hemangiomas are rare. They are present and fully
grown at birth. Those that involute are called RICH (rapidly
involuting) and those that do not are called NICH (noninvoluting,
which may actually grow with the child). There is considerable
overlap between both clinically, by imaging and histology.
Hemangiomas of the cavernous (adult) type tend to occur after 16
years of age and do not involute with age. Most cavernous
hemangiomas in adults are located in the retro-orbital, intraconal
space.
Hemangiomas present as soft masses that increase in size with
Valsalva maneuver (crying in children) or during pregnancy; an
overlying skin hemangioma may also be present. When very large, it
may result in platelet consumption.
See section on PHACES syndrome for more information.
1348
FIGURE 27-23. Axial postcontrast CT shows an avidly enhancing mass
along the right mandible in a patient with an infantile hemangioma.
1349
FIGURE 27-24. Axial T2 in a different patient shows a mildly bright
mass replacing the left parotid gland (arrow) with small vascular flow
voids.
1350
FIGURE 27-25. Corresponding fat-suppressed postcontrast T1 shows avid
enhancement of the hemangioma.
1351
FIGURE 27-26. Axial T2, in a different patient, shows a hemangioma
(arrows) involving the deep and superficial left parotid lobes and
parapharyngeal space.
1352
FIGURE 27-27. Axial fat-suppressed postcontrast T1 in a baby shows a
large solid enhancing mass in the right parotid. Note large vessels in the
deep aspect of mass. This was a hemangioendothelioma, which tends to
occur often in this gland and be very large.
1353
FIGURE 27-28. Axial fat-suppressed postcontrast T1 image, in a different
patient, shows an enhancing lesion in the right masseter muscle.
SUGGESTED READING
Liang MG, Frieden IJ. Infantile and congenital hemangiomas. Semin Pediatr Surg
2014;23:162–167.
1354
Laryngeal Masses
Key Facts
The larynx extends from the valleculae to the space between the
cricoid and first tracheal ring; the supraglottis begins with the tip of
the epiglottis and ends in the laryngeal ventricle; the glottis is the true
vocal cords; the infraglottis extends from the undersurface of the true
vocal cords to the bottom of the cricoid.
Most common pathology in this location: squamous cell carcinoma,
laryngocele, thyroglossal duct cyst, stenosis, and trauma. Other
cancers: lymphoma, adenocarcinoma, minor salivary gland tumors,
and rarely sarcomas.
Laryngeal tumors are treated with radiation, chemotherapy, or surgery
depending on staging. If surgery is performed, the following
guidelines help you to interpret the images:
Most supraglottic tumors are squamous cell carcinomas; if the
tumor stops at the laryngeal ventricle, a supraglottic
laryngectomy is done (best voice-saving procedure).
If a tumor respects the anterior commissure and involves less
than the anterior third of the contralateral true cord, a vertical
hemilaryngectomy may be done (anterior commissure normally
measures <1 mm).
If a tumor has infraglottic extension (1 cm below laryngeal
ventricle), a total laryngectomy is needed.
Report in all laryngeal tumors: nodal stage, cartilage invasion
(relative contraindication to radiation, will upstage the tumor to T3 or
T4 requiring total laryngectomy), involvement of pre- and paraglottic
fat or postcricoid area, extralaryngeal extension, carotid encasement,
and status of infraglottis and of midline. Sclerosis of cartilages may
signify invasion or be just reactive changes.
MRI is more sensitive than CT for cartilage invasion but may
overestimate it due to inability of this technique to distinguish tumor
from inflammation.
1355
FIGURE 27-29. Axial STIR shows an infiltrating glottic tumor with
invasion of the thyroid cartilage bilaterally.
1356
FIGURE 27-30. Corresponding DWI shows increased signal within the
lesion due to restricted diffusion.
1357
FIGURE 27-31. Axial postcontrast CT in a different patient shows
subglottic extension of tumor (white arrow) along the cricoid cartilage
(black arrow).
1358
FIGURE 27-32. Coronal CT reformation, in a different patient, shows
tumor (arrow) in both false and true cords.
1359
FIGURE 27-33. Axial postcontrast CT in a patient with transcartilage
tumor extension shows large right laryngeal low-density (necrotic) mass
destroying right thyroid cartilage and extending to the strap muscles.
1360
FIGURE 27-34. Axial postcontrast CT in a different patient with
lymphoma involving the right paraglottic and pyriform sinus regions.
SUGGESTED READINGS
Baugnon KL, Beitler JJ. Pitfalls in the staging of cancer of the laryngeal
squamous cell carcinoma. Neuroimaging Clin N Am 2013;23:81–105.
Yousem DM, Tufano RP. Laryngeal imaging. Neuroimaging Clin N Am
2004;14:611–624.
Masticator Space Masses

1361
Key Facts
The masticator space normally contains the vertical mandibular
ramus, masseter, temporalis, medial pterygoid and lateral pterygoid
muscles, branches of the third division of the trigeminal nerve, and
the inferior alveolar artery and vein and is enclosed by the superficial
layer of the deep cervical fascia.
Most common lesions occurring here include odontogenic infections
(remember that infections may tract superiorly under the temporalis
muscle), primary or secondary bone tumors, neurogenic tumors,
lymphoma, leukemia, or invasion from squamous cell carcinoma
originating in the retromolar triangle.
1362
FIGURE 27-35. Axial postcontrast T1 shows a left nasopharyngeal
carcinoma (arrows) with extensive invasion of the masticator space.
Tumor enhances almost to the same degree as normal nasopharyngeal
tissue (arrowhead) from which it is difficult to separate.
1363
FIGURE 27-36. Axial postcontrast T1 in a different patient shows a left
parotid mucoepidermoid carcinoma (arrows) invading the left masseter
muscle.
1364
FIGURE 27-37. Axial postcontrast T1 in a different patient with leukemia
shows diffuse enhancement of tumor infiltrating the right masticator space.
1365
FIGURE 27-38. Coronal postcontrast CISS in a patient with lymphoma
shows tumor in left masticator space (arrows) with intracranial extension
along the mandibular division of cranial nerve 5 (arrowhead). There is
also tumor invading the left parotid and masseter muscles (wavy arrow).
1366
FIGURE 27-39. Axial postcontrast CT in a different patient with dental
disease shows an abscess (arrow) in the left masticator space.
1367
phlegmonous changes in the left masticator space with abscess in the
ipsilateral buccal space.
SUGGESTED READING
Fernandes T, Lobo JC, Castro R, et al. Anatomy and pathology of the masticator
space. Insights Imaging 2013;4:605–616.
Nasopharyngeal Masses
1368
Key Facts
Most common tumors: squamous cell carcinoma (>80%),
adenocarcinoma, minor salivary gland tumors, and lymphoma.
Etiology of nasopharyngeal carcinoma is not entirely clear and is
likely multifactorial, including potential risk factors such as tobacco
and alcohol use, diet, genetic predisposition, race, and viral infections
(EBV and HPV). Its incidence is increased in Chinese
(500:1,000,000) and African Americans (still rare: 7:1,000,000),
particularly males 30 to 60 years of age.
Types: keratinizing—sporadic form, localized and related to EBV (in
endemic areas), no nodes, poor response to radiation, and
nonkeratinizing—most common, may be differentiated or
undifferentiated, strongly associated with EBV, disseminated,
favorable prognosis. The basaloid form is an extremely rare but more
aggressive subtype.
Common symptoms: adenopathy (>50%), serous otitis media (due to
obstruction of eustachian tube orifice), nasal obstruction, epistaxis,
and neurologic dysfunction secondary to base of the skull or
cavernous sinus invasion.
Tumors begin in the fossa of Rosenmüller and have local extension
with initial metastases to the lateral retropharyngeal nodes of
Rouviere, spinal accessory (level 5) nodes, and lastly the internal
jugular nodes (levels 2, 3, and 4).
Nodes are present in 50% of patients at diagnosis and decrease
survival by half.
Nasopharyngeal cancer has low signal on T2 images and ADC maps
due to its high cellularity.
1369
FIGURE 27-41. Sagittal postcontrast T1 shows a large nasopharyngeal
carcinoma (arrow).
1370
FIGURE 27-42. Axial T2, in the same patient, shows the mass (arrow) to
have relatively low signal. There are pathologically large retropharyngeal
nodes (arrowheads) and invasion of the prevertebral musculature.
1371
FIGURE 27-43. Corresponding ADC map demonstrates low signal due to
high cellularity and restricted diffusion.
1372
FIGURE 27-44. Sagittal T1 in a different patient shows a necrotic tumor
(arrows) that is invading the skull base.
1373
FIGURE 27-45. Coronal postcontrast T1 in a different patient shows a
nasopharyngeal carcinoma (arrows) with perineural extension along the
third division of cranial nerve 5 (arrowhead) into the left cavernous sinus.
1374
FIGURE 27-46. Axial noncontrast T1 in a different patient with
disseminated nonkeratinizing (undifferentiated) carcinoma shows large
mass with invasion of central skull base.
SUGGESTED READINGS
Abdel Khalek Abdel Razek A, King A. MRI and CT of nasopharyngeal
carcinoma. AJR Am J Roentgenol 2012;198:11–18.
Glastonbury CM, Salzman KL. Pitfalls in the staging of cancer of nasopharyngeal
carcinoma. Neuroimaging Clin N Am 2013;23:9–25.
1375
Nasopharyngeal Angiofibroma
Key Facts
This histologically benign but locally aggressive tumor occurs almost
exclusively in young (hence, “juvenile”) males. Occasionally found in
females and may be associated with hormonal imbalance.
All originate near the pterygopalatine fossa or sphenopalatine
foramen, which may be expanded, and characteristically result in
bowing of the posterior maxillary sinus wall.
Common clinical symptoms include nasal obstruction (90%),
epistaxis (60%), facial deformities, anosmia, and headache.
Tumor tends to spread through anatomic pathways rather than
destruction, and extension in the maxillary and ethmoid sinuses is not
uncommon (30% to 40%); intracranial extension is less common (5%
to 10%).
They are almost exclusively fed by ipsilateral internal maxillary and
ascending pharyngeal arteries.
1376
FIGURE 27-47. Axial postcontrast CT shows an enhancing mass (white
arrows) extending to the right pterygopalatine fossa and nasopharynx.
Note normal pterygopalatine fossa on the left (black arrow) with fat and
vessels.
1377
FIGURE 27-48. Axial fat-suppressed T2 in the same patient at a higher
level shows the mass to be bright and to have multiple flow voids.
1378
FIGURE 27-49. Lateral DSA view, in the same patient, best demonstrates
the hypervascular nature of the tumor.
1379
FIGURE 27-50. Axial noncontrast CT, in a different patient, shows a very
large tumor with significant widening of the left pterygopalatine fossa and
posterior bulging of the maxillary sinus.
1380
avidly enhancing mass extending to the nasopharynx and right
pterygopalatine fossa.
1381
enhancing mass in the right nasal cavity and also involving the right
pterygopalatine fossa (arrows).
SUGGESTED READING
Blount A, Riley KO, Woodworth BA. Juvenile nasopharyngeal angiofibroma.
Nodal Metastases
Key Facts
1382
Fifty percent of squamous cell carcinomas of the upper airway have
nodal metastases at presentation; nodal metastases eventually develop
in up to 80% of these patients. Presence of one nodal metastasis
generally reduces lifespan by one-half.
The superior group of nodes is the lateral pharyngeal nodes (first-
order drainage of nasopharynx) and the jugulodigastric (JD) nodes.
Below the JD node, adenopathy may be classified as follows:
Level 1: submental (1A) and submandibular (1B) nodes (separated by
the anterior belly of the digastric muscle).
Level 2: internal jugular chain above hyoid bone; 2A, anteromedial or
lateral to jugular vein; 2B, posterior to jugular vein.
Level 3: internal jugular chain between hyoid bone and cricoid
cartilage.
Level 4: internal jugular chain below cricoid cartilage.
Level 5: posterior to sternocleidomastoid muscle and anterior to
trapezius muscles (spinal accessory chain of nodes), 5A, between
skull base and lower margin of cricoid cartilage; 5B, between cricoid
cartilage and clavicle.
Level 6: nodes related to thyroid gland.
Level 7: central compartment below the hyoid (tracheoesophageal
and anterior cervical chain).
Internal jugular nodes are the eventual drainage site for the entire
neck; a size >15 mm and/or central low density (necrosis) >3 mm are
compatible with metastases.
Most common cause of nodal metastases is squamous cell carcinoma;
lymphoma is the second most common (particularly Hodgkin) and
results in moderately to markedly enlarged nodes. Malignant nodes
tend to have lower ADC values than benign ones.
Most common cause of completely cavitated nodes is papillary
thyroid carcinoma (particularly at level 4).
Differential diagnosis of calcified nodes includes tuberculosis, treated
lymphoma, and metastases from neuroblastoma and papillary thyroid
carcinoma.
Differential diagnosis of hemorrhagic nodes includes metastases from
renal cell, thyroid, and breast carcinomas.
Nodes that are bright on precontrast MR T1-weighted images include
those containing thyroglobulin and melanoma.
1383
FIGURE 27-53. Axial postcontrast CT shows necrotic bilateral level 1B
(arrowheads) and left level 2A (arrow) nodes.
1384
FIGURE 27-54. Axial T2 image, in a different patient, shows marked
enlargement of a left retropharyngeal node.
1385
FIGURE 27-55. Axial postcontrast CT, in a different patient, shows a
necrotic right level 2A node (arrow) in a patient with an ipsilateral
retromolar squamous cell carcinoma (not shown).
1386
FIGURE 27-56. Axial postcontrast CT, in a different patient, shows left
level 2B (arrowhead) and 5 (arrow) necrotic nodes.
1387
very large right level 6 necrotic node.
1388
FIGURE 27-58. Axial postcontrast CT, in a different patient, shows large
nodes in levels 5, 6, and 7.
SUGGESTED READINGS
Eisenmenger LB, Wiggins RH III. Imaging of head and neck lymph nodes.
Radiol Clin North Am 2015;53:115–132.
Saindane AM. Pitfalls in the staging of cervical lymph node metastasis.
Oral Cavity and Oropharyngeal Space

Masses
Key Facts
Only 7% of masses in this area are malignant, but >90% of
malignancies are squamous cell carcinomas, followed by lymphoma,
1389
adenoid cystic carcinoma, mucoepidermoid carcinoma, and
adenocarcinoma.
In children, the most common lesions at this location are
hemangioma, lymphatic malformations, and tonsillar abscesses.
Common risk factors in adults include tobacco and alcohol use and
HIV infection.
Most masses in this region are found incidentally during routine
physical examination, but pain in the external auditory canal is an
important clinical symptom.
Most tumors in this region arise in the floor of the mouth (divided by
the mylohyoid muscle into submandibular [below] and sublingual
[above] spaces), gingival folds, retromolar trigone, base of the
tongue, and palate.
The presence of sialectasis without a radiopaque stone should prompt
a clinical oral exam to rule out an obstructing mucosal lesion.
Epidermoids and dermoids are distinct slow-growing lesions of the
sublingual space that have a fatty appearance and may be identical to
lipomas.
Lingual thyroid should be included in the differential diagnosis of
masses that enhance in the base of the tongue.
Ranulas are retention cysts of salivary glands that may remain
confined to the sublingual space (simple) or cross the mylohyoid
muscle (diving); diving ranulas have no true walls.
1390
FIGURE 27-59. Axial postcontrast T1 shows carcinoma in left palatine
tonsil region (star) and bilaterally enlarged retropharyngeal lymph nodes
(arrows).
1391
FIGURE 27-60. Midline postcontrast CT reformation in a different patient
shows a mucoepidermoid carcinoma (arrow) in the soft palate.
1392
FIGURE 27-61. Axial postcontrast CT, in a different patient, shows bulky
tumor (arrow) in left tongue base extending posteriorly to the tonsil via the
glossotonsillar sulcus.
1393
large infiltrative squamous cell carcinoma in the left oral tongue invading
the mandible and premandibular tissues (arrow).
1394
right peritonsillar abscess (arrow) with mass effect on the airway.
1395
FIGURE 27-64. Axial postcontrast T1 in a patient with large necrotic
undifferentiated sarcoma arising from the posterior wall of the oropharynx.
SUGGESTED READING
Meesa IR, Srinivasan A. Imaging of the oral cavity. Radiol Clin North Am
2015;53:99–114.
Paragangliomas (Skull Base)

Key Facts
1396
Paragangliomas are rare tumors derived from neural crest cells; most
are histologically benign, but <6% show malignancy.
More common in females, age 40 to 60 years.
Common locations: carotid body, jugular bulb, and cochlear
promontory, but they may occur anywhere in the body.
Glomus jugulare characteristically erode the jugular foramen,
enhance intensely, and contain calcifications and multiple flow voids
(salt-and-pepper appearance if larger than 2.5 cm in diameter). May
extend to tympanic cavity (glomus “jugulotympanicum”).
Remember that the normal carotid body may be seen on CT
angiography but should not measure more than 4 mm.
Glomus jugulare and tympanicum (or jugulotympanic) usually
present with pulsatile tinnitus and jugular foramen syndrome
(involvement of ninth, tenth, and eleventh cranial nerves).
Three percent are multicentric, except in familial paragangliomas,
where up to 25% are multicentric.
After radiation therapy, these tumors show stabilization or reduction
in size, decreased enhancement, and diminished flow voids.
About one-third may be part of a germline mutation (particularly
when multicentric and in patients younger than 40) in the succinate
dehydrogenase gene group (SDHx, with a higher frequency of smaller
tumors) or part of the von Hippel-Lindau, “multiple endocrine
neoplasia,” and other “neural crest” cell syndromes.
Octreotide (somatostatin) or PET (FDG, FDOPA, or FD) radionuclide
scanning is useful to assess the nature of the tumor and find additional
lesions.
1397
FIGURE 27-65. Coronal CT reformat in a patient with glomus
tympanicum shows a mass in the right middle ear cavity along the cochlear
promontory (arrow).
1398
FIGURE 27-66. Corresponding axial postcontrast T1 shows the mass
(arrow) to enhance avidly.
1399
FIGURE 27-67. Octreotide scan, in the same patient, shows avid
radiotracer uptake by the tumor (arrow).
1400
FIGURE 27-68. Coronal CT, in a different patient, shows tumor (M)
destroying temporal bone and extending to hypotympanum (arrow), thus a
glomus jugulotympanicum.
1401
FIGURE 27-69. Axial CT in a different patient shows a right glomus
jugulare (arrows) with osseous destruction.
1402
FIGURE 27-70. Corresponding axial postcontrast T1 shows enhancement
of the mass, which also features small internal flow voids (arrow).
SUGGESTED READING
Castinetti F, Kroiss A, Kumar R, et al. 15 Years of paraganglioma: imaging and
imaging-based treatment of pheochromocytoma and paraganglioma. Endocr
Relat Cancer 2015;22:T135–T145.
Parapharyngeal Space Masses

Key Facts
1403
Normal parapharyngeal space contains fat, third division of
trigeminal nerves, branches of internal maxillary artery, and
ascending pharyngeal artery and veins.
Parapharyngeal space is divided into a prestyloid compartment (80%
of masses occur here and are mostly pleomorphic adenomas), and
poststyloid compartment (20% of masses arise here and are mostly
neurogenic tumors and paragangliomas).
In children, consider second branchial cleft cysts, abscesses, and
pleomorphic adenomas.
Squamous cell carcinoma usually arises from the adjacent pharyngeal
mucosal space and invades the parapharyngeal space secondarily.
Clue: If the parapharyngeal space is not identified in the presence of a
mass in that region, it is likely that the mass arises in the
parapharyngeal space.
Pleomorphic adenomas are typically well circumscribed and very
bright on T2, may have a T2 dark rim, and do not have restricted
diffusion on ADC maps. About 1% to 10% of pleomorphic adenomas
will develop carcinomas in them (carcinoma ex pleomorphic
adenoma), and these show restricted diffusion. Postsurgical
recurrence occurs in 20% to 40% and is seen as nodular T2
hyperintense lesions in the surgical bed.
1404
FIGURE 27-71. Axial postcontrast T1 shows enhancing left pleomorphic
adenoma (star). Note normal fat in opposite parapharyngeal space (arrow).
1405
large pleomorphic adenoma (black arrow) in the right parapharyngeal
space. Note normal left parapharyngeal fat (white arrow).
1406
FIGURE 27-73. Axial ADC map, in a different patient, shows left-sided
pleomorphic adenoma (arrow) without restricted diffusion.
1407
right-sided low-density schwannoma (arrow).
1408
FIGURE 27-75. Axial T2 in a different patient shows a right
parapharyngeal pleomorphic adenoma (arrow) with well-defined and
lobulated margins.
1409
FIGURE 27-76. Corresponding axial postcontrast T1 shows avid but
somewhat heterogeneous enhancement of the lesion (arrow).
SUGGESTED READINGS
Gupta A, Chazen JL, Phillips CD. Imaging evaluation of the parapharyngeal
space. Otolaryngol Clin North Am 2012;45:1223–1232.
Zaghi S, Hendizadeh L, Hung T, et al. MRI criteria for the diagnosis of
pleomorphic adenoma: a validation study. Am J Otolaryngol
2014;35:713–718.
Parotid Space Masses
1410
Key Facts
Normal parotid space contains the parotid gland, nodes,
retromandibular vein (which arbitrarily divides the gland into deep
and superficial portions on imaging studies), facial nerve (which
anatomically divides the gland into deep and superficial portions),
and external carotid artery.
In a child with a parotid mass, consider hemangioma, lymphangioma,
first branchial cleft cyst, and pleomorphic adenoma.
In AIDS patients with parotid masses, consider benign
lymphoepithelial cysts, infection, and lymphoma.
In adults, most common tumors in this space are as follows: benign
mixed tumor (pleomorphic adenoma) (>80%), Warthin tumor
(especially in older males with bilateral parotid masses),
mucoepidermoid carcinoma, adenoid cystic carcinoma (especially
with multiple cranial nerve deficits secondary to perineural tumor
spread), metastases, and lymphoma (especially in patients with
multiple masses and those with history of Sjögren disease).
Facial nerve palsy in the presence of a parotid mass implies
malignancy and poor prognosis.
MRI reveals that benign tumors are hyperintense on T2, while
malignant tumors are hypointense on T2. DWI shows malignant
tumors to have restricted diffusion, although there is overlap with
Warthin tumors, which contain lymphoid tissue and have low ADC
values.
1411
FIGURE 27-77. Axial CT shows multiple intraparotid cysts in an AIDS
patient.
1412
Warthin tumor (arrows) involving the deep aspect of the left parotid gland.
FIGURE 27-79. Axial fat-suppressed T2 shows a bright pleomorphic

adenoma in the left parotid gland.
1413
FIGURE 27-80. Axial fat-suppressed postcontrast T1, in the same patient,
shows avid and heterogeneous enhancement of the tumor.
1414
FIGURE 27-81. Corresponding ADC map shows the mass to have high
signal.
1415
FIGURE 27-82. Axial postcontrast CT in a different patient shows an
enhancing metastasis in the right parotid gland (arrow) secondary to a
cutaneous squamous cell carcinoma.
SUGGESTED READINGS
Christe A, Waldherr C, Hallett R, et al. MR imaging of parotid tumors: typical
lesion characteristics in MR imaging improve discrimination between benign
and malignant disease. AJNR Am J Neuroradiol 2011;32:1202–1207.
Thoeny HC, De Keyzer F, King AD. Diffusion-weighted MR imaging in the head
and neck. Radiology 2012;263:19–32.
1416
Retropharyngeal Space Masses
Key Facts
The retropharyngeal compartment (also called the “retrovisceral”
compartment of the visceral space) is a potential space between the
buccopharyngeal fascia and alar fascia that contains fat and medial
and lateral retropharyngeal nodes (between the prevertebral muscles
and pharyngeal constrictor muscles) and extends from the base of the
skull to T3. It is different than the “prevertebral” space that is limited
posteriorly by the cervical vertebrae and anteriorly by the prevertebral
fascia. The “danger” space is between the alar and prevertebral fascia
and extends from skull base to posterior mediastinum.
Most common lesions of the retropharyngeal space are inflammatory
processes (especially in children), which include reactive adenopathy
and abscesses. In children, calcific tendinitis may produce swelling
and pain.
In adults, adenopathy at this level is suspicious for metastatic disease
until proven otherwise.
Other lesions include hemangioma, lipoma, lymphoma, and direct
invasion by carcinoma.
Occasionally, the ICAs (one or both) are in this location (which can
be a transient finding) and may present as retropharyngeal pulsatile
masses and cause dysphagia. In cases of inflammation, the
corresponding ICA may be narrowed.
1417
FIGURE 27-83. Axial postcontrast CT shows extensive pharyngeal and
retropharyngeal edema (arrow). Note row of vessels along the
buccopharyngeal fascia (arrowhead).
1418
FIGURE 27-84. Axial postcontrast CT in a different patient shows a large
abscess in the retropharyngeal space (star) with peripheral enhancement
and marked mass effect on the airway.
1419
necrotic left retropharyngeal node (of Rouviere, arrow) in a patient with
oropharyngeal cancer.
1420
FIGURE 27-86. Axial postcontrast CT in a different patient shows a
retropharyngeal effusion. Note anteriorly displaced vessels along the
buccopharyngeal fascia.
1421
FIGURE 27-87. Corresponding sagittal CT reformat shows calcification
of the superior fibers of the longus colli tendons (arrow) in this patient
with longus colli tendinitis.
1422
FIGURE 27-88. Axial postcontrast CT shows retropharyngeal course of
the internal carotid arteries bilaterally (arrow).
SUGGESTED READINGS
Debnam JM, Guha-Thakurta N. Retropharyngeal and prevertebral spaces:
anatomic imaging and diagnosis. Otolaryngol Clin North Am
2012;45:1293–1310.
Hoang JK, Branstetter BFt, Eastwood JD, et al. Multiplanar CT and MRI of
collections in the retropharyngeal space: is it an abscess? AJR Am J
Roentgenol 2011;196:W426–W432.
Sialolithiasis
1423
Key Facts
Most common location for stones (80%) is Wharton duct (since it is
the longest duct, has a more uphill course, and is prone to stasis of
saliva, which is more viscous due to higher mucin content).
Most stones in Wharton duct are lodged distally at aperture (punctum)
or where it crosses the mylohyoid muscle.
Stones may also be found in Stensen duct of the parotid gland (10%
to 15%, distally at aperture) and in sublingual glands (>5%).
Most stones are solitary and radiopaque.
Stones may lead to acute sialadenitis and abscess formation in gland
and/or duct.
Stones may be missed by MR; therefore, if an inflammatory process
is considered, CT is best; if a tumor of the salivary gland is suspected,
MRI is the ideal imaging method.
In the presence of sialectasis and absence of a radiopaque stone on
CT, an oral mucosal lesion must be ruled out by clinical exam.
1424
FIGURE 27-89. Axial postcontrast CT shows a stone in the left distal
Wharton duct (arrowhead) and formation of a sialocele in the left
submandibular gland (arrow).
1425
FIGURE 27-90. Coronal postcontrast CT, in a different patient, shows a
stone in the right submandibular duct (arrowhead) at the level of the
mylohyoid muscle and inflammatory changes in the right submandibular
gland (arrow).
1426
FIGURE 27-91. Axial CT, in a different patient, shows a stone (arrow) in
the distal right Stensen duct with periductal inflammatory changes.
1427
FIGURE 27-92. Axial CT, in a different patient, shows a stone in the left
submandibular duct (arrow) with concentric rings of mineralization.
1428
FIGURE 27-93. Axial CT, in a different patient, shows a stone involving
a long segment of the right submandibular duct (arrow).
1429
FIGURE 27-94. Corresponding parasagittal CT reformat shows the stone
(black arrow) and resultant sialectasis (arrowhead) and sialocele (white
arrow).
SUGGESTED READING
Abdullah A, Rivas FF, Srinivasan A. Imaging of the salivary glands. Semin
Roentgenol 2013;48:65–74.
Slow-Flow Vascular Malformations
1430
Key Facts
Three different types:
Capillary: port-wine stains, angiokeratomas, and telangiectasias;

imaging provides a limited role for these as they are superficial and
clinically evident.
Venous malformations:
Most common low-flow vascular malformations; 50% identified
at birth, expand with Valsalva maneuvers, and grow with the
patient.
Phleboliths can be seen in about half of them and are highly
suggestive (identified on CT or sometimes as signal voids on
MR).
Scattered puddles of contrast enhancement may be seen due to
venous lakes.
Lymphatic malformations (lymphangiomas):
These benign nonencapsulated masses contain large dilated
spaces filled with milky fluid.
About >80% of all lymphangiomas occur in the neck
(particularly the lower neck), and up to 10% extend into the
mediastinum.
About 50% of lesions are present at birth, and the majority are
found before 2 years of age; in adults, about 30% of cystic neck
masses are benign and related to lymphatic malformations.
Most are macrocystic and occur below the mylohyoid muscle in
the posterior or less commonly anterior triangles of the neck.
Microcystic ones are more commonly found above the
mylohyoid muscle particularly floor of the mouth and
submandibular region.
They may be associated with syndromes (Turner, Noonan).
By CT or MRI, they may contain fluid levels due to hemorrhage
and presence of unclotted blood and their septa may enhance.
Lymphatics and neck veins form at around the same time and thus
slow-flow malformations can be mixed.
1431
FIGURE 27-95. Axial T2 shows a multiloculated lymphatic malformation
(arrow) anterolateral to the right submandibular gland. Note numerous
fluid levels.
1432
FIGURE 27-96. Sagittal T2 in a different patient shows a large unilocular
lymphatic malformation in the posterior triangle.
1433
FIGURE 27-97. Coronal postcontrast T1 in a different patient shows a
large lymphatic malformation (arrow) in the left neck. Note faint septal
enhancement.
1434
FIGURE 27-98. Axial T2 in a different patient shows a lobulated
multicompartmental mass in the right and posterior neck also involving the
parapharyngeal and retropharyngeal spaces.
1435
FIGURE 27-99. Postcontrast T1, in the same patient, shows avid
enhancement of the lesion consistent with a venous malformation.
1436
FIGURE 27-100. Coronal postcontrast T1 in a different patient shows
signal voids related to phleboliths (arrows) in a venous malformation.
SUGGESTED READING
Griauzde J, Srinivasan A. Imaging of vascular lesions of the head and neck.
Radiol Clin North Am 2015;53:197–213.
Sublingual and Submandibular Space

Masses
1437
Key Facts
The sublingual space is located superomedial to the mylohyoid
muscle and mainly contains the tongue, lingual neurovascular plexus,
sublingual glands and ducts, and Wharton ducts.
Most common lesions: squamous cell carcinoma, tumors of the
sublingual gland, cellulitis and abscesses, calculi, ranulas,
lymphangioma, epi-/dermoid, ectopic thyroid, and hemangioma
(in children).
The submandibular space is located inferolateral to the mylohyoid
muscle, superior to the hyoid bone, and medial to the horizontal
ramus of the mandible and contains most of the submandibular gland,
nodes, anterior belly of the digastric muscle, and facial vein and
artery.
Most common lesions: metastases, invasion by squamous cell
cancer from the mouth, submandibular gland tumors, lipoma,
epi-/dermoid, ranulas, infections, second branchial cleft cyst, and
lymphangioma.
1438
FIGURE 27-101. Axial fat-saturated T2 shows a midline dermoid in the
sublingual space (arrow).
1439
FIGURE 27-102. Axial postcontrast CT in a patient with Ludwig angina
shows extensive inflammatory changes in the submandibular region with
formation of an abscess on the left side.
1440
simple ranula in the left sublingual space (arrow).
1441
FIGURE 27-104. Axial T2, in a different patient, shows a plunging ranula
in the submandibular space (arrow).
1442
low-density submandibular midline epidermoid.
1443
right submandibular gland with surrounding inflammation due to
presumed viral sialoadenitis.
SUGGESTED READING
Agarwal AK, Kanekar SG. Submandibular and sublingual spaces: diagnostic
imaging and evaluation. Otolaryngol Clin North Am 2012;45:1311–1323.
Thyroglossal Duct Cyst
1444
Key Facts
Most common midline mass in children; less common in adults.
Locations include below the hyoid bone (65%), suprahyoid (20%),
and at level of hyoid bone (15%) or a combination of these locations
(anywhere along the descent path of the thyroid anlage, which starts
at the foramen cecum).
They generally measure 2 to 4 cm and may enlarge progressively;
sudden enlargement may be associated with upper respiratory
infections; carcinomas (papillary and squamous types) occur in 1% of
them and are related to ectopic thyroid tissue, which is present in
about a third of cysts.
By imaging, they are uni- or multilocular masses with peripheral
enhancement centered in the strap muscles, and the hyoid bone may
contain a midline cleft. Can present in a paramedian location
particularly if infrahyoid.
If track is not completely resected, they may recur at the base of the
tongue.
Main differential diagnosis: lingual thyroid, dermoid, epidermoid,
abscess, external laryngocele, and necrotic node.
1445
FIGURE 27-107. Axial noncontrast T1 shows a bright cyst in a typical
location (arrow) spreading the strap muscles.
1446
FIGURE 27-108. Midsagittal CT reformation, in a different patient,
shows a cyst (arrow) abutting the inferior aspect of the hyoid bone.
1447
small cyst (arrow) arising at the foramen cecum.
1448
large infrahyoid paramedian cyst with significant mass effect (arrows).
1449
cyst with thick and ill-defined walls due to inflammation (arrow).
1450
frank abscess formation within a thyroglossal duct cyst.
SUGGESTED READING
Zander DA, Smoker WR. Imaging of ectopic thyroid tissue and thyroglossal duct
cysts. Radiographics 2014;34:37–50.
Thyroid Masses
Key Facts
1451
Most common cause of thyroid enlargement (adult and children) is
multinodular goiter (most due to prior viral infection in developed
countries).
Most focal thyroid masses are benign adenomas (but cannot be
distinguished from small carcinomas), colloid cysts, or focal
multinodular goiters.
The most common thyroid carcinomas: papillary (>50%, which has
the best prognosis), follicular (20%), medullary (10%), anaplastic
(>5%, worst prognosis), and Hürthle cell (>2%). Important risk
factors include prior radiation, family history, and multiple endocrine
neoplasia type II (medullary type).
Lymphoma is not unusual, and in some cases, there is a history of
thyroiditis.
Both lymphoma and anaplastic carcinoma tend to invade the trachea.
Tumors of the thyroid gland may result in cystic, calcified, or
extremely vascular nodal metastases, often confined to levels 3 and 4.
Malignant tumors generally show restricted diffusion on ADC maps.
1452
FIGURE 27-113. Axial postcontrast CT shows enlarged thyroid with
multiple low-density regions in a multinodular goiter.
1453
FIGURE 27-114. Coronal postcontrast CT in a different patient with
papillary carcinoma shows a large mass with cystic changes expanding the
right thyroid gland.
1454
thyroid lymphoma completely encircling the airway. Bubble of gas on left
is postbiopsy.
1455
FIGURE 27-116. Axial postcontrast CT, in a different patient, shows an
avidly enhancing hypervascular metastasis in left neck level 3 from a
papillary thyroid carcinoma (arrow).
1456
large mass predominantly in right lobe containing low-density zones and
calcifications. Note airway displacement in this anaplastic carcinoma.
1457
FIGURE 27-118. Coronal postcontrast CT reformation, in a different
patient, shows nearly cystic nodal metastases (arrow) from thyroid
carcinoma.
SUGGESTED READINGS
Bahl M, Sosa JA, Nelson RC, et al. Imaging-detected incidental thyroid nodules
that undergo surgery: a single-center experience over 1 year. AJNR Am J
Neuroradiol 2014;35:2176–2180.
Nguyen XV, Choudhury KR, Eastwood JD, et al. Incidental thyroid nodules on
CT: evaluation of 2 risk-categorization methods for work-up of nodules.
Tornwaldt Cyst
Key Facts
1458
Tornwaldt cysts represent a remnant of the notochordal bursa and are
found in ~6% of the population.
They are located in the superior posterior nasopharynx centrally
between the longus capiti muscles (occasionally, they may be
eccentric).
They are almost always incidentally discovered; but they may cause
abscess, halitosis, and sore throat.
Usually, they measure 1 to 5 mm in diameter (in rare instances, they
may measure up to 3 cm in diameter) and contain highly
proteinaceous fluid, which may produce high signal intensity on both
T1 and T2 and also low T2 signal intensity. Ventrally, they are
covered by a layer of enhancing nasopharyngeal mucosa.
Main differential diagnosis: adenoid tissue and mucosal retention
cysts (commonly multiple and off midline).
FIGURE 27-119. Midsagittal noncontrast T1 shows a typical low-

intensity Tornwaldt cyst (arrow) surrounded by mucosa.
1459
FIGURE 27-120. Axial postcontrast fat-suppressed T1, in the same
patient, shows cyst (arrow) in midline nasopharyngeal mucosa.
1460
FIGURE 27-121. Axial T2, in a different patient, shows a bright
Tornwaldt cyst (arrow).
1461
FIGURE 27-122. Corresponding postcontrast T1 shows a thin layer of
enhancing mucosa covering the cyst.
1462
FIGURE 27-123. Axial noncontrast T1 image, in a different patient,
shows a bright cyst (arrow).
1463
FIGURE 27-124. Corresponding T2 image shows the cyst (arrow) to be
dark.
SUGGESTED READING
Sekiya K, Watanabe M, Nadgir RN, et al. Nasopharyngeal cystic lesions:
Tornwaldt and mucous retention cysts of the nasopharynx: findings on MR
imaging. J Comput Assist Tomogr 2014;38:9–13.
1464
CHAPTER 28
Orbits
Globe Calcifications
Key Facts
Calcifications are related to deposition of calcific hyaline-like
material at the surface or deep within the optic disk (drusen bodies are
commonly bilateral while choroidal osteomas tend to be unilateral).
Drusen bodies are located at the level of the optic nerve head.
Drusen bodies are found in <1% of the population, may be familial,
and are more common in patients with retinitis pigmentosa.
When the drusen body is deep, it may elevate and blur the margins of
the optic disk (“buried” drusen) and clinically mimic papilledema.
Usually drusen bodies are asymptomatic and incidentally found, but
occasionally permanent or episodic visual field defects may be
present.
Choroidal osteomas are located distal to the optic disk and are more
common in patients with tuberous sclerosis.
Choroidal osteomas cannot be differentiated from the more common
idiopathic subchoroidal calcifications.
Disorders of calcium and phosphorus metabolism may also produce
ocular calcifications.
Occasionally, choroidal angiomas will calcify.
The end result of any severe infection, inflammation, or traumatic
process to the eye is globe shrinking and calcification (phthisis bulbi).
Calcifications at the tendinous insertions of the extraocular muscles
(uni- or bilateral) are normal and seen in older individuals.
1465
FIGURE 28-1. Axial CT shows calcified drusen in the optic nerve heads
bilaterally.
FIGURE 28-2. Axial CT, in a different patient, shows left calcified

choroidal osteoma (arrow).
1466
FIGURE 28-3. Axial CT, in a different patient, shows bilaterally calcified
(arrows) small globes in phthisis bulbi.
FIGURE 28-4. Axial CT, in a different patient, shows normal

calcifications at insertions of left medial and lateral recti muscles (limbus
calcifications).
1467
FIGURE 28-5. Axial CT in a different patient shows a contracted and
partially calcified left globe (phthisis bulbi). Note glaucoma valve on the
right side.
SUGGESTED READING
Davis PL, Jay WM. Optic nerve head drusen. Semin Ophthalmol
2003;18:222–242.
Orbital Dermoid
Key Facts
Represents the most common periorbital mass in children; more than
half of craniofacial dermoids occur in or near the orbits.
Inclusion of ectodermal elements along fusion lines of orbit; most
common location is frontozygomatic suture (so-called “lacrimal”
1468
dermoids); occasionally may present as a retro-orbital mass with
proptosis.
Generally asymptomatic (only a nontender mass is present) but
occasionally may rupture (spontaneously or after trauma) leading to
an intense local inflammatory reaction (due to leaked keratinous
contents) and mimicking orbital cellulitis or can rarely become
infected; growth is very slow.
CT shows low density (“fatty” appearance) in 50% of cases; widening
of frontozygomatic suture by mass is typical.
MRI shows high T1 signal if fatty, fat–fluid levels are typical but rare
(more common in large lesions).
If fluid levels are present in an intraconal mass, consider dermoid,
lymphangioma, chocolate cyst, and rare aneurysmal bone cyst
extending into orbit. Remember that the most common retro- ocular
mass to contain a fluid level is a dermoid.
Main differential diagnosis: mucocele (from adjacent paranasal
sinus), venolymphatic malformation, and sebaceous cyst.
FIGURE 28-6. Axial CT shows a low-density dermoid (arrow) in the left

medial canthal region.
1469
FIGURE 28-7. Axial CT in a different patient shows typical location of a
dermoid (arrow) along the right frontozygomatic suture.
1470
FIGURE 28-8. Axial CT, in a different patient, shows a ruptured dermoid
(arrow) with mass effect on the globe and stranding of the orbital fat due
to inflammation.
1471
FIGURE 28-9. Axial T2, in a different patient, shows a small bright
dermoid (arrow).
1472
FIGURE 28-10. Coronal CT, in a different patient, shows fatty dermoid
with fluid level in inferior right orbit.
1473
FIGURE 28-11. Axial T2, in a different patient, shows dependent fluid
level in large left intraconal dermoid.
SUGGESTED READINGS
Chung EM, Murphey MD, Specht CS, et al. From the Archives of the AFIP.
Pediatric orbit tumors and tumorlike lesions: osseous lesions of the orbit.
Golden BA, Jaskolka MS, Ruiz RL. Craniofacial and orbital dermoids in children.
Oral Maxillofac Surg Clin North Am 2012;24:417–425.
Graves Ophthalmopathy
Key Facts
1474
Develops in the majority of Graves’ patients but may occur in
euthyroid patients and can rarely be seen in Hashimoto thyroiditis;
resolves spontaneously in >90% of cases (the remaining patients
develop eye complications); most common cause of proptosis in
adults.
Histologically, it is an inflammatory leukocytic infiltration with
edema and deposition of mucopolysaccharides, end result of which is
fibrosis, lipomatosis, and fatty degeneration.
May lead to optic neuropathy (due to compression of the optic nerve),
diplopia (due to muscle entrapment), corneal ulcers (due to
proptosis), and conjunctival congestion.
May involve any extraocular muscle but most often affects the medial
and inferior recti. It is usually bilateral but may be unilateral or
asymmetric in 15% of cases.
MRI: muscles may be of high T2 signal reflecting edema (the normal
diameter of the belly of the recti muscles is 3 mm).
Other imaging findings: increased amount of retro-ocular fat,
stranding of the intraconal fat, enlarged lacrimal gland, stretched
optic nerve, and enlarged superior ophthalmic vein.
Main differential diagnosis: inflammatory pseudotumor, myositis,
lymphoma, and metastases.
1475
FIGURE 28-12. Coronal CT reformat shows thickening of the extraocular
muscles bilaterally and stranding of the orbital fat with a “dirty”
appearance.
1476
FIGURE 28-13. Axial CT, in a different patient, shows that the muscle
thickening is predominantly at the level of their bellies with sparing of
their tendinous insertions.
1477
FIGURE 28-14. Coronal noncontrast T1, in a different patient, shows
marked enlargement of muscles particularly the medial and inferior recti.
The fat has a “dirty” appearance.
1478
FIGURE 28-15. Corresponding postcontrast T1 shows marked muscle
enhancement.
FIGURE 28-16. Axial CT in a different patient shows unilateral

enlargement of the right medial rectus muscle.
1479
FIGURE 28-17. Coronal CT reformat in a different patient shows
postsurgical changes following orbitotomy with decompression of the left
orbital floor. The superior, medial, and inferior rectus muscles are enlarged
bilaterally but more severely on the left.
SUGGESTED READINGS
Muller-Forell W, Kahaly GJ. Neuroimaging of Graves’ orbitopathy. Best Pract
Res Clin Endocrinol Metab 2012;26:259–271.
Politi LS, Godi C, Cammarata G, et al. Magnetic resonance imaging with
diffusion-weighted imaging in the evaluation of thyroid-associated
orbitopathy: getting below the tip of the iceberg. Eur Radiol
2014;24:1118–1126.
Orbital Cavernous Malformation
1480
(Hemangioma, Adult Type)
Key Facts
Cavernous hemangiomas are the most common retro-ocular
intraconal tumor in adults (found mainly during the second to fourth
decades of life and in females); they are almost always intraconal,
have a fibrous pseudocapsule, receive very little blood supply, and
may rarely calcify; they can be resected relatively easily.
They are thought to be congenital and grow slowly over time (except
for potential periods of rapid growth during puberty and pregnancy).
Many patients have only proptosis without visual defects despite a
relatively large mass.
CT: hyperdense well-defined mass before contrast; calcifications may
be present; + contrast enhancement.
MRI: T1 isointense to muscles, T2 hyperintense with hypointense
capsule, and patchy contrast enhancement. On dynamic contrast-
enhanced MR, they may show filling in with contrast.
Main differential diagnosis: meningioma, schwannoma/neurofibroma,
metastases, venous varix, and venolymphatic malformation.
1481
FIGURE 28-18. Axial postcontrast fat-suppressed T1 shows large right
lobulated intraconal deeply enhancing mass.
1482
FIGURE 28-19. Axial noncontrast CT, in a different patient, shows well-
defined mass in the left orbital apex with expansion of superior orbital
fissure.
1483
FIGURE 28-20. Axial T2, in a different patient, shows small right
intraconal mass of high signal with dark capsule.
1484
FIGURE 28-21. Parasagittal postcontrast CT reformat, in a different
patient, shows a small mass with patchy contrast enhancement and
remodeling of the orbital floor (arrow).
1485
FIGURE 28-22. Axial fat–suppressed T2, different patient, shows a large,
bright, well-circumscribed, and somewhat lobulated mass with proptosis
and mass effect on the optic nerve (arrow).
1486
FIGURE 28-23. Corresponding fat-suppressed postcontrast T1 shows the
mass to enhance avidly.
SUGGESTED READINGS
Rootman DB, Heran MK, Rootman J, et al. Cavernous venous malformations of
the orbit (so-called cavernous haemangioma): a comprehensive evaluation of
their clinical, imaging and histologic nature. Br J Ophthalmol
2014;98:880–888.
Smoker WR, Gentry LR, Yee NK, et al. Vascular lesions of the orbit: more than
meets the eye. Radiographics 2008;28:185–204.
Infantile and Congenital Hemangiomas

Key Facts
1487
Infantile hemangiomas:
Most common benign vascular tumors of infancy; usually absent
at birth (except for sometimes a superficial mark) with rapid
growth in the first few months of life followed by involution
over several years; more frequent in females (particularly as part
of the PHACE association, although most are isolated).
Stain positive for GLUT1, which distinguishes them from other
vascular lesions.
Respond to propranolol (when treatment needed), which has
largely replaced steroids.
Congenital hemangiomas:
As opposed to infantile ones, congenital hemangiomas are rare
and fully formed at birth. These tumors are of two classes:
rapidly involuting congenital hemangioma (RICH) and
noninvoluting congenital hemangioma (NICH); it may be
difficult to tell them apart by imaging, but RICH contains larger
blood vessels.
Most RICH lesions completely involute by about 1 year of age.
NICH type does not involute and grows with the child. Their
natural history may overlap: partially involuting (PICH).
They are GLUT1 negative and do not respond to propranolol.
Only very large ones result in Kasabach-Merritt syndrome.
Main differential diagnosis: venolymphatic malformation, cellulitis,
and lymphoma/leukemia.
1488
FIGURE 28-24. Axial T2 shows an infiltrative lesion in the right orbit
(arrow) with tiny flow voids.
1489
FIGURE 28-25. Corresponding axial postcontrast T1 demonstrates avid
enhancement of the lesion.
1490
FIGURE 28-26. Axial T2 in a different patient demonstrates a large retro-
orbital lesion on the left side (arrow) resulting in proptosis.
1491
FIGURE 28-27. Parasagittal postcontrast T1 shows that the lesion
enhances avidly.
1492
diffuse enhancement of a right orbital tumor.
1493
FIGURE 28-29. Corresponding fat-suppressed postcontrast T1 after 6
months of propranolol treatment shows near-complete resolution of this
infantile hemangioma.
SUGGESTED READING
Liang MG, Frieden IJ. Infantile and congenital hemangiomas. Semin 2 Pediatr
Surg 2014;23:162–167.
Inflammatory Pseudotumor
Key Facts
1494
Idiopathic inflammation (although probably related to lymphoma as
both are “lymphocytic” infiltrates) affecting patients of any age or sex
and presenting with painful proptosis and a red eye (most common
cause for these clinical findings in adults), 75% are unilateral.
IgG4-related disease of the orbit is now increasingly recognized as a
cause of “idiopathic” inflammatory pseudotumor.
Common locations: retro-orbital fat, extraocular muscle(s), optic
nerve, sclera, uvea, and lacrimal gland.
May present as a mass (tumefactive type) or as an enlargement of
extraocular muscles (myositic type, which involves the entire muscle
including its distal tendinous attachments).
Responds readily to steroids.
May be associated with Wegener granulomatosis, fibrosing
mediastinitis, autoimmune thyroiditis, rheumatoid arthritis, and
sclerosing cholangitis.
MRI: T2 isointense to hypointense, + enhancement (may be
indistinguishable from lymphoma). On DWI, pseudotumor has higher
ADC than does lymphoma (i.e., lymphoma has more restricted
diffusion).
Main differential diagnosis: thyroid ophthalmopathy, sarcoidosis,
metastases, Sjögren syndrome, granulomatosis with polyangiitis
(Wegener), and lymphoma/leukemia.
1495
FIGURE 28-30. Axial CT shows abnormal soft tissue in the left lacrimal
glands, which is contiguous with the lateral rectus muscle and involves its
tendinous insertion.
1496
mildly enlarged left medial and lateral recti muscles, thick and enhancing
optic nerve extending intraocularly into the head of the nerve, and some
stranding of the intraconal fat.
1497
FIGURE 28-32. Axial fat-suppressed postcontrast T1, in a different
patient, shows involvement of left sclera, optic nerve sheath, and lacrimal
gland.
1498
FIGURE 28-33. Corresponding ADC map shows no significant restricted
diffusion in the process.
1499
thickening and increased enhancement of left extraocular muscles and
mild stranding of the intraconal fat.
1500
FIGURE 28-35. Coronal postcontrast T1 in a different patient with IgG4-
related disease of the orbit shows enhancing tissue in the extraconal fat
bilaterally.
SUGGESTED READINGS
Haradome K, Haradome H, Usui Y, et al. Orbital lymphoproliferative disorders
(OLPDs): value of MR imaging for differentiating orbital lymphoma from
benign OLPDs. AJNR Am J Neuroradiol 2014;35:1976–1982.
Kapur R, Sepahdari AR, Mafee MF, et al. MR imaging of orbital inflammatory
syndrome, orbital cellulitis, and orbital lymphoid lesions: the role of
1501
diffusion-weighted imaging. AJNR Am J Neuroradiol 2009;30:64–70.
Lacrimal Gland Masses

Key Facts
Enlargement of the lacrimal gland is nonspecific and most often
related to viral infections or related to Sjögren syndrome
(lymphocytic infiltration of tear gland with decreased lacrimation
associated with rheumatoid arthritis, lupus, or scleroderma), Mikulicz
syndrome (nonspecific enlargement of salivary and lacrimal glands),
sarcoidosis, lymphoma, and leukemia.
Most common lacrimal gland tumors: benign mixed or pleomorphic
tumors (25%, typically painless), adenoid cystic carcinoma (25%,
prone to perineural spread), lymphoma (older adults), and metastasis
(rare).
Dermoids are the most common congenital lesions, and imaging
reveals them to be well-defined cyst-like (containing fluid or fat)
masses that may be partially calcified and scallop adjacent bones;
they are found most often in the region of the frontozygomatic suture
in the superolateral aspect of the orbit. Dermoids may become
infected.
1502
FIGURE 28-36. Axial T2 shows bilateral lacrimal gland masses of low
signal intensity (arrows), typical of highly cellular and malignant
neoplasms at this site.
1503
FIGURE 28-37. Corresponding postcontrast T1 shows homogeneous
enhancement of the lacrimal masses. This patient had lymphoma related to
Sjögren disease.
1504
large enhancing and invasive adenocarcinoma that arose in the left
lacrimal gland.
1505
FIGURE 28-39. Axial postcontrast T1 shows a nonspecific avidly
enhancing right lacrimal gland mass (arrow) in this patient with an
adenoid cystic carcinoma.
1506
FIGURE 28-40. Axial postcontrast CT in a child with left lacrimal gland
rhabdomyosarcoma.
1507
patient, shows infiltrative squamous cell carcinoma arising in the lacrimal
sac region.
SUGGESTED READING
Tailor TD, Gupta D, Dalley RW, et al. Orbital neoplasms in adults: clinical,
radiologic, and pathologic review. Radiographics 2013;33:1739–1758.
Orbital Lymphatic Malformation

Key Facts
Benign “hamartomatous” malformation composed of veins and
lymphatics.
May involve any compartment of orbit or several compartments
1508
simultaneously.
They tend to fluctuate in size with upper respiratory tract infections
and progressive relentless growth is typical, which may be
accelerated during pregnancy or puberty and may be sudden due to
acute hemorrhage; generally found in first decade of life.
Patients with lymphatic and venolymphatic malformations may have
an increased incidence of intracranial vascular anomalies.
CT: multiloculated mass showing cystic areas with fluid levels (due
to spontaneous hemorrhage from fragile vessels along the septa),
calcifications, and enhancement of walls.
MRI: fluid levels may be variable signal intensity on T1 and T2
depending on age of bleeds.
Main differential diagnosis: infantile hemangioma, dermoid, cystic
schwannoma, and plexiform neurofibromas.
FIGURE 28-42. Axial contrast-enhanced CT shows lobulated right

intraconal mass of low density and cystic appearance.
1509
FIGURE 28-43. Corresponding T2, in a different patient, shows fluid
levels and high signal intensity from this mostly cystic retroconal
lymphatic malformation.
1510
FIGURE 28-44. Axial T2 in a different patient shows a multilocular
lymphatic malformation (arrow) with fluid levels in the left orbit.
SUGGESTED READINGS
Bisdorff A, Mulliken JB, Carrico J, et al. Intracranial vascular anomalies in
patients with periorbital lymphatic and lymphaticovenous malformations.
Wiegand S, Eivazi B, Bloch LM, et al. Lymphatic malformations of the orbit.
Clin Exp Otorhinolaryngol 2013;6:30–35.
Melanoma
1511
Key Facts
Melanomas arise from the uveal tract, occur almost exclusively in
whites, and are the most common primary ocular tumor in adults,
usually presenting between 40 and 60 years of age (rare in African
Americans).
Most are diagnosed clinically and by sonography.
By funduscopic exam, they may be difficult to visualize if associated
with choroid or retinal detachments (especially hemorrhagic ones).
They are most commonly located in the choroid (85%), ciliary body,
and iris (less aggressive course than the other two sites).
CT: hyperdense mass that shows moderate contrast enhancement.
MRI: T1 bright and T2 dark due to the shortening effects of melanin
(except for 20% of lesions that are amelanotic), + enhancement;
effusions are also well visualized and are slightly T1 and T2 bright
due to protein and blood.
Mortality reaches 70% when tumor size exceeds 12 mm.
Main differential diagnosis: chorioretinitis, choroidal hemangioma,
metastasis, detachment, and cysticercosis.
1512
FIGURE 28-45. Axial noncontrast T1 shows bright mass (arrow) in
superior aspect of right globe.
1513
FIGURE 28-46. Axial T2, in the same patient, shows that the mass
(arrow) is dark.
1514
FIGURE 28-47. Sagittal postcontrast T1, in the same patient, shows
enhancement of the mass (arrow), which is now of higher signal than
before contrast.
1515
FIGURE 28-48. Axial noncontrast T1 in a different patient shows a bright
right choroidal melanoma (arrow) and increased signal within the vitreous
due to hemorrhage.
1516
FIGURE 28-49. Coronal T2, in the same patient, shows the melanoma to
be very dark (arrow).
1517
patient, shows very large melanoma that has destroyed the left globe and
infiltrated the posterior structures.
SUGGESTED READINGS
Houle V, Belair M, Allaire GS. AIRP best cases in radiologic-pathologic
correlation: choroidal melanoma. Radiographics 2011;31:1231–1236.
Tailor TD, Gupta D, Dalley RW, et al. Orbital neoplasms in adults: clinical,
radiologic, and pathologic review. Radiographics 2013;33:1739–1758.
Optic Neuritis
Key Facts
1518
Acute inflammatory demyelination usually idiopathic or viral, also
commonly associated with multiple sclerosis (initial manifestation of
multiple sclerosis in 25% of patients, 60% of patients with optic
neuritis will eventually develop multiple sclerosis); when optic
neuritis is due to multiple sclerosis 50% of patients show cerebral
lesions by MRI.
Fifty percent of patients with neuromyelitis optica present with
isolated optic neuritis, which is associated with more severe and
persistent visual loss and may more commonly extend to the chiasm
compared to multiple sclerosis.
Recurrent optic neuritis in a subset of patients is associated with
antimyelin oligodendrocyte glycoprotein (MOG) antibodies.
More common in females; average age is 33 years.
May also be secondary to infections or inflammatory processes of the
sinonasal cavities, meninges, and orbital tissues (especially
pseudotumor) or to radiation therapy.
MRI: T2 hyperintense and enlarged optic nerve, + enhancement, optic
nerve sheath may also enhance, chronically optic nerve becomes thin
with prominent surrounding CSF space. DWI may show restricted
diffusion acutely and then increased diffusion in chronic stages. DTI
shows loss of anisotropy acutely.
Main differential diagnosis: inflammatory pseudotumor, sarcoidosis,
astrocytoma, meningioma, and infarction.
1519
FIGURE 28-51. Axial fat-suppressed T2 shows increased signal in the left
optic nerve (arrow) in a patient with multiple sclerosis.
1520
FIGURE 28-52. Coronal fat-suppressed postcontrast T1 in a different
patient shows enhancement of the right optic nerve (arrow).
1521
FIGURE 28-53. Axial fat-suppressed postcontrast T1 shows enhancement
of the left optic nerve (arrows) in a patient with neuromyelitis optica.
1522
FIGURE 28-54. Corresponding fat-suppressed coronal T2 shows bright
signal within the left optic nerve (arrow). Compare to the normal signal of
the supratentorial white matter or right optic nerve.
1523
FIGURE 28-55. Axial fat-suppressed postcontrast T1 in a different patient
with neuromyelitis optica shows enhancement of the left optic nerve
(arrow) extending to the chiasm.
1524
FIGURE 28-56. Axial DTI (anisotropy map), in a different patient, shows
loss of anisotropy in left optic nerve (arrow).
SUGGESTED READINGS
Khanna S, Sharma A, Huecker J, et al. Magnetic resonance imaging of optic
neuritis in patients with neuromyelitis optica versus multiple sclerosis. J
Neuroophthalmol 2012;32:216–220.
Wilejto M, Shroff M, Buncic JR, et al. The clinical features, MRI findings, and
outcome of optic neuritis in children. Neurology 2006;67:258–262.
Orbital Cellulitis and Abscess

Key Facts
1525
Preseptal cellulitis is a pyogenic inflammation confined to the
superficial orbital soft tissues, which are separated posteriorly by the
orbital septum (reflection of the periosteum and check ligaments of
eyelids); treatment is usually medical.
Postseptal cellulitis is most common in children but can occur at any
age; it is located posterior to the orbital septum; it is usually
extraconal and results in subperiosteal phlegmon or abscess;
subperiosteal abscesses are usually secondary to sinonasal (ethmoid)
infections, foreign bodies, and fractures (their treatment involves
aggressive antibiotic therapy and/or surgery).
Ophthalmic vein and cavernous sinus septic thrombosis, central
retinal artery/vein occlusion (due to increased intraocular pressures),
epidural/subdural empyemas, cerebritis, and meningitis are important
complications of postseptal inflammation.
Main differential diagnosis: trauma with hematomas,
lymphoma/leukemia, and mucocele from adjacent paranasal sinus.
1526
FIGURE 28-57. Axial postcontrast CT in a patient with preseptal cellulitis
shows formation of an abscess (arrow) under the eyelid.
FIGURE 28-58. Axial T2 in a different patient with mild sinus disease

and left preseptal cellulitis shows periorbital edema (arrow).
1527
FIGURE 28-59. Axial noncontrast CT, in a different patient with sinusitis,
shows pre- and postseptal cellulitis with extensive inflammatory tissue
extending along the medial left orbit.
1528
FIGURE 28-60. Coronal postcontrast CT in a different patient with pre-
and postseptal cellulitis shows a subperiosteal abscess (arrow) and a dirty
appearance of the orbital fat.
1529
patient with left orbital cellulitis shows subtle enhancement of the orbital
fat with perineuritis (arrow) and thickening of extraocular muscles.
1530
patient with orbital cellulitis shows a thrombosed right superior
ophthalmic vein (arrow).
SUGGESTED READINGS
Mathew AV, Craig E, Al-Mahmoud R, et al. Paediatric post-septal and pre-septal
cellulitis: 10 years’ experience at a tertiary-level children’s hospital. Br J
Radiol 2014;87:20130503.
Platnick J, Crum AV, Soohoo S, et al. The globe: infection, inflammation, and
systemic disease. Semin Ultrasound CT MR 2011;32:38–50.
1531
Orbital Cavity Trauma
Key Facts
Most “blow-out” fractures involve the medial wall (at course of
ethmoidal artery) and floor (at course of infraorbital nerve); these
sites are zones of “natural” weakness.
The orbital roof may be fractured in severe trauma, usually resulting
from a direct blow to the forehead and involving the frontal sinus; the
fracture is displaced into the orbit (“blow in”); may be associated
with CSF leaks or cerebral contusions.
The medial orbital wall may be normally dehiscent in some
individuals.
Entrapment is a clinical diagnosis; herniation of muscle per se is not
needed for entrapment, which may happen with just fat herniation or
hematoma/contusion of muscle.
Severe stretching of optic nerve may result in visual deficits.
Hematomas at the optic nerve–scleral junction may indicate nerve
injury.
1532
FIGURE 28-63. Axial CT shows a fracture of the right lamina papyracea
(arrow) with herniation of fat into the ethmoid region.
1533
FIGURE 28-64. Coronal CT reformat, in a different patient, shows a
blow-out fracture of the left orbital floor with herniation of the left inferior
rectus muscle (arrow) and orbital fat.
1534
FIGURE 28-65. Coronal CT, in a different patient, shows a left superior
subperiosteal hematoma.
1535
FIGURE 28-66. Axial CT, in a different patient, shows rupture of the left
globe and extensive pneumo-orbit due to orbital fractures.
1536
FIGURE 28-67. Axial CT, in a different patient, shows hematoma
thickening the left medial rectus muscle and an ipsilateral medial orbital
wall fracture with opacified ethmoids.
1537
FIGURE 28-68. Axial CT (bone windows), in a different patient, shows a
nail in right extraconal space.
SUGGESTED READING
Winegar BA, Gutierrez JE. Imaging of orbital trauma and emergent non-traumatic
conditions. Neuroimaging Clin N Am 2015;25:439–456.
Persistent Hyperplastic Primary Vitreous

(PHPV)
Key Facts
Refers to a persistent, hyperplastic, embryonic hyaloid vascular
system that is usually unilateral; patients may also have seizures,
hearing loss, mental deficiencies, and cataracts.
PHPV is the second most common cause of leukokoria after
retinoblastoma.
The affected eye is usually small (microphthalmia).
CT: hyperdense vitreous, no calcifications; a thin central structure
(Cloquet canal) may be seen extending from the posterior retina to the
lens; the vitreous may enhance, and retinal detachments may be
present.
MRI: T1 and T2 hyperintense (retinoblastoma is usually T2
hypointense), Cloquet canal, and fluid levels.
May be indistinguishable from retinal dysplasia occurring with Norrie
disease, trisomy 13, and Walker-Warburg syndrome.
Main differential diagnosis: retinoblastoma, Coat disease, and
retinopathy of prematurity.
1538
FIGURE 28-69. Axial T1 shows bright left vitreous with a fluid level and
a central linear canal of Cloquet (arrow). The eye is small.
FIGURE 28-70. Sagittal T1 in the same patient nicely shows the canal of
Cloquet.
1539
FIGURE 28-71. Axial T1 (inversion recovery) image, in a different
patient, shows bilateral detachments, which continue anteriorly in the
“tent-like” configuration with the canals of Cloquet.
1540
FIGURE 28-72. Axial T2 in a different patient demonstrates dark
retrolental tissue and a canal of Cloquet with detachment posteriorly.
1541
the retrolental tissue.
SUGGESTED READINGS
Castillo M, Wallace DK, Mukherji SK. Persistent hyperplastic primary vitreous
involving the anterior eye. AJNR Am J Neuroradiol 1997;18:1526–1528.
Gujar SK, Gandhi D. Congenital malformations of the orbit. Neuroimaging Clin
N Am 2011;21:585–602.
Posttreatment Findings
Key Facts
1542
Most common findings of treatment of retinal detachments include
Scleral banding or buckling (apposition of sclera to detached
retina) may or may not encircle the globe completely; solid
silicone bands are CT dense and dark on T1 and T2 images;
silicone sponge bands are of low CT density and also dark on T1
and T2 images.
Intraocular tamponade: nearly complete or partial filling of
vitreous chamber with special gas.
Retinopexy: reattachment of retina with heat (diathermy),
freezing (cryotherapy), or laser (photocoagulation), these
procedures if uncomplicated have no specific imaging findings,
but they may be accompanied by intraocular gas or fluid
tamponades.
Glaucoma drainage devices are alternatives to medical therapy or
conventional trabeculectomy. They consist of a draining tube with
one end in the anterior or posterior chambers or posterior segment
and the other connected to a plate inserted superotemporally and less
commonly inferonasally or inferotemporally under Tenon capsule.
A bleb forms around the glaucoma device from which aqueous humor
is usually resorbed, but which may become encapsulated leading to
drainage failure or growth with mass effect on the orbit.
1543
FIGURE 28-74. Axial CT shows circumferential scleral banding (arrows)
and air within the right globe from pneumatic retinopexy for treatment of
retinal detachment.
1544
FIGURE 28-75. Axial CT in a different patient shows high-density silicon
within the globes treated for retinal detachment.
1545
FIGURE 28-76. Axial CT, in a different patient, shows a glaucoma device
in the left orbit (arrow).
1546
FIGURE 28-77. Axial fat-suppressed T2, in a different patient, shows
dark left intraocular silicone oil. Note posterior staphyloma in both globes
(right > left), which was the cause of the retinal detachment in this patient.
1547
FIGURE 28-78. Coronal T2, in a different patient, shows a small fluid
collection below the right optic nerve sheath (arrow) following
fenestration for treatment of pseudotumor cerebri.
1548
FIGURE 28-79. Axial CT shows metal artifact from radioactive plaque
for treatment of right ocular melanoma.
SUGGESTED READINGS
Adams A, Mankad K, Poitelea C, et al. Post-operative orbital imaging: a focus on
implants and prosthetic devices. Neuroradiology 2014;56:925–935 .
Reiter MJ, Schwope RB, Kini JA, et al. Postoperative imaging of the orbital
contents. Radiographics 2015;35:221–234.
Detachments and Effusions

Key Facts
Retinal:
1549
Represent accumulations of fluid between sensory retina and retinal
pigment epithelium (the sensory retina belongs to CNS and therefore
does not heal), more common in diabetics.
A rhegmatogenous detachment is caused by a tear in the retina.
Exudative detachments result from leakage of fluid without a full-
thickness tear.
May be secondary to pulling of the retina by a contracting vitreous
(“traction” detachment, especially in children with retinopathy of
prematurity or inflammatory disorders, diabetic and sickle cell
retinopathies), subretinal hemorrhage due to trauma, or choroidal
lesions.
By imaging, retinal detachments are more commonly V-shaped due to
retinal attachments at the optic disk and ora serrata but may have any
shape and size, density/intensity varies according to their age,
etiology, and internal organization.
Choroidal:
Represents accumulations of fluid or blood (postsurgical, trauma, or

inflammation) in the subchoroidal space.
Most choroidal detachments occur in the presence of ocular
hypotonia and are due to increased permeability of choroidal
capillaries.
By imaging, choroidal detachments may be lenticular-like or mound-
like abnormalities.
MRI: serous effusions are of low T1 and high T2 signal; hemorrhagic
and inflammatory effusions may be both T1 and T2 bright.
1550
FIGURE 28-80. Axial CT in a patient with history of trauma shows
hyperdensity in the left posterior globe and dislocation of the lens
(arrowhead).
1551
FIGURE 28-81. Corresponding axial T2 shows a V shaped left retinal
detachment with relatively low intensity fluid due to the presence of blood
products.
1552
FIGURE 28-82. Axial T2, in a different patient, shows bilateral medial
small retinal detachments extending to the region of the optic nerve heads.
1553
FIGURE 28-83. Axial T2 in a different patient shows retinal detachments
whose contents are almost as bright as the vitreous. Note the thin detached
membranes (arrowhead).
1554
choroidal detachments nearly filling the left vitreous cavity. Note that they
do not terminate in a V-shaped configuration at the optic nerve head.
1555
FIGURE 28-85. Coronal postcontrast T1 in a different patient shows
bilateral choroidal detachment with enhancement of the detached
membranes.
SUGGESTED READING
Gariano RF, Kim CH. Evaluation and management of suspected retinal
detachment. Am Fam Physician 2004;69:1691–1698.
Key Facts
1556
Most important cause of leukokoria (white pupillary reflex); other
causes for leukokoria include persistent hyperplastic primary vitreous,
retinopathy of prematurity, congenital cataract, toxocariasis, and Coat
disease.
Average age at diagnosis: 13 months (most are found <5 years of
age); nearly 100% of bilateral cases and 15% of unilateral ones are
hereditary (chromosomal defect in 13q).
Twenty-five to thirty percent are bilateral; trilateral retinoblastoma
(both eyes and pineal gland or suprasellar region) is very rare (<1%).
Patients with 13q abnormalities have increased incidence of cerebral
abnormalities.
If tumor extends beyond the globe, mortality is near 100%;
occasionally, it presents as a diffuse infiltrating mass.
Intraocular calcification in a child is a retinoblastoma until proven
otherwise. However, CT is no longer needed to make diagnosis as
MRI features are typical.
MRI: T1 hyperintense, T2 hypointense, + contrast enhancement
(enhancement of the anterior eye segment generally represents
reactive angiogenesis and not tumor), T2* signal voids in areas of
calcification, and pronounced restricted diffusion (lower ADC values
may correlate with poor differentiation and prognosis).
Overall long-term survival is >80% in localized tumors.
Main differential diagnosis: persistent hyperplastic primary vitreous,
Coat disease, and retinopathy of prematurity.
1557
FIGURE 28-86. Axial CT shows mostly calcified retinoblastoma in right
globe.
1558
FIGURE 28-87. Axial postcontrast T1 in a different patient shows a small
enhancing retinoblastoma in the left posterior globe (arrow).
FIGURE 28-88. Corresponding ADC map demonstrates very low signal

from the tumor (arrow).
1559
FIGURE 28-89. Axial T2 in a different patient shows bilateral low-signal-
intensity tumors and membranous detachments.
1560
the anterior eye segment on the left (arrow).
1561
FIGURE 28-91. Axial postcontrast T1 in a different child with advanced
disease shows intracranial extension of the tumor along the left optic
nerve. Note enhancement of the left trigeminal nerve (arrow).
SUGGESTED READINGS
Abdel Razek AA, Elkhamary S, Al-Mesfer S, et al. Correlation of apparent
diffusion coefficient at 3T with prognostic parameters of retinoblastoma.
Rodjan F, de Graaf P, van der Valk P, et al. Detection of calcifications in
retinoblastoma using gradient-echo MR imaging sequences: comparative
study between in vivo MR imaging and ex vivo high-resolution CT. AJNR Am
J Neuroradiol 2015;36:355–360.
1562
Ocular Trauma
Key Facts
Most cases are related to blunt trauma, and there is a significantly
increased risk of ocular injury in the presence of maxillofacial
fractures.
May result in foreign bodies, hemorrhagic choroidal and/or retinal
effusions and detachments, dislocation of lens (which can be partial
or complete), hypotonia and collapse from perforations or globe
explosions, optic nerve avulsion or stretching, hematoma, and
extraocular muscle hematoma and/or entrapment.
Globe rupture can be subtle on imaging and may only be evident as
decreased depth of the anterior chamber.
Thin section CT accurately localizes metallic and nonmetallic foreign
bodies; avoid MRI with metallic orbital/ocular foreign bodies.
By CT, dry wood is hypodense, fresh wood has intermediate to
slightly increased density (similar to fresh blood), plastic may be
hypo- or hyperdense, and glass is hyperdense.
1563
FIGURE 28-92. Axial CT shows rupture of the left globe with wrinkling
of the posterior ocular contour resulting in a “flat tire” or “mushroom”
appearance.
1564
FIGURE 28-93. Axial CT, in a different patient, shows dislocation of the
left lens into the posterior segment (arrow).
1565
FIGURE 28-94. Axial CT, in a different patient, shows left intraocular
metallic foreign body.
1566
FIGURE 28-95. Axial CT, in a different patient, shows rupture of the
right globe, vitreous and retinal hemorrhage, and posterior displacement of
the lens with increased depth of the anterior chamber.
1567
FIGURE 28-96. Axial CT, in a different patient, shows burst right globe
with hemorrhage in all compartments.
1568
FIGURE 28-97. Axial CT, in a different patient with left cornea
perforation, shows diminished size of the aqueous chambers (arrow) with
approximation of lens to cornea (compare to normal right ones).
SUGGESTED READING
Sung EK, Nadgir RN, Fujita A, et al. Injuries of the globe: what can the
radiologist offer? Radiographics 2014;34:764–776.
1569
CHAPTER 29
Sinuses
Acute (Uncomplicated) Sinusitis

Key Facts
Most common etiologies: viral upper respiratory tract infection,
bacterial, allergic, and fungal, it is a clinical diagnosis, and isolated
imaging abnormalities cannot be used to make the diagnosis.
Although fluid levels are the most common manifestation, they
actually occur in >50% of cases (fluid levels are also seen in trauma
[hemorrhage], intubated patients, barotrauma, and bleeding
disorders). Fluid in acute sinusitis tends to be mucoid and near-water
density before inspissation occurs.
A fluid level in a paranasal sinus may only be significant when
clinical findings suggest infectious sinusitis.
Fluid level in the frontal sinus is most specific for infectious sinusitis
but not pathognomonic.
Fluid level in the sphenoid sinus may also be associated with a base
of skull fracture with CSF leakage.
Allergic sinusitis tends to be more diffuse, and bacterial sinusitis is
more commonly localized. Isolated maxillary sinusitis may be related
to an adjacent odontogenic source.
In adults, a solitary opacified (or with fluid level, particularly
refractory to antibiotics or long-standing) paranasal sinus should raise
the possibility of an underlying tumor.
Fluid levels in the presence of nasal or oral tubes are not diagnostic
for infection.
1570
FIGURE 29-1. Axial CT shows mucosal thickening in the left maxillary
sinus with a fluid level.
1571
FIGURE 29-2. Coronal CT shows bilateral maxillary sinus fluid levels
and mucosal thickening.
1572
FIGURE 29-3. Axial CT, in a different patient, shows bilateral maxillary
sinus fluid levels, left-sided mucosal thickening, and thick walls
suggesting chronic disease with superimposed acute sinusitis.
1573
FIGURE 29-4. Coronal CT in a baby with ciliary dysmotility shows
bilateral maxillary and ethmoid mucosal thickening with left-sided frothy
secretions.
1574
FIGURE 29-5. Coronal CT in a patient with right enophthalmos shows
small ipsilateral maxillary sinus, lateralization of uncinate process, and
middle turbinate as well as mucosal thickening all compatible with the
silent sinus syndrome. These patients may present with acute exacerbation
of their chronic process.
SUGGESTED READING
Joshi VM, Sansi R. Imaging in sinonasal inflammatory disease. Neuroimaging
Clin N Am 2015;25:549–568.
Anatomical Variants of the Sinonasal

Cavity
1575
Key Facts
Developmental anomalies of the ostiomeatal unit include concha
bullosa, paradoxical middle turbinates, septal deviation, enlarged
ethmoid bullae, infraorbital (Haller) cells (extension of an anterior or
posterior ethmoidal cell), and sphenoethmoidal (Onodi) cells
(posterior extension of an ethmoidal cell superolateral to the sphenoid
sinus, which increases the surgical risk of optic nerve or carotid
injury).
A concha bullosa (aeration of the middle and rarely superior
turbinates) is present in 30% to 50% of the population and is
commonly bilateral; if large, it may deviate the nasal septum or
become superinfected (concha bullitis) and develop a mucocele or
pyomucocele if its ostium is occluded.
Paradoxical middle turbinates are common and diagnosed when their
curvature is the reverse of the curvature of the inferior turbinates; if
small, they are usually bilateral; if large, they are unilateral and may
produce septal deviation.
Hypoplasia of the middle turbinates is uncommon and does not
produce symptoms.
Enlarged ethmoid bullae and/or infraorbital cells may result in
obstruction of the ipsi- or contralateral infundibula.
1576
FIGURE 29-6. Coronal CT shows large left infraorbital (Haller) cell with
accompanying medial deviation of the ipsilateral uncinate process.
1577
FIGURE 29-7. Coronal CT in a different patient shows sphenoethmoidal
(Onodi) cells (arrow). Note their relation to the optic canals (arrowhead)
and the sphenoid sinuses inferior to them (star).
1578
FIGURE 29-8. Coronal CT, in a different patient, shows large bilateral
concha bullosae in middle turbinates with left septal deviation and
opacified left maxillary sinus.
1579
FIGURE 29-9. Coronal CT, in a different patient, shows aerated uncinate
processes. There is mild mucosal thickening in the alveolar recesses of
both maxillary sinuses and right septal deviation.
1580
FIGURE 29-10. Coronal CT, in a different patient, shows a left
infraorbital cell (arrow), paradoxical left inferior turbinate (arrowhead),
bilateral concha bullosae (wavy arrow), and left septal deviation.
1581
FIGURE 29-11. Coronal CT in a different patient shows expansion of a
left concha bullosa by a mucocele (star) and obstructive disease in the left
maxillary sinus.
SUGGESTED READING
Lund VJ, Stammberger H, Fokkens WJ, et al. European position paper on the
anatomical terminology of the internal nose and paranasal sinuses. Rhinol
Suppl 2014;(24):1–34.
Choanal Narrowing
1582
Key Facts
Can be of two types: stenosis and atresia (note: severe stenosis
behaves clinically like atresia), further subdivided into purely bony
(70%), membranous with soft tissue (plugs), and mixed (10%); purely
membranous atresia/narrowing is rare.
For bone stenosis, always measure the thickness of the palate, the
diameter of the choana (if any), and thickness of the vomer; for soft
tissue stenosis/atresia, always measure the thickness of the plug and
obtain CT after administration of decongestants.
More commonly unilateral than bilateral, more frequent in females,
and with a slight right-sided predominance.
Bilateral is generally found in newborns, is accompanied by systemic
abnormalities, is part of many syndromes, needs immediate treatment
regardless of secondary type.
Unilateral presents later in childhood and even in adults; most
common symptom is chronic unilateral obstruction with or without
infection; may require surgical treatment.
If accompanied by hypotelorism, always examine the brain for
midline abnormalities.
Remember to obtain CT with high-resolution bone settings, angle
images about 10 degrees cephalad to hard palate, give patients
decongestants before, and if needed aspirate secretions.
Size of normal choanae: never <3 to 4 mm; size of normal vomer:
never more than 5 to 6 mm.
1583
FIGURE 29-12. Axial CT showing bilateral stenosis with large soft tissue
plugs.
1584
fusion of hard palate with ventral clivus in severe stenosis (also called
nasopharyngeal atresia).
1585
FIGURE 29-14. Axial CT, in a different patient, shows right bony
stenosis with thick plate and left stenosis with thin plate.
1586
FIGURE 29-15. Parasagittal CT reformation, in a different patient, shows
thin bone atretic plate (arrow).
1587
FIGURE 29-16. Axial CT in a different patient shows right-sided atresia
with a thin bony plate (arrowhead) and soft tissue plug, medialization of
the right pterygopalatine fossa, and thickening of the vomer (arrow).
1588
FIGURE 29-17. Axial CT, in a different patient, shows bony atresia
bilaterally.
SUGGESTED READING
Adil E, Huntley C, Choudhary A, et al. Congenital nasal obstruction: clinical and
radiologic review. Eur J Pediatr 2012;171:641–650.
CSF Leaks
Key Facts
CSF leaks may be secondary to trauma (95% occur within the first 3
months), surgery, or spontaneous (congenital bone dehiscences,
1589
frontal sinus osteoma); leaks may arise in sinonasal cavities or
temporal bones.
Spontaneous CSF leaks are more common in obese, middle-aged
women.
Imaging studies need to be done when patient is actively leaking.
Beta-transferrin test on secretion determines it is CSF.
Thin (1-mm) section CT generally determines site of bone defect and
precludes other tests. High-resolution steady-state MR sequences
(CISS or similar) may be able to demonstrate the leak if CT findings
are not certain.
Occasionally, contrast CT cisternography and radionuclide
cisternography are needed.
FIGURE 29-18. Coronal CT in a patient with right rhinorrhea shows bone

defect (arrow) in ethmoid roof with underlying sinus opacification.
1590
FIGURE 29-19. Coronal CISS image, in the same patient, shows defect
(arrow) in cribriform plate with bright CSF in underlying ethmoid sinus.
FIGURE 29-20. Coronal CT, in a different patient, shows defect (arrow)

in roof of right lateral recess of sphenoid sinus and fluid level in sinus.
1591
FIGURE 29-21. Nuclear medicine study in the same patient after lumbar
puncture shows radiotracer accumulation in sphenoid sinus (arrows).
1592
FIGURE 29-22. Coronal CT reformat in a different patient shows CSF
leak into the left sphenoid sinus due to an osseous defect (arrow).
SUGGESTED READINGS
Lloyd KM, DelGaudio JM, Hudgins PA. Imaging of skull base cerebrospinal
fluid leaks in adults. Radiology 2008;248:725–736.
Schuknecht B, Simmen D, Briner HR, et al. Nontraumatic skull base defects with
spontaneous CSF rhinorrhea and arachnoid herniation: imaging findings and
correlation with endoscopic sinus surgery in 27 patients. AJNR Am J
Neuroradiol 2008;29:542–549.
Fibro-osseous Lesions of Sinonasal
1593
Cavities
Key Facts
Osteomas are the most common tumors in the paranasal sinuses; they
are zones of compact bone covered by sinus mucosa occurring in the
frontal and ethmoid sinuses or any facial bone; they may be
secondary to infection or prior trauma, and most are asymptomatic
but may result in headaches and occasionally produce obstructive
sinusitis or rarely spontaneous pneumocephalus; they are more
commonly found in individuals aged 15 to 40 years. They may be
very dense without cancellous bone (eburnated or “ivory” osteoma);
mature, which are softer and cancellous-like (osteoma spongiosum);
or mixed.
Osteoid osteomas occasionally arise in the frontal or ethmoid regions.
Osteoblastomas may occur in the maxilla, frontal, or sphenoethmoidal
regions.
Monostotic fibrous dysplasia (more common) involves the maxilla or
mandible in 20% to 25% of cases; polyostotic fibrous dysplasia (less
common) occurs almost exclusively in females and involves the skull
and facial bones in 40% to 60% of cases.
Fibrous dysplasia encroaches sinonasal cavities and neurovascular
foramina.
Malignant degeneration occurs in <1% of fibrous dysplasia patients
(osteosarcoma, fibrosarcoma, and chondrosarcoma).
Ossifying fibromas are rare, more common in females, usually
diagnosed between the third and fourth decades, and 75% occur in the
mandible followed by the maxilla. Tumor consistency may range
from soft tissue to eggshell calcification to heavy cementum-like
calcification.
1594
FIGURE 29-23. Axial CT shows extensive fibrous dysplasia replacing the
right maxillary sinus and the base of the skull. Note unilateral enlargement
of the face in this patient with precocious puberty and McCune-Albright
syndrome.
1595
FIGURE 29-24. Sagittal CT reformat in a different patient shows a very
dense “ivory” osteoma in the frontal sinus (arrow).
1596
FIGURE 29-25. Axial CT, in a different patient, shows osteoma with a
more cancellous appearance in the right anterior ethmoid sinuses.
1597
FIGURE 29-26. Coronal CT reformat, in a different patient, shows an
ossifying fibroma in the right maxillary sinus. Note thin shell of
calcification (arrows) and erosion of teeth apices (arrowhead).
SUGGESTED READING
Eller R, Sillers M. Common fibro-osseous lesions of the paranasal sinuses.
Fungal Sinusitis
Key Facts
Aspergillosis (in healthy and immunosuppressed patients) and
mucormycosis (in immunosuppressed patients) are the most common
causes (others include candidiasis, histoplasmosis, cryptococcus, and
1598
coccidioidomycosis).
Three types:
Allergic (noninvasive) fungal sinusitis (more common in young
males):
Maxillary and ethmoid sinuses are most commonly
involved; isolated involvement of the sphenoid sinus
suggests aspergillosis.
Occurs in the setting of sinonasal polyposis.
Secretions of high density on plain films or CT
(calcification seen on CT in 50% of cases) and of T2 low
signal intensity (sometimes with pronounced signal void
due to deposition of iron and manganese) on MRI suggest
the diagnosis. May result in bone erosion and intracranial
extension.
Main differential diagnosis: chronic sinusitis, polyposis.
Invasive fungal sinusitis (more common in diabetics and
immunosuppressed patients):
May present as an acute and rapidly progressive or a more
indolent chronic form; may have only a small amount of
mucosal thickening in affected sinus (most commonly
maxillary and ethmoid), extension outside of sinus is
typical despite absence of bone erosion. Chronic form tends
to show more dense and coarse calcifications.
May progress intracranially; aspergillosis may cause a
vasculitis, mycotic aneurysms, and vessel thrombosis;
mucormycosis may involve the orbits and cavernous
sinuses.
Main differential diagnosis: infectious acute sinusitis,
Wegener granulomatosis, lymphoma.
Mycetoma (also called a fungus ball, indolent, presents at any
age, and most common in maxillary sinus):
Main differential diagnosis: chronic sinusitis, allergic
fungal sinusitis, mucocele, inverted papilloma.
1599
FIGURE 29-27. Axial CT in a patient with allergic fungal sinusitis shows
high-density material completely filling the ethmoid and sphenoid sinuses
bilaterally.
1600
FIGURE 29-28. Axial postcontrast MR in a different patient with allergic
fungal sinusitis shows extensive enhancement of tissue within the ethmoid
and sphenoid sinuses.
1601
FIGURE 29-29. Corresponding axial T2 shows dark signal flow voids
within the enhancing tissue that may resemble air, due to heavy metal
deposition.
1602
FIGURE 29-30. Coronal CT reformat, in a different patient, shows a mass
with areas of calcification in the right maxillary sinus and nasal cavity
(arrow) as well as evidence of chronic osteitis.
1603
FIGURE 29-31. Corresponding coronal T2 shows profound hypointensity
within the mass that is more extensive than the areas of calcification
(arrow). This was a fungus ball.
1604
lack of enhancement of the nasal mucosa (“black turbinate sign,” arrow)
due to necrosis (note thin rim of residual enhancement in the right inferior
turbinate, arrowhead).
SUGGESTED READINGS
Raz E, Win W, Hagiwara M, et al. Fungal sinusitis. Neuroimaging Clin N Am
2015;25:569–576.
Safder S, Carpenter JS, Roberts TD, et al. The “Black Turbinate” sign: an early
MR imaging finding of nasal mucormycosis. AJNR Am J Neuroradiol
2010;31:771–774.
1605
Papilloma
Key Facts
Rare, comprise 4% of all sinonasal tumors, arise from ectodermally
derived ciliated respiratory mucosa lining the sinonasal cavities
(Schneiderian papillomas). Occur more commonly in males (except
for the oncocytic type) between 40 and 50 years of age.
Greater than 50% of papillomas arise from the nasal septum and are
called “fungiform (everted) papillomas.”
Forty-seven percent are inverting papillomas that arise in the lateral
nasal wall and extend (invert) into the adjacent maxillary and ethmoid
sinuses or, less commonly, into the sphenoid, cribriform plate, and
frontal sinuses.
About 3% are oncocytic (cylindrical cell and columnar) papillomas
(similar in location to inverting ones).
Papillomas tend to be unilateral.
Associated malignancy (mainly squamous cell carcinoma) is found in
3% to 24% of papillomas and tends to be seen more commonly with
the inverting type (although this is controversial).
En bloc removal of the lesion is the goal because imaging cannot
distinguish between benign and malignant lesions.
Main differential diagnosis: polyp, angiofibroma, rhinoscleroma.
1606
FIGURE 29-33. Axial CT shows a mass with areas of calcification in the
right nasal cavity and sphenoid sinus (arrows). CT does not allow
delineation of the lesion margins.
1607
FIGURE 29-34. Corresponding axial T2 demonstrates the mass to be
heterogeneous but predominantly hypointense. Note bright obstructive
secretions/mucosal thickening in the right maxillary sinus.
1608
FIGURE 29-35. Sagittal postcontrast T1, in the same patient, shows the
typical “cerebriform” pattern of inverted papillomas.
1609
FIGURE 29-36. Axial postcontrast CT, in a different patient, shows the
“cerebriform” pattern of an inverted papilloma in the left nasal cavity and
maxillary sinus, which extrudes through the nostril.
1610
FIGURE 29-37. Coronal T2 in a different patient shows a predominantly
hypointense papilloma in the sphenoid sinus.
1611
FIGURE 29-38. Axial T2, in a different patient, shows dark carcinoma
arising in a papilloma surrounded by bright retained secretions. The
diagnosis of carcinoma is generally a pathological one and cannot be made
a priori.
SUGGESTED READING
Jeon TY, Kim HJ, Chung SK, et al. Sinonasal inverted papilloma: value of
convoluted cerebriform pattern on MR imaging. AJNR Am J Neuroradiol
2008;29:1556–1560.
Mucocele
1612
Key Facts
Mucoceles are the most common etiology for expanded sinuses with
thinned walls (may be absent). They are epithelium-lined cysts that
are produced by the obstruction of ostia or of individual sinus
compartments from chronic inflammation or allergies (as well as
trauma and underlying tumors).
Locations: frontal (65%), ethmoid (25%), maxillary (10%), and
sphenoid sinus (2%, although this location is being recognized as
being more frequent because of the use of MRI). May rarely occur
within an interfrontal septal sinus cell, which may grow enough to
block both frontal sinus outflow tracts.
Facial deformity is the most common initial complaint; presence of
pain should suggest the possibility of superimposed infection
(mucopyocele). Those in the maxillary sinuses are less commonly
symptomatic.
MRI: most are of low signal intensity on T1 and hyperintense on T2;
inspissated mucoceles are hypointense on both T1 and T2 and may
simulate dilated but aerated sinuses; surrounding mucosa is T2 bright
and enhances.
Main differential diagnosis: polyp, slow-growing tumor, cephaloceles
(particularly in the sphenoid or frontal sinuses).
1613
FIGURE 29-39. Axial CT shows expansile and rounded right anterior
ethmoidal mucocele with erosion of bone laterally.
1614
FIGURE 29-40. Axial CT in a different patient shows a hyperdense
mucocele expanding the right frontal sinus with a focus of osseous
dehiscence in the inner table (arrow).
1615
FIGURE 29-41. Parasagittal postcontrast T1, in a different patient, shows
frontal (arrowhead) and maxillary (arrow) sinus mucoceles. Note that the
mucosal contents do not enhance. Their mucosal lining does show
enhancement.
1616
FIGURE 29-42. Coronal CT in a different patient with chronic sinusitis
shows large mucoceles involving the left frontal sinus and a left concha
bullosa.
1617
FIGURE 29-43. Axial postcontrast T1, in a different patient, shows right
maxillary sinus mucocele with peripheral enhancing smooth mucosa.
1618
FIGURE 29-44. Axial CT in a different patient who was clinically
deemed to have a mucocele shows an osseous defect in the lateral
sphenoid sinus wall (arrowhead). This was a cephalocele (arrow).
SUGGESTED READING
Tsitouridis I, Michaelides M, Bintoudi A, et al. Frontoethmoidal mucoceles: CT
and MRI evaluation. Neuroradiol J 2007;20:586–596.
Mucous Retention Cysts

Key Facts
May be sequelae of inflammatory sinusitis, allergy, or trauma, but are
also highly prevalent in asymptomatic patients without history of
rhinosinusitis.
1619
They represent obstruction of a minor salivary gland or a mucous-
secreting gland and should be distinguished from just polypoid
mucosal thickening.
Occur in more than 10% of the population, most commonly along the
floor of the maxillary sinus.
They are usually incidental and asymptomatic and should not be
expansile or result in bone remodeling as mucoceles do. They grow
slowly and many regress.
If small, they have an upward convex border; if large, their superior
surface becomes flattened, and they may simulate a fluid level; if very
large, they may obstruct the sinus ostia.
Caution: in adults, early paranasal sinus carcinoma may appear
identical to a mucous retention cyst.
As with most inflammatory sinus disease, retention cysts are bright
on T2 images.
FIGURE 29-45. Axial CT shows mostly low-density mucosal thickening

in both maxillary sinuses (left > right).
1620
FIGURE 29-46. Corresponding bone window settings better show the
“polypoid” nature of the mucosal thickening.
1621
FIGURE 29-47. Axial T2, in the same patient, shows bright swollen
polypoid mucosa.
1622
FIGURE 29-48. Corresponding postcontrast T1 shows that mucosa on
surface of mucous retention cysts enhances, while the retained submucosal
secretions do not enhance.
1623
FIGURE 29-49. Sagittal noncontrast T1 in a different patient shows
maxillary mucous retention cysts with different intensities due to varying
protein contents.
SUGGESTED READING
Clin N Am 2015;25:549–568.
Ostiomeatal Unit, Obstruction

Key Facts
1624
The ostiomeatal unit (OMU) is formed by:
1. Uncinate process: thin bone lamina belonging to the ethmoid bone,
which begins anteriorly at the lacrimal bone and extends posteriorly
to the inferior nasal concha.2. Ethmoidal infundibulum: air space
located superolateral to the uncinate process and inferior to the
ethmoidal bulla.3. Semilunar hiatus: air space above the uncinate
process and inferior to the ethmoidal bulla, communicating the
infundibulum with the middle meatus.4. Ostia for the maxillary,
anterior, and middle ethmoidal complex and frontal recess form the
medial aspect of the infundibulum.
OMU may be obstructed by mucosal thickening, polyps, enlarged or
pneumatized uncinate process, deviated nasal septum with or without
spurs, concha bullosa of the middle turbinate, large ethmoid bullae,
and paradoxical middle turbinates.
FIGURE 29-50. Coronal CT shows polypoid mucosal thickening (arrow)
1625
blocking the left infundibulum.
FIGURE 29-51. Coronal CT, in a different patient, shows polypoid

mucosal thickening obstructing both infundibula. Middle turbinates are
very hypoplastic.
1626
FIGURE 29-52. Coronal CT in a child shows complete opacification of
the right maxillary sinus with obstruction of the ostiomeatal unit and
obstruction of the left ethmoidal infundibulum (arrow) and maxillary
ostium.
SUGGESTED READING
Beale TJ, Madani G, Morley SJ. Imaging of the paranasal sinuses and nasal
cavity: normal anatomy and clinically relevant anatomical variants. Semin
Ultrasound CT MR 2009;30:2–16.
Sinonasal Polyps
Key Facts
1627
Polyps are usually the sequelae of inflammation, vasomotor and/or
infectious rhinitis, diabetes, and cystic fibrosis.
Polyps in children are uncommon in the absence of cystic fibrosis;
they are found in 4% of the population and are more common in
males; and asthma is an important predisposing factor.
Polyps may be solitary or multiple and occur in the nasal cavity or in
any sinus (but are more common in the maxillary sinus) and may
enlarge and become a conglomerate mass, which expands the sinuses
and the infundibula, erode their septae, or result in loss of bone
density in the ethmoid trabeculae, turbinates, and nasal septum.
Occasionally, they behave aggressively and erode bone (producing
intracranial extension).
Polyps may protrude from a sinus into nasal cavities (antrochoanal
and rarely sphenochoanal and ethmochoanal polyps).
Polyposis may be infected with fungi (generally Aspergillus) and by
CT show high density and/or calcifications (25% to 50%, due to
calcium phosphate and calcium sulfate in necrotic mycetomas).
Infarcted polyps may be hyperdense on noncontrast CT; presence of
low T2 signal intensity does not imply malignancy but correlates with
desiccated secretions and/or fungal infection.
Main differential diagnosis: retention and mucosal cysts, allergic
fungal sinusitis, Wegener granulomatosis.
1628
FIGURE 29-53. Axial noncontrast CT shows severe sinonasal polyposis
with areas of osseous dehiscence in the maxillary sinuses and at the skull
base. Note the presence of dense secretions.
1629
FIGURE 29-54. Coronal CT in a different patient shows extensive
polyposis with trabecular thinning and osseous expansion.
1630
FIGURE 29-55. Corresponding coronal postcontrast T1 demonstrates
enhancement of most of the polypoid tissue and intracranial extension.
1631
FIGURE 29-56. Fat-suppressed postcontrast T1, in a different patient,
shows extensive disease with near-complete obliteration of the sinonasal
airway.
1632
FIGURE 29-57. Coronal CT reformat, in a different patient, shows an
antrochoanal polyp prolapsing into the left nasal cavity.
1633
FIGURE 29-58. Corresponding parasagittal CT reformat shows the polyp
extending posteriorly to the choana (arrow).
SUGGESTED READINGS
Huang BY, Senior BA, Castillo M. Current trends in sinonasal imaging.
Clin N Am 2015;25:549–568.
Paranasal Sinus Malignancy

Key Facts
1634
Squamous cell carcinoma is the most common malignancy (80%);
advanced local disease is common at diagnosis; maxillary sinus is
most commonly affected followed by the nasal cavity, and 20% of
patients have nodal metastases at diagnosis.
Undifferentiated carcinoma and lymphoma are not uncommon but are
indistinguishable by imaging from squamous cell carcinoma.
Adenoid cystic carcinoma accounts for 10% of sinonasal
malignancies, involves the ethmoid sinuses more often, may have a
benign appearance (at least initially), and is highly neurotropic with a
propensity for perineural spread.
Primary sarcomas (e.g., osteosarcoma and chondrosarcoma) are rare
and destructive tumors that contain calcifications by CT.
Olfactory neuroblastomas (esthesioneuroblastomas) are rare, arise
from the olfactory epithelium, tend to extend intracranially and
intraorbitally, and frequently calcify. Peritumoral cysts at their
intracranial interface are characteristic.
Many malignant tumors of the paranasal sinuses have intermediate to
low signal intensity on T2 and on ADC maps, melanomas may be
bright on T1 and have low T2 signal, and differential diagnosis for
this appearance includes air, desiccated secretions, fungal infections
(calcium, iron, and manganese), hemorrhage, bone, and enamel.
Processes that simulate carcinoma include mucormycosis,
granulomatosis and polyangiitis (Wegener), midline granuloma, and
cocaine abuse.
1635
FIGURE 29-59. Axial CT shows large destructive adenocarcinoma
arising in the left maxillary sinus and extending to the skull base.
1636
heterogeneously enhancing squamous cell carcinoma in the right maxillary
sinus (arrows) extending to the retromaxillary tissues.
1637
FIGURE 29-61. Corresponding coronal postcontrast T1 shows the mass to
enhance to a lesser degree than the nasal mucosa.
1638
FIGURE 29-62. Coronal postcontrast T1 in a different patient shows an
olfactory neuroblastoma (star) with characteristic cysts at its intracranial
interface (arrow). The left maxillary sinus shows marked mucosal swelling
but is not involved by tumor (arrowhead).
1639
FIGURE 29-63. Midsagittal postcontrast T1 in a different patient shows a
large sinonasal undifferentiated carcinoma (SNUC) involving the entire
nasal cavity and extending intracranially.
1640
FIGURE 29-64. Axial noncontrast T1 in a patient with a left ethmoid
primary melanoma shows the tumor to contain bright regions.
SUGGESTED READINGS
Sen S, Chandra A, Mukhopadhyay S, et al. Sinonasal tumors: computed
tomography and MR imaging features. Neuroimaging Clin N Am
2015;25:595–618.
Sen S, Chandra A, Mukhopadhyay S, et al. Imaging approach to sinonasal
neoplasms. Neuroimaging Clin N Am 2015;25:577–593.
1641
Granulomatosis with Polyangiitis
(Wegener Granulomatosis)
Key Facts
A destructive aseptic process characterized by noncaseating
granulomata and vasculitis most commonly presenting in the fourth to
fifth decades of life.
Can affect any organ or tissue but tends to involve midline facial
structures (may result in a large sinonasal cavity); affects the nasal
cavity (particularly septum), paranasal sinuses (with secondary orbital
involvement), and temporal bone.
Initially: septal perforation and erosion (particularly anterior ethmoid
region), nodular mucosal thickening, enhancing soft tissue masses are
present.
Late: paranasal sinuses become small, thick walled, and occasionally
filled with fibro-osseous–appearing tissues (neo-osteogenesis),
particularly the maxillary antra.
Low T1 and T2 signal and enhances.
Simultaneous involvement of nasal cavities/paranasal sinuses and
orbits should raise the suspicion of granulomatosis with polyangiitis
(but carcinoma is a much more common cause for this). Hard palate
is less commonly involved.
Main differential diagnosis: cocaine use, sarcoidosis, invasive fungal
sinusitis, lymphoma.
1642
FIGURE 29-65. Axial CT shows a large midline sinonasal cavity with
fibro-osseous replacement of the maxillary antra.
1643
FIGURE 29-66. Coronal CT, in the same patient, shows destruction of the
nasal septum and ethmoid structures including turbinates, with
preservation of the hard palate.
1644
FIGURE 29-67. Coronal CT in a different patient shows fibro-osseous
replacement of the antra and destruction of the hard palate with relative
preservation of the upper ethmoid structures.
1645
FIGURE 29-68. Coronal CT, in a different patient, shows an atelectatic
right maxillary sinus with thick walls, nasal septal perforation, and partial
destruction of the turbinates. There is nodular soft tissue in the left
maxillary antrum.
1646
FIGURE 29-69. Postcontrast T1 image, in a different patient, shows
enhancing soft tissue in the right maxillary sinus that extends into the
retromaxillary structures.
1647
FIGURE 29-70. Axial CT, in a different patient, shows opacification of
ethmoid and sphenoid cavities with erosion of septa and extension into
both orbits.
SUGGESTED READING
Pakalniskis MG, Berg AD, Policeni BA, et al. The many faces of granulomatosis
with polyangiitis: a review of the head and neck imaging manifestations. AJR
Am J Roentgenol 2015;205:W619–W629.
1648
CHAPTER 30
Temporal Bone
Bell Palsy
Key Facts
Most common cause of unilateral facial nerve palsy (70%).
Characterized by acute onset (peaks at 48 hours) and spontaneous
resolution within 4 to 6 months.
Possible etiologies include viruses (latent infection in geniculate
ganglion), ischemia, immunological disorders, and polyneuropathy
associated with other CNS or systemic disorders.
Fifteen percent of patients have an atypical course characterized by
slow progression and no remission.
One to five percent of all facial nerve palsies are bilateral and are
most often viral in nature (herpes, varicella, cytomegalovirus).
MRI: enhancement of the seventh nerve, imaging may not be needed
for unilateral uncomplicated palsies but only for atypical ones
(recurrent, progressive, bilateral). Enhancement may persist several
months after clinical recovery.
Enhancement of the descending portion of the facial nerve is a normal
finding seen in 76% of MRI studies due to a rich circumneural
vascular plexus.
Differential diagnoses: Ramsay Hunt syndrome (secondary to herpes
zoster infection), sarcoidosis, Lyme disease, lymphoma, perineural
tumor spread, and early facial nerve schwannoma.
Main differential diagnoses: tumor (primary and perineural spread)
and postoperative enhancement.
1649
FIGURE 30-1. Axial postcontrast T1 shows bilateral cochlear
enhancement (arrows) in a patient with sensorineural hearing loss and
right facial nerve palsy.
1650
enhancement of right facial nerve (straight arrow) and of left basal turn
(curved arrow) of cochlea and left vestibule (arrowhead). Findings were
thought to be due to viral infection.
1651
FIGURE 30-3. Coronal T1, in a different patient, shows enhancement of
the right geniculate ganglion (arrow).
1652
enhancement of facial nerve (arrow) as it courses in the superior portion of
the IAC.
1653
enhancement of the right facial nerve at the fundus, labyrinthine segment,
and geniculate ganglion (arrow).
1654
asymmetric enhancement of the right mastoid segment (arrow). Compare
to normal enhancement on the left (arrowhead).
SUGGESTED READING
Singh AK, Bathla G, Altmeyer W, et al. Imaging spectrum of facial nerve lesions.
Curr Probl Diagn Radiol 2015;44:60–75.
Cholesteatoma, Acquired (Secondary)

Key Facts
1655
Typically arises from epithelial-lined tympanic membrane retraction
pockets (due to Eustachian tube dysfunction and negative pressures)
that become trapped in the middle ear with subsequent deposition of
cells and keratin debris.
Forms a mass-like lesion in the middle ear that erodes the ossicles and
bone.
Pars flaccida cholesteatomas (most common type) begin in Prussak
space (therefore erode the scutum) and extend to the epitympanum,
aditus ad antrum, and mastoid antrum.
Pars tensa cholesteatomas begin in the mesotympanum and involve
the sinus tympani and erode the ossicles (which is the most common
associated abnormality on CT). They are less common than the pars
flaccida ones.
Complications: dehiscence of the tegmen tympani with intracranial
extension (produces meningitis, venous thrombosis, abscess, or CSF
leak), erosion of labyrinth with creation of perilymphatic fistula,
pneumolabyrinth, facial nerve palsy, hearing loss, and
automastoidectomy (especially with “mural” cholesteatomas). Higher
recurrence rates are seen following canal wall-up compared to wall-
down mastoidectomies.
They are bright on DWI; non–echoplanar DWI techniques are
preferred as they lack susceptibility artifact, allow thinner sections,
and can detect lesions as small as 2 to 3 mm (need to be larger than 5
mm to be appreciable on conventional echoplanar DWI).
Cholesteatomas do not enhance after Gd administration.
Main differential diagnoses: when small, none as their appearance is
typical, if large and extend to middle ear and cause bone erosion,
tumors, congenital middle ear cholesteatoma, and paraganglioma.
1656
FIGURE 30-7. Coronal CT in early cholesteatoma shows thickening of
pars flaccida (arrow) due to retraction pocket filled with desquamated
debris.
1657
FIGURE 30-8. Coronal CT reformat in a different patient without surgical
history shows the classic appearance of an automastoidectomy.
1658
FIGURE 30-9. Corresponding axial DWI shows the cholesteatoma to be
bright.
1659
nonenhancing cholesteatoma (arrow) in mastoid antrum.
1660
FIGURE 30-11. Axial CT in a different patient shows a large
cholesteatoma with dehiscence of the facial nerve canal, lateral
semicircular canal (arrow), and sigmoid plate.
1661
FIGURE 30-12. Coronal non–echoplanar DWI clearly shows a bright
cholesteatoma that was poorly seen on echoplanar technique due to
susceptibility artifact.
SUGGESTED READINGS
Alvo A, Garrido C, Salas A, et al. Use of non-echo-planar diffusion-weighted MR
imaging for the detection of cholesteatomas in high-risk tympanic retraction
pockets. AJNR Am J Neuroradiol 2014;35:1820–1824.
Dremmen MH, Hofman PA, Hof JR, et al. The diagnostic accuracy of non-echo-
planar diffusion-weighted imaging in the detection of residual and/or
recurrent cholesteatoma of the temporal bone. AJNR Am J Neuroradiol
2012;33:439–444.
Cholesterol Granuloma
1662
Key Facts
Results from blood entering mucosa-lined air cells with proposed
mechanisms including either obstruction (due to Eustachian tube
dysfunction with vacuum phenomenon) of aerated petrous apex or
exposed bone marrow with accumulation of secretions and repeated
hemorrhages.
Seen in association with chronic middle ear inflammation and is the
most common lesion of the petrous apex. Can occur at any site where
there are mucosa-lined air cells.
Presents with headache and/or dysfunction of cranial nerves six and
seven, but many are incidental findings.
Expansile and bright on both T1 and T2 (due to presence of
cholesterol crystals) may show peripheral contrast enhancement and
areas of hemorrhage.
Should not be confused with congenital cholesteatoma (epidermoid)
of the petrous apex, which is usually of CSF-like signal intensity on
MR (however, occasionally, epidermoids are bright on both T1 and
T2 due to high cholesterol content—“white epidermoid”—and
indistinguishable from the more common cholesterol granuloma).
Main differential diagnoses: asymmetric fat/bone marrow, ICA
aneurysm, trapped fluid, apicitis, chordoma, and metastasis.
1663
FIGURE 30-13. Axial noncontrast T1 shows a well-circumscribed
expansile bright lesion in the left petrous apex.
1664
FIGURE 30-14. Axial postcontrast T1 shows a rim of peripheral
enhancement.
1665
FIGURE 30-15. Axial CT, in a different patient, shows very large
expansile cholesterol granuloma, which has eroded into the internal
auditory canal and basal turn of cochlea.
1666
FIGURE 30-16. Axial CT, in a different patient, shows opacification but
no remodeling/erosion of the air cells in the petrous apex compatible with
trapped secretions.
1667
FIGURE 30-17. Axial T2 in a different patient shows dark blood products
(arrow) within a cholesterol granuloma in the right petrous apex.
1668
FIGURE 30-18. Axial postcontrast T1 in a patient with Gradenigo
syndrome shows abscess (arrow) in right petrous apex and enhancing
mucosa in the mastoid.
SUGGESTED READING
Isaacson B. Cholesterol granuloma and other petrous apex lesions. Otolaryngol
Clin North Am 2015;48:361–373.
Facial Nerve Schwannoma

Key Facts
Account for 5% of facial nerve palsies, particularly unilateral (the
remainder are of viral or posttraumatic etiology).
Onset of facial nerve palsy is slow and progressive.
Tend to arise in the geniculate ganglion but may involve any of its
1669
segments and result in smooth scalloping of the surrounding bone.
Identification of extension along the labyrinthine segment of the
facial nerve is important in making the correct presurgical diagnosis
as it is only schwannomas that result in significant thickening and
enhancement in this region.
May be seen as part of NF2; intracranial schwannomas are less
common in NF3.
Main differential diagnoses: facial nerve hemangioma (more
aggressive bony changes with a permeative appearance), perineural
tumor spread, vestibular schwannoma, viral neuritis (Bell’s and
Ramsay Hunt syndrome), parotid mass (when extracranial), and
meningioma.
FIGURE 30-19. Coronal postcontrast T1 shows enhancing schwannomas

involving the descending and intraparotid (star) portions of cranial nerve
VII.
1670
FIGURE 30-20. Axial CISS image, in a different patient, shows dark
signal in a facial nerve tumor involving the IAC (arrow) and region of
geniculate ganglion (arrowhead).
1671
FIGURE 30-21. Axial CT, in a different patient, shows a mass involving
the left geniculate ganglion and the tympanic segment of the facial nerve
where it surrounds the ossicles.
1672
FIGURE 30-22. Corresponding postcontrast T1 shows avid enhancement
of the schwannoma (arrow).
1673
FIGURE 30-23. Axial fat-saturated postcontrast T1 in a different patient
shows a schwannoma involving the cisternal, meatal, and labyrinthine
segments and geniculate ganglion.
1674
FIGURE 30-24. Coronal fat-saturated postcontrast T1 in a different
patient shows a schwannoma in the left geniculate ganglion (arrow).
SUGGESTED READINGS
Toulgoat F, Sarrazin JL, Benoudiba F, et al. Facial nerve: from anatomy to
pathology. Diagn Interv Imaging 2013;94:1033–1042.
Wiggins RH III, Harnsberger HR, Salzman KL, et al. The many faces of facial
nerve schwannoma. AJNR Am J Neuroradiol 2006;27:694–699.
Temporal Bone Fractures

Key Facts
Longitudinal type:
Most common (70% to 80%), results from blows to temporoparietal

region and leads to conductive hearing loss secondary to ossicular
1675
chain dislocations (most common: incudostapedial [>2 mm
separation] and malleoincudal). Tympanic membrane is often
perforated (increased incidence of postfracture cholesteatoma).
Facial palsy (10% to 20%) is delayed and generally resolves
spontaneously.
Air in the temporomandibular joint is an indirect sign of temporal
bone fracture. Air in the labyrinth is indicative of underlying fracture.
Transverse type:
Second most common (10% to 20%) type (some authors believe that
mixed or complex fractures are more common than either) results
from frontal or occipital blows.
Produces sensorineural hearing loss and vertigo due to involvement
of the otic capsule and/or transection/concussion of eighth cranial
nerve. Facial palsy is more common and usually permanent due to
nerve transection.
Otic capsule violating versus capsule sparing:
Determination of otic capsule involvement has better correlation with

clinical outcomes and complications than the traditional
classification.
About 95% of fractures spare the otic capsule, involvement of which
is more common with occipital blows and increases the risk of CSF
leak/fistulas, sensorineural hearing loss, and seventh cranial nerve
palsy.
1676
FIGURE 30-25. Axial CT shows longitudinal fracture (arrow) and
separation of malleus and incus.
1677
FIGURE 30-26. Axial CT, in a different patient, shows air (arrow) in
vestibule due to a fracture.
FIGURE 30-27. Axial CT, in a different patient, shows missing incus due
to complete dislocation. Only the head of the malleus is seen.
1678
FIGURE 30-28. Axial CT, in a different patient, shows mild
malleoincudal separation (arrow) and air cell opacification.
1679
FIGURE 30-29. Axial CT in a different patient shows an oblique fracture
with disruption of the ossicles (arrowhead) and violation of the otic
capsule (arrow).
1680
FIGURE 30-30. Axial CT in a different patient with prior history of a
temporal bone fracture shows a cephalocele (arrowhead) and fluid in the
mastoid air cells (arrows) due to CSF leak.
SUGGESTED READINGS
Collins JM, Krishnamoorthy AK, Kubal WS, et al. Multidetector CT of temporal
bone fractures. Semin Ultrasound CT MR 2012;33:418–431.
Juliano AF, Ginat DT, Moonis G. Imaging review of the temporal bone: part II.
Traumatic, postoperative, and noninflammatory nonneoplastic conditions.
Radiology 2015;276:655–672.
1681
Glomus Tympanicum
Key Facts
Paraganglioma occurring at the cochlear promontory and arising from
the plexus formed by the nerves of Jacobson (tympanic branch of
ninth cranial nerve) and Arnold (auricular ramus of tenth cranial
nerve).
Generally found in women >30 years of age and is the most common
tumor of the middle ear in adults.
Symptoms: pulsatile tinnitus, hearing loss, aural fullness, and facial
nerve palsy (30%). They can occasionally bleed; otalgia is rare.
They are very vascular tumors, and their blood supply is mostly via
external carotid artery branches (especially the ascending pharyngeal
artery); therefore, presurgery embolization is relatively easy.
Presents as retrotympanic “red mass” whose differential diagnosis
includes aberrant internal carotid artery, persistent stapedial artery,
dehiscent jugular bulb (or diverticulum), cholesterol granuloma, or
hemangioma.
Only 2% are multicentric (less than paragangliomas arising
elsewhere), they are prone to be locally invasive, and 5% are
malignant (see section on paragangliomas for more information).
Main differential diagnoses: aberrant ICA, dehiscent jugular bulb,
congenital middle ear cholesteatoma, and schwannoma of facial
nerve.
1682
FIGURE 30-31. Coronal CT shows soft tissue mass (arrow) over cochlear
promontory.
1683
FIGURE 30-32. Coronal postcontrast CT reformat in a different patient
shows very avid (almost vascular appearing) enhancement of a glomus
tympanicum (arrow).
1684
FIGURE 30-33. Lateral DSA view, different patient, after ECA injection
shows large carotid body tumor (arrow ) and smaller but also
hypervascular glomus tympanicum (arrowhead). (Case courtesy of H.
Alvarez, Chapel Hill, NC.)
1685
FIGURE 30-34. Axial noncontrast CT, in a different patient, shows tumor
along the cochlear promontory (arrow) and postobstructive secretions in
the mastoid air cells.
1686
FIGURE 30-35. Corresponding postcontrast T1 shows avid enhancement
of the tumor (arrow).
1687
FIGURE 30-36. Indium-111 Octreoscan in the same patient shows uptake
within the tumor (arrow).
SUGGESTED READING
Sweeney AD, Carlson ML, Wanna GB, et al. Glomus tympanicum tumors.
Labyrinthitis Obliterans
Key Facts
1688
Refers to an inflammatory (possibly viral, syphilis) process, trauma,
and prior surgery that result in sensorineural hearing loss and vertigo.
May be of the “fibrosing” type (better seen with MRI) initially and
then of the “ossifying” type (seen with MRI and CT).
Etiologies: tympanogenic, meningogenic (bacterial meningitis),
hematogenic (viral), autoimmune, toxic, ischemic, and posttraumatic
(including iatrogenic).
Unilateral disease is usually tympanogenic in origin; bilateral is
usually meningogenic or hematogenic in nature.
CT: focal (at round window and/or basilar turn of cochlea) or diffuse
ossification of the membranous labyrinthine.
MRI (particularly CISS images): normal T2 brightness of labyrinthine
structures is not well seen. The scala tympani of the basal turn of the
cochlea (posteriorly) is the most frequently affected site and should
be scrutinized as findings may be subtle and isolated. There may be
enhancement in the early stages.
FIGURE 30-37. Axial CT shows increased density in the scala tympani of

the basal turn of the cochlea (arrow) with preservation of fluid in the scala
1689
vestibuli anteriorly.
FIGURE 30-38. Axial CT, in a different patient, shows fuzzy borders and
near-complete effacement of the cochlea (arrow) by bone proliferation
(ossifying labyrinthitis). The vestibule and semicircular canals are
involved too and not well seen.
1690
FIGURE 30-39. Coronal CT, same patient, shows bone filling most of the
cochlea (arrow).
1691
FIGURE 30-40. Axial CISS image, different patient, shows normal bright
right cochlea (arrow). The left cochlea (arrowhead) is only faintly seen.
1692
FIGURE 30-41. Axial CT in a different patient shows heterogeneously
increased density in the apical and middle turns of the cochlea
(arrowhead) and modiolus and obliteration of the lateral semicircular
canal (arrow).
1693
FIGURE 30-42. Corresponding CISS image shows decreased signal in the
right cochlea (arrow) and vestibule (arrowhead). Compare to normal left
side.
SUGGESTED READING
Booth TN, Roland P, Kutz JW Jr, et al. High-resolution 3-D T2-weighted imaging
in the diagnosis of labyrinthitis ossificans: emphasis on subtle cochlear
involvement. Pediatr Radiol 2013;43:1584–1590.
Otospongiosis
Key Facts
1694
Also called “otosclerosis.” It occurs in 1% of the population, is more
common in women, and can be bilateral in 85% of patients.
Patients present with progressive hearing loss with onset between the
fourth and fifth decades of life.
Two types: fenestral (more common) and retrofenestral.
Both types are characterized by an osteodystrophy affecting the bony
labyrinth with development of fibrous and vascularized changes in
bone that later on may result in new bone formation (thus the name
“otosclerosis”).
In the fenestral type, the process affects the margins (particularly the
anterior one) of the oval window and stapes footplate and produces
conductive hearing loss, treatment is stapedectomy, postoperative
complications need to be imaged with MR, and main differential
diagnoses include infection, Paget disease, and fibrous dysplasia.
In the retrofenestral (cochlear) type, the process affects the otic
capsule (especially around the cochlea) and produces sensorineural
deafness; treatment is vitamin D, fluoride, or cochlear implantation.
Almost always seen in conjunction with fenestral involvement.
Main differential diagnoses: osteogenesis imperfecta, Paget disease,
fibrous dysplasia, radiation-induced changes, and syphilis.
1695
FIGURE 30-43. Axial CT shows lucency (arrows) around the cochlea in a
case of retrofenestral otospongiosis.
FIGURE 30-44. Coronal CT in the same patient confirms lucency

(arrows) surrounding the cochlea.
1696
FIGURE 30-45. Axial CT, in a different patient, shows lucency (arrow)
anterior to the oval window in the region of the fissula ante fenestram.
1697
FIGURE 30-46. Coronal CT in the same patient confirms the typical
“ground-glass” lucency (arrow) of otospongiosis.
1698
FIGURE 30-47. Coronal CT reformat in a different patient with
retrofenestral otospongiosis shows mild pericochlear lucency (arrows). A
stapes prosthesis (arrowhead) is partially seen.
SUGGESTED READINGS
Juliano AF, Ginat DT, Moonis G. Imaging review of the temporal bone: part II.
Traumatic, postoperative, and noninflammatory nonneoplastic conditions.
Radiology 2015;276:655–672.
Whetstone J, Nguyen A, Nguyen-Huynh A, et al. Surgical and clinical
confirmation of temporal bone CT findings in patients with otosclerosis with
failed stapes surgery. AJNR Am J Neuroradiol 2014;35:1195–1201.
1699
Vascular Anomalies and Variants, Middle
Ear
Key Facts
Generally present as vascular retrotympanic masses that are clinically
indistinguishable from glomus tympanicum.
Only seldom are associated with conductive hearing loss but not
uncommonly produce tinnitus.
Jugular bulb variants include asymmetrical jugular foramen (most
common and generally on the right); high–riding jugular bulb and
jugular bulb diverticulum (found in 6% of the population) are used
interchangeably and refer to the jugular bulb extending up to the level
of the basal cochlear turn; dehiscent jugular bulb is less common and
refers to intratympanic herniation of the bulb via dehiscent bone.
“Aberrant carotid artery” is a misnomer and results from enlargement
of the inferior tympanic artery as it anastomoses with a large
caroticotympanic artery due to regression of the cervical internal
carotid artery (ICA); may be confirmed by MRA. As a general rule,
the carotid artery should not extend laterally beyond the cochlear
promontory.
Persistent stapedial artery is very rare and may be associated with an
aberrant ICA; characteristic findings include absence of foramen
spinosum and enlarged tympanic portion of the facial nerve as this
artery usually follows it. It may result in conductive hearing loss due
to ankylosis of the stapes or rarely erode the otic capsule and lead to
sensorineural hearing loss.
1700
FIGURE 30-48. Axial CT shows a high–riding jugular bulb (arrow) with
a very thin and nearly dehiscent plate separating it from the middle ear
cavity.
1701
FIGURE 30-49. Axial CT in a different patient shows a jugular bulb
diverticulum (arrowhead) projecting into the tympanic space (“dehiscent
jugular bulb”).
1702
FIGURE 30-50. Coronal CT, in a different patient, shows lateralized
internal carotid artery (arrow) projecting into the tympanic space.
1703
FIGURE 30-51. Axial CT, in a different patient, shows “high–riding”
jugular bulb (arrow) extending to the midcochlear level.
1704
FIGURE 30-52. Axial CT, in a different patient, shows a small
canaliculus arising from the petrous segment of the internal carotid artery
(arrow, left) and continuing as a persistent stapedial artery (arrow, right).
SUGGESTED READING
Blazic S, Milojevic M, Grgurevic U, et al. Anatomical variations and relations of
large blood vessels to the tympanic cavity. Surg Radiol Anat
2015;37:327–331.
Enlarged Endolymphatic Sac (Large

Vestibular Aqueduct) Syndrome
1705
Key Facts
One percent of patients with congenital sensorineural hearing loss
have abnormalities detected by imaging studies; most commonly
recognized one is probably a large vestibular aqueduct.
Vestibular aqueduct syndrome is the most commonly recognized
cause of congenital hearing loss and may be bilateral in up to 90% of
patients.
Enlarged vestibular aqueduct may occur in the setting of autosomal
recessive SLC26A4 gene mutations as part of Pendred syndrome.
Normal vestibular aqueduct extends from the vestibule to the
posterior aspect of the petrous bone and contains the endolymphatic
duct whose function is equilibration of endolymphatic fluid pressure.
CT: dilated vestibular aqueduct (vestibular aqueduct should be no
wider than a semicircular canal or more than 1.5 mm at its midpoint).
Large vestibular aqueduct may be associated with cochlear anomalies
(from incomplete partition type II [Mondini] to absence of the
modiolus).
Enlargement of the endolymphatic sac may occur in the presence of a
normal size aqueduct, and it may present as a mass in the
cerebellopontine angle region and is better seen on MRI.
Fluid levels and occasional contrast enhancement may be seen.
1706
FIGURE 30-53. Axial CT in a patient with incomplete partition type II
(Mondini) shows dysplastic cochlea (arrowhead), large vestibular
aqueduct (black arrow), and large vestibule (white arrow).
1707
FIGURE 30-54. Axial CT in a different patient shows a dilated vestibular
aqueduct (arrow).
1708
FIGURE 30-55. Axial CISS image, in a different patient, shows enlarged
vestibular aqueducts bilaterally (arrows).
1709
FIGURE 30-56. Axial CISS image, in a different patient, shows large
right endolymphatic sac (arrow) and prominent left vestibular aqueduct
(arrowhead).
1710
FIGURE 30-57. Axial CISS image, in a different patient, shows very
large left-sided endolymphatic sac (arrow).
1711
mildly large and enhancing endolymphatic sacs (arrows).
SUGGESTED READINGS
Huang BY, Zdanski C, Castillo M. Pediatric sensorineural hearing loss, part 1:
Practical aspects for neuroradiologists. AJNR Am J Neuroradiol
2012;33:211–217.
Young JY, Ryan ME, Young NM. Preoperative imaging of sensorineural hearing
loss in pediatric candidates for cochlear implantation. Radiographics
2014;34:E133–E149.
Vestibular Schwannoma
Key Facts
Most common posterior fossa tumor in adults and second most
common intracranial extra-axial tumor after meningioma in adults.
Seventy-five percent to 80% of masses in the cerebellopontine angle
1712
cistern are vestibular schwannomas.
Arise from the Scarpa ganglion at the glial cell–Schwann cell junction
(Obersteiner-Redlich zone) in the superior division of the vestibular
nerve.
More common in females, 40 to 60 years of age.
Bilateral eighth nerve schwannomas = NF2.
Most common symptoms: sensorineural hearing loss, tinnitus,
headache, and disequilibrium.
Facial nerve palsy is uncommon as this nerve is fairly resistant to
pressure.
Five to twenty-two percent of vestibular schwannomas are atypical by
imaging and have associated arachnoid cysts or central necrosis or are
partially or completely cystic. There may be increased cochlear signal
on FLAIR due to increased protein in the perilymphatic space.
Main differential diagnoses: meningioma, facial nerve schwannoma,
metastasis, viral infection (Ramsay Hunt syndrome), and lipoma (low
signal on fat-suppressed MR images).
1713
FIGURE 30-59. Coronal postcontrast T1 shows an oblong enhancing
mass in the right internal auditory canal (arrow).
1714
FIGURE 30-60. Axial T2 in a different patient shows a large vestibular
schwannoma in the right cerebellopontine angle with a small canalicular
component and mass effect on the brainstem.
1715
FIGURE 30-61. Axial postcontrast T1 in a different patient shows avid
enhancement of a vestibular schwannoma with cisternal and canalicular
components.
1716
FIGURE 30-62. Axial CISS image, in a different patient, shows a small
tumor in the left IAC fundus (arrow) probably extending to the basal turn
at the insertion of the modiolus.
1717
FIGURE 30-63. Axial postcontrast T1 image, in a different patient, shows
a tiny tumor (arrow) in the fundus of the right IAC.
1718
schwannoma within the right vestibule (arrow).
SUGGESTED READINGS
Ginat DT, Martuza RL. Postoperative imaging of vestibular schwannomas.
Neurosurg Focus 2012;33:E18.
Silk PS, Lane JI, Driscoll CL. Surgical approaches to vestibular schwannomas:
what the radiologist needs to know. Radiographics 2009;29:1955–1970.
1719
Index
A
Aberrant carotid artery
Abscess(es)
cerebral
orbital
of the spine epidural
Abuse, brain trauma of child
Acute disseminated encephalomyelitis (ADEM)
Adenoma, pituitary
Agenesis of corpus callosum
Agyria/pachygyria complex
AIDS. See Human immunodeficiency virus infection;
Infection/Inflammation
Alexander disease
Allergic reactions
during angiography
to contrast medium, prevention of
management of acute
anaphylaxis See (Hypotension)
bronchospasm
facial/laryngeal edema
hypertension, severe
pulmonary edema
seizures
urticaria
vagal reaction See (Hypotension)
Alobar holoprosencephalies
Alzheimer disease
1720
Amino acid disorders
Aminophylline, for reaction to contrast media
Amyotrophic lateral sclerosis
Anaphylaxis
Anaplastic astrocytoma
Aneurysm (s)
angiography for evaluation of
of brain, imaging fundamentals and facts of
anterior communicating artery
basilar artery tip
extradural See (Pseudoaneurysm)
giant
infratentorial
intracranial, multiple
middle cerebral artery
posterior communicating artery
vasospasm
Aneurysmal bone cyst (ABC)
Angiofibroma, nasopharyngeal
Angiography, general guidelines
allergic reactions
aneurysm evaluation
anticoagulants, oral
anticoagulation during
carotid stenosis
consent for
contrast medium for
hypertension, precautions for patients with
injection rates
for adults, suggested
for children, suggested
injection sites
neuroangiographic catheters for
patient chart
postangiogram items
notes
orders
1721
preangiogram items
laboratory tests
notes
orders
premedication
pulses
stroke-like symptoms, appearance of
for vasculitis evaluation
for vertebral arteries
Angioma
cavernous
of brain
of spinal cord
pial
Ankylosing spondylitis in spine
Annular tears, of spine
Anterior cerebral artery (ACA) infarction
Anterior communicating artery aneurysm
Anterior nasal masses
Anticoagulation treatment, during angiography
Antidepressants, effect on seizure threshold
Antipsychotics, effect on seizure threshold
Anxiolysis
adults
children
Aqueductal stenosis
Arachnoid cyst
Arachnoiditis, spinal
Arterial dissection, trauma
Arteriovenous fistula
Arteriovenous malformation (AVM)
and fistula, dural
spinal cord
Aspirin, over-the-counter drugs containing
Astrocytoma
anaplastic
brain stem
1722
low grade
pilocytic
spinal cord
Atropine, for reaction to contrast media
Autosomal recessive disorder
Axonal injury, diffuse and intermediary injuries
B
Balo sclerosis
Band heterotopia
Basal ganglia
Basilar artery
aneurysm of tip
thrombosis
Bell palsy
Benign compression fractures
Bilateral facet dislocation
Bleeding time, over-the-counter drugs that increase
Bone cyst, aneurysmal
Border zone infarcts
Brachial plexus, imaging fundamentals and facts
fatty tumors
inflammatory and infectious plexitis
radiation-induced plexitis
viral and idiopathic plexitis
metastases
nerve sheath tumors
pancoast tumors
traumatic injuries
avulsion and stretch injuries
vascular injuries
Brain
aneurysms, imaging fundamentals and facts of
computed tomography (CT)
contrast
craniosynostosis (spiral CT)
noncontrast, routine
1723
degenerative disorders, imaging fundamentals and facts
hemorrhage, nontraumatic, imaging fundamentals and facts
infections/inflammation, imaging fundamentals and facts
leukodystrophies
magnetic resonance imaging (MRI) protocols
neonatal
perfusion
pulsatile tinnitus
stroke
trauma
tumor
with and without contrast
malformations
congenital, imaging, fundamentals and facts
vascular, imaging, fundamentals and facts
metabolic disorders, imaging fundamentals and facts
neurocutaneous syndromes, imaging fundamentals and facts
stroke, imaging fundamentals and facts
subdural hematoma
trauma, imaging fundamentals and facts
tumors, imaging fundamentals and facts
extra-axial
intra-axial
Brain plexus, MRI protocols
Brain stem astrocytoma
Branchial cleft cysts
Bronchodilators, effect on seizure threshold
Bronchospasm
Buccal space mass
C
CADASIL (Cerebral autosomal dominant arteriopathy with subcortical
infarctions and leukoencephalopathy)
Calcifications
cerebral
globe
Canavan disease
1724
Capillary telangiectasia
Carbon monoxide intoxication
Carcinomas, choroid plexus
Carotid artery
aberrant
cavernous sinus fistula of
internal, stenosis
Carotid space mass
Carotid stenosis
angiography for
internal carotid artery
Caudal agenesis syndrome
Caudal agenesis (regression) syndrome
Cavernous malformation
of brain
spinal cord
C1-C2 punctures for myelography
Cellulitis, orbital
Central neurocytoma
Cerebellar artery aneurysm, posterior inferior (PICA)
Cerebellar infarction, acute
Cerebral abscess
Cerebral amyloid angiopathy
Cerebral angiogram
Cerebral artery
anterior (ACA), infarction
middle
bifurcation aneurysm of
infarction, acute (less than 24 hours), CT
infarction, acute (less than 24 hours), MRI
infarction, subacute (2 to 21days), CT
infarction, subacute (2 to 21days), MRI
posterior (PCA), infarction
Cerebral autosomal dominant arteriopathy with subcortical infarctions and
leukoencephalopathy (CADASIL)
Cerebral calcifications
1725
Cerebral microhemorrhages
Cerebral pyogenic abscess
Cerebral vasculitis
Cerebrospinal fluid (CSF) leak
Cervical myelograms
Chance-type fractures
Charcot-Marie-Tooth disease
Chiari malformation
type I
type II
Child abuse, brain trauma of
Chloral hydrate
Choanal narrowing
Cholesteatoma, acquired (secondary)
Cholesterol granuloma
Chordoma
Choriocarcinoma
Choroidal detachments and effusions
Choroid plexus
carcinoma
tumors
Clopidogrel (Plavix)
CNS stimulants, effect on seizure threshold
Colloid cyst
Compression fractures of spine
benign
malignant
Computed tomographic angiography (CTA)
head
neck/carotids
Computed tomography (CT)
brain
contrast
craniosynostosis (spiral CT)
noncontrast, routine
contrast medium for
CSF leak
1726
CTA
head
neck/carotids
CT perfusion
maxillofacial
neck
orbits
pituitary
sinuses, paranasal
contrast
noncontrast
preoperative for computer navigation
spine
cervical, routine
thoracic/lumbar, routine
spiral (helical) CT
temporal bones
Congenital malformations
of the brain, imaging fundamentals and facts
agenesis of corpus callosum
agyria/pachygyria complex
anterior nasal masses
aqueductal stenosis
band heterotopia
Chiari malformation
cortical dysplasias
craniosynostoses
Dandy–Walker syndrome
focal gray matter heterotopias
holoprosencephalies
Joubert syndrome
posterior encephaloceles
schizencephalies
septooptic dysplasia
of the spine, imaging fundamentals and facts
caudal agenesis (regression) syndrome
dermoid and epidermoid
1727
diastematomyelia
lipomas
lipomyelomeningocele and lipomyelocele
myelomeningocele and myelocele
Consent form
for angiography
for myelography
Contrast media
allergies, prevention of reaction in patients with
CT contrast
drugs for treatment of reactions to
aminophylline
atropine
dexamethasone sodium phosphate
diazepam (Valium)
diphenhydramine (Benadryl)
epinephrine
endotracheal tubes
gadolinium-based contrast agents
iodinated contrast (renal insufficiency)
MR contrast
adult
children
for sedation
oral
parenteral
seizure threshold, drug groups that lower
antidepressants
antidepressants, tricyclic
antipsychotics
bronchodilators
CNS stimulants
immunomodifiers
MAO inhibitors
narcotics
phenothiazine derivatives
Contrecoup injuries
1728
Contusion(s)
of brain
of the spine
Corpus callosum, infarctions
Cortical dysplasias
Cortical vein thrombosis
Craniofacial arteriovenous metameric syndrome (CAMS), arteriovenous
malformations in
Craniopharyngioma
Craniosynostoses
Craniosynostosis, computed tomography (CT)
Creutzfeldt–Jakob disease
Cryptococcus
Cyst(s)
aneurysmal
branchial cleft
facet
fissural
mucous retention, in paranasal sinuses
perineural (Tarlov)
spinal cord
synovial
thyroglossal duct
Tornwaldt
Cysticercosis
Cytomegalovirus (CMV)
D
Dandy–Walker syndrome
Degenerative disorders
Alzheimer disease
amyotrophic lateral sclerosis
carbon monoxide intoxication
hippocampal sclerosis
Huntington disease
hyperglycemia
1729
intracranial hypotension
liver failure, total parenteral nutrition
methanol intoxication
multisystem atrophy
Osmotic demyelination syndrome
pantothenate kinase deficiency
pseudotumor cerebri
radiation injury
of spine, imaging fundamentals and facts
annular tears
facet cysts, lumbar
herniated disc
ossified posterior longitudinal ligament
Paget disease
schmorl nodes
spondylolysis and spondylolisthesis, lumbar spine
Dejerine–Sottas disease
Dementia, Alzheimer disease
Dermoid
Dermoid and epidermoid spinal malformations
Dermoid tumors, orbital
Detachments and effusions, orbital
Developmental venous anomalies (DVA)
Dexamethasone sodium phosphate, for reaction to contrast media
Diastematomyelia
Diazepam (Valium)
as anxiolytic
dosage for sedation
Diphenhydramine (Benadryl), for reaction to contrast media
Discitis, pyogenic
Drugs
affecting performance of invasive procedures
allergic reactions
to contrast medium, prevention of
management of acute
anxiolytics
aspirin, over-the-counter drugs containing
1730
bleeding time, over-the-counter drugs that increase
contrast extravasation
contrast media See (Contrast media)
Drusen bodies
Dural arteriovenous malformations and fistulas
Dysembryoplastic neuroepithelial tumor (DNET)
Dysplasia
cortical
septooptic
Dysplastic cerebellar gangliocytoma
E
Ear, vascular anomalies in middle
Edema
facial/laryngeal
pulmonary
Effusions, orbital
EMLA Cream, for IV insertion in children
Encephalitis
Rasmussen
viral
Encephaloceles
occipital and parietal
sincipital
Encephalomyelitis disseminated, acute
Encephalopathy
hypertensive
hypoxic-ischemic
Wernicke
Endolymphatic sac syndrome, enlarged
Endotracheal tubes
Eosinophilic granuloma of the spinal cord
Ependymoma
brain
spinal
Ependymomas and subependymomas
Epidermoid
1731
Epidermoid spinal malformations
Epidermoid tumors of brain
Epidural abscess
Epidural blood patch
Epidural hematoma
Epinephrine, for reaction to contrast media
Extra-axial brain tumors, imaging fundamentals and facts
Extrapontine myelinolysis
Eye of the tiger sign
F
Facet cysts, spinal
Facial/laryngeal edema
Facial nerve schwannoma
Fentanyl, dosages for sedation
Fibromuscular dysplasia (FMD)
Fibro-osseous lesions of sinonasal cavities
Filar lipoma
Fissural cyst
Flumazenil (Romazicon)
Focal gray matter heterotopias
Fracture(s)
benign compression
Hangman fracture
Jefferson fracture
odontoid
orbital cavity
pathologic (malignant) compression
of skull
of temporal bone, longitudinal
Fungal sinusitis
Fungiform papillomas
G
Gadolinium-based contrast agents
Gangliogliomas
Ganglion cysts
1732
Germinal matrix hemorrhage
Giant aneurysm, in brain
Glioblastoma
Gliomatosis cerebri
Globe calcifications
Glomus tympanicum
Glutaric aciduria type 1
Goiter, multinodular
Granuloma
cholesterol
eosinophilia
Graves ophthalmopathy
Gray matter heterotopia, focal
Guillain–Barré syndrome
H
Hallervorden–Spatz syndrome
Hamartoma
Hangman fracture
Head. See also Orbits
orbital pathology, imaging fundamentals and facts
sinonasal cavities, imaging fundamentals and facts
temporal bone, imaging fundamentals and facts
Headache, postmyelography
Head and neck, neck masses See (Neck)
Hemangioblastoma
Hemangioma
of neck
orbital cavernous
vertebral body
Hematoma
brachial plexus
brain
epidural
subdural
spinal epidural and subdural
Hemorrhage, brain
1733
nontraumatic, imaging fundamentals and facts of
cerebral amyloid angiopathy
cerebral microhemorrhages
germinal matrix hemorrhage, grades I and II
germinal matrix hemorrhage, grades III and IV
hypertensive, acute
periventricular leukomalacia
Hemorrhagic encephalomyelitis, acute
Hemorrhagic infarcation in brain
Hemorrhagic transformation in brain
Heparin
Herniated disc
Herpes
type I
type II
Heterotopia
band
focal gray matter
Hippocampal sclerosis
Holoprosencephalies
Homocystinuria
Human immunodeficiency virus infection
Huntington disease
Hydromyelia
Hygroma, brain
Hyperglycemia
Hypertension, precautions for patients with, during angiography
Hypertensive encephalopathy
Hypertrophic polyneuropathies,spinal
Hypotension
with bradycardia
with tachycardia
Hypoxia in brain, generalized
Hypoxic—ischemic encephalopathy
I
Iatrogenic disorders
1734
liver failure and total parenteral nutrition
osmotic demyelination syndrome
radiation injury
Ibuprofen
Idiopathic plexitis
Imaging. See also Computed tomography (CT)
computed tomography (CT) protocols
Immunomodifiers, effect on seizure threshold
Infarction
anterior cerebral artery (ACA)
cerebellar, acute
corpus callosum
hemorrhagic, in brain
lacunar
middle cerebral artery infarction
acute (less than 24 hours), MRI
subacute (2 to 21 days)
posterior cerebral artery
spinal cord
watershed
Infection/inflammation
cerebral abscess
Creutzfeldt–Jakob disease
cryptococcus
cysticercosis
disseminated encephalomyelitis, acute
human immunodeficiency virus
leukoencephalopathy, progressive multifocal
meningitis
multiple sclerosis
neuromyelitis optica
Rasmussen encephalitis
sarcoidosis
toxoplasmosis
tuberculosis
1735
viral encephalitis
arachnoiditis
Guillain-Barré syndrome
hypertrophic polyneuropathies
phlegmon
rheumatoid arthritis
spondylitis, discitis, and epidural abscess, pyogenic
tuberculosis
Infratentorial aneurysms
Infratentorial tumors
brainstem astrocytoma
dysplastic cerebellar gangliocytoma
ependymoma and subependymoma
hemangioblastoma
medulloblastoma
pilocytic astrocytoma
Injection rates, for angiography
for adults, suggested
for children, suggested
Injection sites, for angiography
Intra-axial tumors
infratentorial tumors See (Infratentorial tumors)
supratentorial tumors See (Supratentorial tumors)
Intracranial aneurysms, multiple
Intracranial hypotension
Intraocular tamponade
Invasive procedures, drugs that may affect performance of
Iodinated contrast (renal insufficiency)
Iohexol
Iopamidol
Ischemia in brain, generalized
Ivy sign
J
Jefferson fracture
Joubert syndrome
1736
K
Korsakoff psychosis
Krabbe disease
L
Labyrinthitis obliteransff
Lacrimal gland mass
Lacunar infarctions
Laminar necrosis
Laryngeal mass
Leigh disease
Leptomeningeal cysts in skull fracture
Leptomeningeal metastases
Leukodystrophies
adrenoleukodystrophy
Alexander disease
Canavan disease
Krabbe disease
metachromatic
Pelizaeus–Merzbacher disease
Leukomalacia, periventricular
Lhermitte–Duclos disease
Lidocaine and prilocaine (EMLA Cream), for IV insertion in children
Lidocaine, for reaction to contrast media
Lipoma
spinal
Lipomyelomeningocele and lipomyelocele, spinal
Liposarcoma, brachial plexus
Lobar holoprosencephalies
Locked-in syndrome
Lorazepam (Ativan)
as anxiolytic
dosages for sedation
Lymphoma
1737
Magnetic resonance imaging (MRI) protocols
brain
neonatal
perfusion
pulsatile tinnitus
stroke
trauma
tumor
with and without contrast
brain plexus
malformations and anomalies See also (Congenital malformations)
of spine
neck
carotid arteries
general
pituitary
spine
cervical
lumbar
thoracic
temporomandibular joints
venogram
Malformations and anomalies, of brain
Maple syrup disease
Masticator space mass
Maxillofacial bones, computed tomography (CT) of trauma
Medications. See Drugs
Medulloblastoma
Melanoma, orbital
MELAS (myopathy, encephalopathy, lactic acidosis and strokes)
Meningioma, spinal
Meningitis
complicated
uncomplicated
Menke kinky hair syndrome
Meperidine (Demerol), dosages for sedation
MERRF (myoclonic epilepsy with ragged red fibers)
1738
Metabolic disorders, of the brain, imaging fundamentals and facts
amino acid disorders
cerebral calcifications
mitochondrial disorders
mucopolysaccharidoses
Wernicke encephalopathy
Wilson disease
Metastases
brachial plexus
nodal
spinal cord and leptomeningeal
vertebral
Methanol intoxication
Midazolam (Versed)
as anxiolytic
dosages for sedation
Midline holoprosencephalies
Mitochondrial disorders
Morphine sulfate, dosages for sedation
Moyamoya, in brain
Mucocele, sinonasal
Mucous retention cysts
Multiple sclerosis
Multisystem atrophy
Myelography
C1-C2 punctures
cervical
complete myelogram
consent form
contrast media, suggested usage of adults
CT scan, postmyelogram
general guidelines
injection site
lumbar
padiatric patients
patient chart
1739
postmyelogram orders
postmyelography headache
puff method
thoracic
vasovagal reaction
Myelomeningocele and myelocele, spinal
N
Naloxone (Narcan)
Narcotics, effect on seizure threshold
Nasopharyngeal angiofibroma
Nasopharyngeal mass
Neck
computed tomography (CT) protocol
masses in the, imaging fundamentals and facts
angiofibroma, nasopharyngeal
branchial cleft cysts
buccal space
carotid space
fissural cyst
hemangiomas
laryngeal
masticator space
nasopharyngeal
nodal metastases
oropharyngeal space
paragangliomas
parapharyngeal space
parotid space
retropharyngeal space
sialolithiasis
slow-flow vascular malformations
sublingual and submandibular space
thyroglossal duct
thyroid
Tornwaldt
1740
Necrosis, laminar brain
Necrotizing leukoencephalopathy
Nerve root, avulsions
Neuritis, optic
Neuroangiographic catheters
Neurocutaneous syndromes, of the brain, imaging fundamentals and facts
myelin vacuolization
neurofibromatosis type 2
neurofibromatosis type 1
Phace(s) syndrome
schwannomatosis
Sturge–Weber syndrome
tuberous sclerosis
von Hippel-Lindau disease
Neurocytoma, central
Neurofibromatosis
type 1
astrocytoma and neurofibroma
myelin vacuolization
type 2
Neuromyelitis optica
Neuronal tumors
central neurocytoma
dysembryoplastic neuroepithelial tumor
gangliogliomas
Nitroprusside, for reaction to contrast media
Nodal metastases
Nonsteroidal anti-inflammatory agents, over-the-counter drugs containing
O
Occipitoatlantal separation
Ocular trauma
Oligodendroglioma
Optic neuritis
Oral cavity mass
Orbital cavity trauma
Orbits. See also Head
1741
pathology of, imaging fundamentals and facts
cellulitis and abscess
dermoid
detachments and effusions
globe calcifications
Graves ophthalmopathy
infantile and congenital hemangiomas
lacrimal gland mass
lymphatic malformation
melanoma
optic neuritis
orbital cavernous hemangioma
orbital cavity trauma
orbital dermoid
persistent hyperplastic primary vitreous
posttreatment changes
pseudotumor
retinoblastoma (PNET-RB)
trauma, ocular
Oropharyngeal space mass
Ossified posterior longitudinal ligament
Osteoma
choroidal
osteoid
Ostiomeatal unit, obstruction
Otospongiosis (otosclerosis)
P
Paget disease
Pancoast tumor involving the brachial plexus
Pantothenate kinase deficiency
Papilloma
choroid plexus
fungiform
of paranasal sinuses
Paranasal sinuses. See also Sinuses
1742
malignancy
Parapharyngeal space mass
Parotid space mass
Pars tensa cholesteatomas
Pathologic (malignant) compression fractures
Patient notes
for angiography
for myelography
Pediatric patients
computed tomography protocols
preparation for myelography
Pelizaeus–Merzbacher disease
Pentobarbital sodium (Nembutal)
Perineural cysts
Perisylvian syndrome
Periventricular leukomalacia
Persistent hyperplastic primary vitreous (PHPV)
Persistent stapedial artery
Petrous apex, cysts of
Phace(s) syndrome
Phenothiazine derivatives, effect on seizure threshold
Phenothiazines
Phentolamine, for reaction to contrast media
Phenylketonuria
Phlegmon
Pilocytic astrocytoma
Pineal cysts
Pineal gland tumors
Pineoblastomas
Pituitary adenoma
Pituitary gland
adenoma of
Platelet inhibitors
Plavix (clopidogrel)
Plexitis
1743
radiation-induced
viral and idiopathic
Pneumocephalus
Polyneuropathies, hypertrophic
Polyposis, sinonasal
Postangiogram items
notes
orders
Posterior cerebral artery (PCA) infarction
Posterior communicating artery aneurysm
Posterior encephaloceles
Posterior inferior cerebellar artery (PICA) aneurysm
Posterior longitudinal ligament, ossified
Posterior reversible encephalopathy syndrome (PRES)
Postmyelography
CT scan
headache
orders
Posttraumatic aneurysms. See Pseudoaneurysm
Preangiogram items
laboratory tests
notes
orders
Primary inflammatory demyelinating polyneuropathy
Progressive multifocal leukoencephalopathy
Protamine sulfate
Pseudoaneurysm
in brachial plexus
in brain
Pseudosynovial cysts
Pseudotumor cerebri
Pseudotumor, inflammatory orbital
Puff method in myelography
Pulmonary edema
Pulsatile tinnitus
Pulses for angiography protocol
1744
R
Radiation-induced plexitis
Radiation injury
Ranitidine, for reaction to contrast media
Ranulas
Rasmussen encephalitis
Renal insufficiency (iodinated contrast)
Rendu–Osler–Weber disease, arteriovenous malformations in
Retinal detachments and effusions
Retinopexy
Retropharyngeal space mass
Rhematoid arthritis, spinal
S
Sarcoidosis
Schizencephalies
Schmorl nodes
Schwannoma
brain
facial nerve
spinal
vestibular
Scleral banding/buckling
Sedation
intraprocedural care
lidocaine and prilocaine (EMLA Cream)
oral drugs
chloral hydrate
diazepam (Valium)
lorazepam (Ativan)
midazolam (Versed)
morphine sulfate
pentobarbital sodium (Nembutal)
parenteral drugs
diazepam (Valium)
fentanyl
1745
lorazepam (Ativan)
meperidine (Demerol)
midazolam (Versed)
morphine sulfate
pentobarbital sodium (Nembutal)
post-procedural care
preprocedural care
release of patient
reversal agents
flumazenil (Romazicon)
naloxone (Narcan)
sedation scale
topical agents
Sedation scale
Seizure threshold, drug groups that lower
antidepressants
tricyclic
antipsychotics
bronchodilators
CNS stimulants
immunomodifiers
MAO inhibitors
narcotics
phenothiazine derivatives
Semilobar holoprosencephalies
Septooptic dysplasia. See also Holoprosencephalies
Sialolithiasis
Sincipital encephaloceles
Sinonasal polyposis
Sinuses
imaging fundamentals and facts
acute (uncomplicated)
choanal narrowing
CSF leak
fibro-osseous lesions of sinonasal cavities
fungal
1746
mucocele
mucous retention cysts
ostiomeatal unit, obstruction
papilloma
paranasal sinus malignancy
sinonasal polyposis
Wegener granulomatosis
Sinusitis
acute (uncomplicated)
fungal
Skull fracture
Sodium bicarbonate, for reaction to contrast media
Spinal cord
astrocytoma
cavernous malformation
contusions
cyst
ependymoma
infarction
metastases
Spine
congenital malformations, imaging fundamentals and facts
infection/inflammation, imaging fundamentals and facts
tumors, imaging fundamentals and facts
vascular disorders, imaging fundamentals and facts
Spiral (helical) computed tomography (CT)
Spondylitis and discitis of the spine, pyogenic
Spondylolisthesis, lumbar
Spondylolysis, lumbar
Stapedial artery, persistent
Stenosis
aqueductal
internal carotid artery
Stroke
imaging fundamentals and facts of
1747
anterior cerebral artery infarction
basilar artery thrombosis
CADASIL
cerebellar infarction, acute
cerebral vasculitis
corpus callosum infarctions
cortical vein thrombosis
fibromuscular dysplasia
hemorrhagic infarction
hypertensive encephalopathy
hypoxia, generalized
internal carotid artery stenosis
ischemia, generalized
lacunar infarctions
middle cerebral artery infarction
acute (less than 24 hours), CT
acute (less than 24 hours), MRI
subacute (2 to 21 days), CT
subacute (2 to 21 days), MRI
moyamoya
posterior cerebral artery infarction
venous sinus occlusion
venous system occlusion, deep
Wallerian degeneration
watershed infarctions
Stroke-like symptoms, appearance during angiography
Sturge–Weber syndrome
Subacute combined degeneration
Subarachnoid hemorrhage, traumatic
Subdural hematoma
brain
spinal
Subependymal giant cell astrocytoma
Sublingual space mass
Submandibular space mass
Supratentorial tumors
1748
astrocytoma
anaplastic
low grade
ependymomas and subependymomas
glioblastoma
gliomatosis cerebri
metastases
neuronal tumors
central neurocytoma
dysembryoplastic neuroepithelial tumor
gangliogliomas
oligodendroglioma
Synovial cyst, lumbar
Syringomyelia
T
Tarlov cyst
Telangiectasia, capillary, effects in brain
Temporal bone
imaging fundamentals and facts
Bell palsy
cholesteatoma, acquired (secondary)
cholesterol granuloma
ear, vascular anomalies in middle
fracture of
longitudinal
transverse
labyrinthitis obliterans
otospongiosis
schwannoma
facial nerve
vestibular
vascular anomalies, middle ear
vestibular aqueduct syndrome
Temporomandibular joints, magnetic resonance imaging (MRI) protocols
Teratoma
1749
Thoracic myelogram
Thrombosis
basilar artery
cortical vein
venous sinus occlusion
Thyroglossal duct cyst
Thyroid mass
Tinnitus, pulsatile
Topical agents for IV insertion in children
Tornwaldt cyst
Total parenteral nutrition
Toxoplasmosis
Trauma
to brain, imaging fundamentals and facts
arterial dissection
axonal injury, diffuse, and intermediary injuries
from child abuse
contusions
epidural hematoma
hygroma
pneumocephalus
skull fractures
subarachnoid hemorrhage, traumatic
subdural hematoma
benign compression fractures
chance-type fractures
compression fractures, malignant
contusion
facet dislocation
Hangman fracture
Jefferson fracture
occipitoatlantal separation
odontoid fractures
vertebral artery injury
Tricyclic antidepressants, effect on seizure threshold
1750
Tuberculosis
brain
spine
Tuberous sclerosis of brain
harmartoma
subependymal giant cell astrocytoma
Tumors. See also specfic tumors
of the brachial plexus
metastases
Pancoast tumor involving
schwannoma
of the brain, extra-axial, imaging fundamentals and facts
arachnoid cyst
choroid plexus
colloid cyst
craniopharyngioma
dermoid
epidermoid
lipoma
meningioma
pineal gland
pituitary adenoma
of the brain, intra-axial, imaging fundamentals and facts
astrocytoma See (Astrocytoma)
dysembryoplastic neuroepithelial tumors
dysplastic cerebellar gangliocytoma
gangliogliomas
glioblastoma multiforme
gliomatosis cerebri
hemangioblastoma
lymphoma
medulloblastoma
metastases
neurocytoma, central
neuronal cell
oligoastrocytoma
oligodendroglioma
1751
pilocytic astrocytoma
of sinonasal cavities, malignant
aneurysmal bone cyst
astrocytoma
chordoma
eosinophilic granuloma
ependymoma
hemangioma, vertebral body
hydromyelia
leptomeningeal metastases
meningioma
metastases See (Metastases)
osteoid osteoma
perineural (Tarlov) cyst
schwannoma
spinal cord cyst
subacute combined degeneration
U
Unilateral facet dislocation
Urticaria
V
Vagal reaction
Vascular anomalies and variants, middle ear
Vascular injuries, brachial plexus and
Vascular malformations and anomalies
brain, imaging fundamentals and facts
arteriovenous malformation (AVM)
capillary telangiectasia
carotid artery-cavernous sinus fistula of
dural arteriovenous malformations and fistula
vein of Galen malformation
venous malformation
1752
spinal, imaging fundamentals and facts
arteriovenous fistula
arteriovenous malformations
hematomas, epidural and subdural
infarction
Vasculitis
angiography for evaluation of
cerebral
Vasospasm
Vasovagal reaction in myelography
Vein of Galen malformation
Venous angioma
Venous malformation of brain, developmental
Venous sinus occlusion
Venous system occlusion of brain, deep
Vertebral arteries, angiography of
Vertebral artery injury
Vertebral body hemangioma
Vertebral metastases
Vestibular aqueduct syndrome, large
Vestibular schwannoma
Viral encephalitis
Viral plexitis
von Hippel-Lindau disease
W
Wallerian degeneration
Watershed infarctions
Wegener granulomatosis
Wernicke encephalopathy
While matter brain disorders. See Leukodystrophies
Wilson disease
Wyburn–Mason syndrome, arteriovenous malformations in
1753
目录
Half Title 2
Title 3
Copyright 5
Dedication 7
Preface 8
Acknowledgments.html 10
Contents 11
PART 1: IMAGING PROTOCOLS AND
21
GUIDELINES
1 CT Protocols 22
Brain without Contrast 22
Brain with Contrast Administration 23
Deep Brain Stimulator Head Protocol 24
Paranasal Sinus, Screening 24
Paranasal Sinuses with Contrast 25
Paranasal Sinuses, Preoperative for Computer Navigation 26
Maxillofacial without Contrast 26
Maxillofacial with IV Contrast 27
Orbits 28
Temporal Bones 28
Neck 29
CSF Leak 30
Craniosynostosis 30
Routine C-Spine 31
Routine T-/L-Spine 31
CTA Head 32
CTA Neck/Carotids 33
CT Perfusion 34
Pituitary Protocol 34
2 MRI Protocols 36
1754
Brain without and with Contrast 36
Neonatal Brain 37
Brain, Stroke 37
Brain, Tumor 37
Brain, Trauma 38
Brain, Perfusion 38
Carotid Arteries, Neck 38
Venogram 39
Brain, Pulsatile Tinnitus 39
Pituitary 39
Neck, General 40
Temporomandibular Joints 40
Cervical Spine 40
Thoracic Spine 40
Lumbar Spine 41
Brachial Plexus 41
3 Myelography Protocols 43
General Guidelines 43
4 Digital Subtraction Angiography Protocols 49
General Guidelines 49
5 Sedation and Anxiolysis Protocols 55
Conscious Sedation 55
Anxiolysis 61
6 Medications in Neuroradiology 63
Medications for Contrast Media Reactions 63
Endotracheal Tubes 65
Prevention of contrast reactions in allergic patients 65
Management of contrast reactions 65
Medications That May Affect the Performance of Invasive
68
Procedures
Medications (Generic Names) That May Lower Seizure
69
Threshold
Over-the-Counter Medications That Increase Bleeding Time 70
CT Contrast Allergy 72
1755
Iodinated Contrast in Renal Insufficiency 72
MR Contrast Administration in Adults (>18 Years of Age) 74
MR Contrast Administration in Children (<16 and >2 Years
75
of Age)
Contrast Extravasation 75
PART 2: IMAGING FUNDAMENTALS 77
SECTION A: Brain Imaging 78
7 Trauma 79
Arterial Dissection 79
Child Abuse 86
Contusions 90
Diffuse Axonal Injury and Intermediary Injuries 96
Epidural Hematoma 103
Pneumocephalus 107
Skull Fractures 112
Subdural Hematoma and Hygroma 119
Traumatic Subarachnoid Hemorrhage 126
8 Stroke 133
Acute Cerebellar Infarct 133
Acute (<24 Hours) Middle Cerebral Artery Infarct, CT 139
Acute (<24 Hours) Middle Cerebral Artery Infarct, MRI 146
Acute Anterior (ACA) and Posterior (PCA ) Cerebral
155
Artery Infarcts
Basilar Artery Occlusion 163
Cadasil 169
Corpus Callosum Infarctions 177
Cortical Vein Thrombosis 184
Deep Venous System Occlusion 191
Generalized Brain Hypoxia/Ischemia 198
Fibromuscular Dysplasia (FMD) 204
Hemorrhagic Infarct and Hemorrhagic Transformation 209
Hypertensive Encephalopathy 215
Stenosis, Extracranial ICA 222
Lacunar Infarctions 227
1756
Moyamoya 231
Subacute (2 to 21 Days) Middle Cerebral Artery Infarct,
237
CT
Subacute Infarction, MRI 241
Cerebral Vasculitis 248
Venous Sinus Occlusion 255
Wallerian Degeneration 262
Watershed Cerebral Infarctions 269
9 Nontraumatic Hemorrhage 274
Acute Hypertensive Hemorrhages 274
Cerebral Amyloid Angiopathy 280
Cerebral Microhemorrhages 288
Hemorrhage in the Premature Brain 293
Periventricular Leukomalacia (PVL) 299
10 Aneurysms 307
Anterior Communicating (AComm) Artery Aneurysms 307
Basilar Artery Tip Aneurysms 313
Giant Aneurysms 320
Infratentorial Aneurysms 326
Middle Cerebral Artery Bifurcation Aneurysms 334
Multiple Intracranial Aneurysms 340
Posterior Communicating Artery Aneurysms 346
Pseudoaneurysms 351
Vasospasm (after SAH) 357
11 Vascular Malformations 365
Arteriovenous Malformations (AVM) 365
Capillary Telangiectasias 371
Carotid Artery–Cavernous Sinus Fistulas 376
Cavernous Malformations 383
Developmental Venous Anomalies (DVA) 391
Dural Arteriovenous Fistulas 397
Vein of Galen Malformations 404
12 Extra-Axial Masses 412
Arachnoid Cyst 412
1757
Choroid Plexus Tumors 418
Colloid Cyst 425
Craniopharyngioma 433
Dermoid 439
Epidermoid 446
Lipoma 453
Meningioma 460
Pineal Gland Tumors 468
Pituitary Adenoma 475
13 Intra-Axial Tumors 483
Supratentorial Tumors 483
Anaplastic Astrocytoma 483
Ependymomas and Subependymomas 489
Glioblastoma 497
Gliomatosis Cerebri 504
Astrocytoma, Low Grade 510
Lymphoma 518
Metastases 525
Neuronal Tumors 532
Oligodendroglioma 539
Infratentorial Tumors 546
Brainstem Astrocytoma 546
Ependymoma and Subependymoma 553
Hemangioblastoma 559
Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos
566
Disease)
Medulloblastoma 572
Pilocytic Astrocytoma 580
14 Infections and Inflammations 587
Acute Disseminated Encephalomyelitis (ADEM) 587
Cerebral Pyogenic Abscess 594
Creutzfeldt-Jakob Disease (CJD) 601
Cryptococcus 608
Cysticercosis 615
1758
Human Immunodeficiency Virus Infection 621
Meningitis, Complications 628
Meningitis (Uncomplicated) 635
Multiple Sclerosis 640
Neuromyelitis Optica 646
Progressive Multifocal Leukoencephalopathy (PML) 654
Rasmussen Encephalitis 660
Sarcoidosis 668
Tuberculosis 674
Toxoplasmosis 682
Viral Encephalitis 689
15 Leukodystrophies 696
Adrenoleukodystrophy 696
Alexander Disease 702
Canavan Disease 708
Krabbe Disease 713
Metachromatic Leukodystrophy 718
Pelizaeus-Merzbacher Disease 723
16 Metabolic Disorders 729
Amino Acid Disorders 729
Cerebral Calcifications 734
Mitochondrial Disorders 739
Mucopolysaccharidoses 746
Wernicke Encephalopathy 751
Wilson Disease (Hepatolenticular Degeneration) 757
17 Degenerative and Iatrogenic Disorders 764
Alzheimer Disease 764
Amyotrophic Lateral Sclerosis 769
Carbon Monoxide and Methanol Intoxication 774
Hippocampal Sclerosis 779
Huntington Disease (Chorea) 785
Nonketotic Hyperglycemia 792
Intracranial Hypotension 797
Liver Insufficiency and Total Parenteral Nutrition 804
1759
Multisystem Atrophy 809
Osmotic Demyelination Syndrome 814
Pantothenate Kinase Deficiency (Hallervorden-Spatz
821
Syndrome)
Pseudotumor Cerebri 827
Radiation Injury 832
18 Congenital Malformations 840
Agenesis of Corpus Callosum 840
Agyria/Pachygyria Complex and Band Heterotopia 846
Anterior Nasal Masses 854
Aqueductal Stenosis 861
Chiari Malformation Type I 866
Chiari Malformation Type II 871
Cortical Dysplasias 875
Craniosynostoses 881
Dandy-Walker Complex 887
Focal Gray Matter Heterotopias 892
Holoprosencephalies 898
Joubert Syndrome 905
Posterior (Occipital and/or Parietal) Encephaloceles 910
Schizencephalies 917
Septooptic Dysplasia 921
19 Neurocutaneous Syndromes 927
NF-1 927
NF-2 934
PHACE(S) Syndrome 941
Sturge-Weber Syndrome 948
Schwannomatosis (NF-3) 955
Tuberous Sclerosis 960
von Hippel-Lindau Disease 967
SECTION B: Spinal Imaging 974
20 Brachial Plexus 975
Inflammatory and Infectious Plexitis 975
Traumatic Brachial Plexus Injuries 979
1760
Brachial Plexus Tumors 986
21 Congenital Malformations 994
Caudal Agenesis (Regression) Syndrome 994
Diastematomyelia (Split-Cord Malformation) 1001
Dermoid and Epidermoid 1008
Lipomas 1015
Lipomyelomeningocele and Lipomyelocele 1022
Myelomeningocele and Myelocele 1028
22 Degenerative Spine 1036
Annular Fissures 1036
Facet Cysts 1039
Herniated Disc 1047
Ossified Posterior Longitudinal Ligament (OPLL) 1053
Paget Disease 1061
Schmorl Nodes 1068
Spondylolysis and Spondylolisthesis, Lumbar Spine 1074
23 Infection Inflammation 1082
Ankylosing Spondylitis (Seronegative
1082
Spondyloarthropathy)
Arachnoiditis 1089
Guillain-Barré Syndrome 1095
Hypertrophic Polyneuropathies 1100
Rheumatoid Arthritis 1108
Spondylitis, Discitis, and Epidural Abscess, Pyogenic 1115
Tuberculosis 1122
24 Spine Tumors and Tumor-like Conditions 1129
Aneurysmal Bone Cyst (ABC) 1129
Spinal Cord Astrocytoma 1135
Chordoma 1142
Spinal Cord Cysts, Nonneoplastic 1149
Eosinophilic Granuloma 1155
Spinal Cord Ependymoma 1162
Vertebral Body Hemangioma 1169
Osteoid Osteoma 1175
1761
Perineural (Tarlov) Cysts 1183
Spinal Schwannoma and Meningioma 1187
Spinal Cord and Leptomeningeal Metastases 1195
Subacute Combined Degeneration 1202
Vertebral Metastases 1209
25 Trauma 1217
Occipitoatlantal Separation 1217
Benign Compression Fractures 1224
Pathologic (Malignant) Compression Fractures 1231
Chance-Type Fractures 1238
Facet Dislocation 1245
Hangman Fracture 1252
Jefferson Fracture 1259
Odontoid Fractures 1264
Spinal Cord Injury 1271
Vertebral Artery Injury 1279
26 Vascular Disorders 1286
Spinal Cord Infarction 1286
Spinal Epidural and Subdural Hematomas 1293
Spinal Arteriovenous Fistula 1300
Spinal Cord Arteriovenous Malformations 1307
Spinal Cord Cavernous Malformation 1314
SECTION C: Ear, Nose, and Throat Imaging 1322
27 Neck Masses 1323
Branchial Cleft Cysts 1323
Buccal Space Masses 1330
Carotid Space Masses 1336
Fissural Cysts 1342
Hemangiomas 1347
Laryngeal Masses 1355
Masticator Space Masses 1361
Nasopharyngeal Masses 1368
Nasopharyngeal Angiofibroma 1376
Nodal Metastases 1382
1762
Oral Cavity and Oropharyngeal Space Masses 1389
Paragangliomas (Skull Base) 1396
Parapharyngeal Space Masses 1403
Parotid Space Masses 1410
Retropharyngeal Space Masses 1417
Sialolithiasis 1423
Slow-Flow Vascular Malformations 1430
Sublingual and Submandibular Space Masses 1437
Thyroglossal Duct Cyst 1444
Thyroid Masses 1451
Tornwaldt Cyst 1458
28 Orbits 1465
Globe Calcifications 1465
Orbital Dermoid 1468
Graves Ophthalmopathy 1474
Orbital Cavernous Malformation (Hemangioma, Adult
1480
Type)
Infantile and Congenital Hemangiomas 1487
Inflammatory Pseudotumor 1494
Lacrimal Gland Masses 1502
Orbital Lymphatic Malformation 1508
Melanoma 1511
Optic Neuritis 1518
Orbital Cellulitis and Abscess 1525
Orbital Cavity Trauma 1532
Persistent Hyperplastic Primary Vitreous (PHPV) 1538
Posttreatment Findings 1542
Detachments and Effusions 1549
Retinoblastoma (PNET-RB) 1556
Ocular Trauma 1563
29 Sinuses 1570
Acute (Uncomplicated) Sinusitis 1570
Anatomical Variants of the Sinonasal Cavity 1575
Choanal Narrowing 1582
1763
CSF Leaks 1589
Fibro-osseous Lesions of Sinonasal Cavities 1593
Fungal Sinusitis 1598
Papilloma 1606
Mucocele 1612
Mucous Retention Cysts 1619
Ostiomeatal Unit, Obstruction 1624
Sinonasal Polyps 1627
Paranasal Sinus Malignancy 1634
Granulomatosis with Polyangiitis (Wegener
1642
Granulomatosis)
30 Temporal Bone 1649
Bell Palsy 1649
Cholesteatoma, Acquired (Secondary) 1655
Cholesterol Granuloma 1662
Facial Nerve Schwannoma 1669
Temporal Bone Fractures 1675
Glomus Tympanicum 1682
Labyrinthitis Obliterans 1688
Otospongiosis 1694
Vascular Anomalies and Variants, Middle Ear 1700
Enlarged Endolymphatic Sac (Large Vestibular
1705
Aqueduct) Syndrome
Vestibular Schwannoma 1712
Index 1720
1764

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Neuroradiology

Carlos Zamora, MD, PhD

Mauricio Castillo, MD, FACR

Copyright © 2017 Wolters Kluwer

Copyright © 2012 by Lippincott Williams & Wilkins, a Wolters Kluwer business.

Library of Congress Cataloging-in-Publication Data

Given continuous, rapid advances in medical science and health information,

There is no doubt that neuroimaging plays a central role in the ever-

Carlos Zamora, MD, PhD

Carlos Zamora, MD, PhD

PART 1 ■ IMAGING PROTOCOLS AND

SECTION A ■ Brain Imaging

Brain without Contrast

0 to 6 months: 100 mAs

PACS: Transmit all images.

Brain with Contrast Administration

0 to 6 months: 100 mAs

IV Contrast: After completion of unenhanced scan

Deep Brain Stimulator Head Protocol

Paranasal Sinus, Screening

Paranasal Sinuses with Contrast

Paranasal Sinuses, Preoperative for

Maxillofacial without Contrast

Maxillofacial with IV Contrast

The following is a sample of the most commonly performed neuro-MRI

Brain without and with Contrast

*ASL, arterial spin labeled perfusion may be used additionally to contrast-enhanced

Carotid Arteries, Neck

Brain, Pulsatile Tinnitus

*MRA centered at base of skull.

*Obtain with mouth in open and closed positions.

11. If a complete myelogram is requested, do the thoracic area last

*Filming sequence is 2 to 4 frames per second using digital subtraction equipment. If

VA, vertebral artery.

9. If the patient is complaining of stroke-like symptoms, do no do

Lidocaine 2.5% and Prilocaine 2.5% (EMLA Cream)

Releasing the Patient

Medications for Contrast Media Reactions

Prevention of contrast reactions in allergic

Management of contrast reactions

Hypotension with Tachycardia (Anaphylaxis)

Hypotension with Bradycardia (Vagal

Medications That May Affect the

Over-the-Counter Medications That

*Contrast allergy premedication: prednisone 50 mg PO 13 hours, 7 hours,

Iodinated Contrast in Renal Insufficiency

Guidelines for Use of Gadolinium-Based

Acute or chronic severe renal insufficiency as evidenced by GFR <30

Acute renal insufficiency of any severity due to the hepatorenal

Adults with normal renal function Standard dose (0.1 mmol/kg)

MR Contrast Administration in Adults

Subdural Hematoma and Hygroma

Traumatic Subarachnoid Hemorrhage

Acute Cerebellar Infarct

Acute (<24 Hours) Middle Cerebral Artery

Acute (<24 Hours) Middle Cerebral Artery

Acute Anterior (ACA) and Posterior

Corpus Callosum Infarctions

Deep Venous System Occlusion

Generalized Brain Hypoxia/Ischemia

Fibromuscular Dysplasia (FMD)

Hemorrhagic Infarct and Hemorrhagic

Subacute (2 to 21 Days) Middle Cerebral