(A Case-Based Approach) Yang Tang, Sugoto Mukherjee, Max Wintermark - Emergency Neuroradiology - A Case-Based Approach-Cambridge University Press (2015)

Emergency Neuroradiology
A Case-Based Approach
Emergency Neuroradiology
A Case-Based Approach
Yang Tang MD PhD
Assistant Professor and Attending Radiologist in Neuroradiology and Emergency Radiology,
Virginia Commonwealth University Medical Center, Richmond, VA, USA
Sugoto Mukherjee MD
Assistant Professor, Department of Radiology and Medical Imaging, University of Virginia Health System,
Charlottesville, VA, USA
Max Wintermark MD MAS MBA

Professor of Radiology and Chief of Neuroradiology, Stanford University, Stanford, CA, USA
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C Yang Tang, Sugoto Mukherjee, and Max Wintermark 2015
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............................................................................
Every effort has been made in preparing this book to provide accurate and
up-to-date information which is in accord with accepted standards and practice
at the time of publication. Although case histories are drawn from actual cases,
every effort has been made to disguise the identities of the individuals involved.
Nevertheless, the authors, editors, and publishers can make no warranties that the
information contained herein is totally free from error, not least because clinical
standards are constantly changing through research and regulation. The authors,
editors, and publishers therefore disclaim all liability for direct or consequential
damages resulting from the use of material contained in this book. Readers
are strongly advised to pay careful attention to information provided by the
manufacturer of any drugs or equipment that they plan to use.
To our families, for the love and unwavering support!
Contents
List of contributors page x
Foreword by Mauricio Castillo xi
Preface xiii
List of abbreviations xiv
Case 2.3: Diffuse axonal injury 71
Section 1 – Brain Case 2.4: Traumatic carotid–cavernous fistula and
Section editor: Yang Tang carotid artery injury 73
1. Cerebrovascular diseases 1 3. Cerebral demyelinating and inflammatory
Yang Tang, Xinli Du, Sugoto Mukherjee, and diseases 75
Max Wintermark Yang Tang, Xinli Du, Max Wintermark, and
Sugoto Mukherjee
Case 1.1: Anterior circulation stroke 1
Case 1.2: Distal basilar thrombosis 6 Case 3.1: Tumefactive multiple sclerosis 75
Case 1.3: Spontaneous carotid dissection 8 Case 3.2: Acute disseminated encephalomyelitis 77
Case 1.4: Adult hypoxic–ischemic injury 11 Case 3.3: Neurosarcoidosis 79
Case 1.5: Neonatal hypoxic–ischemic injury 14 Case 3.4: Neuromyelitis optica – brain 82
Case 1.6: Reperfusion hemorrhage after carotid Case 3.5: Lupus cerebritis 85
stenting 17 Case 3.6: Neuro-Behçet’s syndrome 87
Case 1.7: Thrombotic microangiopathy 20
4. Intracranial infections 89
Case 1.8: Reversible cerebral vasoconstriction
syndrome 23 Yang Tang, Xinli Du, Sugoto Mukherjee, and
Case 1.9: Artery of Percheron infarction 25 Max Wintermark
Case 1.10: Primary angiitis of central nervous Case 4.1: Bacterial meningitis 89
system 27 Case 4.2: Pyogenic abscess 92
Case 1.11: Cerebral venous thrombosis 30 Case 4.3: CNS tuberculosis 95
Case 1.12: Lobar hemorrhage due to dural sinus Case 4.4: Cerebral fungal infection 98
thrombosis 34 Case 4.5: Herpes encephalitis 102
Case 1.13: Cerebral amyloid angiopathy 37 Case 4.6: Nonherpetic viral encephalitis 104
Case 1.14: Cavernous malformation 39 Case 4.7: Lyme neuroborreliosis 107
Case 1.15: Cerebral aneurysms 42 Case 4.8: Cerebellitis 109
Case 1.16: Vasospasm and delayed cerebral Case 4.9: Parenchymal neurocysticercosis 112
ischemia after subarachnoid Case 4.10: Ventricular neurocysticercosis 114
hemorrhage 47 Case 4.11: Creutzfeldt–Jakob disease 116
Case 1.17: Cerebral arteriovenous malformation 50 Case 4.12: Neurosyphilis 118
Case 1.18: Dural arteriovenous fistula 53 Case 4.13: Cerebral toxoplasmosis 120
Case 1.19: Cerebral arteriovenous fistula 56 Case 4.14: CNS cryptococcosis 122
Case 1.20: Moyamoya disease 57 Case 4.15: Progressive multifocal
Case 1.21: Spontaneous carotid–cavernous fistula 60 leukoencephalopathy 125
Case 1.22: Carotid blow-out syndrome 62 Case 4.16: Cytomegalovirus infection 127
Case 1.23: Sinus pericranii 64
5. Brain tumors and tumor-like conditions 129
2. Head trauma 66 Rajkamal S. Khangura, Max Wintermark,
Yang Tang, Max Wintermark, and Sugoto Mukherjee Sugoto Mukherjee, and Yang Tang
Case 2.1: Epidural hematoma 66 Case 5.1: Glioblastoma multiforme 129
Case 2.2: Cerebral herniation syndrome 68 Case 5.2: Gliomatosis cerebri 132
vii
Contents
Case 5.3: Oligodendroglioma 135 Case 7.7: Temporal bone fractures 228
Case 5.4: Primary CNS lymphoma 137 Case 7.8: Eagle syndrome 230
Case 5.5: Brainstem glioma 141 Case 7.9: Laryngeal injury 232
Case 5.6: Pediatric posterior fossa tumors 144
8. Head and neck infections 234
Case 5.7: Central neurocytoma 148
Case 5.8: Dysembryoplastic neuroepithelial tumor 150 Jason DeBerry, Max Wintermark, Sugoto Mukherjee, and
Case 5.9: Metastatic neuroblastoma 152 Yang Tang
Case 5.10: Tumefactive perivascular space 154 Case 8.1: Acute tonsillitis and peritonsillar abscess 234
Case 5.11: Hamartoma of tuber cineureum 156 Case 8.2: Lemierre’s syndrome 236
Case 5.12: Epidermoid 158 Case 8.3: Odontogenic abscess 238
Case 5.13: Colloid cyst 161 Case 8.4: Ludwig’s angina 240
Case 5.14: Ruptured arachnoid cyst 163 Case 8.5: Adult supraglottitis 242
Case 5.15: Ruptured intracranial dermoid cyst 165 Case 8.6: Acute retropharyngeal calcific tendinitis 244
Case 5.16: Choroid plexus papilloma 167 Case 8.7: Retropharyngeal abscess and
Case 5.17: Craniopharyngioma 169 descending necrotizing mediastinitis 246
Case 5.18: Intracranial metastasis 171 Case 8.8: Orbital cellulitis 248
6. Miscellaneous cerebral emergencies 174 Case 8.9: Invasive fungal sinusitis 250
Case 8.10: Petrous apicitis 253
Yang Tang, Matthew R. Parry, Sugoto Mukherjee, and
Case 8.11: Skull base osteomyelitis 256
Max Wintermark
Case 8.12: Mastoiditis with complications 258
Case 6.1: Posterior reversible encephalopathy
9. Orbits 260
syndrome 174
Case 6.2: Acute hepatic (hyperammonemic) Thomas J. E. Muttikal, Yang Tang, Max Wintermark, and
encephalopathy 177 Sugoto Mukherjee
Case 6.3: Wernicke’s encephalopathy 179 Case 9.1: Optic neuritis 260
Case 6.4: Delayed post-hypoxic Case 9.2: Ophthalmic artery aneurysm 262
leukoencephalopathy 181 Case 9.3: Orbital varix 264
Case 6.5: Central pontine myelinolysis 183 Case 9.4: Orbital cavernous hemangioma 266
Case 6.6: Metronidazole toxicity 185 Case 9.5: Orbital pseudotumor 268
Case 6.7: Radiation necrosis 187 Case 9.6: Orbital lymphoma 270
Case 6.8: Leigh syndrome 193 Case 9.7: Thyroid ophthalmopathy 272
Case 6.9: Maple-syrup urine disease 196 Case 9.8: Pituitary apoplexy 274
Case 6.10: Limbic encephalitis 198 Case 9.9: Cavernous sinus lymphoma 276
Case 6.11: Idiopathic intracranial hypertension 200
Case 6.12: Intracranial hypotension due to CSF 10. Paranasal sinuses 279
leak 202 Jason DeBerry, Max Wintermark, Sugoto Mukherjee, and
Case 6.13: Peri-ictal signal changes 206 Yang Tang
Case 6.14: Mesial temporal sclerosis 209 Case 10.1: Allergic fungal sinusitis 279
Case 6.15: Wallerian degeneration 210 Case 10.2: Squamous cell carcinoma of maxillary
Case 6.16: Amyotrophic lateral sclerosis 212 sinus 281
Case 10.3: Esthesioneuroblastoma 282
Case 10.4: Inverted papilloma 284
Section 2 – Head and neck Case 10.5: Frontoethmoidal
Section editor: Sugoto Mukherjee meningoencephalocele 286
7. Facial trauma 214 11. Temporal bone 288

David Chiao, Yang Tang, Max Wintermark, and Michael Reardon, Yang Tang, Max Wintermark, and
Sugoto Mukherjee Sugoto Mukherjee
Case 7.1: Orbital blow-out fractures 214 Case 11.1: External auditory canal cholesteatoma 288
Case 7.2: Globe injury 216 Case 11.2: Middle ear cholesteatoma 290
Case 7.3: Naso-orbital-ethmoidal fractures 218 Case 11.3: Glomus jugulotympanicum
Case 7.4: Zygomaticomaxillary complex fractures 221 paraganglioma 292
Case 7.5: Le Fort fractures 223 Case 11.4: Petrous apex cholesterol granuloma 294
Case 7.6: Mandibular fractures 226 Case 11.5: Labyrinthitis 296
viii
Contents
Case 11.6: Endolymphatic sac tumor 298 Case 15.8: Central cord syndrome 343
Case 11.7: Bell’s palsy 300 Case 15.9: Thoracolumbar fractures 345
Case 15.10: Ankylosing spondylitis 348
12. Head and neck tumors 302
Case 15.11: Brachial plexus injury 350
David Chiao, Sugoto Mukherjee, Yang Tang, and
Max Wintermark 16. Spinal infectious and inflammatory diseases 352
Thomas J. E. Muttikal, Max Wintermark, Sugoto
Case 12.1: Laryngeal carcinoma 302
Mukherjee, and Yang Tang
Case 12.2: Metastatic nodal mass mimicking
brachial cleft cyst 304 Case 16.1: Neuromyelitis optica – spine 352
Case 12.3: Vocal cord paralysis 306 Case 16.2: HIV-associated vacuolar myelopathy 354
Case 12.4: Skull base tumor 309 Case 16.3: Transverse myelitis 356
Case 16.4: Guillain–Barré syndrome 358
13. Pediatric head and neck conditions 311
Case 16.5: Neurosarcoidosis – spine 360
Michael Reardon, Yang Tang, Max Wintermark, and Case 16.6: Spondylodiscitis 362
Sugoto Mukherjee
17. Spinal tumors 364
Case 13.1: Persistent hyperplastic primary vitreous 311
Catherine Shaeffer, Max Wintermark, Sugoto Mukherjee,
Case 13.2: Choanal atresia and pyriform aperture
and Yang Tang
stenosis 313
Case 13.3: Juvenile nasopharyngeal angiofibroma 315 Case 17.1: Astrocytoma of cord 364
Case 13.4: Langerhans cell histiocytosis 317 Case 17.2: Ependymoma of cord 366
Case 13.5: Vascular malformations 320 Case 17.3: Myxopapillary ependymoma 368
Case 17.4: Spinal hemangioblastoma 370
Case 17.5: Spinal paraganglioma 372
Section 3 – Spine Case 17.6: Meningioma of foramen magnum 374
Case 17.7: Spinal cord metastasis 376
Section editor: Max Wintermark
Case 17.8: Spinal leptomeningeal metastasis 378
14. Spinal vascular diseases 322 Case 17.9: Vertebral metastasis with cord
Carlos Leiva-Salinas, Yang Tang, Sugoto Mukherjee, and compression 380
Max Wintermark
18. Miscellaneous spine emergencies 382
Case 14.1: Cord infarction 322 Thomas J. E. Muttikal, David Clifton, Yang Tang, Sugoto
Case 14.2: Perimedullary arteriovenous fistula 324 Mukherjee, and Max Wintermark
Case 14.3: Intramedullary cavernous
Case 18.1: Disc extrusion 382
malformation 326
Case 18.2: Spinal epidural abscess 384
15. Spinal trauma 328 Case 18.3: Spinal epidural hematoma 386
David T. Powell, Max Wintermark, Sugoto Mukherjee, Case 18.4: Rheumatoid arthritis of the spine 388
and Yang Tang Case 18.5: Periodontoid pseudotumor 390
Case 18.6: Vertebral plana 392
Case 15.1: Atlanto-occipital dislocation 328 Case 18.7: Arachnoid web 394
Case 15.2: Occipital condylar fracture 330
Case 15.3: Jefferson fracture 332
Case 15.4: Odontoid fracture 334
Case 15.5: Hangman fracture 336
Case 15.6: Hyperflexion injury 338 Index 396
Case 15.7: Hyperextension injury 341
ix
Contributors
David Chiao MD MPH University of Virginia Health System, Charlottesville,

Resident Physician, Department of Radiology and Medical VA, USA
Imaging, University of Virginia Health System, Charlottesville,
VA, USA Thomas J. E. Muttikal MD
Clinical Instructor, Division of Neuroradiology, Department
David Clifton MD of Radiology and Medical Imaging, University of Virginia
Resident Physician, Department of Radiology and Medical Health System, Charlottesville, VA, USA
VA, USA Matthew R. Parry MD
Resident Physician, Department of Radiology, Virginia
Jason DeBerry MD Commonwealth University Medical Center, Richmond, VA,
Resident Physician, Department of Radiology and Medical USA
VA, USA David T. Powell MD
Neuroradiology Fellow, Department of Radiology,
Xinli Du MD PhD University of Texas Southwestern Medical Center, Dallas, TX,
Staff Physician, Department of Neurology, Hunter Holmes USA
McGuire Veterans Affair Medical Center, Richmond, VA, USA
Michael Reardon MD
Rajkamal S. Khangura MD Assistant Professor, Division of Neuroradiology, Department
Resident Physician, Department of Radiology, Virginia of Radiology and Medical Imaging, University of Virginia
Commonwealth University Medical Center, Richmond, VA, Health System, Charlottesville, VA, USA
USA
Catherine Shaeffer MD
Carlos Leiva-Salinas MD Resident Physician, Department of Radiology and Medical
Clinical Instructor, Division of Neuroradiology, Imaging, University of Virginia Health System, Charlottesville,
Department of Radiology and Medical Imaging, VA, USA
x
Foreword
To whom does Emergency Radiology belong? Radiology spe- Emergency Neuroradiology: A Case-Based Approach is the
cialists, radiology generalists, emergency physicians? The truth title of the book you have in your hands – and its name implies
is that it probably “belongs” to all, depending on where one expert knowledge, easily delivered and digestible. Beautiful
works. At most teaching hospitals, trainees initially interpret all images are accompanied by pithy text and to-the-point infor-
studies, which are later reviewed by specialists, while at other mation. Cases are grouped into large and general sections, mak-
hospital generalists (sometimes called night hawks) do it all, ing them easy to find in a hurry. Beyond the usual emergent situ-
and at even smaller community hospitals the emergency room ations, some cases such as “sinus pericranii” may be useful when
personnel may be in charge of rendering the initial imaging facing this entity as an incidental finding in the ED (such as a
interpretations for acutely sick patients. Regardless of who does patient presenting with a bump on the head). CT abounds but
the initial interpretation of these studies, our knowledge about MRI, which is increasingly used in emergencies, is also amply
how to interpret them should come from the best and most represented. We neuroradiologists know that often head emer-
experienced specialists, and that is where this case-based book gencies are accompanied by neck and spine ones. Thus, very
by Drs. Tang, Mukherjee, and Wintermark makes its mark. complete sections on head & neck and spine emergencies are
Why another case-based book? The way we teach and learn also included.
has drastically changed in the last 15 years. While most radi- There is no question that this book will be frequently used
ologists of my generation learned by reading (prose) books, in the emergency department, where it belongs – but it should
younger individuals no longer do it this way. Millennials and also remind many of us as why we embark on Neuroradiology:
Generation Z obtain and process knowledge differently, that is, it is fun. An expert perusing this book will find its illustrations
their knowledge is no longer built in blocks but in a pyramidal beautiful and enjoyable, and will still be able to learn something
fashion by laying a foundation and then building on top of it via from it.
the process of accumulating small information bites, synthesiz-
ing them, coordinating them, and ending with a good rounded Mauricio Castillo MD FACR
fund of knowledge (or a tall pyramid!). While I learned mostly University of North Carolina School of Medicine,
from text and imagination, newer generations learn mostly in a Chapel Hill, NC, USA
pictorial fashion, which is perhaps easier and more lasting. This
new book does the latter.
xi
Preface
Diseases affecting the brain, head and neck, and spine are preva- includes over 150 selected cases, which are divided into three
lent in the emergency setting. Traumatic, vascular, or infectious sections and eighteen chapters, and cover the common as well
events are more likely to present acutely, while exacerbations as some uncommon emergent cases in brain, head and neck,
or complications of underlying chronic diseases such as inflam- and spine neuroradiology. Each case vignette consists of a short
matory, neoplastic, metabolic, degenerative, or even congenital history, images, findings, and diagnosis, followed by focused
processes can also present in an urgent fashion and may pose a discussion of differential diagnosis and key points, and supple-
significant diagnostic challenge to clinicians and radiologists. mented with a short list of suggested readings. Readers can use
Therefore, there is a strong need to adequately prepare radi- it either as a primary learning tool or as a quick on-call reference
ologists, especially our trainees, for on-call neuroradiological guide.
emergencies. We would like to thank our colleagues at the Virginia Com-
Although many excellent, comprehensive neuroradiology monwealth University and University of Virginia Medical Cen-
textbooks are available, we feel that the most effective way of ters for their contributions. A number of residents and fellows
preparing for neuroradiological emergencies is through a con- have participated in writing up the cases and providing valuable
centrated series of case reviews. Our aim in this book is to feedback. We would also like to thank the editorial staff at Cam-
develop a teaching curriculum specific for emergency neuro- bridge University Press for making this book possible, and, last
radiology and to supplement the large-volume reference books but not least, Dr. Mauricio Castillo for writing a foreword to the
with a concise book, using a case-based, picture-rich format. It book.
xiii
Abbreviations
ACA anterior cerebral artery DWI diffusion-weighted imaging

ACE angiotensin-converting enzyme ECA external carotid artery
A-comm anterior communicating artery EDH epidural hematoma
ADC apparent diffusion coefficient EOM extraocular muscle
ADEM acute demyelinating encephalomyelitis EPM extrapontine myelinolysis
AIDP acute inflammatory demyelinating ESR erythrocyte sedimentation rate
polyneuropathy ELST endolymphatic sac tumor
AIDS acquired immune deficiency syndrome FDG fludeoxyglucose (18F)
ALS amyotrophic lateral sclerosis FLAIR fluid-attenuated inversion recovery
AOD atlanto-occipital dislocation GBM glioblastoma multiforme
AP anteroposterior GBS Guillain–Barré syndrome
AQP aquaporin GC gliomatosis cerebri
AS ankylosing spondylitis GRE gradient-recalled echo
ATRT atypical teratoid–rhabdoid tumor HAART highly active antiretroviral therapy
AV arteriovenous HIV human immunodeficiency virus
AVF arteriovenous fistula HPV human papilloma virus
AVM arteriovenous malformation HSV herpes simplex virus
CAA cerebral amyloid angiopathy HUS hemolytic uremic syndrome
CBF cerebral blood flow IAC internal auditory canal
CBV cerebral blood volume ICA internal cerebral artery
CCF carotid–cavernous fistula ICP intracranial pressure
CECT contrast-enhanced computed tomography ICV internal cerebral vein
CJD Creutzfeldt–Jakob disease IIH idiopathic intracranial hypertension
CM cavernous malformation IRIS immune reconstitution inflammatory syndrome
CMV cytomegalovirus JNA juvenile nasopharyngeal angiofibroma
CNS central nervous system LCH Langerhans cell histiocytosis
CPM central pontine myelinolysis LNB Lyme neuroborreliosis
CPPD calcium pyrophosphate deposition MCA middle cerebral artery
CRP C-reactive protein MDCT multiple-detector computed tomography
CSF cerebrospinal fluid MIP maximum-intensity projection
CTA computed tomography angiography MPRAGE magnetization prepared rapid gradient echo
CTV computed tomography venography MRA magnetic resonance angiography
CVD cortical venous drainage MRI magnetic resonance imaging
DAI diffuse axonal inury MRV magnetic resonance venography
DAVF dural arteriovenous fistula MS multiple sclerosis
DCI delayed cerebral ischemia MSUD maple-syrup urine disease
DIC disseminated intravascular coagulation MTS mesial temporal sclerosis
DNET dysembryoplastic neuroepithelial tumor MTT mean transit time
DNM descending necrotizing mediastinitis NAA N-acetylaspartate
DSA digital subtraction angiography NBS neuro-Behçet’s syndrome
DVA developmental venous anomaly NCC neurocysticercosis
xiv
Abbreviations
NECT non-enhanced computed tomography RCVS reversible cerebral vasoconstriction syndrome

NMO neuromyelitis optica SAH subarachnoid hemorrhage
NOE naso-orbito-ethmoidal SCA superior cerebellar artery
PACNS primary angiitis of central nervous system SCC squamous cell carcinoma
PADI posterior atlantodental interval SLE systemic lupus erythematosus
PCA posterior cerebral artery STIR short tau inversion recovery
P-comm posterior communicating artery TB tuberculosis
PCNSL primary CNS lymphoma TIA transient ischemic attack
PCR polymerase chain reaction TMA thrombotic microangiopathy
PET positron emission tomography TMJ temporomandibular joint
PHPV persistent hyperplastic primary vitreous tPA tissue plasminogen activator
PICA posterior inferior cerebellar artery TOF time of flight
PML progressive multifocal leukoencephalopathy TTD time to drain
PNET primitive neuroectodermal tumor TTP thrombotic thrombocytopenic purpura
PRES posterior reversible encephalopathy syndrome VHL von Hippel–Lindau
PTA peritonsillar abscess WD Wallerian degeneration
rCBV relative cerebral blood volume ZMC zygomaticomaxillary complex
xv
Section 1 Brain
Chapter
Cerebrovascular diseases
1 Yang Tang, Xinli Du, Sugoto Mukherjee, and Max Wintermark
Case 1.1
Figure 1.1.1 (patient 1) Figure 1.1.2 (patient 1)
1
Section 1: Brain
Figure 1.1.5 (patient 1)

2
Chapter 1: Cerebrovascular diseases
3
Section 1: Brain
Figures 1.1.7–1.1.9 CT perfusion images show moderately prolonged TTD

(Fig. 1.1.7), decreased CBF (Fig. 1.1.8), and preserved CBV (Fig. 1.1.9) in the entire
left MCA territory (circle), consistent with tissue at ischemic risk. This patient
underwent emergent mechanical thrombectomy and had good clinical
outcome.
Patient 3
Figure 1.1.10 Axial CTA of the head shows an intraluminal thrombus in the
distal supraclinoid right ICA with abrupt cutoff (white arrow). The entire right
MCA territory shows markedly diminished vascularity.
Figures 1.1.11–1.1.13 CT perfusion images show markedly prolonged TTD

(Fig. 1.1.11), decreased CBF (Fig. 1.1.12), and CBV (Fig. 1.1.13) in the entire right
MCA territory (circle), consistent with core infarction. This patient died in a few
days from massive cerebral edema leading to herniation.
Diagnosis
Patient 1: left MCA thrombus with benign oligemia in the
left MCA territory.
Patient 2: left ICA thrombus with tissue at ischemic risk in
the left MCA territory.
Patient 3: right ICA thrombus with large right MCA
infarction.
Differential diagnosis
r None.
History
Patient 1: 55-year-old man presents with acute onset of
right-sided weakness.
Key points
r There are three major ischemic stroke subtypes: large-
Patient 2: 43-year-old man with recent cardiac surgery artery atherosclerotic infarctions, cardioembolic
presents with sudden right-sided weakness and aphasia. infarctions, and lacunar infarctions.
Patient 3: 62-year-old woman with history of atrial r The concepts of core, penumbra, and benign oligemia:
fibrillation develops sudden left-sided weakness. b The infarcted tissue with irreversible cell death is
defined as the “core.”
Findings b Functionally impaired yet still viable and salvageable
tissue surrounding the core is commonly termed
Patient 1
“penumbra” or “tissue at risk.” The penumbra will
Figure 1.1.1 NECT of the head shows a focal hyperdensity in the distal M1 progress to infarction unless timely reperfusion occurs
segment of left MCA (arrow), suggestive of an intraluminal thrombus. No large
infarction is identified. either spontaneously or as a result of treatment, and its
fate largely depends on the severity/duration of
Figure 1.1.2 Axial CTA of the head confirms an intraluminal thrombus with ischemia and the availability of collateral circulation.
acute cutoff of distal M1 segment (black arrow) but good flow reconstitution in b Further away from the ischemic core, there is tissue
the M2 branches (white arrows).
with “benign oligemia,” which is hypoperfused yet
Figures 1.1.3–1.1.5 CT perfusion images show mildly prolonged time to functionally intact. This tissue will survive even
drain (TTD) (Fig. 1.1.3), preserved cerebral blood flow (CBF) (Fig. 1.1.4), and
preserved cerebral blood volume (CBV) (Fig. 1.1.5) in the left parietal/temporal
if reperfusion does not occur. It is important to
region (circle) of the posterior left MCA distribution, consistent with benign distinguish benign oligemia from true penumbra to
oligemia. No large ischemic infarct or tissue at risk is identified. Patient received avoid the overestimation of tissue at risk.
intravenous tPA and had significant clinical improvement. Follow-up head CT
in 24 hours (not shown) did not show large territory infarction or hemorrhage. r Acute stroke imaging:
b NECT is the initial modality, to exclude intracranial
hemorrhage and other etiologies that may simulate
Patient 2
acute stroke, such as mass or hydrocephalus. Although
Figure 1.1.6 Coronal CTA of the head and neck shows abrupt occlusion of
the proximal left ICA (curved white arrow) due to an intraluminal thrombus.
not sensitive, CT signs of early ischemia include
Diminished flow is observed in the left cerebral hemisphere compared to the hyperdense vessels, loss of gray–white differentiation,
right. parenchymal hypodensity, and gyral swelling.
4
Hypodensity over one-third of MCA territory is Table 1.1.1 Interpretation of CT perfusion in ischemic stroke
commonly considered a contraindication for MTT/TTD CBF CBV
thrombolysis. Alberta Stroke Program Early CT Score Benign oligemia ↑ normal normal or ↑
(ASPECTS) has been developed to standardize the
Penumbra ↑↑ ↓ Normal or ↓
detection and reporting of the extent of early ischemia
Core ↑↑↑ ↓↓ ↓↓
using a 10-point scale.
b CTA is the modality of choice to evaluate the CBF, cerebral blood flow; CBV, cerebral blood volume; MTT, mean transit
time; TTD, time to drain.
extracranial/intracranial vascular anatomy and detect ↑, mildly elevated; ↑↑, moderately elevated; ↑↑↑, markedly elevated.
the site of vascular occlusion. In addition, it can
provide valuable information on the status of
collateral circulation, which is a major factor b CT perfusion is an attractive alternative because of its
determining the rate of core expansion and the wider availability, fewer contraindications, and
patient’s outcome. potential for quantitative assessment. It should be
b Currently, there is considerable debate regarding the noted that there is a critical need for standardization, as
utility of penumbra imaging beyond initial NECT and interoperator, intraoperator, and intervendor software
CTA. A recent study found no benefit of penumbra differences can greatly influence the results, and the
imaging in selecting patients who would differentially published parameter thresholds are not easily
benefit from endovascular therapy. Regardless, transferrable between institutions.
advanced imaging with either CT perfusion or MRI/ b In our institution, CT perfusion studies are interpreted
MR perfusion has been a focus of intense research with the following general guidelines, as illustrated in
investigation and has been adopted by many centers as the cases and Table 1.1.1. Cerebral blood volume
part of their stroke imaging protocol. (CBV): the marker for core infarct. Cerebral blood flow
b MRI with diffusion-weighted imaging is the standard (CBF): the marker for penumbra. Time to drain (TTD)
of infarct core characterization. An infarct volume of and mean transit time (MTT): the markers for oligemia
less than 70 ml has been suggested as a threshold for or hypoperfusion.
selecting patients for endovascular treatment, as r Treatment: the utility of intravenous tPA thrombolysis
patients with higher infarct volumes have unfavorable within the first 4.5 hours of stroke onset has been well
outcomes regardless of treatment. MR perfusion established, while the risk of intracranial hemorrhage
performed concurrently can help estimate the size associated with intravenous tPA beyond the 4.5-hour
of penumbra. However, the limited availability of MRI window outweighs the benefit. Endovascular treatment
scanners and individual safety contraindications using intra-arterial thrombolysis or mechanical
significantly restrict its use in the emergent embolectomy beyond the 4.5-hour window is still
setting. investigational.
Further reading
Kidwell CS, Jahan R, Gornbein J, Alger JR, Pexman JH, Barber PA, Hill MD, Sevick RJ, Yoo AJ, Verduzco LA, Schaefer PW, Hirsch
Nenov V, Ajani Z, et al. A trial of imaging Demchuk AM, Hudon ME, et al. Use of JA, Rabinov JD, Gonzalez RG. MRI-based
selection and endovascular treatment for the Alberta Stroke Program Early CT selection for intra-arterial stroke therapy:
ischemic stroke. N Engl J Med 2013; Score (ASPECTS) for assessing CT scans value of pretreatment diffusion-weighted
368(10): 914–923. in patients with acute stroke. AJNR Am J imaging lesion volume in selecting
Kucinski T, Naumann D, Knab R, Schoder Neuroradiol 2001; 22(8): 1534–1542. patients with acute stroke who will
V, Wegener S, Fiehler J, et al. Tissue Souza LC, Yoo AJ, Chaudhry ZA, Payabvash benefit from early recanalization. Stroke
at risk is overestimated in perfusion- S, Kemmling A, Schaefer PW, et al. 2009; 40(6): 2046–2054.
weighted imaging: MR imaging in Malignant CTA collateral profile is highly Zhu G, Michel P, Zhang W, Wintermark M.
acute stroke patients without vessel specific for large admission DWI infarct Standardization of stroke perfusion CT
recanalization. AJNR Am J Neuroradiol core and poor outcome in acute stroke. for reperfusion therapy. Transl Stroke Res
2005; 26(4): 815–819. AJNR Am J Neuroradiol 2012; 33(7): 2012; 3(2): 221–227.
1331–1336.
5
Section 1: Brain
Case 1.2
Figure 1.2.1 Figure 1.2.2
6
Diagnosis
Distal basilar thrombosis.
r The diagnosis is usually straightforward, with
characteristic vascular distribution and filling defects in
the basilar top on CTA/MRA. Occasionally, if only thalami
are involved, the following entities should be considered:
b Artery of Percheron infarction.
b Venous thrombosis of internal cerebral veins.
b Hypoxic–ischemic encephalopathy.
b Wernicke’s encephalopathy.
Key points
r “Top of basilar” syndrome refers to thromboembolic
occlusion of the distal basilar artery and its terminal
branches including posterior cerebral arteries and superior
cerebellar arteries, leading to ischemic infarction of the
above vascular territories, including the thalami, occipital
lobes, rostral midbrain, and superior cerebellum.
Figure 1.2.5 r Clinically, patients present with acute visual, oculomotor,
and behavioral symptoms, while motor symptoms may not
be obvious.
r Imaging findings:
History
50-year-old man with history of atrial fibrillation is found b CT: may be normal in the early stage and show
unresponsive for an unknown duration. hypodensity/edema if infarction occurs. Hyperdense
embolus/thrombus in the distal basilar artery may be
seen in some cases.
Findings b CTA/MRA/DSA: thrombus/embolus the distal basilar
Figure 1.2.1 NECT shows areas of low attenuation in the right thalamus and artery. It is also important to assess the patency of PCA
occipital lobe, consistent with acute infarction.
and SCA.
b CT or MRI perfusion: may add further information on
Figure 1.2.2 Coronal CTA of the head shows a filling defect in the distal
basilar artery (block arrow), consistent with a thrombus. There is occlusion of the status of affected vascular territories (infarction
the origins of the right posterior cerebral artery (PCA) and superior cerebellar versus viable tissue at ischemic risk), and select patients
artery (SCA). The proximal left PCA (curved arrow) and SCA (arrow) are
patent. for endovascular intervention.
b MRI: diffusion restriction if infarction occurs.
Figures 1.2.3–1.2.5 CT perfusion images demonstrate prolonged TTD r It is associated with poor prognosis, frequently leading to
(Fig. 1.2.3), decreased CBF (Fig. 1.2.4), and decreased CBV (Fig. 1.2.5) in the
right thalamus and bilateral occipital lobes, right greater than left, consistent death or “locked in” syndrome.
with ischemic infarction in the bilateral PCA distributions. Note that the left r Treatment: endovascular recanalization is the only
PCA infarction is not yet obvious on NECT. Additional images (not shown)
also revealed infarctions of superior cerebellum in the SCA treatment that may improve clinical outcomes, and it must
distribution. be performed early.
Further reading
Barkhof F, Valk J. “Top of the basilar” Davis SM, Donnan GA. Basilar artery
syndrome: a comparison of clinical and thrombosis: recanalization is the key.
MR findings. Neuroradiology 1988; 30(4): Stroke 2006; 37(9): 2440.
293–298.
7
Section 1: Brain
Case 1.3
8
r Atherosclerosis: commonly at carotid bifurcation or of

History vertebral origin. In comparison, carotid artery dissection
44-year-old man presents with sudden right-arm weakness typically involves the postbulbar segment at the skull base,
and speech difficulty. and vertebral dissection tends to occur at the C1–2 and
C6–7 levels or at the intradural segment. A long segment of
Findings tapered narrowing favors dissections, whereas focal
narrowing with associated calcification is typical for
Figure 1.3.1 Axial DWI shows diffusion restriction in left parietal lobe,
consistent with acute ischemic infarction. atherosclerosis. Other findings such as intimal flap or
pseudoaneurysm are seen only with dissection.
Figures 1.3.2, 1.3.3 Axial T2 (Fig. 1.3.2) and axial T1 with fat saturation r Fibromuscular dysplasia: typically beaded appearance.
(Fig. 1.3.3) demonstrate a crescent of T2 and T1 hyperintensity (arrows)
surrounding a diminished left ICA flow void (curved arrow) at the skull base, However, arterial dissection is a common complication of
consistent with dissection with an intramural hematoma. fibromuscular dysplasia.
Figure 1.3.4 TOF MRA source image again demonstrates the eccentric
intramural hematoma (white arrow), which is hyperintense compared to
adjacent muscles but less so than the flow-related enhancement of residual left
Key points
ICA lumen (curved arrow). r Dissection is a common cause of ischemic stroke in a
young patient. It refers to separation of the arterial wall
Figure 1.3.5 TOF MRA MIP image shows a long segment of tapered layers by intramural hematoma, which may result from
narrowing (arrows) of distal left ICA with an associated pseudoaneurysm
(curved arrow). intimal tear or rupture of the vasa vasorum. While
subintimal dissection typically causes luminal stenosis/
Figure 1.3.6 DSA of left ICA prior to carotid stenting confirms the luminal occlusion, subadventitial dissection may result in
narrowing (arrows) and pseudoaneurysm (curved arrow).
dissecting aneurysm and vessel rupture contained by
thrombus, leading to pseudoaneurysm. Extracranial
dissection is more common than intracranial dissection.
Diagnosis r Besides traumatic injury, many inciting factors such as
Spontaneous extracranial carotid dissection. sports, chiropractor manipulation, sneezing and coughing,
etc. have been associated with spontaneous dissection.
Differential diagnosis In addition, a number of patients have underlying
r Arterial thrombosis: once an artery is thombosed, it is arteriopathies such as fibrous dysplasia, Marfan syndrome,
difficult to determine whether underlying dissection is cystic medial necrosis, and type IV Ehlers–Danlos
present. syndrome.
9
Section 1: Brain
r Clinical presentations: ischemic stroke or TIA, due either b MRI/MRA is the modality of choice to assess the
to an embolic or to a hemodynamic phenomenon, is the cerebral infarction and directly image the intramural
most common presentation. Intracranial dissection may hematoma, which eccentrically or circumferentially
occasionally result in subarachnoid hemorrhage. Local surrounds a normal or narrowed flow void (crescent or
symptoms such as headache and neck pain are common. target sign), widens the external diameter of the artery,
Carotid dissection may result in Horner’s syndrome due and is typically hyperintense on fat-saturated T1 and
to oculosympathetic paresis. Other symptoms include T2 in the subacute phase (in the acute phase, it can be
tinnitus and cranial neuropathies, etc. isointense or slightly hyperintense on T1 and T2
r Imaging: sequences).
b DSA has been largely replaced by noninvasive CT or b CTA has the same sensitivity as MRI/MRA, and a CTA
MRA. Angiographic findings include string sign, source image can demonstrate hyperdense intramural
tapered stenosis/occlusion, flame-shaped occlusion, hematoma.
intimal flap, or pseudoaneurysm.
Further reading
Provenzale JM. Dissection of the internal Redekop GJ. Extracranial carotid and Schievink WI. Spontaneous dissection of the
carotid and vertebral arteries: imaging vertebral artery dissection: a review. Can carotid and vertebral arteries. N Engl J
features. AJR Am J Roentgenol 1995; J Neurol Sci 2008; 35(2): 146–152. Med 2001; 344(12): 898–906.
165(5): 1099–1104.
10
Case 1.4
11
Section 1: Brain
12
r Creutzfeldt–Jakob disease.
History r Large-territory acute ischemia.
Patient 1: 65-year-old man is resuscitated after a cardiac
arrest.
Patient 2: 30-year-old man remains unresponsive 7 days
Key points
r Adult global hypoxic–ischemic brain injuries are most
after a motor vehicle crash that resulted in severe thoracic
injuries. often caused by insults such as cardiac arrest, vascular
catastrophe, drowning, or asphyxiation.
r Mild insults are manifested by border-zone ischemic
infarcts. In severe insults, gray-matter structures including
Findings the basal ganglia, thalami, cerebral cortex, cerebellum, and
Patient 1 hippocampi are preferentially affected due to high energy
Figures 1.4.1–1.4.4 Axial DWI (Figs. 1.4.1, 1.4.2) and ADC (Figs. 1.4.3, 1.4.4)
demands.
demonstrate diffusion restriction of the bilateral basal ganglia, cerebral and r In adults, CT is usually the first imaging modality, which
cerebellar hemispheres. may show diffuse cerebral edema with effacement of the
cortical sulci, decreased cortical attenuation, loss of normal
Figures 1.4.5, 1.4.6 Axial FLAIR images show associated cortical swelling
and hyperintensity. gray–white differentiation, and ill definition of central
gray-matter structures (basal ganglia and thalami).
Sometimes, an appearance of pseudo-subarachnoid
Patient 2 hemorrhage may be seen on CT, likely due to a
Figures 1.4.7, 1.4.8 Axial NECT demonstrates extensive cerebral edema combination of cortical edema, displacement of CSF, and
with sulcal/cisternal effacement and loss of normal gray–white matter
differentiation. Note the linear hyperdensities within the cortical sulci, distention of the superficial vasculature.
representing blood vessels, which should not be mistaken for subarachnoid r If CT is negative or inconclusive, MRI is the modality of
hemorrhage. choice for confirmation and assessment of the extent of
injury.
b DWI/ADC: diffusion restriction becomes positive
Diagnosis within the first 24 hours. The DWI signal abnormality
Hypoxic–ischemic injury.
normally peaks at approximately 3 days and becomes
pseudonormalized after the first week.
b T1/T2/FLAIR: often normal or only have subtle
Differential diagnosis abnormalities within the first 24 hours. After 24 hours,
r Toxic encephalopathy such as carbon monoxide or T1 hypointensity, T2/FLAIR hyperintensity and
methanol intoxication. cortical swelling become more obvious.
r Hypoglycemia. b In the chronic stage, encephalomalacia/laminar
r Acute hepatic encephalopathy. necrosis will develop.
Further reading
Huang BY, Castillo M. Hypoxic–ischemic McKinney AM, Teksam M, Felice R, Casey
brain injury: imaging findings from birth SO, Cranford R, Truwit CL, et al.
to adulthood. Radiographics 2008; 28(2): Diffusion-weighted imaging in the
417–439; quiz 617. setting of diffuse cortical laminar
necrosis and hypoxic–ischemic
encephalopathy. AJNR Am J Neuroradiol
2004; 25(10): 1659–1665.
13
Section 1: Brain
Case 1.5
14
r Additional considerations for bilateral basal ganglia
abnormalities in neonates include:
b Mitochondrial disease.
b Other inborn errors of metabolism.
b Kernicterus.
b Hypoglycemia.
Key points
r Hypoxic–ischemic injury (HII) to the brain is a major
cause of mortality and morbidity in the perinatal period.
Obstetric complications such as cardiac arrest, placental
abruption, and umbilical cord or uterine ruptures are the
most common risk factors.
r HII in neonates can be divided into profound (severe) and
partial (mild to moderate) injuries.
r Profound injuries primarily involve the deep gray matter
(basal ganglia, ventrolateral thalami, posterior limb of
internal capsules, and dorsal brainstem) and occasionally
the perirolandic cortex, since these areas are actively
Figure 1.5.5
myelinating and most susceptible to energy depletion.
r In partial injuries, the cortical/subcortical watershed zones
are usually more affected, while the central structures
History mentioned above are relatively spared due to
Preterm infant (34 weeks gestational age), delivered autoregulation/blood shunting. In preterm infants, partial
emergently after placental abruption, develops convulsions injuries frequently lead to germinal matrix hemorrhage
shortly after birth. Apgar score is 0 at 1 minute and 0 at and periventricular leukomalacia.
5 minutes. r Since most of these patients are hemodynamically
unstable, transcranial ultrasound, which can be easily
performed at bedside, is usually the first-line imaging
Findings modality. Germinal matrix hemorrhage can be identified
Figures 1.5.1, 1.5.2 Coronal (Fig. 1.5.1) and sagittal (Fig. 1.5.2) with ultrasound and divided into four grades, which
transfontanelle ultrasound performed on postnatal day 1 shows increased correlate with prognosis:
echogenicity in the left caudothalamic groove (arrow) without intraventricular
extension, consistent with grade I germinal matrix hemorrhage. b Grade I: Hemorrhage confined to the germinal matrix.
b Grade II: Intraventricular hemorrhagic extension
Figures 1.5.3, 1.5.4 MRI on postnatal day 12. Axial T1 (Fig. 1.5.3) shows
increased T1 signal in the bilateral ventrolateral thalami (black arrows) and without hydrocephalus.
posterior putamina (white arrows). Axial T2 (Fig. 1.5.4) demonstrates b Grade III: Intraventricular extension with
corresponding hypointense signal in the ventrolateral thalami (black arrows), hydrocephalus.
while posterior putamina have nearly normal signal. Also note the extensive T2 b Grade IV: Parenchymal hemorrhage secondary to
hyperintensity in the white matter, especially posteriorly. Early subacute
intraventricular hemorrhage is noted, which is T1 hyperintense and T2 venous infarction.
hypointense, along with mild ventriculomegaly, consistent with progression to r The deep structures and cerebral convexities cannot be well
grade III intraventricular hemorrhage.
assessed with ultrasound, and MRI is usually the next
Figure 1.5.5 Axial T1 MRI performed 8 weeks after birth demonstrates T1 study to evaluate suspected parenchymal injuries, with the
hyperintensity in the ventrolateral thalami (black arrows), posterior limb of
internal capsules (white arrows), and right posterior putamen (block arrow).
advantage of lack of ionizing radiation compared to CT.
The MRI findings are variable and largely depend on the
timing of the study.
Diagnosis b DWI: diffusion restriction 1–5 days (usually more
Profound neonatal hypoxic–ischemic injury and grade III apparent on ADC maps). After one week, the diffusion
intraventricular hemorrhage. signal may become pseudonormalized.
15
Section 1: Brain
b Proton MR spectroscopy: increased lactate and the first 24 hours. After day 2, T1 shortening develops
decreased NAA in the central gray matter and centrum and persists for several months. T2 is typically
semiovale. hyperintense during the first week due to edema, then
b T1/T2: T1 and T2 images are most useful after the becomes hypointense after the first week. In the chronic
end of the first week, when DWI becomes pseudo- stage, laminar necrosis/encephalomalacia will ensue.
normalized. T1 and T2 are frequently normal during
Further reading
Huang BY, Castillo M. Hypoxic–ischemic
brain injury: imaging findings from birth
to adulthood. Radiographics 2008; 28(2):
417–439; quiz 617.
16
Case 1.6
17
Section 1: Brain
18
r Other causes of intraparenchymal hemorrhage such as

History
from tumor, AVM, or amyloid angiopathy.
57-year-old presents with sudden onset of left-sided
amaurosis fugax and word-finding difficulty.
Findings Key points

r This case illustrates the typical imaging appearance
Figure 1.6.1 Axial T2 MRI shows loss of normal flow void of the left ICA
(arrow). No infarction is seen on additional images. of carotid dissection and hyperacute hyperperfusion
hemorrhage after a carotid stenting procedure. See
Figure 1.6.2 Sagittal MIP image from the neck CTA shows a long segment of Case 1.3 for a discussion of carotid dissection.
tapered narrowing of cervical left ICA from approximately 2 cm distal to the r Hyperperfusion syndrome is a rare complication of
bifurcation to the skull base (arrows). The configuration, location, and lack of
calcification are suggestive of dissection. recanalization procedures that can occur after either
carotid stenting or endarterectomy. Two different types of
Figure 1.6.3 Axial CTA source image demonstrates slightly hyperdense hyperperfusion injuries have been reported, both of which
intramural hematoma (arrows) surrounding the severely compressed lumen.
occur to the hemisphere that the recanalization procedure
Figures 1.6.4–1.6.6 CT perfusion images demonstrate abnormal perfusion has been performed on.
of the left MCA territory (circle), with marked prolongation of time to drain r The more common type usually develops between the fifth
(Fig. 1.6.4), decreased cerebral blood flow (Fig. 1.6.5), and preserved cerebral
blood volume (Fig. 1.6.6), consistent with tissue at significant ischemic risk. and seventh days after the procedure (delayed type). It is
believed to be due to impaired cerebral autoregulation
Figure 1.6.7 Lateral DSA of left ICA performed prior to the carotid stenting from vascular stenosis. Patients typically present with
confirms the dissection, with a long segment of cervical ICA stenosis tapering ipsilateral headache/retro-orbital pain, focal neurological
to near occlusion at the skull base (white arrow), compressed by an intramural
hematoma and a false lumen (black arrow). deficits, seizure, nausea, vomiting, or other signs of
increased intracranial pressure. CT or MRI can show
Figure 1.6.8 The patient underwent successful carotid stenting, but reversible vasogenic edema. CT or MR perfusion studies
developed mental status change and right-side paralysis several hours after the
completion of the procedure. NECT shows intraparenchymal hemorrhage in would reveal a hyperperfusion pattern, with decreased
the left temporal lobe with subarachnoid extension. Note early left mean transit time and increased cerebral blood flow and
transtentorial hernation with effacement of the basilar cistern. The patient volume, opposite to the ischemic pattern. Intracranial
underwent emergent decompressive craniectomy and eventually made a
good clinical recovery. hemorrhage can occur but is not an obligate component
for this type.
r A second type of hyperperfusion injury, as shown in the
Diagnosis current case, typically occurs within a few hours after the
Reperfusion hemorrhage, after carotid stenting for procedure (hyperacute type). It is always associated with
spontaneous carotid dissection. intracranial hemorrhage and has a worse prognosis. The
pathophysiology is postulated to be due to rupture of small
arteries that are exposed to suddenly normalized perfusion
Differential diagnosis pressure after recanalization of a high-grade stenosis.
r Hypertensive hemorrhage. r Treatment: strict blood pressure control during and after
r Hemorrhagic transformation of ischemic infarction. the procedure.
Further reading
Buhk JH, Cepek L, Knauth M. Hyperacute Karapanayiotides T, Meuli R, Devuyst G,
intracerebral hemorrhage complicating Piechowski-Jozwiak B, Dewarrat A,
carotid stenting should be distinguished Ruchat P, et al. Postcarotid
from hyperperfusion syndrome. AJNR endarterectomy hyperperfusion or
Am J Neuroradiol 2006; 27(7): 1508–1513. reperfusion syndrome. Stroke 2005;
36(1): 21–26.
19
Section 1: Brain
Case 1.7
20
History Diagnosis
49-year-old patient with history of myelodysplastic disorder Thrombotic microangiopathy (TMA).
status post stem-cell transplant and chemotherapy, develops
pancytopenia, neutropenic fever, and altered mental status.
r Vasculitis.
Findings r Venous thrombosis.
Figure 1.7.1 Initial axial NECT shows a small amount of intraparenchymal r Embolic infarctions.
hemorrhage in the left frontal operculum (white arrow). There is also subtle low r Inherited hypercoagulable states, such as protein C
attenuation in the right thalamus (white curved arrow), suggestive of infarction.
Hyperdensities are noted of the bilateral internal cerebral veins (ICV) (black deficiency, protein S deficiency, factor V Leiden mutation,
arrow), consistent with venous thrombosis. Subsequent MRV confirms ICV antiphospholipid antibody syndrome, etc.
thrombosis, but other deep cerebral veins and dural sinuses (not shown) are
patent.
Figure 1.7.2 MIP image of 3D time of flight brain MRA demonstrates signal
loss of left PCA (arrow), consistent with occlusion or high-grade stenosis. Other
Key points
major intracranial arteries are patent. r This patient has developed ischemic and hemorrhagic
infarctions in multiple vascular territories. The basal
Figures 1.7.3, 1.7.4 Axial DWI images show multifocal areas of diffusion
restriction in the bilateral cerebral hemispheres, cerebellum, and thalami, ganglia/thalamic infarctions are likely due to ICV
consistent with ischemic infarctions. The distribution of infarctions does not thrombosis, and infarction in the left occipital lobe is likely
conform to a single arterial or venous territory. from occlusion of the left PCA. However, other multifocal
areas of cerebral lobar and cerebellar infarctions cannot be
Figures 1.7.5, 1.7.6 Axial T2 images show hypointense foci in the posterior
left frontal lobe (Fig. 1.7.5) and left frontal operculum (Fig. 1.7.6, white arrow), explained by large arterial or venous occlusion on vascular
consistent with hemorrhagic infarcts. In Fig. 1.7.6, note the multifocal areas of imaging and are presumably due to thrombotic
T2 hyperintensity in bilateral cerebral hemispheres, caudate nuclei/putamina, microangiopathy (TMA).
and thalami, consistent with infarctions as demonstrated in the DWI images.
Also note the hypointense signal within the bilateral internal cerebral veins, in
r A number of disorders associated with endothelial injury
keeping with thrombosis (black arrow). can eventually lead to platelet aggregation, thrombosis,
21
Section 1: Brain
and vascular occlusion. Collectively termed TMA, these r A spectrum of neuroimaging findings are related to TMA,
disorders include thrombotic thrombocytopenic purpura including cortical and subcortical ischemic and
(TTP), hemolytic uremic syndrome (HUS), disseminated hemorrhagic infarctions, posterior reversible
intravascular coagulation (DIC), and malignant encephalopathy, and venous thrombosis. The incidence of
hypertension. Clinically, these disorders may manifest as TMA varies with the underlying diagnosis, with the most
end-organ ischemia and/or hemorrhage. common diagnosis being DIC.
Further reading
Ellchuk TN, Shah LM, Hewlett RH, Osborn
AG. Suspicious neuroimaging pattern of
thrombotic microangiopathy. AJNR Am J
Neuroradiol 2011; 32(4): 734–738.
22
Case 1.8
23
Section 1: Brain
History neurological symptoms, and transient, multifocal

vasoconstriction of cerebral arteries lasting several weeks
50-year-old woman with a history of depression develops
to months.
severe headache, nausea, and visual disturbance for 3 days. r This disorder has been described throughout the medical
literature in the past with various names including
Findings Call–Fleming syndrome, benign angiopathy of the central
Figure 1.8.1 Axial NECT shows a small intraparenchymal hemorrhage in the nervous system, migranous vasospasm, drug-induced
right occipital lobe without mass effect. vasculitis, postpartum angiopathy, etc. It typically affects
young to middle-aged women. Although pathophysiology
Figures 1.8.2, 1.8.3 Head CTA coronal (Fig. 1.8.2) and sagittal (Fig. 1.8.3) MIP
images demonstrate multifocal stenosis of right PCA (arrows). Left PCA also is still poorly understood, commonly recognized triggering
shows mild luminal irregularity to a lesser extent (Fig. 1.8.2). factors include sympathomimetic or vasoactive agents,
peripartum, strenuous exercise, sexual activity, and
Figure 1.8.4 Head CTA sagittal MIP image shows short segments of stenosis
in the bilateral distal ACA branches (arrows).
excessive alcohol drinking.
r On CT or MRI imaging, patients with RCVS may present
Repeated CTA in three months (not shown) showed complete resolution of with isolated cortical subarachnoid hemorrhage (most
the above vascular abnormalities. common), intraparenchymal hemorrhage, ischemic
infarctions, or PRES-like pictures. CSF analysis is mostly
Diagnosis normal. Vascular imaging (DSA, CTA, or MRA) can
show multisegmental arterial stenosis alternating with
Reversible cerebral vasoconstriction syndrome (RCVS).
normal-caliber or dilated vessels in multiple vascular
territories (“string of beads” appearance). Resolution
Differential diagnosis of angiographic abnormalities within 3 months of the
r The main differential diagnosis is vasculitis. Patients with initial episode is considered as the hallmark of RCVS,
vasculitis nearly always have abnormal CSF findings, and distinguishing it from true vasculitis.
their angiographic appearance persists on the follow-up r The clinical recovery depends highly on the extent of brain
imaging. Biopsy may be required for definitive diagnosis. parenchymal damage, which may not always be reversible.
Treatment consists of the removal of triggers, and
Key points calcium-channel blockers in selected patients.
r RCVS is a recently recognized clinical–radiological entity
characterized by severe “thunderclap” headache, focal
Further reading
Ducros A, Boukobza M, Porcher R, Sarov M, Marder CP, Donohue MM, Weinstein JR,
Valade D, Bousser MG. The clinical and Fink KR. Multimodal imaging of
radiological spectrum of reversible reversible cerebral vasoconstriction
cerebral vasoconstriction syndrome. A syndrome: a series of 6 cases. AJNR Am J
prospective series of 67 patients. Brain Neuroradiol 2012; 33(7): 1403–1410.
2007; 130(12): 3091–3101.
24
Case 1.9
25
Section 1: Brain
Figure 1.9.5 Axial FLAIR image demonstrates corresponding FLAIR

hyperintensity.
Diagnosis
Artery of Percheron infarction.
r Top of basilar syndrome from distal basilar embolus:
usually with infarctions of occipital lobes and cerebellum
in the territories of PCA and SCA. See Case 1.2.
r Venous infarction from deep venous thrombosis.
r Infiltrating glioma or lymphoma.
r Metabolic disorders such as Wernicke’s encephalopathy.
Key points
r Artery of Percheron is a rare anatomic variant: a single
thalamoperforating branch arising from the PCA P1
segment supplying the bilateral paramedian
thalamic–mesencephalic junctions.
Figure 1.9.5
r It is estimated to occur in 0.1–0.3% of all ischemic
strokes.
r Clinical presentations: paramedian thalamic syndrome
including altered mental status, vertical gaze palsy, and
History memory impairment.
70-year-old female presents with unresponsiveness. r Imaging: characteristic ischemic patterns of infarctions –
bilateral paramedian thalamic infarctions, with or without
Findings anterior thalamic or rostral midbrain involvement, best
Figures 1.9.1–1.9.3 Axial DWI images show symmetric areas of seen on DWI and FLAIR. Subacute infarctions can
hyperintensity in the bilateral paramedian thalami (Figs. 1.9.1, 1.9.2) and left enhance.
midbrain (Fig. 1.9.3). r Hyperintensity along the pial surface of the midbrain in
Figure 1.9.4 Axial ADC shows hypointensity of bilateral paramedian thalami,
the interpeduncular fossa on axial DWI and FLAIR images
consistent with acute infarction. (the “V sign”) is present in 67% of patients.
Further reading
Lazzaro NA, Wright B, Castillo M, Fischbein Matheus MG, Castillo M. Imaging of acute
NJ, Glastonbury CM, Hildenbrand PG, bilateral paramedian thalamic and
et al. Artery of Percheron infarction: mesencephalic infarcts. AJNR Am J
imaging patterns and clinical spectrum. Neuroradiol 2003; 24(10): 2005–2008.
AJNR Am J Neuroradiol 2010; 31(7):
1283–1289.
26
Case 1.10
27
Section 1: Brain
28
History septic emboli, and vasospasm from subarachnoid

hemorrhage.
Patient 1: 24-year-old previously healthy man develops
status epilepticus.
Patient 2: 55-year-old woman presents with multiple recent
Key points
r PACNS is a rare disease characterized by nonatheromatous
strokes.
inflammation and necrosis of the cerebral vasculature
without involvement of other organs.
Findings r It mostly affects middle-aged men. The clinical
Patient 1 presentations are often nonspecific, including headache,
Figures 1.10.1, 1.10.2 Coronal FLAIR (Fig. 1.10.1) and axial T2 (Fig. 1.10.2) encephalopathy, seizure, strokes, and subarachnoid or
MRI demonstrate multifocal hyperintense cortical/subcortical lesions bilaterally, intraparenchymal hemorrhages.
including right middle and inferior frontal gyri (Fig. 1.10.1, white arrows), right r Imaging:
orbitofrontal gyrus (Fig. 1.10.2, curved arrow) and bilateral hippocampi (black
arrows). b MRI is the imaging modality of choice and is abnormal
in 90–100% of patients, although the findings are
Figure 1.10.3 Axial DWI reveals diffusion restriction in some of these cortical
lesions. None of these lesions enhances on the post-contrast images (not mostly nonspecific. It may show T2/FLAIR
shown). CTA of the head (not shown) is also normal. hyperintensity in the subcortical/deep white matter,
deep gray matter, and/or cerebral cortex. Infarctions
Patient 2 are usually seen bilaterally in multiple vascular
territories and with different ages. Other patterns
Figures 1.10.4, 1.10.5 Axial DWI demonstrates multiple areas of restricted
diffusion in the bilateral cerebral hemispheres, consistent with acute include diffuse small-vessel ischemic demyelination,
infarctions. The majority of these infarctions are cortical, with an additional confluent white-matter lesions, which can be mistaken
focus in the left frontal white matter. for multiple sclerosis, or rarely mass lesions, which can
Figure 1.10.6 Axial T1 shows an old cortical infarction in the right occipital
be confused with neoplasm. Leptomeningeal or
lobe with laminar necrosis. parenchymal enhancement and cerebral hemorrhage
can also occur.
Figure 1.10.7 3D volume-rendered CTA image reveals multifocal stenosis of b The CSF analysis is abnormal in 80–90% of patients
both anterior and posterior circulations, with “beaded” appearance.
with PACNS, typically showing mild pleocytosis and
Figure 1.10.8 A curved planar reformatted image of left MCA provides a elevation of protein. The combination of negative MRI
detailed view of multifocal stenosis. and CSF analysis has an excellent negative predictive
value.
b Cerebral angiography may reveal findings of multifocal
Diagnosis stenosis alternating with normal or dilated segments
Primary angiitis of central nervous system (PACNS). with a “beaded” appearance. Of note, the sensitivity of
angiography is poor, as PACNS frequently involves
Differential diagnosis small vessels beyond the resolution of angiography, and
r A broad range of differential diagnoses should be therefore a negative angiogram does not exclude the
considered, as many patients (such as patient 1) have diagnosis of PACNS. Furthermore, the angiographic
uncharacteristic findings on brain CT/MRI and findings are not specific to PACNS. A wide range of
unrevealing neurovascular studies (CTA, MRA or DSA). vasculopathies, as mentioned in the differential
These include various infectious encephalitis/ diagnosis section, can cause similar findings. Thus, a
meningoencephalitis, demyelinating process, systemic positive angiogram does not make the diagnosis. The
CNS vasculitis, CNS lymphoma, sarcoidosis, gold standard for the diagnosis of PACNS remains
paraneoplastic syndrome, and mitochondrial disease. brain and leptomeningeal biopsy, although the
r If neurovascular studies show alternating multisegmental pathology is frequently false-negative from sampling
stenosis and dilation, the differential diagnoses include error, because of the patchy and segmental nature of
atherosclerosis, systemic CNS vasculitis, reversible the disease.
vasoconstriction syndrome, moyamoya disease, r Treatment consists of corticosteroid or cyclophosphamide.
Further reading
Birnbaum J, Hellmann DB. Primary angiitis Pomper MG, Miller TJ, Stone JH, Tidmore
of the central nervous system. Arch WC, Hellmann DB. CNS vasculitis in
Neurol 2009; 66(6): 704–709. autoimmune disease: MR imaging
findings and correlation with
angiography. AJNR Am J Neuroradiol
1999; 20(1): 75–85.
29
Section 1: Brain
Case 1.11
30
31
Section 1: Brain
Figure 1.11.10 CT venogram confirms right sigmoid sinus thrombosis (black

History arrow). Note the normally enhancing left sigmoid sinus on the venogram
Patient 1: 16-year-old girl presents with headache and (white arrow).
vomiting followed by rapid neurological deterioration.
Patient 2: 72-year-old asymptomatic man was found to have Diagnosis
papilledema on routine eye exam.
Cerebral venous thrombosis.
Findings Differential diagnosis

Patient 1 r Be aware of several important diagnostic pitfalls:
Figure 1.11.1 NECT shows increased density in the internal cerebral veins b On NECT, the dense clot sign may be mimicked
(arrow), veins of Galen (curved arrow), and straight sinus (block arrow),
consistent with venous thrombosis. by hemoconcentration due to dehydration or
polycythemia, adjacent subdural or subarachnoid
Figures 1.11.2–1.11.4 Axial DWI (Fig. 1.11.2), ADC (Fig. 1.11.3), and FLAIR hemorrhage.
(Fig. 1.11.4) demonstrate cytotoxic edema in the bilateral caudates, putamina, b On MRI, the appearance of acute or chronic thrombi
and bifrontal periventricular white matter, with diffusion restriction and FLAIR
hyperintensity. There is also vasogenic edema in the right thalamus without may mimic patent sinuses with complex or slow
diffusion restriction. The signal abnormalities are in the drainage territory of flow.
deep cerebral veins. b Congenitally hypoplastic sinus or arachnoid
Figure 1.11.5 Axial T1 demonstrates high signal within the internal cerebral granulations may mimic thrombosis on MRV or
veins (arrow), veins of Galen (curved arrow), and torcula (block arrow), CTV.
consistent with subacute thrombi. b Subacute thrombi may mimic patent flow on the time
Figure 1.11.6 Sagittal post-contrast T1 shows extensive filling defects in the
of flight (TOF) MRV due to intrinsic T1 shortening.
superior sagittal sinus and straight sinus (∗ ), consistent with thrombosis.
Key points
Patient 2 r Cerebral venous thrombosis is an uncommon but
Figures 1.11.7–1.11.9 Axial T1 (Fig. 1.11.7) and T2 (Fig. 1.11.8) demonstrate potentially fatal neurological disease.
abnormal signal in the right sigmoid sinus (black arrow), which is T1 isointense r Numerous predisposing factors have been implicated,
and T2 hyperintense, compared to the normal flow void of left sigmoid sinus
(white arrow). There is a similar degree of enhancement of bilateral sigmoid including local processes adjacent to the dural sinuses such
sinuses on the post-contrast axial T1 (Fig. 1.11.9). as infection, trauma, or tumor compression/invasion, as
32
well as hypercoagulability secondary to systemic processes – Subacute stage: hyperintense on T1,

such as dehydration, oral contraceptive, peripartum hyperintense on T2.
status, malignancy, protein C and protein S deficiencies, – Chronic stage: isointense on T1, hyperintense
etc. on T2.
r Increased venous pressure may lead to parenchymal Post-contrast T1: filling defects within the sinuses.
vasogenic edema, cytotoxic edema, or intracranial Of note, chronic thrombi may enhance similarly to
hemorrhage. the normal sinus (see patient 2, Fig. 1.11.9). Sinus
r Parenchymal lesions that are not conforming to typical enhancement on post-contrast T1 does not
arterial distributions, or that predominantly involve the necessarily confirm patency, and CT/MR
subcortical regions with relative sparing of the cortices, venography is often necessary for a definitive
should raise the suspicion for venous infarcts. diagnosis.
r Superior sagittal sinus thrombosis may cause edema/ The thrombi may show susceptibility on gradient-
hemorrhage in the parasagittal regions. Thrombosis of the recalled echo (GRE) sequence and high diffusion
transverse sinus or vein of Labbé can lead to changes in the signal on DWI.
temporal lobes. Deep venous occlusions typically involve Time of flight (TOF) MRV: absence of flow-related
the thalami, basal ganglia, and periventricular white enhancement in the occluded sinuses or cortical
matter. veins.
r Imaging: Contrast-enhanced MRV or CTV: filling defects or
b NECT: hyperattenuating thrombi in the occluded veins lack of enhancement in the occluded sinus/cortical
(dense clot sign). veins.
b CECT: filling defects in the dural sinuses surrounded r Treatment:
by contrast (empty delta sign). b Anticoagulation.
b MRI: b Endovascular mechanical thrombectomy may be
T1 and T2 signal intensities vary depending on the required in severe cases.
age of thrombi. b In contrast to arterial ischemic infarcts, many
– Acute stage: isointense on T1, hypointense on parenchymal abnormalities secondary to venous
T2. occlusion are reversible if treated early.
Further reading
Leach JL, Fortuna RB, Jones BV, Poon CS, Chang JK, Swarnkar A, Johnson
Gaskill-Shipley MF. Imaging of cerebral MH, Wasenko J. Radiologic diagnosis of
venous thrombosis: current techniques, cerebral venous thrombosis: pictorial
spectrum of findings, and diagnostic review. AJR Am J Roentgenol 2007;
pitfalls. Radiographics 2006; 26(Suppl 1): 189(6 Suppl): S64–75.
S19–S41; discussion S42–S43.
33
Section 1: Brain
Case 1.12
34
35
Section 1: Brain
r
History r
Vascular malformation (AVM, AVF, aneurysm).
Hemorrhagic tumor.
Patient 1: 57-year-old man is unconscious. r Hemorrhagic transformation of arterial infarction.
Patient 2: 70-year-old man presents with transient r Amyloid angiopathy.
right-sided weakness. r Anticoagulation.
r Vasculitis.
Findings
Patient 1 Key points
Figure 1.12.1 NECT shows large left temporal lobar hemorrhage (∗ ) with
r Dural sinus thrombosis is an uncommon but important
adjacent edema. cause of lobar hemorrhages. Parenchymal hemorrhages
can be seen in up to one-third of cases of cerebral venous
Figure 1.12.2 NECT at a more inferior level reveals asymmetric hyperdensity
in the left transverse sinus (arrow), suspicious for dural sinus thrombosis. thrombosis.
r Hemorrhage in the frontal and parietal lobes may be due to
Figure 1.12.3 Axial T1 MRI without contrast demonstrates hyperintensity thrombosis of the superior sagittal sinus, while temporal
of left transverse sinus (arrow), consistent with subacute thrombus. T1
hyperintense subacute left temporal hematoma is also noted (curved arrow).
and occipital lobes are in the drainage territory of the
transverse sinus or vein of Labbé (inferior anastomotic
Figure 1.12.4 CT venogram confirms occlusion of left transverse sinus vein). Hemorrhage caused by cerebral venous thrombosis
(arrow) and patency of right transverse sinus (block arrow). is typically cortical with subcortical extension. Large
hematoma is typically flame-shaped with an irregular
Patient 2 border and adjacent edema reflecting hemorrhagic
Figure 1.12.5 NECT shows two small foci of subcortical parenchymal
infarction (patient 1). Smaller isolated subcortical
hemorrhages in the left parietal lobe. hemorrhages without edema or mass effect may also be
seen (patient 2).
Figures 1.12.6, 1.12.7 Axial T2 MRI (Fig. 1.12.6) and axial T1 MRI (Fig. 1.12.7) r Imaging: although CT or MRI can be diagnostic of venous
demonstrate acute left parietal hematoma (arrow), which is slightly T2
hyperintense and T1 isointense, and with a hematocrit level. thrombosis by showing dense sinus sign (CT) or altered
flow signal in the dural sinuses (MRI), these findings are
Figure 1.12.8 Contrast-enhanced MR venogram reveals nonocclusive sometime equivocal and difficult to distinguish from
thrombus in the superior sagittal sinus (arrow).
artifact. As illustrated in patient 2, the CT density of
superior sagittal sinus is not significantly hyperdense, and
the MRI signals (isointense on T1, slightly hyperintense on
Diagnosis T2, likely reflecting acute thrombus) can be confused with
Lobar hemorrhage due to dural sinus thrombosis. complex venous flow. It is therefore important to always
keep cerebral venous thrombosis in the differential
Differential diagnosis diagnosis of otherwise unexplained parenchymal
r Trauma. hemorrhages. Contrast-enhanced MR or CT venography is
r Hypertension. the diagnostic modality of choice.
Further reading
Leach JL, Fortuna RB, Jones BV, Gaskill-
Shipley MF. Imaging of cerebral venous
thrombosis: current techniques,
spectrum of findings, and diagnostic
pitfalls. Radiographics 2006; 26(Suppl 1):
S19–S41; discussion S42–S43.
36
Case 1.13
37
Section 1: Brain
History medium-sized vessels of the cerebral cortex, subcortical

white matter, and leptomeninges.
68-year-old female with multiple recent falls and altered r Clinical presentations are often nonspecific and include
mental status.
intracranial hemorrhage, TIA, and cognitive impairment.
r CAA should be strongly suspected in patients over the age
Findings of 60 with multiple lobar hemorrhages in the absence of
Figure 1.13.1 NECT of the head shows an intraparenchymal hematoma in hypertension.
the left parietal lobe and confluent periventricular white matter low-density r According to Boston criteria, CAA can be only definitively
changes.
diagnosed by postmortem examination of the brain. MRI,
Figures 1.13.2, 1.13.3 Axial T1 (Fig. 1.13.2) and axial T2 (Fig. 1.13.3) MRI especially gradient-recalled echo (GRE) sequence, plays a
demonstrate early subacute intraparenchymal hemorrhage (T1 hyperintense major role in establishing the diagnosis of “probable or
and T2 hypointense) in the left parietal lobe (black arrow), and late subacute
intraparenchymal hemorrhage (T1 hyperintense and T2 hyperintense with a possible CAA” in live patients.
rim of hemosiderin deposition) in the right frontal lobe (white arrow). r Imaging features:
b Intracerebral hemorrhages of differential ages in
Figure 1.13.4 Axial GRE MRI reveals multiple foci of cortical/subcortical
microhemorrhages (arrows), suggestive of amyloid deposition. cortical/subcortical distribution.
b CAA-related macrohemorrhages typically exhibit
irregular borders, and may be associated with
Diagnosis subarachnoid, subdural, or intraventricular
Cerebral amyloid angiopathy (CAA)-related lobar hemorrhage and hemosiderosis.
hemorrhages. b GRE MRI sequence shows characteristic
microhemorrhages in the cortical/subcortical
Differential diagnosis distribution, which is the key for diagnosis.
r For acute lobar hemorrhage, please refer to Case 1.12. b Leukoencephalopathy: consisting of patchy or
r For multiple microhemorrhages: confluent white-matter T2 hyperintensities, which are
b Chronic hypertension. potentially reversible.
b Diffuse axonal injury. b Cerebral atrophy.
b Hemorrhagic metastasis. r Treatment:
b Type 4 cavernous malformation. b The management of acute CAA-related hemorrhage is
b Vasculitis.
similar to spontaneous hemorrhage of other causes.
b Avoid anticoagulants and antiplatelet agents, because of
Key points the high rate of recurrent hemorrhages.
r CAA is a cerebrovascular disorder characterized by the b Rare forms of CAA-related inflammation may be
deposition of ␤-amyloid protein in the small and responsive to immunosuppressive therapy.
Further reading
Blitstein MK, Tung GA. MRI of cerebral Chao CP, Kotsenas AL, Broderick DF.
microhemorrhages. AJR Am J Roentgenol Cerebral amyloid angiopathy: CT and
2007; 189(3): 720–725. MR imaging findings. Radiographics
2006; 26(5): 1517–1531.
38
Case 1.14
39
Section 1: Brain
History Diagnosis
Patient 1: 37-year-old female presents with acute right Cavernous malformation (CM).
hemiparesis and dysarthria.
Patient 2: 10-year-old boy presents with seizure. Differential diagnosis
r For microhemorrhages (type 4 CM), please refer to
Case 1.13.
Findings r For large CM:
b Thrombosed aneurysm.
Patient 1
b Hemorrhagic tumor.
Figure 1.14.1 NECT demonstrates a round mass in the left pons with
peripheral hyperdensity.
b Hemorrhage from other etiologies such as trauma,
hypertension, arteriovenous malformation, etc.
Figure 1.14.2 Sagittal T1 MRI shows that the mass is predominantly T1
hyperintense, compatible with subacute hemorrhage. Key points
Figure 1.14.3 Axial T2 MRI demonstrates a mass with mixed T2 signal
r CM, also called cavernoma, cavernous angioma, or
intensity with fluid–fluid levels (black arrow) and surrounding edema (white cavernous hemangioma, is an angiographically occult
arrow).
vascular malformation that is frequently associated with
Figure 1.14.4 The gradient-recalled echo (GRE) sequence reveals developemental venous anomaly (DVA).
susceptibility from the presence of blood products with a hemosiderin ring r Histologically, it consists of well-circumscribed sinusoidal
(arrows). vascular channels containing blood in various stages
without intervening brain parenchyma.
r Clinical presentation depends on location. Many lesions
Patient 2 are asymptomatic. Lesions associated with DVA are more
Figure 1.14.5 Axial T2 MRI shows a lobulated, predominantly T2 likely to hemorrhage. Other presentations include seizures
hyperintense mass in the left parietal lobe with a surrounding hemosiderin ring and cranial nerve palsies, etc. Brainstem lesions have a
(arrows). worse prognosis with a higher rate of recurrent
hemorrhage and progressive neurologic decline.
Figure 1.14.6 Axial post-contrast T1 MRI demonstrates patchy enhancement r CT: hyperdense mass with variable degrees of calcification,
of this lesion (arrows). There is an adjacent developmental venous anomaly
with “Medusa head” appearance of multiple tributaries draining into an usually without significant mass effect or edema unless
enlarged collector vein (curved arrow). there is recent hemorrhage or thrombosis.
40
r MRI: variable T1 and T2 signal intensity depending on the b Type 4: punctate microhemorrhages with blooming
age of blood. T2 may show fluid–fluid level and a complete artifacts on the gradient-recalled echo.
hemosiderin ring. Post-contrast T1 shows absent or mild r Frequently, the imaging appearance may not be
enhancement, and the associated DVA. pathognomonic due to recent thrombosis or hemorrhage
r CM can be classified into four types based on MR features:
and a follow-up MRI series would be beneficial if
b Type 1: subacute hemorrhage with T1 hyperintensity. immediate surgical intervention is not warranted.
b Type 2: “popcorn” lesion containing hemorrhage of r Treatment: observation for asymptomatic lesion. Surgical
various ages, with mixed T1 and T2 signal intensity. resection for lesions causing large hemorrhage, intractable
b Type 3: chronic hemorrhage, with T1 and T2 seizures, or progressive neurologic deficits. Stereotactic
hypointensity. radiosurgery is an option for surgically inaccessible lesions.
Further reading
Rivera PP, Willinsky RA, Porter PJ. Zabramski JM, Wascher TM, Spetzler RF,
Intracranial cavernous malformations. Johnson B, Golfinos J, Drayer BP, et al.
Neuroimaging Clin N Am 2003; 13(1): The natural history of familial cavernous
27–40. malformations: results of an ongoing
study. J Neurosurg 1994; 80(3): 422–432.
41
Section 1: Brain
Case 1.15
42
43
Section 1: Brain
History Patient 4
Figure 1.15.7 Axial NECT demonstrates subarachnoid hemorrhage
Five patients present with severe headaches. surrounding the pons. The thickest blood clot is located at the right
cerebellopontine angle cistern (arrow).
Figure 1.15.8 AP DSA image from the right vertebral artery injection shows
Findings three aneurysms, at the origin of the right PICA (arrow), the tip of the basilar
artery (curved arrow), and the origin of the right SCA (block arrow). The source
Patient 1 of the hemorrhage is thought to be the PICA aneurysm based on the location
of thickest clot.
Figure 1.15.1 Axial NECT demonstrates diffuse subarachnoid hemorrhages
within the basilar cisterns, bilateral sylvian fissures, interhemispheric fissure,
cortical sulci, and occipital horns of lateral ventricles. Hydrocephalus is noted. Patient 5
Figure 1.15.9 Axial NECT demonstrates subtle subarachnoid hemorrhage in
Figure 1.15.2 Sagittal MIP image of head CTA shows a posteriorly projecting the left parietal/occipital sulci (circle).
saccular aneurysm in the region of the posterior communicating artery (arrow).
Figure 1.15.10 Sagittal MIP image of head CTA shows a small, peripherally
located aneurysm arising from the distal branch of left PCA (arrow).
Patient 2 Diagnosis
Figure 1.15.3 Axial NECT demonstrates an intraparenchymal hematoma
centered in the left insula and lentiform nucleus with local mass effect.
Cerebral aneurysms.
Patient 1: saccular P-comm aneurysm.
Figure 1.15.4 Coronal MIP image of head CTA shows a laterally projecting
saccular aneurysm at the left MCA bifurcation (arrow). Patient 2: saccular MCA bifurcation aneurysm.
Patient 3: fusiform MCA aneurysm.
Patient 4: multiple posterior circulation aneurysms,
including a dissecting PICA aneurysm.
Patient 3
Patient 5: mycotic distal PCA aneurysm.
Figure 1.15.5 Axial NECT demonstrates a serpentine, heterogeneously
hyperdense mass along the left sylvian fissure (arrows).
Figure 1.15.6 Axial MIP image of head CTA shows a fusiform aneurysm of the r For saccular aneurysm:
left MCA (arrows). The nonopacified thrombosed portion of the aneurysm
corresponds to the hyperdensity on the NECT (block arrows, Figs. 1.15.5 and b Vascular infundibulum (triangular shaped, ⬍ 3 mm in
1.15.6). size, a vessel arises from the apex).
44
r Other causes of subarachnoid hemorrhage: b Unruptured aneurysms are usually asymptomatic and
b Trauma. identified incidentally.
b Nonaneurysmal perimesencephalic subarachnoid b Thromboembolism can result from thrombosed or
hemorrhage. dissecting aneurysms.
b Venous thrombosis. b Local mass effects from giant aneurysm, compression
b Vasculitis. of optic nerves from ophthalmic aneurysm, and third
b Posterior reversible encephalopathy syndrome (PRES). nerve palsy from P-comm aneurysm, etc.
b Reversible cerebral vasoconstriction syndrome (RCVS). r Imaging:
b Vascular malformations. b NECT:
Standard for detecting SAH, with accuracy of
98–99%.
The pattern of SAH distribution and site of thickest
Key points clot may provide clues as to the location of the
r Cerebral aneurysms can be classified based on the
bleeding aneurysm.
morphology, etiology, location, size, etc. b CTA:
b Saccular aneurysm is the most common type and Modality of choice for the initial workup of acute
usually occurs at vascular branch points of the circle of SAH.
Willis. The most common locations include A-comm, Reliably detects aneurysms ⬎ 3 mm using 64-slice
P-comm, MCA bifurcation, tip of the basilar artery, MDCT scanners.
ICA terminus, and PICA origin. Aneurysms are Demonstration of adjacent bony anatomy and 3D
multiple in 20–25% patients. volume-rendered images are useful for treatment
b Other unusual types of aneurysms: planning.
Fusiform aneurysm: long segment of fusiform Limitations: limited sensitivity in detecting
enlargement of nonbranching vascular segment, cavernous aneurysms because of contrast
which may be secondary to atherosclerotic opacification of cavernous sinus, and in detecting
dolichoectasia, or nonatherosclerotic vasculopathies aneurysms at the skull base becacuse of beam
(inherited disease, collagen vascular disease, hardening artifact from bony structures. Not as
infections, etc.). accurate as MRA in following the treated
Dissecting aneurysm: can occur at both aneurysms, due to metallic artifacts of the coils and
vertebrobasilar and carotid circulations, but the clips.
most common location is the intracranial vertebral b MRA:
artery at the origin of PICA. Technique: time of flight (TOF) and contrast-
Blood-blister aneurysm: small aneurysm at the enhanced MRA.
nonbranch point of artery, typically at the Usually not used in the acute setting because of
supraclinoid ICA. Likely due to arterial dissection. patient motion and other MRI artifacts (flow
Difficult to treat, with a high rate of recurrence and turbulence and intrinsic T1 shortening from blood
rebleeding. products, etc.).
Giant aneurysm: ⬎ 2.5 cm, usually partially MRA is the preferred noninvasive modality for
thrombosed, symptoms related to mass effect are follow-up of the treated aneurysms, owing to its
common. Common locations include cavernous high sensitivity to residual flow and insensitivity to
ICA, MCA, and basilar tip. the metallic artifacts.
Mycotic aneurysm: usually small, peripheral b DSA:
aneurysms caused by arterial wall damage from Still considered reference standard for diagnosing
septic emboli. aneurysms. 3D rotational angiography can reliably
r Clinical presentation: detect aneurysms ⬎ 1.5 mm.
Endovascular treatment can be performed in the
b Intracranial hemorrhage, especially subarachnoid
same setting.
hemorrhage, is the most common presentation of
r Treatments:
ruptured aneurysms. Complications of subarachnoid
hemorrhage include hydrocephalus, vasospasm, b Surgical clipping and endovascular coiling are the most
rebleeding, cerebral edema, and treatment-related commonly used techniques.
complications. b Anatomy of the aneurysm, such as size, location, other
b Cavernous aneurysm rupture results in carotid– morphological features, should be considered in
cavernous fistula and/or epistaxis but is not a cause of choosing the most appropriate treatment for each
SAH because of its extradural location. patient.
45
Section 1: Brain
b In general, endovascular coiling is the preferred b Newer endovascular techniques are evolving, including
technique, with significantly lower mobidity compared stent-assisted coiling, balloon-assisted coiling, flow
to surgical clipping, as established by the International divertors, and liquid embolic material (Onyx).
Subarachnoid Aneurysm Trial (ISAT).
Further reading
Hacein-Bey L, Provenzale JM. Current Meyers PM, Schumacher HC, Higashida RT,
imaging assessment and treatment of Derdeyn CP, Nesbit GM, Sacks D, et al.
intracranial aneurysms. AJR Am J Reporting standards for endovascular
Roentgenol 2011; 196(1): 32–44. repair of saccular intracranial cerebral
aneurysms. J Neurointerv Surg 2010; 2(4):
312–323.
46
Case 1.16
47
Section 1: Brain
History Key points

41-year-old female, who is 11 days status post coil r Delayed cerebral ischemia (DCI) is a serious complication
embolization of ruptured left P-comm aneurysm, develops of aneurysmal subarachnoid hemorrhage with significant
word-finding difficulty and right-sided weakness. mortality and morbidity. It is presumably due to
vasospasm caused by degraded blood products within the
subarachnoid space.
r DCI typically occurs 4–14 days and peaks at 7–8 days after
Findings
aneurysm rupture, but it can be present as early as at the
Figure 1.16.1 3D volume-rendered head CTA image demonstrates luminal time of admission. Clinically, patients usually present
narrowing and irregularity of left M1 (arrow) and A1 (curved arrow) segments
compared to the contralateral side, consistent with vasospasm. with decreased level of consciousness and/or new focal
neurological deficits (hemiparesis, hemiplegia, aphasia,
Figures 1.16.2–1.16.4 CT perfusion images show abnormal perfusion etc.) that cannot be attributed to other etiologies such as
pattern in the left cerebral hemisphere, with prolongation of TTD (Fig. 1.16.2),
decreased CBF (Fig. 1.16.3), and preserved CBV (Fig. 1.16.4), suggestive of tissue subarachnoid hemorrhage or hydrocephalus. However,
at ischemic risk. DCI can be clinically silent in many patients.
r Imaging:
Figures 1.16.5, 1.16.6 AP DSA of left internal carotid injection confirms
vasospasm of left M1 and A1 segments (Fig. 1.16.5), which improves after b CT or MRI can detect new cerebral infarctions, which
intra-arterial injection of verapamil (Fig. 1.16.6). are not present at the time of aneurysm rupture.
b Two patterns of infarctions have been identified,
including large vessel territory infarction, typically near
the site of the ruptured aneurysm, and multiple
Diagnosis widespread subcortical infarctions, which are often
Vasospasm and delayed cerebral ischemia after unrelated to the site of aneurysm rupture.
subarachnoid hemorrhage. b Vascular studies including transcranial Doppler, CTA,
MRA, and DSA can demonstrate segmental stenosis
and contour irregularity. However, the clinical
Differential diagnosis presentations and angiographic findings may not
r None in the appropriate clinical setting, although correlate.
angiographic appearance is similar to vasculitis. See b Perfusion studies, especially CT perfusion, can be used
Case 1.10. to show the hemodynamic consequence of vasospasm,
48
and are reported to have high sensitivity and specificity r Treatment: triple-H therapy (hypertension, hypervolemia,
to detect DCI. Both prolonged MTT and reduced CBF hemodilution), calcium-channel blocker, magnesium,
are associated with subsequent infarction. endovascular therapy (intra-arterial injection of
nimodipine or balloon angioplasty).
Further reading
Sanelli PC, Kishore S, Gupta A, Mangat H,
Rosengart A, Kamel H, 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(12): 2209–2214.
49
Section 1: Brain
Case 1.17
50
History Key points

68-year-old woman presents with severe headache and r Pial AVMs are abnormal connections between arteries and
altered mental status. veins through an intervening tangle of vessels (nidus) and
lack of a true capillary bed.
Findings r The most common clinical presentations are intracranial
hemorrhage or seizures.
Figure 1.17.1 Axial NECT shows intraparenchymal hemorrhage in the inferior r The diagnostic criteria include the presence of a nidus
right frontal lobe and insula (curved arrow). An additional hyperdense structure
is seen in the right ventricular trigone (arrow). within the brain parenchyma, which can be seen on
cross-sectional studies or DSA, and arteriovenous
Figure 1.17.2 More inferiorly in the right temporal lobe there are partially
calcified serpentine structures suspicious for arteriovenous malformation
shunting, which is best seen on dynamic angiographic
(AVM). studies.
r The arterial feeders and venous drainage of an AVM
Figures 1.17.3, 1.17.4 Axial CTA (Fig. 1.17.3) shows an enhancing compact depend on the location of the nidus. For superficial AVMs,
AVM nidus in the right temporal lobe, which corresponds to a cluster of
abnormal flow voids on T2 MRI image (Fig. 1.17.4). the main arterial supplies are mostly through the pial
arteries, and venous drainages are through the cortical
Figures 1.17.5, 1.17.6 3D volume-rendered CTA (Fig. 1.17.5) and lateral veins. Deep and ventricular AVMs tend to recruit
DSA from right ICA injection (Fig. 1.17.6). The AVM is supplied by the right MCA,
with both superficial and deep venous drainage. The deep drainage is through
perforator and choroidal arterial supplies, with the venous
the right vein of Galen with an associated large venous pouch in the right drainage typically being through the deep venous system.
lateral ventricle (Figs. 1.17.1, 1.17.5, 1.17.6, arrows). r The Spetzler–Martin grading system has been widely used
to assign points based on the following features of the
AVM:
Diagnosis b Size of nidus: ⬍ 3 cm = 1; 3–6 cm = 2; ⬎ 6 cm = 3.
Pial arteriovenous malformation (Spetzler–Martin
b Eloquence of involved brain: noneloquent = 0;
grade III).
eloquent = 1.
b Venous drainage pattern: superficial only = 0;
Differential diagnosis deep = 1.
r AV fistula r The grade correlates with surgical outcome. The current
r Proliferative angiopathy. case is a grade III lesion, with two points given for its size
r Developmental venous anomaly. and one point given for the deep venous drainage.
51
Section 1: Brain
r In addition, certain AVM angioarchitectural features such posterior fossa location are associated with an increased
as intranidal aneurysm, venous pouch, venous stenosis, risk of hemorrhage and should be reported.
deep venous drainage, single venous drainage, and r Treatment options for brain AVMs include surgery,
endovascular embolization, and radiosurgery.
Further reading
Geibprasert S, Pongpech S, Jiarakongmun P,
Shroff MM, Armstrong DC, Krings T.
Radiologic assessment of brain
arteriovenous malformations: what
clinicians need to know. Radiographics
2010; 30(2): 483–501.
52
Case 1.18
53
Section 1: Brain
Figure 1.18.6
Figure 1.18.5
r Pial AVM.
r Cerebral AVF.
History
68-year-old man with sudden-onset headache and
right-sided weakness.
Key points
r DAVFs are pathological direct communications between
Findings dural arteries and dural venous sinuses, meningeal veins,
or cortical veins without a true nidus, which distinguishes
Figure 1.18.1 NECT shows left parietal intraparenchymal hemorrhage.
them from the parenchymal or pial AVMs. Although the
Figure 1.18.2 Axial T2 MRI shows an aneurysm (black arrow) in the vicinity of precise etiology is still debated, many DAVFs seem to be
hemorrhage. related to recanalization of dural sinus thrombosis.
r The majority of patients present in adulthood. Pulsitile
Figure 1.18.3 Axial CTA MIP image again demonstrates the venous aneurysm
(black arrow), as well as multiple prominent left cortical veins (white arrows).
tinnitus is a common symptom, resulting from increased
blood flow through the transverse and sigmoid sinuses.
Figure 1.18.4 Sagittal CTA MIP image reveals a dural arteriovenous fistula More severe presentations include intracranial
(DAVF) near the midpoint of the superior sagittal sinus (black arrow). In hemorrhage, seizures, focal neurological symptoms,
addition, there is a second DAVF at the cribriform plate (white arrow). Both
DAVFs have associated cortical venous drainages (block arrows). altered mental status, and dementia, which are likely
attributable to retrograde cortical venous drainage (CVD)
Figures 1.18.5, 1.18.6 AP and lateral DSA images of the right external and resultant venous hypertension.
carotid artery confirm the DAVF at the midpoint of superior sagittal sinus, r DAVFs are commonly stratified based on the Cognard or
supplied by both superficial temporal artery (white arrow) and middle
meningeal artery (black arrow). Associated cortical venous drainages (block Borden classification systems, and the venous drainage
arrow) and venous aneurysm (∗ ) are evident. Also note the faint blush at the pattern is the most important determinant of clinical
cribriform plate representing the second DAVF (Fig. 1.18.5, black curved arrow).
presentation, prognosis, and management. Lack of CVD is
associated with a benign natural history, while the presence
of CVD is an ominous feature associated with increased
Diagnosis rate of hemorrhage, nonhemorrhagic deficits, and
Dural arteriovenous fistulas (Borden type II). mortality.
54
r Neuroimaging: b Noninvasive neurovascular imaging, especially CTA,

b In the emergent setting, NECT is usually the initial is useful in treatment planning by delineating the
modality, which can identify hemorrhage and edema relationship of DAVFs to adjacent structures. Catheter
from venous hypertension associated with high-risk angiography, however, remains the gold standard.
lesions. r Treatment options include endovascular embolization
b MRI is more helpful in demonstrating abnormal flow (transarterial or transvenous) or open craniotomies.
void, dilated vessels, venous aneurysms, vascular
enhancement, etc.
Further reading
Borden JA, Wu JK, Shucart WA. A proposed dural arteriovenous fistulas: clinical and imaging findings, and treatment. AJNR
classification for spinal and cranial dural angiographic correlation with a revised Am J Neuroradiol 2012; 33(6): 1007–1013.
arteriovenous fistulous malformations classification of venous drainage. Kwon BJ, Han MH, Kang HS, Chang KH.
and implications for treatment. J Radiology 1995; 194(3): 671–680. MR imaging findings of intracranial
Neurosurg 1995; 82(2): 166–179. Gandhi D, Chen J, Pearl M, Huang J, dural arteriovenous fistulas: relations
Cognard C, Gobin YP, Pierot L, Bailly AL, Gemmete JJ, Kathuria S. Intracranial with venous drainage patterns. AJNR Am
Houdart E, Casasco A, et al. Cerebral dural arteriovenous fistulas: classification, J Neuroradiol 2005; 26(10): 2500–2507.
55
Section 1: Brain
Case 1.19
Diagnosis
Cerebral arteriovenous fistula (cerebral AVF).
r AVM.
r Dural AVF.
Key points
r Cerebral or pial AVF is a rare form of arteriovenous
malformation. It represents an abrupt transition from the
arterial feeder(s) to the draining vein without a discrete
nidus, distinguishing it from the classic cerebral AVMs.
Also, as opposed to dural AVF, it derives its arterial supply
from pial or cortical arterial vessels, instead of meningeal
arteries. It can arise from any cerebral artery and is
frequently associated with large venous aneurysms.
r Cerebral AVFs are most often diagnosed during the first
5 years of life, and are rarely seen in adults.
Figure 1.19.1 r The clinical presentations include congestive heart failure,
macrocrania, intracranial hemorrhage, seizures, and focal
History neurological deficits. An association with hereditary
hemorrhagic telangiectasia has been reported.
2-year-old boy with acute altered neurological status. r Treatment options include endovascular embolization or
open surgery.
Findings
Figure 1.19.1 Sagittal MIP of head CTA shows a large aneurysm (∗ ), supplied
by the callosomarginal branch of the right anterior cerebral artery (curved
arrow) and drained into the superior sagittal sinus through a single draining
vein (arrow).
Further reading
Weon YC, Yoshida Y, Sachet M, Mahadevan
J, Alvarez H, Rodesch G, et al.
Supratentorial cerebral arteriovenous
fistulas (AVFs) in children: review of 41
cases with 63 non choroidal single-hole
AVFs. Acta Neurochir (Wien) 2005;
147(1): 17–31.
56
Case 1.20
Figure 1.20.2
Figure 1.20.1
57
Section 1: Brain
Findings
Figure 1.20.1 NECT shows diffuse intraventricular hemorrhage and
hydrocephalus.
Figures 1.20.2, 1.20.3 Axial (Fig. 1.20.2) and coronal (Fig. 1.20.3) head CTA
MIP images demonstrate occlusion of bilateral A1 and M1 segments, which are
replaced by numerous lenticulostriate collaterals (arrows).
Figure 1.20.4 Axial T2 reveals small flow voids in the basilar cistern from
lenticulostriate and thalamostriate collaterals.
Figure 1.20.5 Axial FLAIR shows increased signal in the cortical sulci due to
slow flow of the engorged pial collaterals (white arrows). Also note the small
flow voids in the basal ganglia from the lenticulostriate collaterals (black
arrows).
Figure 1.20.6 AP projection DSA of left ICA injection demonstrates occlusion

of A1 and M1 segments, and numerous lenticulostriate collaterals with “puff of
smoke” appearance.
Figure 1.20.7 Lateral projection DSA of right ICA injection demonstrates

occlusion of A1 and M1 segments, and multiple lenticulostriate collaterals
(black arrow). Note that the right PCA (curved arrow) is supplied by right ICA
(fetal origin). Pial collaterals from right PCA reconstitute some ACA and MCA
branches (white arrows).
Figure 1.20.7
Diagnosis
History Idiopathic moyamoya disease.
21-year-old woman presents with sudden onset of headache
and altered mental status.
58
Differential diagnosis prevalent in children, while hemorrhagic strokes (mainly

r Similar angiographic findings have been described in many intraparenchymal and intraventricular hemorrhage) are
more common in adults.
other medical conditions (collectively termed “moyamoya r Imaging:
syndrome”), including:
b Sickle cell disease. b NECT can show cerebral infarction or hemorrhage.
b Atherosclerosis. b MRI: T2 shows collateral flow voids in the basilar
b Meningitis. cistern and basal ganglia. FLAIR can show bright sulci
b Down syndrome. (leptomeningeal “ivy” sign) due to slow flow of
b Neurofibromatosis type I. engorged pial collaterals. Post-contrast T1 can show
b Cranial radiation. lenticulostriate collateral and leptomeningeal collateral
enhancement.
b CTA/MRA/DSA: stenosis/occlusion of distal ICA and
Key points proximal circle of Willis vessels with collateral
r Moyamoya disease is a chronic cerebrovascular disease
formation.
characterized by progressive stenosis or occlusion of the
r Treatment: treatment of acute infarction or hemorrhage.
distal ICA and proximal circle of Willis vessels with
collateral formation. Moyamoya means puff of smoke in Consider surgical revasculization for patients with
Japanese, and this has been used to describe the ischemic-type or who have progressive cognitive
characteristic angiographic appearance of the collateral symptoms. Perfusion studies (SPECT, CT, or MRI
network. It has a higher incidence among the eastern Asian perfusion) may help identify patients with inadequate
than the European populations and likely has a strong cerebral blood flow reserve. Surgical techniques include
genetic etiology. direct superficial temporal artery to middle cerebral artery
r The clinical manifestations of moyamoya are variable. bypass or encephaloduroarteriosynangiosis (EDAS).
Ischemic disease, such as TIA or infarction, is more
Further reading
Hasuo K, Mihara F, Matsushima T. MRI and Ohta T, Tanaka H, Kuroiwa T. Diffuse Suzuki J, Kodama N. Moyamoya disease: a
MR angiography in moyamoya disease. J leptomeningeal enhancement, “ivy sign,” review. Stroke 1983; 14(1): 104–109.
Magn Reson Imaging 1998; 8(4): 762–766. in magnetic resonance images of
moyamoya disease in childhood: case
report. Neurosurgery 1995; 37(5):
1009–1012.
59
Section 1: Brain
Case 1.21
60
Figures 1.21.4, 1.21.5 AP and lateral DSA images of left internal carotid
artery in the arterial phase show abnormal early filling of left cavernous sinus
(∗ ), consistent with carotid–cavernous fistula. The fistula is supplied by the
meningohypophyseal trunk (curved arrow), a branch of the ICA, mainly drained
by the left superior ophthalmic vein (arrow).
Diagnosis
Type B carotid–cavernous fistula (CCF).
r Traumatic carotid–cavernous fistula.
r Other types of dural AV fistula or AVM.
Key points
r CCFs can be classified into direct and indirect types based
on angiographic findings. CCFs secondary to trauma or
aneurysm rupture are high-flow direct shunts (type A),
while other types are typically low-flow, spontaneous dural
AV fistulas between cavernous sinus and meningeal
Figure 1.21.5 branches of internal carotid artery (type B), external
carotid artery (type C), or both (type D).
r The majority of spontaneous CCFs are idiopathic, although
History certain predisposing factors have been implicated
63-year-old woman with progressive left-eye double vision including pregnancy, minor trauma, straining,
and proptosis. atherosclerotic disease, and collagen vascular disease.
r These shunts occur more frequently in middle-aged
Findings women, and present insidiously, with symptoms generally
being less severe than those of direct CCFs. The clinical
Figure 1.21.1 Axial T2 MRI demonstrates asymmetric dilation of the left presentations include diplopia, chemosis, proptosis, dilated
superior ophthalmic vein (arrow).
episcleral veins, diminished vision, cranial nerve IV and III
Figure 1.21.2 Axial T2 MRI shows a bulging contour of the left cavernous paresis, etc.
sinus (arrows), which contains an abnormal flow void (curved arrow). r Although they have a tendency to close spontaneously, a
number of patients will develop progressive symptoms and
Figure 1.21.3 Axial CTA reveals vascular engorgement of left cavernous sinus
(arrow). require endovascular treatment.
Further reading
Barrow DL, Spector RH, Braun IF, Landman
JA, Tindall SC, Tindall GT. Classification
and treatment of spontaneous
carotid–cavernous sinus fistulas. J
Neurosurg 1985; 62(2): 248–256.
61
Section 1: Brain
Case 1.22
62
History common carotid, cervical internal carotid, or external

carotid artery and its branches.
45-year-old male, with history of nasopharyngeal carcinoma r Risk factors include extensive head and neck surgery,
status post radical neck dissection and chemoradiation
radiation, wound breakdown with exposed carotid artery,
therapy, presents with massive epistaxis.
infection, and tumor recurrence.
r It can be classified into three categories:
Findings b Threatened: physical exam or radiologic evidence
Figure 1.22.1 Axial T2 MRI demonstrates an ovoid T2-hypointense lesion (∗ ) of inevitable hemorrhage if action is not taken
associated with the right internal carotid artery flow void (block arrow) at the
skull base. There is adjacent material (arrows) with intermediate T2 signal.
immediately (e.g., exposed artery).
b Impending: evidence of sentinel bleed (e.g., blood in
Figures 1.22.2–1.22.4 Axial pre-contrast T1 (Fig. 1.22.2) shows that this mouth) that resolves spontaneously or with pressure.
mass (∗ ) is T1 hypointense, with avid enhancement on the post-contrast axial b Acute: active bleeding that is not resolved by packing
(Fig. 1.22.3) and sagittal (Fig. 1.22.4) T1 sequences, consistent with a
pseudoaneurysm. Note surrounding non-enhancing material (arrows) with low and pressure.
to intermediate T1 signal representing acute hemorrhage. r Imaging:
b CT angiography is the preferred noninvasive imaging
Diagnosis modality. It can reveal vascular findings such as active
Carotid “blow-out” syndrome. contrast extravasation, pseudoaneurysm, arteriovenous
fistula, and luminal irregularity. It also provides
information on the extraluminal soft-tissue
Differential diagnosis abnormality and facilitates treatment planning.
r None in the appropriate clinical setting. b MRI/MRA: similar to CTA, but CTA is preferred
because of its rapid acquisition and availability.
Key points b DSA: remains the modality of choice. Endovascular
r Carotid blow-out syndrome is a life-threatening vascular intervention can be performed at the same time.
emergency of extracranial carotid arteries and is one of the r Treatment: airway protection, intravenous fluid and blood
most feared complications associated with surgery and/or transfusion, surgical sacrifice or endovascular occlusion of
radiation therapy of head and neck cancers. It can involve carotid artery, endovascular stent grafting.
Further reading
Mazumdar A, Derdeyn CP, Holloway W,
Moran CJ, Cross DT, 3rd. Update on
endovascular management of the carotid
blowout syndrome. Neuroimaging Clin N
Am 2009; 19(2): 271–281.
63
Section 1: Brain
Case 1.23
History Key points

3-year-old boy presents with an enlarging scalp “bump” and r Sinus pericranii is an unusual venous anomaly
focal tenderness. characterized by direct communication of intracranial
dural sinuses and epicranial varicose veins.
Findings r Most lesions are congenital. Some cases are associated with
other congenital venous anomaly, such as in this case.
Figures 1.23.1, 1.23.2 Sagittal (Fig. 1.23.1) and axial (Fig. 1.23.2) CT r Typically presents as a soft scalp mass, which increases in
venogram images demonstrate multiple scalp varicose veins (Fig. 1.23.2, black
arrows), draining into the superior sagittal sinus via a transosseous collector size with maneuvers that increase the intracranial pressure.
(Figs. 1.23.1, 1.23.2, black curved arrow), through a well-corticated midline skull Other common symptoms include headache and focal
defect. Note a soft-tissue mass posterior to the collector vein (Figs. 1.23.1,
1.23.2, white arrows), which is not opacified by contrast and is thought to
pain. Thrombosis of the epicranial varix can result in loss
represent a thrombosed varix. Also note the persistent embryonic falcine sinus of compressibility of the lesion and focal pain.
(Fig. 1.23.1, ∗ ). r The most common location is midline, with scalp varix
draining into the superior sagittal sinus.
r Rarely occurs in a lateral location, communicating with the
Diagnosis
transverse sinus or a developmental venous anomaly.
Sinus pericranii. r Imaging:
b CT: scalp soft-tissue mass and underlying well-defined
Differential diagnosis bone defect.
r Other common scalp/calvarial masses in children: b CTV/MRV/angiography: all components of sinus
b Dermoid or epidermoid. pericranii, including the epicranial veins, emissary
b Encephalocele. veins, and dural sinuses.
b Hemangioma. b MRI: T1/T2 shows flow voids, enhances on the
b Langerhans cell histiocytosis. post-contrast T1. May show thrombosed veins.
b Metastatic tumor (neuroblastoma, leukemia, etc.). r Treatment: surgery, endovascular therapy.
64
Further reading
Akram H, Prezerakos G, Haliasos N, Carpenter JS, Rosen CL, Bailes JE, Gailloud
O’Donovan D, Low H. Sinus pericranii: P. Sinus pericranii: clinical and imaging
an overview and literature review of a rare findings in two cases of spontaneous
cranial venous anomaly (a review of the partial thrombosis. AJNR Am J
existing literature with case examples). Neuroradiol 2004; 25(1): 121–125.
Neurosurg Rev 2012; 35(1): 15–26.
65
Section 1 Brain
Chapter
Head trauma
2 Yang Tang, Max Wintermark, and Sugoto Mukherjee
Case 2.1

66
Chapter 2: Head trauma
History Differential diagnosis

Three patients after motor vehicle crashes. r Subdural hematoma.
Findings Key points

r Epidural hematoma (EDH) refers to hemorrhage into the
Patient 1 potential space between the inner table of skull and the
Figure 2.1.1 NECT demonstrates a biconvex heterogeneous epidural outer layer of dura.
hematoma (∗ ) along the right frontal convexity with mass effect. The r It is usually associated with skull fractures, as well as other
low-density material within the hematoma represents acute unclotted blood.
There is a nondisplaced right frontal calvarial fracture (white curved arrow) and
concomitant injuries such as subdural hemorrhage,
overlying soft-tissue swelling. subarachnoid hemorrhage, parenchymal
hemorrhage/contusion.
r The majority of epidural hematoma is arterial (90%), most
Patient 2 commonly in the parietotemporal region and associated
Figure 2.1.2 Axial head CTA shows a right occipital epidural hematoma (∗ ), with injuries to the middle meningeal artery. The epidural
which displaces the right transverse sinus (white arrow) anteriorly. The distal
right transverse sinus and sigmoid sinus are occluded (black arrows). Note a hematoma is usually biconvex in shape, does not cross the
nondisplaced right occipital bone fracture (curved black arrow) with overlying skull sutures, and does not extend into the
soft-tissue swelling. interhemispheric fissure, as compared to the subdural
hematoma.
r Venous epidural hematoma (10%) is usually related to
Patient 3
fractures near the dural sinuses, and can involve multiple
Figure 2.1.3 NECT shows a bioccipital hyperacute epidural hematoma (∗ )
with hematocrit level. Also note the right cerebellar intraparenchymal intracranial compartments. Dural sinuses displaced by the
hemorrhage (arrow), intraventricular hemorrhage within the fourth ventricle hematomas can occasionally be occluded.
(curved arrow), and subarachnoid hemorrhage (block arrow) in the ambient r CT is the diagnostic study of choice in the emergency
cistern.
setting. Hyperacute EDH may contain both
Figure 2.1.4 Sagittal MIP image from the head CTA also demonstrates the high-attenuation clot and low-attenuation swirling lucency,
occipital epidural hematoma (∗ ). There is downward tonsillar herniation (block which represents active bleeding with unretracted clots.
arrow), with crowding of the foramen magnum and compression of the
brainstem. Note the lack of opacification of the basilar artery (black arrow), in
Subacute EDH has more homogeneously high attenuation
comparison to the normally enhancing anterior cerebral arteries, reflecting as active bleeding stops and organized clot forms. Chronic
high intracranial pressure in the posterior fossa. EDH has mixed or low attenuation as the clot breaks down.
Enhancing membrane formation may result from
neovascularity and formation of granulation tissue.
Diagnosis r Treatment: surgical evacuation if the hematoma causes
Arterial and venous epidural hematoma. significant mass effect.
Further reading
Osborn A, Salzman K, Barkovich A, editors.
Diagnostic Imaging Brain, 2nd edn. Salt
Lake City, UT: Amirsys; 2010.
67
Section 1: Brain
Case 2.2
68
History Diagnosis
Patient 1: 68-year-old female presents with multiple recent Cerebral herniation syndrome.
falls and altered mental status.
Patient 2: 43-year-old man with known cryptococcal
meningitis develops bradycardia and hypotension. Differential diagnosis
r Intracranial hypotension.
r Chiari I malformation.
Findings
Figure 2.2.1 NECT demonstrates bifrontal subdural hemorrhages (left greater r Cerebral herniation is a deadly complication of increased
than right) causing rightward midline shift, compression of the left lateral
ventricle, and dilation of the right lateral ventricle. intracranial pressure. It can be caused by a number of
diseases, including traumatic or nontraumatic intracranial
Figure 2.2.2 NECT shows medial deviation of the left uncus (black arrows) hemorrhages, brain tumor, large ischemic infarction, or
with obliteration of the CSF space surrounding the midbrain, consistent with
descending transtentorial herniation.
infection.
r Types of cerebral herniation:
Figure 2.2.3 Axial MIP image of head CTA better demonstrates the direct b Subfalcine herniation (midline shift):
mass effect of left uncus on the left PCA (arrow). The right PCA (block arrow)
is also compressed against the right tentorial notch. A focus of pontine Most common type.
hemorrhage is present (curved arrow). Cingulate gyrus is displaced under the falx cerebri.
Compression of ipsilateral ventricle and dilation of
Figure 2.2.4 The patient underwent emergent decompressive craniectomy,
but postsurgical head CT reveals ischemic infarctions of the left thalamus contralateral ventricle due to CSF obstruction at the
(arrow) and bilateral medial occipital lobes (block arrow) in the bilateral PCA foramen of Monro.
distribution. A new left frontal intraparenchymal hemorrhage is also noted. Compression of anterior cerebral artery by the falx
may lead to ischemic infarction.
b Unilateral descending transtentorial herniation (uncal
Patient 2 herniation):
Figure 2.2.5 NECT shows upward herniation of cerebellum (arrows) with Second most common.
effacement of quadrigeminal cistern and obstructive hydrocephalus. Medial temporal lobe is displaced inferomedially
Figure 2.2.6 NECT shows downward cerebellar tonsil herniation (arrows) into through the tentorial incisura, causing effacement of
the cisterna magna. suprasellar cistern.
69
Section 1: Brain
Compression of ipsilateral oculomotor nerve, lobes through the incisura, compressing the
resulting in loss of the normal pupillary reflex brainstem.
(“blown pupil”). b Ascending transtentorial herniation: upward
Contralateral cerebral peduncle is compressed herniation of cerebellum through the incisura with
against the tentorial notch, causing ipsilateral effacement of quadrigeminal cistern.
hemiplegia (false localizing sign). b Tonsillar herniation: downward displacement of
Compression of ipsilateral PCA may lead to cerebellar tonsils into the foramen magnum.
ischemic infarction. b Transcranial herniation (“fungus cerebri”): brain
Hemorrhage into the midbrain and pons (Duret parenchyma herniates through the calvarial/dural
hemorrhage) can occur due to tearing of perforating defect.
branches of the basilar artery or venous thrombosis. r Treatment: osmotic therapy (mannitol, hypertonic saline).
b Bilateral descending transtentorial herniation (central
Emergent decompressive craniectomy. Poor prognosis.
herniation): downward herniation of bilateral temporal
Further reading
Johnson PL, Eckard DA, Chason DP,
Brecheisen MA, Batnitzky S. Imaging
of acquired cerebral herniations.
Neuroimaging Clin N Am 2002; 12(2):
217–228.
70
Case 2.3
Figure 2.3.2
Figure 2.3.1
71
Section 1: Brain
History Key points

32-year-old man presents after motor vehicle crash with r DAI results from traumatic acceleration/deceleration or
Glasgow Coma Scale (GCS) score of 8. rotational injuries, and most frequently occurs after
high-speed motor vehicle crash.
r Patients with severe DAI are usually unconscious at the
Findings time of injury and remain in the persistent vegetative state.
Figure 2.3.1 NECT shows a small focus of intraparenchymal hemorrhage at
r The regions commonly affected by DAI include gray–white
the right frontal gray–white junction (arrow). matter junction, body and splenium of the corpus
callosum, dorsolateral midbrain, and upper pons, while
Figures 2.3.2, 2.3.3 Axial GRE MRI images demonstrate multiple foci of
susceptibility in the right frontal subcortical white matter (Fig. 2.3.2), as well as
other regions such as internal capsule, deep gray matter,
right thalamus, splenium of corpus callosum, and left frontal lobe (Fig. 2.3.3), and cerebellar peduncles can also be involved.
consistent with hemorrhagic diffuse axonal injury (DAI). r Imaging:
Figures 2.3.4–2.3.6 Axial FLAIR (Fig. 2.3.4), DWI (Fig. 2.3.5), and GRE
b DAI lesions are typically punctate or ovoid lesions,
(Fig. 2.3.6) images in a different trauma patient. There are multiple FLAIR which can be nonhemorrhagic (majority) or
hyperintense foci in the white matter of bilateral hemispheres and the body of hemorrhagic.
corpus callosum (Fig. 2.3.4), which demonstrate diffusion restriction (Fig. 2.3.5)
but no associated susceptibility (Fig. 2.3.6), consistent with nonhemorrhagic
b CT is not sensitive for DAI, but larger foci may show
diffuse axonal injuries. hyperdensity (hemorrhagic) or hypodensity
(nonhemorrhagic).
b On MRI, DAI lesions are typically T2/FLAIR
Diagnosis hypointense (hemorrhagic) or hyperintense
Diffuse axonal injury (DAI). (nonhemorrhagic). Hemorrhagic lesions show
susceptibility on GRE/SWI sequence. Both
hemorrhagic and nonhemorrhagic lesions may show
Differential diagnosis diffusion restriction on DWI. Diffusion tensor imaging
r Embolic infarctions, especially fat embolism following is the most sensitive sequence and shows reduced
long-bone fracture. anisotropy.
Further reading
Topal NB, Hakyemez B, Erdogan C, Bulut
M, Koksal O, Akkose S, et al. MR
imaging in the detection of diffuse axonal
injury with mild traumatic brain injury.
Neurol Res 2008; 30(9): 974–978.
72
Case 2.4
73
Section 1: Brain
r As a paired structure, the right and left cavernous sinuses

History
communicate with each other via the intercavernous sinus,
31-year-old man presents with bilateral exophthalmos after
also known as the circular sinus.
motor vehicle crash. r The major tributaries of cavernous sinuses include
superior/inferior ophthalmic veins, sphenoparietal sinus,
Findings and basal vein of Rosenthal, although the flow can be easily
Figure 2.4.1 Axial head CTA demonstrates abnormal enhancement and reversed in the setting of CCF. The major egress routes
contour bulge of bilateral cavernous sinuses (black arrows), intercavernous include superior/inferior petrosal sinuses, pterygoid and
sinus (∗ ), and clival plexus (block arrow), consistent with traumatic
carotid–cavernous fistula (CCF).
clival venous plexuses, eventually draining into the internal
jugular veins.
Figure 2.4.2 Axial head CTA shows engorgement of bilateral superior r CCFs are classified into four subtypes:
ophthalmic veins (block arrows).
b Type A (direct): between ICA and cavernous sinus.
Figure 2.4.3 Sagittal neck CTA shows extracranial dissection of right ICA b Type B (indirect): between the meningeal branches of
(block arrow), which tapers to occlusion. the ICA and cavernous sinus. See Case 1.21.
b Type C (indirect): between the meningeal branches of
Figure 2.4.4 AP DSA of left ICA demonstrates focal injury and narrowing of
left cavernous ICA (curved black arrow), which terminates into a large CCF. Note the ECA and cavernous sinus.
the tremendous filling of bilateral cavernous sinuses (∗ ) and numerous tributary b Type D (indirect): between the meningeal branches of
and egress veins including superior ophthalmic veins (block arrows), facial veins
(arrow), and multiple cortical and deep cerebral veins. There is no antegrade
ICA and ECA, and cavernous sinus.
filling of anterior and middle cerebral arteries, owing to steal phenomenon. r Traumatic CCF (type A) is a high-flow shunt due to
laceration or transection of cavernous ICA, typically
resulting from severe head/facial trauma. It usually
Diagnosis presents early with ophthalmologic symptoms including
Traumatic carotid–cavernous fistula (CCF), bilateral carotid pulsatile exophthalmos, chemosis, orbital bruits, visual
artery injury. disturbances, but occasionally cerebral hemorrhage or
infarction can occur.
Differential diagnosis r As the initial diagnostic modality, CTA is preferred over
r None. MRA. It can be used to evaluate the injury to the ICAs, size
and location of fistula tract, morphology of cavernous
sinus, and dilation of tributary and egress veins. DSA
Key points remains the diagnostic standard.
r CCF is an abnormal communication between the carotid r Treatment: endovascular therapy (either transarterial or
artery and the venous channels of the cavernous sinus. transvenous) with detachable balloon or coiling.
Further reading
Barrow DL, Spector RH, Braun IF, Landman Chen CC, Chang PC, Shy CG, Chen WS,
JA, Tindall SC, Tindall GT. Classification Hung HC. CT angiography and MR
and treatment of spontaneous angiography in the evaluation of carotid
carotid–cavernous sinus fistulas. J cavernous sinus fistula prior to
Neurosurg 1985; 62(2): 248–256. embolization: a comparison of
techniques. AJNR Am J Neuroradiol 2005;
26(9): 2349–2356.
74
Section 1 Brain
Chapter
Cerebral demyelinating and inflammatory diseases
3 Yang Tang, Xinli Du, Max Wintermark, and Sugoto Mukherjee
Case 3.1
75
Section 1: Brain
r Tumor: glioma, metastasis, lymphoma.
r Infection: pyogenic abscess, tuberculosis, fungal infection,
Lyme disease, etc.
r Vasculitis: primary CNS angiitis, sarcoidosis, lupus, etc.
r Progressive multifocal leukoencephalopathy (PML).
Key points
r Multiple sclerosis (MS) is an autoimmune-mediated
demyelinating disease that can affect brain, spinal cord,
and optic nerves.
r Clinical presentations are variable and depend on the
location of the lesions.
r Imaging:
b Typical MS lesions are small ovoid or linear lesions in
the periventricular or perivenular distribution
(“Dawson fingers”). Lesions involving the
callososeptal interface are also considered
characteristic of MS.
b Atypical imaging features of MS lesions include large
Figure 3.1.5
size (⬎ 2 cm), edema, mass effect, and/or enhancement
(often referred to as “tumefactive”), and these
History frequently pose a diagnostic dilemma.
38-year-old female presents with progressive bilateral lower b Compared to tumor/abscess, tumefactive
extremity sensory and motor deficits over the past 3 months. demyelinating lesions tend to have little mass effect
relative to their size, frequently with an incomplete ring
Findings enhancement.
b Other distinguishing features include dilated central
Figure 3.1.1 Axial T2 shows a large hyperintense lesion in the left parietal
cortical and subcortical region with adjacent edema but without significant
veins within the lesion, rapid response to corticosteroid
mass effect (block arrow). Note additional smaller patchy T2 hyperintense treatment, and decreased relative CBV on perfusion
periventricular lesions (arrows). imaging.
b On proton MR spectroscopy, tumefactive lesions may
Figures 3.1.2, 3.1.3 Axial (Fig. 3.1.2) and sagittal post-contrast T1 (Fig. 3.1.3)
images reveal incomplete ring enhancement of this lesion (block arrow). Note produce an identical spectrum to a high-grade glioma
additional smaller enhancing lesion (white arrow). with suppressed level of NAA, increased level of
choline, and detectable lactate, therefore not
Figure 3.1.4 Sagittal FLAIR demonstrates additional smaller lesions involving
the left optic nerve (white arrow), subcortical white matter (black arrow), and
contributory the differentiation of these two
callososeptal interface (white curved arrow). entities.
r Although it was originally proposed as an intermediate
Figure 3.1.5 Sagittal T2 thoracic spine shows a longitudinally extensive T2
hyperintense cord lesion from T9 to T12. lesion between multiple sclerosis and acute disseminated
encephalomyelitis, the development of multiple sclerosis is
the most common clinical outcome among patients
Diagnosis presenting with tumefactive features.
Tumefactive multiple sclerosis. r Treatment: corticosteroid, disease-modifying agents.
Further reading
Given CA, Stevens BS, Lee C. The MRI Lucchinetti CF, Gavrilova RH, Metz I, Parisi
appearance of tumefactive demyelinating JE, Scheithauer BW, Weigand S, et al.
lesions. AJR Am J Roentgenol 2004; Clinical and radiographic spectrum of
182(1): 195–199. pathologically confirmed tumefactive
multiple sclerosis. Brain 2008; 131(7):
1759–1775.
76
Chapter 3: Cerebral demyelinating and inflammatory diseases
Case 3.2
Figure 3.2.1
Figure 3.2.2
History
10-year-old boy with recent upper respiratory infection
develops fever, vomiting, and decreased consciousness.
Findings
Figures 3.2.1–3.2.3 Axial FLAIR images demonstrate multiple asymmetric
hyperintense lesions in the bilateral hemispheric subcortical white matter,
cortex, and pons. These lesions are not apparent on T1 images, nor do they
show post-contrast enhancement or diffusion restriction (not shown).
Diagnosis
Acute disseminated encephalomyelitis (ADEM).
r The most important differential diagnosis is multiple
sclerosis (MS). Although some imaging features (such as
involvement of gray matter, lack of periventricular and
callosal lesions, lesions of similar age) as well as CSF
Figure 3.2.3 findings (pleocytosis and lack of oligoclonal bands) may
77
Section 1: Brain
favor the diagnosis of ADEM, there are no established r Imaging:

criteria to reliably distinguish these two entities, and a b On MRI, ADEM lesions are typically multiple,
significant number of ADEM patients eventually receive asymmetrical, poorly marginated T2/FLAIR
the diagnosis of MS. hyperintensities, involving peripheral subcortical white
r Other entities, including infectious meningoencephalitis,
matter. Gray-matter structures including cortex, basal
vasculitis, neurosarcoidosis, antiphospholipid antibody ganglia, and thalamus, as well as brainstem and
syndrome, and mitochondrial disease, can resemble cerebellum, are frequently involved as well.
ADEM both clinically and on imaging. b ADEM lesions are usually inconspicuous on T1 and
uncommonly enhancing after contrast injection. When
Key points enhancement is present, most lesions typically enhance
r ADEM is a CNS autoimmune demyelinating disorder simultaneously, suggestive of similar age of
predominantly affecting children. Although classically demyelination.
monophasic, recurrent and multiphasic ADEM are well b Diffusion characteristics are variable: acute lesions may
recognized. show restriction, while subacute lesions have facilitated
r Predisposing factors such as recent febrile infection or diffusion.
vaccination have been reported in the majority of cases. b Acute hemorrhagic leukoencephalitis (Hurst disease) is
Clinical presentations include headache, fever, vomiting, a rare condition that is considered to be a hyperacute,
focal neurological signs, seizures, encephalopathic changes fulminant variant of ADEM.
(behavioral disturbances and altered consciousness), r Treatment: high-dose glucocorticoids, intravenous
etc. immune globulin, and plasma exchange.
Further reading
Menge T, Hemmer B, Nessler S, Wiendl H, Rossi A. Imaging of acute disseminated
Neuhaus O, Hartung HP, et al. Acute encephalomyelitis. Neuroimaging Clin N
disseminated encephalomyelitis: an Am 2008; 18(1): 149–61; ix.
update. Arch Neurol 2005; 62(11):
1673–1680.
78
Case 3.3
79
Section 1: Brain


Multiple patients with the same diagnosis. r Neurosarcoidosis is a great mimicker of many CNS
diseases.
Findings b Basilar leptomeningitis can be seen in tuberculous, and
in fungal or carcinomatous meningitis.
Patient 1 b Pituitary/infundibulum involvement can be seen in
Figure 3.3.1 Sagittal post-contrast T1 demonstrates enhancement of Langerhans cell histiocytosis.
hypothalamic infundibulum, interpeduncular cistern, ventral surface of the b Dural masses may be indistinguishable from other
brainstem, and cerebellar folia, consistent with basilar meningitis.
extra-axial lesions such as meningioma, lymphoma,
Figure 3.3.2 Axial post-contrast T1 demonstrates scattered nodular other granulomatous diseases, and idiopathic chronic
leptomeningeal enhancement (black arrows), including bilateral cranial nerves pachymeningitis.
V (white arrows).
b Parenchymal masses can mimic tumor or infections.
Figure 3.3.3 Axial post-contrast T1 of the orbits demonstrates thickening and b The white-matter involvement may mimic
enhancement of intracranial segments of the bilateral optic nerves (black demyelinating process or vasculitis.
arrows).
Key points
Patient 2 r Sarcoidosis is a systemic granulomatous disease of
Figure 3.3.4 Axial T2 shows a large area of vasogenic edema in the right unknown origin, characterized by the presence of
frontal lobe. Note T2 hypointense plaque-like extra-axial mass (black arrows).
noncaseating granulomas.
r While most patients have systemic, especially pulmonary,
Figure 3.3.5 Axial post-contrast T1 shows avid enhancement of the
extra-axial mass, mimicking a meningioma. manifestations, a small percentage of patients only have
CNS disease.
r CNS sarcoidosis can present with a variety of nonspecific
Patient 3 clinical presentations, including headache, visual
Figure 3.3.6 Axial T2 demonstrates confluent white-matter hyperintensity. impairment, seizure, and diplopia.
r History and extracranial manifestations of sarcoidosis
often play a key role in diagnosis, but biopsy is frequently
Diagnosis required. Serum ACE level is elevated in ⬍ 50% of cases,
Neurosarcoidosis. and normal CSF ACE level does not exclude the diagnosis.
80
r A wide spectrum of imaging findings in neurosarcoidosis b Dural thickening/enhancement or dural based masses:
has been well documented, including: may occur in the supratentorial or infratentorial
b Chronic leptomeningitis: preferentially involving compartment, and can be concurrent with
basilar cistern, hypothalamus, and pituitary stalk. It can leptomeningitis.
b Parenchymal masses: may occur in a random location
extend to the cortical sulci and infiltrate along the
Virchow–Robin perivascular space to cause a or along the expected distribution of perivascular
small-vessel vasculitis type of appearance. This may spaces, likely spread from subarachnoid disease. Of
result in communicating or noncommunicating note, both parenchymal and dural based lesions are
hydrocephalus. frequently T2 hypointense to the gray matter, reflecting
b Cranial nerve enhancement: often associated with the compact cellular nature of the granulomatous
widespread leptomeningitis. While all cranial nerves process.
b Non-enhancing T2 hyperintense white-matter disease.
can be involved, optic nerves are most frequently
b Spinal involvement. See Case 16.5.
affected. Cranial nerves III, V, VII/VIII complex are
also commonly involved. r Treatment: corticosteroid, other immunosuppressants.
Further reading
Shah R, Roberson GH, Cure JK. Correlation
of MR imaging findings and clinical
manifestations in neurosarcoidosis. AJNR
Am J Neuroradiol 2009; 30(5): 953–961.
81
Section 1: Brain
Case 3.4
82
History Key points

41-year-old woman with subacute onset of double vision r NMO, aka Devic disease, is a rare CNS demyelinating
and bilateral lower extremity weakness. disorder characterized by recurrent bouts of optic neuritis
and transverse myelitis. It can affect both adults and
children, with a strong predominance in nonCaucasian
Findings females.
Figure 3.4.1 Sagittal T2 demonstrates longitudinally extensive hyperintense r Although historically considered as a variant of multiple
signal of the thoracic cord (over five vertebral segments). sclerosis, NMO is now believed to be a distinct disease
Figure 3.4.2 Axial T2 reveals that the signal abnormality mostly involves the entity with unique immuno-pathological and imaging
central gray matter. features. NMO IgG, an autoantibody against water channel
aquaporin 4 (AQP4), has been reported to be 73% sensitive
Figure 3.4.3 Axial post-contrast T1 of the brain demonstrates enhancement and 91% specific in distinguishing it from multiple
of the prechiasmatic segment of the left optic nerve and optic chiasm.
sclerosis.
Figures 3.4.4, 3.4.5, 3.4.6 Axial FLAIR images reveal hyperintensity in the r Imaging:
bilateral hypothalami (Fig. 3.4.4), and along the left corticospinal tract in the left b NMO lesions in the spinal cord are typically
midbrain cerebral peduncle (Fig. 3.4.5) and medullary pyramid (Fig. 3.4.6).
longitudinally extensive with involvement of more than
three vertebral segments. On the axial images, NMO
Diagnosis lesions frequently involve a large portion of gray and
Neuromyelitis optica (NMO). white matter, sometimes the entirety of cross-sectional
area of the cord, as opposed to typical MS plaques,
which are more discrete and peripheral dorsal/lateral
Differential diagnosis white matter lesions. See Case 16.1 for further
r Multiple sclerosis. discussion.
r ADEM. b Optic nerve involvement, manifested by swelling, T2
r Viral encephalomyelitis. hyperintensity and contrast enhancement, is by itself
r Systemic autoimmune disease (such as SLE, Sjögren indistinguishable from other demyelinating,
syndrome, sarcoidosis, Behçet’s disease). inflammatory or infectious optic neuritis.
r Idiopathic transverse myelitis. b Although brain MRI of the majority of NMO patients
r Cord infarction. are either normal or only show nonspecific white
r Spinal dural arteriovenous fistula. matter disease, the typical NMO lesions follow the
83
Section 1: Brain
distribution of AQP4 and are usually observed around r Treatment: intravenous glucocorticoid, plasmapheresis.
the third and fourth ventricles including thalamus,
hypothalamus, brainstem, and cerebellum.
Further reading
Lennon VA, Wingerchuk DM, Kryzer TJ, Makhani N, Bigi S, Banwell B, Shroff M. VA. Neuromyelitis optica brain lesions
Pittock SJ, Lucchinetti CF, Fujihara K, Diagnosing neuromyelitis optica. localized at sites of high aquaporin 4
et al. A serum autoantibody marker of Neuroimaging Clin N Am 2013; 23(2): expression. Arch Neurol 2006; 63(7):
neuromyelitis optica: distinction 279–291. 964–968.
from multiple sclerosis. Lancet 2004; Pittock SJ, Weinshenker BG, Lucchinetti CF,
364(9451): 2106–2112. Wingerchuk DM, Corboy JR, Lennon
84
Case 3.5
85
Section 1: Brain
r Demyelinating disease.
History r Infectious encephalitis.
Patient 1: 39-year-old woman with history of systemic lupus r Venous infarctions.
erythematous (SLE) presents with seizure and obtundation.
Patient 2: 45-year-old woman with history of SLE presents
with altered mental status.
Key points
r Neuropsychiatric symptoms are common in patients with
Patient 3: 18-year-old female with history of SLE and recent
systemic lupus erythematous (SLE), including stroke,
seizures
seizure, decreased consciousness, cognitive dysfunction,
psychiatric symptoms, etc.
Findings r Once thought to be the major cause of CNS lupus, true
Patient 1 CNS vasculitis has been found to be a rare finding in
patients with SLE. Rather, the pathogenesis of CNS lupus is
Figures 3.5.1, 3.5.2 Axial FLAIR images demonstrate hyperintense signals in
the right parietal (Fig. 3.5.1) and left occipital cortical/subcortical regions likely multifactorial, and a number of autoantibodies and
(Fig. 3.5.2). There is no restricted diffusion on DWI (not shown), consistent with cytokines may play a significant role. Other secondary
vasogenic edema. Note the foci of hypointense signal with these areas (black factors such as infection, metabolic derangement,
arrow), consistent with hemorrhage. Additional cortical edema is also noted in
the right temporal/occipital region (white arrow). hypertension, and drug toxicity also contribute to the
neuropsychiatric symptoms.
r On neuroimaging, acute manifestations of CNS lupus may
Patient 2 include acute infarctions, hemorrhage, generalized or
Figure 3.5.3 Axial FLAIR demonstrates symmetric hyperintensity in the focal cerebral edema. PRES has also been increasingly
bilateral basal ganglia and temporal/occipital lobes. No restricted diffusion or
enhancement. recognized as a common manifestation of CNS lupus as
demonstrated in patient 3, likely due to hypertension.
Chronic findings include brain atrophy and multifocal
Patient 3 white-matter T2 hyperintensity from microinfarctions,
Figure 3.5.4 Axial T2 shows patchy hyperintensity involving the which may be indistinguishable from other causes of
cortex/subcortical white matter of the right temporal/occipital lobe and left
occipital lobe. white-matter disease such as chronic microangiopathic
changes, multiple sclerosis, or other types of CNS
vasculitis.
Diagnosis r Treatment: immunosuppression (steroid,
Lupus cerebritis. cyclophosphamide, etc.).
r Posterior reversible encephalopathy syndrome (PRES).
r Other vasculitis.
Further reading
Futrell N, Schultz LR, Millikan C. Central Gatla N, Annapureddy N, Sequeira W, Jolly
nervous system disease in patients with M. Posterior reversible encephalopathy
systemic lupus erythematosus. Neurology syndrome in systemic lupus
1992; 42(9): 1649–1657. erythematosus. J Clin Rheumatol 2013;
19(6): 334–340.
86
Case 3.6
87
Section 1: Brain
History Key points

27-year-old man with history of recurrent orogenital ulcers r Behçet’s disease is an idiopathic chronic relapsing
and uveitis, presents with subacute onset of headache, multisystem vascular-inflammatory disease of unknown
ataxia, and multiple cranial nerve palsies. origin.
r The classical triad includes oral and genital ulcerations as
Findings well as uveitis, but the disease can affect many other
systems including cardiopulmonary, gastrointestinal, and
Figures 3.6.1, 3.6.2, 3.6.3 Serial axial FLAIR images show extensive nervous systems. Clinically, NBS may resemble multiple
hyperintensity extending from the basal ganglia to the midbrain (Fig. 3.6.1),
pons (Fig. 3.6.2), pontomedullary junction, and middle cerebellar peduncles, sclerosis.
right greater than left (Fig. 3.6.3). Note slight expansion of right midbrain and r Imaging:
sparing of red nuclei (Fig. 3.6.2).
b Parenchymal NBS: most commonly affects midbrain,
Figure 3.6.4 Axial post-contrast T1 shows a midline pontine lesion with faint pons, basal ganglia/internal capsule, and
enhancement. thalamus/hypothalamus, with T2/FLAIR
hyperintensity ± patchy enhancement. NBS lesions are
Diagnosis typically asymmetric, and extend long fiber tracts with
characteristic sparing of the red nucleus. Some lesions
Neuro-Behçet’s syndrome (NBS).
are hemorrhagic, seen on GRE sequence.
b Cerebral venous thrombosis is another common
Differential diagnosis neuroimaging finding.
r Infiltrative glioma or gliomatosis cerebri. b A small percentage of patients present with aseptic
r Other vasculitis. meningitis on MRI.
r Demyelinating disease (MS, ADEM). r Treatment: steroids, disease-modifying agents such as
r Infectious rhomboencephalitis. azathioprine, mycophenolate, methotrexate, and
r Paraneoplastic brainstem encephalitis. cyclophosphamide.
Further reading
Kocer N, Islak C, Siva A, Saip S, Akman C, Siva A, Saip S. The spectrum of nervous
Kantarci O, et al. CNS involvement in system involvement in Behçet’s syndrome
neuro-Behçet syndrome: an MR study. and its differential diagnosis. J Neurol
AJNR Am J Neuroradiol 1999; 20(6): 2009; 256(4): 513–529.
1015–1024.
88
Section 1 Brain
Chapter
Intracranial infections
4 Yang Tang, Xinli Du, Sugoto Mukherjee, and Max Wintermark
Case 4.1

89
Section 1: Brain

Findings
Patient 1
Figure 4.1.1 Axial FLAIR shows hyperintensity within the bilateral cortical
sulci, representing inflammatory exudates from bacterial meningitis.
Figure 4.1.2 Post-contrast T1 demonstrates leptomeningeal enhancement.
Figure 4.1.3 Axial DWI reveals diffusion restriction of posterior limb of left
internal capsule, consistent with ischemic infarction. This may be due to
vasospasm or infectious arteritis of a perforating artery.
Patient 2
Figure 4.1.4 Coronal reformation of sinus CT demonstrates a defect in the
left cribriform plate (arrow) from inadvertent injury during the surgery. Also
note other sinonasal postsurgical changes.
Figure 4.1.5 Axial NECT shows poor definition of left cerebral sulci compared
to the right. Those on the left are filled with inflammatory exudates.
Figures 4.1.6, 4.1.7 Axial FLAIR (Fig. 4.1.6) reveals hyperintensity within
bilateral cortical sulci, worse on the left. Also note the subtle left frontal/parietal
cortical swelling, with corresponding high signal on DWI (Fig. 4.1.7). These
lesions do not follow arterial distribution, and are thought to be due either to
venous ischemia from thrombophlebitis, or to cerebritis.
Figure 4.1.7 (patient 2) Diagnosis

Bacterial meningitis.
History
Patient 1: 5-month-old boy with history of recent Differential diagnosis
otomastoiditis, develops high fever and seizure. r Differential diagnosis for leptomeningeal enhancement:
Patient 2: 23-year-old female with history of recent b Other infectious meningitis: TB, fungal, neurosyphilis.
endoscopic sinus surgery, develops CSF leak, fever, and b Metastatic carcinomatous meningitis, leukemia/
acute-onset right-sided weakness. lymphoma.
90
Chapter 4: Intracranial infections
b Neurosarcoidosis or other systemic inflammatory b Cerebritis: sometimes difficult to distinguish from

disorders. cortical infarction. In the follow-up study, cerebritis
r Differential diagnosis for FLAIR hyperintense signal may evolve into abscess or resolve if treated
within the cortical sulci: appropriately.
b Abscess.
b Exudates from meningitis.
b Subdural effusion and empyema: in contrast to
b Subarachnoid hemorrhage.
b Artifacts from oxygen supplementation and effusion, empyema typically shows increased FLAIR
signal and diffusion restriction due to viscous and
anesthetics.
b Slow flow of pial vessels from proximal vascular proteinaceous contents. Also frequently shows thicker
peripheral enhancement.
occlusion/stenosis. b Hydrocephalus: typically communicating
b Retained contrast in renal failure patients.
hydrocephalus.
b Ventriculitis/ependymitis.
Key points r Imaging: frequently normal. CSF analysis is best
r Definition: bacterial infection of leptomeninges and
for diagnosis, and imaging is best used to detect
subarachnoid space. complications.
r Route of infection: hematogenous spread, direct spread
b CT: may show sulcal effacement from inflammatory
from adjacent sinonasal or otologic infections, penetrating
exudates in the subarachoid space, as shown in
injury.
r Complications: patient 2.
b MRI:
b Infarctions due to vasospasm and/or thrombosis. FLAIR: hyperintense signal in the subarachnoid
Can be arterial or venous. space.
Arterial: occlusion of perforating arteries leads to Post-contrast T1: leptomeningeal enhancement.
infarction of basal ganglia/internal capsules/ DWI: best to demonstrate ischemic infarctions,
thalami. Occlusion of larger arteries leads to abscess, and subdural empyema, which show
territorial infarctions. diffusion restriction.
Venous: results from thrombophlebitis of cortical MRI/MRV: shows arterial/venous occlusion or
veins or dural sinuses. Do not conform to arterial stenosis.
distribution, and frequently hemorrhagic.
Further reading
Atlas SW, editor. Magnetic Resonance Osborn A, Salzman K, Barkovich A, editors.
Imaging of the Brain and Spine, 4th edn. Diagnostic Imaging Brain, 2nd edn. Salt
Philadelphia, PA: Lippincott Williams & Lake City, UT: Amirsys; 2010.
Wilkins; 2009.
91
Section 1: Brain
Case 4.2
92
History Diagnosis
Patient 1: 63-year-old woman presents with Pyogenic abscess.
methicillin-resistant Staphylococcus aureus septicemia from
infected cardiac pacemaker leads. Differential diagnosis
Patient 2: 22-year-old man with history of tetralogy of Fallot r Ring-enhancing lesions:
develops fever and vision changes. b Glioblastoma multiforme.
Patient 3: 20-year-old man with recent sinusitis presents b Cerebral metastasis.
with fever and altered mental status. b Pyogenic abscess and other nonpyogenic infections
(TB, fungal, or parasitic infection).
b Radiation necrosis.
Findings b Tumefactive demyelination.
Patient 1 b Subacute infarction.
Figure 4.2.1 CECT shows multiple ring-enhancing lesions of bilateral cerebral
b Resolving hematoma.
hemispheres at the gray–white matter junction. The largest one is in the left
frontal lobe with adjacent edema.
Key points
r Abscesses can arise from hematogenous spread of systemic
Patient 2
infection, contiguous spread from sinonasal, otogenic, or
Figure 4.2.2 Axial T2 demonstrates a left parieto-occipital mass with
sourrounding edema. Note the necrotic center with heterogeneous T2
odontogenic infections, or secondary to brain surgery or
hyperintense signal and a regular, thin T2 hypointense rim (arrows). trauma.
r Common organisms include staphylococci, streptococci,
Figures 4.2.3, 4.2.4 Axial pre-contrast T1 (Fig. 4.2.3) shows the T1 pneumococci, gram-negative and anaerobic bacilli, etc.
hyperintense rim, with avid enhancement on the post-contrast image r The evolution of brain abscess involves four continuous
(Fig. 4.2.4).
pathological stages: early cerebritis, late cerebritis, early
capsule formation, and late capsule formation.
Patient 3 r Clinical symptoms are nonspecific and most commonly
Figure 4.2.5 Sagittal post-contrast T1 shows a ring-enhancing centrally include fever, headache, seizure, focal neurological deficits,
necrotic lesion in the right frontal lobe with adjacent edema. Note acute and altered mental status.
ethmoid sinusitis (black arrow) with intracranial extension (white arrow).
r Abscess may occur anywhere in the brain parenchyma, but
Figure 4.2.6 Axial DWI reveals diffusion restriction of the right frontal mass. most commonly in the frontal and parietal lobes. Multiple
93
Section 1: Brain
abscesses suggest septic emboli, which preferentially aspect due to less perfusion. Ependymitis and
involve the gray–white matter junction. ventriculitis may occur as a result of intraventricular
r Imaging: abscess rupture.
b Cerebritis manifests as ill-defined cortical/subcortical b The liquefied necrotic center of the abscess typically
low-attenuation (CT) or T2 hyperintense edema (MRI), shows restricted diffusion related to the viscosity of
which may show patchy enchancement and diffusion purulent material. Although not pathognomonic, this
restriction. helps to distinguish abscess from necrotic primary or
b The typical appearance of a well-capsulated abscess is a secondary brain tumors. Of note, nonpyogenic fungal
fluid collection with a thin enhancing rim on or parasitic abscesses may not restrict diffusion.
b On MR spectroscopy, the necrotic center would
post-contrast CT or T1 MRI, surrounded by vasogenic
edema. demonstrate characteristic spectra including lactate,
b The rim is distinctively hypointense on T2 and acetate, succinate, alanine, pyruvate, and amino acid
isointense to slightly hyperintense on T1 compared to peaks, which also helps distinguish it from necrotic
the white matter, due to the presence of collagen, tumor.
b On perfusion MRI, the capsule should have low rCBV
hemorrhage, or free radicals. The ring should be
smooth, regular in thickness and thin-walled compared to necrotic tumor.
(⬍ 5 mm). It may be thinner along the ventricular r Treatment: antibiotics, surgery.
Further reading
Desprechins B, Stadnik T, Koerts G, Enzmann DR, Britt RH, Yeager AS. of amino acids detected at 1H MR
Shabana W, Breucq C, Osteaux M. Use Experimental brain abscess evolution: spectroscopy – initial results. Radiology
of diffusion-weighted MR imaging in computed tomographic and 1999; 213(3): 785–793.
differential diagnosis between neuropathologic correlation. Radiology Haimes AB, Zimmerman RD, Morgello S,
intracerebral necrotic tumors and 1979; 133(1): 113–122. Weingarten K, Becker RD, Jennis R,
cerebral abscesses. AJNR Am J Grand S, Passaro G, Ziegler A, Esteve F, et al. MR imaging of brain abscesses. AJR
Neuroradiol 1999; 20(7): 1252– Boujet C, Hoffmann D, et al. Necrotic Am J Roentgenol 1989; 152(5): 1073–
1257. tumor versus brain abscess: importance 1085.
94
Case 4.3
95
Section 1: Brain
Diagnosis
CNS tuberculosis (TB).
r Other causes of basilar meningitis:
b Other infectious meningitis (pyogenic, fungal,
syphilitic, etc.).
b Inflammatory (neurosarcoidosis, rheumatoid arthritis,
etc.).
b Meningeal carcinomatosis.
b Lymphoma.
r Tuberculoma:
b Other infections (pyogenic abscess, fungal granuloma,
toxoplasmosis, cysticercosis, etc.).
b Tumor.
Key points
r CNS TB includes three categories: basilar meningitis, brain
Figure 4.3.5 (patient 2) parenchymal TB, and spinal meningitis/arachnoiditis.
r Nearly always secondary infections, most often from the
pulmonary source.
r Clinically, patients present with low-grade fever, headache,
History altered mental status, and meningeal signs.
Patient 1: 42-year-old man presents with acute psychosis, r CSF analysis typically shows mononuclear pleocytosis,
hallucination. and fever after a prodrome of flu-like illness.
increased protein, and decreased glucose. CSF polymerase
CSF shows mild pleocytosis and elevated protein.
chain reaction (PCR) and culture are confirmatory
Patient 2: 44-year-old recent immigrant presents with fever tests.
and headache. r TB meningitis preferentially involves the basilar cistern,
and can extend to the surface of brain hemispheres. The
Findings thick exudate in the basilar cistern may impair the
absorption of CSF, leading to communicating
Patient 1
hydrocephalus. Vasculitis can cause cerebral infarctions.
Figures 4.3.1, 4.3.2 Axial and coronal post-contrast T1 with fat saturation r The most common parenchymal form of CNS TB is
demonstrate enhancing material in the basilar cisterns and along bilateral
sylvian fissures. Note dilation of lateral ventricles, consistent with tuberculoma, which may be secondary to hematogenous
communicating hydrocephalus. spread of primary infection, or parenchymal extension of
meningitis.
Figure 4.3.3 Post-contrast sagittal T1 of the lumbar spine demonstrates
diffuse enhancement of the cauda equina nerve roots, consistent with b On CT, some cases may show a “target sign,” which is a
arachnoiditis. central calcification or enhancement surrounded by
hypodensity and rim enhancement.
Patient 2 b On MRI, the imaging appearance depends on whether
Figure 4.3.4 Post-contrast axial T1 shows a lobulated mass in the left insular the tuberculoma caseates. Noncaseating granulomas
region with mixed necrotic and solid enhancing components. would be T1 hypointense, T2 hyperintense and
demonstrate nodular homogeneous enhancement. The
Figure 4.3.5 Axial T2 shows that the necrotic centers (arrows) are iso- to
slightly hypointense to the cortex. Mild surrounding edema is present. This was center of caseating granuloma is frequently isointense
proven to be a caseating tuberculoma on biopsy. or hypointense to brain parenchyma, due to the
96
presence of paramagnetic free radicals or greater rim enhancement. Edema and mass effect can be
cellularity. If the center is more liquefactive, it can be observed.
T2 hyperintense. Post-contrast images typically show r Treatment: anti-TB treatments.
Further reading
Burrill J, Williams CJ, Bain G, Conder G,
Hine AL, Misra RR. Tuberculosis: a
radiologic review. Radiographics 2007;
27(5): 1255–1273.
97
Section 1: Brain
Case 4.4

98
99
Section 1: Brain
100

Patient 1: 54-year-old female status post-chemotherapy and r Other infections: pyogenic abscess, septic emboli, TB,
stem-cell transplant for myelodysplastic syndrome, develops toxoplasmosis, etc.
fever and generalized weakness. This patient also has r Neoplasm: glioblastoma multiforme (GBM), metastasis,
multiple lung lesions on chest CT. lymphoma.
Patient 2: 22-year-old previously healthy man develops
progressive left-sided weakness. Key points
Patient 3: 79-year-old man who was recently diagnosed with r A number of fungi can affect humans, including both
pulmonary aspergillosis, develops multiple intracranial immunocompromised and immunocompetent hosts.
lesions. Aspergillus, Mucor, Candida, and Cryptococcus are
ubiquitous, while other fungi such as Coccidioides,
Findings Histoplasma, and Blastomyces are endemic.
r Two major forms of fungi based on morphology:
Patient 1 b Yeast (Histoplasma and Cryptococcus): small size,
Figures 4.4.1–4.4.4 Multiple intraparenchymal lesions. The largest one is in
the right frontal lobe, which demonstrates low attenuation on NECT (Fig. 4.4.1).
reaches the meningeal microcirculation, causes acute
It is heterogeneously FLAIR hyperintense (Fig. 4.4.2), with a small amount of or chronic meningitis, parenchymal disease is less
intralesional hemorrhage seen as susceptibility on B0 DWI sequence (Fig. 4.4.3, common but also occurs.
curved arrow). Post-contrast axial T1 reveals mild patchy peripheral b Hyphae (Aspergillus and Mucor): large size, invades
enhancement (Fig. 4.4.4). Also note additional smaller lesions in the posterior
right and left frontal lobes, best seen on FLAIR (Fig. 4.4.2). larger vessels, causes parenchymal disease and
vasculopathy.
r Aspergillosis:
Patient 2
Figures 4.4.5–4.4.7 A large infiltrative lesion involves the right frontal and b Most common fungal infection, especially in
temporal lobes and the basal ganglia. It is heterogeneous on T2 (Fig. 4.4.5). Part immunocompromised patients (solid organ and
of the lesion in the right frontal lobe demonstrates avid enhancement with a
small necrotic center (Fig. 4.4.6, black arrow), while part of the lesion in the right
hematopoietic stem-cell transplants, AIDS, diabetes).
frontal and temporal operculum shows intense perivascular enhancement b Route of spread: hematogenous dissemination from
(Fig. 4.4.6, white arrows) on post-contrast sagittal T1. This lesion was biopsy- pulmonary infection, or direct spread from
proven to be a mycetoma caused by Bipolaris sp., a rare fungus belonging to
the phaeohyphomycosis family. This patient decompensated clinically a few
sinonasal/orbital infection.
days after surgery. Repeat MRI post-contrast sagittal T1 image shows a new b Causes infectious vasculopathy: initially acute
lobular enhancing lesion near the vertebrobasilar junction (Fig. 4.4.7, arrow), hemorrhagic infarction, and later an infectious
representing a mycotic aneurysm. Note the non-enhancing T1 isointense
material (∗ ) in the prepontine and premedullary cistern consistent with acute
cerebritis, occasionally evolving into an
subarachnoid hemorrhage from aneurysm rupture. Autopsy confirmed fibrin abscess. Usually in anterior or middle cerebral artery
clot within the basilar artery with acute inflammation and fungal vascular wall territory. Involvement of basal ganglia, thalami, and
invasion.
corpus callosum are characteristic.
b Three imaging patterns:
Patient 3 Multiple cortical and subcortical areas of decreased
Figures 4.4.8–4.4.12 Multiple intracranial lesions. The largest right periatrial CT attenuation or T2 hyperintensity, with or
lesion is T2 hyperintense with surrounding vasogenic edema (Fig. 4.4.8). The without hemorrhage seen on T1 or GRE.
lesion has a hemorrhagic ring, which shows slight hyperintensity on axial T1 Multiple ring-enhancing abscesses.
(Fig. 4.4.9) and susceptibility on gradient-recall sequence (Fig. 4.4.10). Note the
peripheral ring enhancement on sagittal post-contrast T1 (Fig. 4.4.11) and Dural enhancement with adjacent enhancing lesions
restricted diffusion (Fig. 4.4.12), consistent with an abscess. in the paranasal sinuses, involved calvarium, or
optic nerve/orbital fat. The pattern represents direct
extension of sinonasal disease.
Diagnosis r Treatment: antifungal therapy, surgery.
Cerebral fungal infection.
Further reading
DeLone DR, Goldstein RA, Petermann G, Mathur M, Johnson CE, Sze G. Fungal
Salamat MS, Miles JM, Knechtle SJ, et al. infections of the central nervous system.
Disseminated aspergillosis involving the Neuroimaging Clin N Am 2012; 22(4):
brain: distribution and imaging 609–632.
characteristics. AJNR Am J Neuroradiol
1999; 20(9): 1597–1604.
101
Section 1: Brain
Case 4.5
102
Diagnosis
Herpes encephalitis.
r Other viral encephalitis.
r Limbic encephalitis.
r Status epilepticus.
r Infiltrative neoplasm.
r Ischemic infarction.
Key points
r Most common viral encephalitis.
r Herpes simplex virus 1 (HSV-1) is the most common
causative agent, frequently due to reactivation of latent
infection in trigeminal ganglion.
r Although brain biopsy is the definitive diagnosis, CSF PCR
offers rapid diagnosis.
r Imaging:
Figure 4.5.5 b Characteristic involvement of limbic system (medial
temporal lobe, inferior frontal lobe, insula, and
cingulate gyrus), frequently bilateral but asymmetric.
History Basal ganglia are usually spared.
60-year-old woman presents with fever, altered mental b CT: negative in early phase. Hypodensities without or
status, and seizure. with hemorrhage in later phase.
b MRI:
FLAIR/T2: hyperintensity of limbic system.
Findings
DWI: restricted diffusion.

Figure 4.5.1 NECT shows hypodensities of left temporal lobe, insula, inferior T1/GRE: T1 hyperintensity and blooming in
frontal lobe, and right insula.
hemorrhagic lesion.
Figures 4.5.2, 4.5.3 Axial FLAIR demonstrates hyperintensity of bilateral
Post-contrast T1: absent or mild patchy
temporal lobes, left frontal lobe, and bilateral insula. enhancement in early disease. Gyriform cortical
enhancement as disease progresses.
Figure 4.5.4 Coronal post-contrast T1 demonstrates gyriform enhancement
of left temporal lobe and patchy enhancement of bilateral insula and left r Treatment: intravenous acyclovir should be initiated before
thalamus. PCR confirmation.
Figure 4.5.5 The left temporal lobe shows diffusion restriction.
Further reading
Baringer JR. Herpes simplex infections of
the nervous system. Neurol Clin 2008;
26(3): 657–674, viii.
103
Section 1: Brain
Case 4.6
104
r Encephalitis by other infectious agents (HSV, pyogenic,
mycobacterial, and fungal infections, etc.).
r ADEM.
r Infiltrative neoplasms.
r Autoimmune encephalitis, including paraneoplastic
syndrome.
r Toxic/metabolic encephalopathy.
r Vasculitis.
Key points
r Definition: encephalitis is a multifocal or diffuse
inflammatory process of brain parenchyma, commonly
although not exclusively caused by viral infection.
b If meninges are involved, it is termed
meningoencephalitis.
b If spinal cord is involved, it is termed
myeloencephalitis.
r Etiology:
b Over 100 viruses have been associated with acute CNS
Figure 4.6.5 (patient 3) infections.
b Among these, herpes simplex virus (HSV) is the most
common cause of sporadic viral encephalitis.
b Other than HSV, viral encephalitis in the United States
History is most often due to arthropod-borne virus (such as
Patient 1: 12-year-old girl presents with acute onset of fever eastern equine, western equine, St. Louis, and West Nile
and confusion. viruses).
Patient 2: 21-year-old female presents with 1-week history b Enterovirus is a common cause of aseptic meningitis,
of headache, fever, neck stiffness, and seizure activity. although it rarely causes encephalitis.
Patient 3: 54-year-old man presents with fever, altered b Viral encephalitis can be either primary infectious or
mental status, and seizure. postinfectious (ADEM).
r Clinical presentations:
Findings b Aseptic meningitis: nonspecific, including fever,
headache, nausea and vomiting, photophobia, and stiff
Patient 1
neck.
Figure 4.6.1 Axial FLAIR shows asymmetric hyperintensity of bilateral frontal b Encephalitis: abnormalities in brain function, including
lobes and basal ganglia.
altered mental status, motor or sensory deficits,
personality changes, seizures, etc.
Patient 2 r Diagnosis: based on clinical presentation, serology, CSF
Figures 4.6.2, 4.6.3 Axial FLAIR (Fig. 4.6.2) demonstrates hyperintensity in analysis, and imaging findings. CSF analysis typically
the bilateral medial temporal lobes and cerebral peduncles. There is
hyperintensity along the ventricular margin. Diffusion restriction is seen in the shows a lymphocytic pleocytosis and elevated protein. A
corresponding areas on DWI sequence (Fig. 4.6.3). Also note the cortical edema specific causative viral agent is often not identified.
in the bilateral temporal and occipital lobes (Fig. 4.6.2). r Imaging:
b Imaging findings are often nonspecific and difficult to
Patient 3 distinguish from other disease processes.
b MRI is more sensitive than CT.
Figures 4.6.4, 4.6.5 Axial FLAIR demonstrates confluent hyperintensity in
the bilateral medial temporal lobes, hypothalamus, midbrain (Fig. 4.6.4), dorsal b T2/FLAIR: scattered or confluent hyperintensity
pons, and cerebellum (Fig. 4.6.5). involving the cortex, white matter, deep gray nuclei,
brainstem, and cerebellum. Certain viruses tend to
affect specific locations:
Diagnosis HSV: limbic system.
Nonherpetic viral encephalitis. East equine encephalitis: basal ganglia and thalami.
105
Section 1: Brain
St. Louis encephalitis: substantia nigra. b DWI: acute lesions may show diffusion restriction, and
West Nile virus: basal ganglia, thalami, medial often precede the findings on T2/FLAIR.
temporal lobes, brainstem, cerebellum, spinal cord, r Treatment:
and cauda equina. b Mostly supportive. No specific therapies for most CNS
Japanese encephalitis: basal ganglia and thalami.
b T1: isointense or hypointense. Contrast enhancement is viral infections.
b However, empiric treatment for HSV-1 infection with
variable.
b FLAIR is more sensitive than post-contrast T1 to detect acyclovir should always be initiated as soon as possible
if the patient has encephalitis without apparent
meningitis.
b T2/GRE: may show patchy hemorrhages. explanation.
Further reading
Gupta RK, Jain KK, Kumar S. Imaging of
nonspecific (nonherpetic) acute viral
infections. Neuroimaging Clin N Am
2008; 18(1): 41–52; vii.
106
Case 4.7
107
Section 1: Brain
r Vasculitis.
r Sarcoidosis.
r Lymphoma.
r Metastasis.
Key points
r Lyme disease is a tick-borne illness caused by spirochete
Borrelia burgdorferi, which is transmitted to humans by the
infected Ixodes tick bites.
r Preferentially affects skin (with a characteristic skin rash –
erythema migrans), joint, heart, and nervous system.
Approximately 10–15% of patients with untreated disease
will develop neurological involvement (LNB), most
commonly including lymphocytic meningitis/
meningoencephalitis, cranial neuropathy, and
radiculoneuritis.
r The diagnosis of LNB requires a history of tick exposure,
manifestations of nervous system disease, and supportive
laboratory data, which includes positive Lyme serologies.
CSF pleocytosis (typically lymphocytic and/or monocytic),
moderately elevated protein, and normal glucose
Figure 4.7.5 concentration are often seen in patients with acute
LNB. Detection of Lyme antibodies or a positive PCR
of a CSF sample are the most reliable indicators of CNS
infection.
History r Imaging:
14-year-old girl presents with headache and multiple cranial
b Brain MRI is frequently normal or nonspecific in
nerve palsies.
patients suspected of LNB.
b In positive cases, multiple periventricular and/or
Findings subcortical T2/FLAIR hyperintense lesions can be
Figures 4.7.1–4.7.3 Post-contrast axial T1 images demonstrate symmetric identified, which can mimic the appearance of multiple
enhancement of bilateral cranial nerves III (Fig. 4.7.1, arrows), V (Fig. 4.7.2, sclerosis. Lesions may also be seen in basal ganglia or
arrows), and VII (Fig. 4.7.3, arrows).
brainstem.
Figure 4.7.4 Axial T2 image shows hyperintense lesions involving the right
b Multifocal parenchymal or meningeal enhancement
temporal periventricular white matter (curved arrow), genu (arrow), and may occur in cases of meningoencephalitis.
splenium (block arrow) of the corpus callosum. b Cranial nerve enhancement can be seen, which can be
Figure 4.7.5 One lesion in the genu (arrow) of the corpus callosum shows either symmetric or asymmetric. The seventh nerve
post-contrast enhancement. enhancement is the most common, while third, fifth,
and lower cranial nerves can also be involved.
b Myelopathy is rare and is characterized by diffuse or
Diagnosis multifocal T2 hyperintense cord lesions.
Lyme neuroborreliosis (LNB). b Spinal radiculitis manifests as nerve root enhancement
on post-contrast sequences.
Differential diagnosis b Ocular complications most commonly include optic
r Demyelinating disease (multiple sclerosis, ADEM). neuritis and uveitis.
r Other infectious meningitis/meningoencephalitis. r Treatment: antibiotics.
Further reading
Hildenbrand P, Craven DE, Jones R,
Nemeskal P. Lyme neuroborreliosis:
manifestations of a rapidly emerging
zoonosis. AJNR Am J Neuroradiol 2009;
30(6): 1079–1087.
108
Case 4.8
109
Section 1: Brain

r ADEM: typically multifocal and asymmetric on imaging,
Patient 1: 16-year-old male with recent upper respiratory
with additional involvement of cerebrum, brainstem, and
infection presents with fever, headache, and ataxia.
spinal cord.
Patient 2: 3-year-old girl presents with fever, altered mental r Ischemic infarction from bilateral vertebral dissections or
status, ataxia, and mutism. vasculitis.
r Global hypoxic/ischemic injury: isolated cerebellar injury
Findings is rare.
r Drug intoxication, such as anticonvulsants, alcohol,
Patient 1 opiates, etc.
Figure 4.8.1 NECT shows hypodensity in bilateral cerebellar hemispheres r PRES: isolated cerebellar involvement is rare.
causing compression of fourth ventricle. r Paraneoplastic syndrome or other autoimmune
Figure 4.8.2 Axial FLAIR confirms bilateral cerebellar edema with effacement encephalitis.
of fourth ventricle. Additional images demonstrate mild hydrocephalus. r Mitochondrial diseases and inborn errors of metabolism
Post-contrast T1 (not shown) does not show enhancement.
are additional considerations in young children.
Figure 4.8.3 Axial DWI reveals normal diffusion signal in the cerebellum,
consistent with vasogenic edema. Key points
r Acute cerebellitis is a postinfectious or postvaccinal
Patient 2 disorder that predominantly affects children and young
Figure 4.8.4 Axial FLAIR shows mildly increased signal intensity in bilateral
adults. Multiple organisms, predominantly viral, have been
middle cerebellar peduncles. implicated in the pathogenesis.
r The typical clinical presentation consists of headache,
Figures 4.8.5, 4.8.6 Axial DWI (Fig. 4.8.5) and ADC (Fig. 4.8.6) demonstrate altered mental status, fever, and cerebellar symptoms.
diffusion restriction in bilateral middle cerebellar peduncles, consistent with
cytotoxic edema. No enhancement is seen on the post-contrast T1 (not
r The CSF analysis typically shows mild lymphocytic
shown). The diffusion abnormality resolved on the follow-up MRI 5 days later pleocytosis.
after a course of intravenous methylprednisolone. r MRI is the imaging modality of choice, as CT is not
sensitive. The typical imaging appearance consists of
bilateral or, less frequently, unilateral cerebellar
Diagnosis hemispheric cortical edema and T2 hyperintensity, as
Acute cerebellitis. demonstrated in patient 1. In severe cases, cerebellar
110
edema may cause tonsillar herniation and obstructive reported. On MRI, these patients typically have transient
hydrocephalus. Leptomeningeal enhancement along the diffusion restriction in the middle cerebellar peduncles
cerebellar folia can also be seen, and cerebellar atrophy and deep cerebellar nuclei, which resolves shortly
may develop in the chronic stage. afterwards, as demonstrated in patient 2. Cerebellar
r A variant imaging appearance of cerebellitis has been hemisphere can be spared in some of these
reported, mostly in children with rotavirus cerebellitis, patients.
although other infectious agents have also been r Treatment: corticosteroid.
Further reading
De Bruecker Y, Claus F, Demaerel P, Ballaux Takanashi J, Miyamoto T, Ando N, Kubota
F, Sciot R, Lagae L, et al. MRI findings in T, Oka M, Kato Z, et al. Clinical and
acute cerebellitis. Eur Radiol 2004; 14(8): radiological features of rotavirus
1478–1483. cerebellitis. AJNR Am J Neuroradiol 2010;
31(9): 1591–1595.
111
Section 1: Brain
Case 4.9
Figure 4.9.1
Figure 4.9.2
112
r Based on the location of involvement, NCC can be

History
classified into subarachnoid/cisternal (most common),
5-year-old Mexican immigrant presents with seizure.
parenchymal, intraventricular, and spinal forms.
r Parenchymal NCC is frequently observed near the
Findings gray–white junction on cross-sectional imaging. Some
Figure 4.9.1 NECT shows low density in the subcortical white matter of the authors suggest that this actually represents subarachnoid
left occipital lobe, consistent with vasogenic edema. cysticercosis located in deep sulci or perivascular spaces
that are sealed off by inflammation, and not true intra-axial
Figures 4.9.2, 4.9.3 Axial T2 (Fig. 4.9.2) and FLAIR (Fig. 4.9.3) MRI images
demonstrate a thin-walled cystic lesion in the left occipital lobe with lesions, although parenchymal NCC is still considered as a
surrounding edema. There is a central dot within the cystic lesion, representing distinct form of NCC.
a scolex. r Based on imaging and pathological findings, parenchymal
Figure 4.9.4 Post-contrast axial T1 demonstrates peripheral enhancement of NCC can be divided into four stages: vesicular, colloidal
the cystic lesion. Note the faint enhancement of the scolex as well. vesicular, granular nodular, and calcified nodular forms.
b In the vesicular stage, the larva is alive and there is no
inflammation. The lesion typically presents as a cyst
Diagnosis of CSF-like signal without surrounding edema or
Parenchymal neurocysticercosis (NCC), colloidal vesicular enhancement. In many cases, the scolex can be seen as
stage. an eccentric nodule within the cyst (“hole with dot”
sign), which allows a definitive imaging diagnosis.
Differential diagnosis b In the colloidal vesicular stage, the larva degenerates
r Miscellaneous infections (pyogenic abscess, septic emboli, and inflammation develops. The signal of the cyst may
tuberculosis, fungal, or other parasitic infections). be distinct from CSF and becomes hyperintense on
r Neoplasms (primary or metastatic). T1, T2, and FLAIR due to proteinaceous material.
Perilesional edema and peripheral contrast
Key points enhancement are evident.
b In the granular nodular stage, the larva is dead. The
r NCC is the most common parasitic infection affecting the
perilesional edema and enhancement gradually
CNS, caused by the larva of the tapeworm Taenia solium.
subside.
Humans are usually infected via the fecal–oral route and b In the nodular calcified stage, the lesion is completely
serve as the intermediate host.
r The clinical presentations are variable and nonspecific, calcified.
including seizure and headache (most common), r Treatment: antiparasitic agents, antiepileptics,
hydrocephalus, and less commonly meningitis. corticosteroid, surgery.
Further reading
Kimura-Hayama ET, Higuera JA, Noujaim SE, Rossi MD, Rao SK, Cacciarelli
Corona-Cedillo R, Chavez-Macias L, AA, Mendonca RA, Wang AM, et al. CT
Perochena A, Quiroz-Rojas LY, et al. and MR imaging of neurocysticercosis.
Neurocysticercosis: radiologic– AJR Am J Roentgenol 1999; 173(6):
pathologic correlation. Radiographics 1485–1490.
2010; 30(6): 1705–1719.
113
Section 1: Brain
Case 4.10
114
Figures 4.10.2, 4.10.3 Axial FLAIR (Fig. 4.10.2) demonstrates a thin-walled

cyst of CSF signal within the fourth ventricle and a small amount of edema in
the surrounding brain parenchyma. Note the FLAIR hyperintense tubular
structure within the cyst, which is partially calcified on the gradient-recalled
echo (GRE) sequence (Fig. 4.10.3), consistent with a scolex.
Figures 4.10.4, 4.10.5 Pre-contrast axial T1 (Fig. 4.10.4) reveals isointensity

of the scolex to the brain parenchyma. Neither the cyst nor the scolex shows
perceptible enhancement on the post-contrast sagittal T1 (Fig. 4.10.5),
compatible with cysticercosis at the vesicular stage.
Diagnosis
Ventricular neurocysticercosis (NCC).
r Other cysts within the fourth ventricle, such as epidermoid
cyst, arachnoid cyst, ependymal cyst.
r Trapped fourth ventricle from prior infection or
subarachnoid hemorrhage.
r Cystic tumor with “nodule in a cyst” appearance, such as
hemangioblastoma.
Figure 4.10.5 Key points

r Intraventricular neurocysticercosis is the third most
common form of NCC after subarachnoid/cisternal and
parenchymal.
r The fourth ventricle is the most common site, followed by
History the lateral ventricles, third ventricle, and aqueduct.
34-year-old man presents with progressive dizziness and r Isolated ventricular NCC without subarachnoid or
headache for 3 months.
parenchymal involvement is common, and has been
reported in one-third of cases.
r It frequently presents clinically with symptoms related to
Findings hydrocephalus and ventriculitis such as the current case.
r Definitive imaging diagnosis can be made if a scolex is
Figure 4.10.1 NECT shows cystic expansion of the fourth ventricle, which
contains a slightly hyperdense central focus. There is also mild dilation of the identified, which is usually more obvious on T1 and
right temporal horn, consistent with hydrocephalus. FLAIR. See Case 4.9 for further discussion.
Further reading
Kimura-Hayama ET, Higuera JA, Noujaim SE, Rossi MD, Rao SK, Cacciarelli
Corona-Cedillo R, Chavez-Macias L, AA, Mendonca RA, Wang AM, et al. CT
Perochena A, Quiroz-Rojas LY, et al. and MR imaging of neurocysticercosis.
Neurocysticercosis: radiologic– AJR Am J Roentgenol 1999; 173(6):
pathologic correlation. Radiographics 1485–1490.
2010; 30(6): 1705–1719.
115
Section 1: Brain
Case 4.11
116
History 1 000 000. It includes sporadic, familial, iatrogenic, and

variant forms. The vast majority of CJD cases are
Patient 1: 56-year-old man presents with rapidly progressive
sporadic.
dementia. r The clinical presentations include rapidly progressive
Patient 2: 68-year-old man presents with progressive mental deterioration, often with behavioral abnormalities,
dementia and myoclonus jerks. and myoclonus. Extrapyramidal signs such as hypokinesia
and cerebellar manifestations are also common. Patients
Findings usually die within 1 year of symptom onset.
r While brain biopsy/autopsy remains the gold-standard test
Patient 1
for diagnosis, typical clinical presentations, MRI findings,
Figures 4.11.1, 4.11.2 Axial DWI (Fig. 4.11.1) shows restricted diffusion of
bilateral caudate nuclei, putamina, frontal and insular cortices, with characteristic EEG findings of periodic bi- or triphasic
corresponding hyperintensity on axial FLAIR images (Fig. 4.11.2). sharp wave complexes, as well as identification of CSF
14–3–3 and tau proteins, are sufficient to establish the
Patient 2 diagnosis in most cases.
Figures 4.11.3, 4.11.4 Axial DWI (Fig. 4.11.3) shows restricted diffusion in r Imaging: MRI is highly sensitive and specific for the
the inferior frontal, temporal, and occipital cortices, worse on the right, with
corresponding hyperintensity on axial FLAIR (Fig. 4.11.4).
diagnosis of CJD. It predominantly involves the gray
matter and is characterized by progressive FLAIR/T2
hyperintensity and diffusion restriction of basal ganglia
Diagnosis (caudate and putamen ⬎ globus pallidus), thalamus and
Creutzfeldt–Jakob disease (CJD). cerebral cortex. Primary sensorimotor cortex is relatively
spared. In some cases, basal ganglia are spared. The
Differential diagnosis abnormal signal may be unilateral or bilateral, focal or
r Viral encephalitis. diffuse, and asymmetrical or symmetrical. The affected
r Hypoxic–ischemic injury. areas typically do not demonstrate post-contrast
r Acute hepatic encephalopathy, extrapontine osmotic enhancement or mass effect, as opposed to other types of
myelinolysis, other metabolic diseases, etc. acute encephalitis. In variant CJD (“mad cow disease”),
r Drug toxicity. characteristic T2 and diffusion restriction in the pulvinar
r Mitochondrial disease. nuclei of the thalami, termed the “pulvinar sign,” is
pathognomonic.
Key points r Treatment: no effective treatment.
r CJD is the most common human prion diseases, although
it is still quite rare with an incidence of approximately 1 in
Further reading
Kallenberg K, Schulz-Schaeffer WJ, Jastrow Zeidler M, Sellar RJ, Collie DA, Knight R,
U, Poser S, Meissner B, Tschampa HJ, Stewart G, Macleod MA, et al. The
et al. Creutzfeldt–Jakob disease: pulvinar sign on magnetic resonance
comparative analysis of MR imaging imaging in variant Creutzfeldt–Jakob
sequences. AJNR Am J Neuroradiol 2006; disease. Lancet 2000; 355(9213):
27(7): 1459–1462. 1412–1418.
117
Section 1: Brain
Case 4.12
History
24-year-old man with no significant past medical history
presents with word-finding difficulty and right-sided
weakness, and later develops psychosis, agitation, fever, and
seizure.
Findings
Figures 4.12.1, 4.12.2 Axial DWI (Fig. 4.12.1) and FLAIR (Fig. 4.12.2) MRI
images show multiple foci of acute infarctions of left cerebral hemisphere
at the ACA/MCA border zone (circle), with restricted diffusion (Fig. 4.12.1)
and FLAIR hyperintensity (Fig. 4.12.2). Also note FLAIR hyperintensity in the
cortical sulci (arrows), which reflects proteinaceous material within the
CSF.
Figure 4.12.3 CTA coronal MIP image demonstrates severe stenosis of

bilateral ICA terminus and origins of bilateral A1 and M1 segments (arrows).
Bilateral ACA and MCA also demonstrate luminal irregularity, with alternating
segments of stenosis and dilation, consistent with vasculitis.
Diagnosis
Figure 4.12.3 Meningovascular neurosyphilis.
118
Differential diagnosis etc. CSF analysis typically shows pleocytosis and elevated
r Meningovascular type: CNS vasculitis from other protein in cases of neurosyphilis. Although reactive
CSF-VDRL establishes the diagnosis, it may be falsely
infectious or noninfectious etiologies, stroke from other
negative in many patients.
sources (hypercoagulability, embolic). r Neuroimaging:
r Gummatous type: neoplasms, other infections,
inflammatory diseases such as sarcoidosis. b In many patients, neuroimaging appearance is either
r Limbic encephalitis: paraneoplastic or autoimmune normal or nonspecific such as cerebral atrophy or white
encephalitis, HSV encephalitis. matter lesions.
b As with other meningitides, syphilitic meningitis can
Key points lead to arteritis of the brain or spinal cord and result
r Neurosyphilis refers to CNS infection by the spirochete in ischemic infarctions. This form of neurosyphilis
Treponema pallidum, and it can occur at any stage after the (meningovascular) may present as an ischemic stroke
initial infection. There has been a dramatic rise in the in a young person such as the current case, and has
incidence of syphilis and neurosyphilis in the age of been reported to affect about 25% of patients in one
HIV/AIDS endemics. series. CTA, MRA, or catheter angiography would
r The major clinical types of symptomatic neurosyphilis show findings of vasculitis with focal or diffuse arterial
include syphilic meningitis, meningovascular (a narrowing, occlusion, or dilation.
b Gummas are another manifestation of
combination of meningitis and arteritis), general paresis,
and tabes dorsalis. The latter two are a “tertiary form” of meningovascular neurosyphilis, and typically manifest
neurosyphilis and are rarely seen in the antibiotics era. as mass lesions with nodular or ring enhancement with
Ocular or otologic forms can also occur. adjacent edema and mass effect.
r Laboratory tests for syphilis include nontreponemal tests b Rarely, neurosyphilis can presents as limbic
such as venereal disease research laboratory (VDRL) and encephalitis on MRI with FLAIR/T2 hyperintensity in
rapid plasma reagin (RPR), as well as treponemal tests such the medial temporal lobes.
as fluorescent treponemal antibody absorption (FTA-ABS), r Treatment: antibiotics (penicillin G etc.) 10–14 days.
Further reading
Brightbill TC, Ihmeidan IH, Post MJ, Berger
JR, Katz DA. Neurosyphilis in
HIV-positive and HIV-negative patients:
neuroimaging findings. AJNR Am J
Neuroradiol 1995; 16(4): 703–711.
119
Section 1: Brain
Case 4.13
Figure 4.13.2
Figure 4.13.1
120
r Lymphoma: both toxoplasmosis and lymphoma commonly
affect AIDS patients and can have identical imaging
appearance.
b Presumed toxoplasmosis lesions are usually treated
empirically and followed with imaging in 2–4 weeks.
Lack of improvement suggests an alternative diagnosis.
b Advanced imaging may be beneficial in some patients
to differentiate these two entities: lymphoma will be
positive in thallium-201 SPECT and FDG-PET, while
toxoplasmosis will be negative. MR perfusion typically
shows an elevated relative cerebral blood volume
(rCBV) in lymphoma but not in toxoplasmosis.
r Other differential considerations include cryptococcosis,
tuberculosis, pyogenic abscess, systemic metastasis, etc.
Key points
r Cerebral toxoplasmosis is the most common opportunistic
CNS infection in AIDS patients. It results from
reactivation of a latent infection by Toxoplasma gondii, an
Figure 4.13.5 intracellular protozoan.
r Clinical presentations are nonspecific, including fever,
malaise, headache, personality changes, seizure, focal
neurological symptoms, etc.
History r Imaging: although occasionally presenting as a solitary
34-year-old HIV-positive woman presents with left-sided lesion, cerebral toxoplasmosis is commonly multifocal,
weakness. preferentially affecting the basal ganglia, thalamus, and
corticomedullary junction.
Findings b On CT, it manifests as areas of low attenuation.
Figure 4.13.1 NECT shows an area of low density in right midbrain, thalamus, b On MRI, toxoplasmosis abscesses are typically T2
and posterior limb of right internal capsule. hypo- or isointense lesions surrounded by vasogenic
Figure 4.13.2 Axial T2 demonstrates an area of hypointensity in the right
edema. Post-contrast T1 images reveal nodular or ring
midbrain, surrounded by hyperintense edema. enhancement. An eccentric nodule on the
post-contrast imaging (“target sign”), is highly
Figures 4.13.3, 4.13.4 Axial (Fig. 4.13.3) and sagittal (Fig. 4.13.4) suggestive of the diagnosis but is only seen in a
post-contrast T1 images demonstrate faint enhancement.
minority of patients. On DWI, toxoplasmosis lesions
Figure 4.13.5 Axial DWI shows no diffusion abnormality. can demonstrate a wide range of signal characteristics,
from mild restricted diffusion to facilitated diffusion.
MR spectroscopy may show increased lipids/lactate
Diagnosis and reduced choline and NAA but is nonspecific.
Cerebral toxoplasmosis. r Treatment: pyrimethamine, sulfadiazine, clindamycin.
Further reading
Schroeder PC, Post MJ, Oschatz E, Stadler distinguishing central nervous system nervous system infections associated with
A, Bruce-Gregorios J, Thurnher MM. toxoplasmosis from lymphoma. human immunodeficiency virus
Analysis of the utility of Neuroradiology 2006; 48(10): 715–720. infection: radiologic–pathologic
diffusion-weighted MRI and apparent Smith AB, Smirniotopoulos JG, Rushing EJ. correlation. Radiographics 2008; 28(7):
diffusion coefficient values in From the archives of the AFIP: central 2033–2058.
121
Section 1: Brain
Case 4.14
122

Patient 1: 63-year-old man with rheumatoid arthritis on r Tuberculosis.
steroid presents with altered mental status and fever. r Toxoplasmosis.
r Lymphoma.
Patient 2: 32-year-old HIV-positive man presents with r Sarcoidosis.
headache and fever. r Leptomeningeal metastasis, etc.
Findings Key points

Patient 1 r Cryptococcus neoformans is a ubiquitous encapsulated
Figures 4.14.1, 4.14.2 Sagittal and coronal post-contrast T1 demonstrate fungus found in soil contaminated by bird feces. CNS
nodular leptomeningeal enhancement in the basilar cistern, along the
cerebellar folia and sylvian fissures.
infection occurs by means of hematogenous dissemination
from a primary pulmonary focus.
Figure 4.14.3 Axial T2 shows marked edema in the tegmentum of midbrain r It is the most common causative agent of CNS fungal
(∗ ) and bilateral temporal lobes (arrows). Note dilated temporal horns with infection and is the third most common cause of CNS
transependymal CSF edema, consistent with hydrocephalus.
infection in AIDS patients, behind HIV and Toxoplasma.
Figure 4.14.4 Axial DWI shows restricted diffusion in the corresponding r Most patients with cryptococcal infection are
regions, in keeping with cytotoxic edema. immunocompromised. Besides HIV/AIDS, other
predisposing factors include steroid therapy, solid-organ
transplantation, cancer (particularly hematologic
Patient 2 malignancy), and other conditions such as sarcoidosis.
Figure 4.14.5 Axial FLAIR demonstrates hyperintensity of bilateral basal r Most patients initially present with nonspecific signs and
ganglia, representing gelatinous pseudocysts in the dilated perivascular spaces. symptoms of meningoencephalitis, including fever,
Figure 4.14.6 Axial post-contrast T1 shows minimal enhancement.
headache, neck stiffness, photophobia, lethargy,
personality change, etc.
r Lumbar puncture typically shows elevated opening
pressure. A CSF india ink preparation may demonstrate
Diagnosis typical encapsulated yeasts. A positive cryptococcal
CNS cryptococcosis. polysaccharide antigen in the CSF or serum also strongly
123
Section 1: Brain
suggests the presence of infection, and the definitive degree of meningeal enhancement varies, and it may be
diagnosis can be made by positive culture from CSF. absent in immunocompromised patients since they are
r The imaging findings consist of meningoencephalitis, unable to mount an effective inflammatory response.
cryptococcomas, gelatinous pseudocysts, and b Cryptococci also tend to fill and dilate the perivascular
hydrocephalus. spaces of the basal ganglia, thalami, periventricular
b Hydrocephalus, either communicating or white matter, midbrain, and cerebellum, which appear
noncommunicating, is the most frequent finding. on MRI as characteristic T2/FLAIR hyperintense,
b Meningoencephalitis results in T2 hyperintensity non-enhancing or minimally enhancing “gelatinous
within the region of parenchymal involvement. The pseudocysts.”
Further reading
Harris DE, Enterline DS. Neuroimaging of Tien RD, Chu PK, Hesselink JR, Duberg A,
AIDS. I. Fungal infections of the central Wiley C. Intracranial cryptococcosis in
nervous system. Neuroimaging Clin N Am immunocompromised patients: CT and
1997; 7(2): 187–198. MR findings in 29 cases. AJNR Am J
Neuroradiol 1991; 12(2): 283–289.
124
Case 4.15
125
Section 1: Brain
History Key points

53-year-old HIV-positive man presents with altered mental r PML is a CNS demyelinating disease caused by
status. reactivation of JC polyomavirus. It predominantly affects
the HIV/AIDS population, but can also occur in patients
with hematologic/oncologic/rheumatologic conditions, in
Findings the setting of organ transplantation, chronic immuno-
suppression, or treatment with monoclonal antibodies.
Figure 4.15.1 Axial FLAIR shows asymmetric hyperintensity in the bilateral r The clinical presentations of PML are nonspecific and vary
parietal periventricular white matter, right greater than left. The abnormality on
the right extends to the subcortical white matter with scalloping of the greatly depending on the involved locations. The diagnosis
gray–white junction. No significant mass effect. is usually suggested by neuroimaging, and confirmed by
detection of CSF JC virus DNA by PCR, or occasionally
Figure 4.15.2 Axial DWI demonstrates restricted diffusion along the
peripheral aspects of the white matter lesions.
through brain biopsy in difficult cases.
r The characteristic MRI appearance is multifocal,
Figures 4.15.3, 4.15.4 Pre- (Fig. 4.15.3) and post-contrast (Fig. 4.15.4) axial asymmetric, confluent T2 hyperintense and T1
T1 images show hypointensity in the corresponding areas but no hypointense lesions in the subcortical and periventricular
enhancement.
white matter, with diffusion restriction along the margins.
r PML most frequently affects the parieto-occipital lobes,
followed by the frontal lobes, but infratentorial white
Diagnosis matter especially middle cerebellar peduncles, brainstem,
Progressive multifocal leukoencephalopathy (PML). gray matter, or even spinal cord can also be affected.
r Absence of inflammation is considered as a histopathologic
hallmark of classic PML (cPML), which explains the lack of
enhancement and mass effect.
Differential diagnosis r Recently, inflammatory PML (iPML) has been increasingly
r In HIV-positive patients, the main differential diagnosis is recognized, mostly in the setting of immune reconstitution
HIV/AIDS encephalitis. inflammatory syndrome (IRIS) among HIV patients
r In patients with negative or unknown HIV status, the receiving HAART therapy. On imaging, iPML lesions are
differential diagnosis is broad and includes PRES, viral characterized by mass effect, vasogenic edema, and
encephalitis, ADEM, vasculitis, tumefactive demyelination, enhancement distinguishing it from cPML.
lymphoma, etc. r Treatment: HAART.
Further reading
Bag AK, Cure JK, Chapman PR, Roberson
GH, Shah R. JC virus infection of the
brain. AJNR Am J Neuroradiol 2010;
31(9): 1564–1576.
126
Case 4.16
127
Section 1: Brain
Figure 4.16.5 Axial post-contrast T1 of the lumbar spine reveals smooth

enhancement of cauda equina nerve roots, consistent with polyradiculitis.
Diagnosis
Cytomegalovirus infection.
r Bacterial meningitis/ventriculitis.
r Cryptococcal meningitis.
r HIV encephalitis.
r Toxoplasmosis.
r Lymphoma.
Key points
r Cytomegalovirus (CMV) infection is an uncommon but
serious complication in immunocompromised patients. It
occurs in up to 2% of patients with AIDS, primarily in
those with a CD4 T-cell count below 50/mm3 . It may result
either from reactivation of latent infection, or as a primary
infection acquired through organ or bone-marrow
Figure 4.16.5
transplant from seropositive donors.
r The manifestations of CMV neurological disease are
variable, including ventriculitis/ependymitis,
History meningoencephalitis, chorioretinitis, and radiculomyelitis.
36-year-old man with AIDS presents with fever, multiple The detection of CMV DNA by polymerase chain reaction
cranial nerve palsies, and lower extremity weakness. (PCR) or CMV antigen in CSF is highly sensitive and
specific for the diagnosis.
Findings r The imaging appearance of CMV infection is often normal
or nonspecific. Ventriculitis may manifest as ependymal
Figures 4.16.1–4.16.3 Post-contrast axial (Fig. 4.16.1), coronal (Fig. 4.16.2),
and sagittal (Fig. 4.16.3) T1 MRI demonstrate thin, smooth ependymal
enhancement, debris within the ventricles, and
enhancement. There is mild ventriculomegaly, especially at the temporal horns ventriculomegaly. T2/FLAIR images may demonstrate
(Fig. 4.16.2). ependymal/subependymal and periventricular T2
hyperintensity. In rare cases, it may manifest as a
Figure 4.16.4 Axial FLAIR shows cortical and periventricular hyperintensity
from edema. ring-enhancing or space-occupying lesion.
Further reading
Fink KR, Thapa MM, Ishak GE, Pruthi S. Smith AB, Smirniotopoulos JG, Rushing EJ.
Neuroimaging of pediatric central From the archives of the AFIP: central
nervous system cytomegalovirus nervous system infections associated with
infection. Radiographics 2010; 30(7): human immunodeficiency virus
1779–1796. infection: radiologic–pathologic
correlation. Radiographics 2008; 28(7):
2033–2058.
128
Section 1 Brain
Chapter
Brain tumors and tumor-like conditions
5 Rajkamal S. Khangura, Max Wintermark, Sugoto Mukherjee, and Yang Tang
Case 5.1

129
Section 1: Brain
130
Chapter 5: Brain tumors and tumor-like conditions
History Key points

Two patients present with the same diagnosis. r Subtypes of astrocytoma:
b Localized astrocytoma (WHO grade I), such as
pediatric pilocytic astrocytoma, pleomorphic
Findings xanthoastrocytoma, and subependymal giant cell
Patient 1 astrocytoma.
b Diffuse infiltrative astrocytoma (WHO grade II).
Figures 5.1.1–5.1.5 A heterogeneous “butterfly”-shaped mass involving the
bilateral frontal lobes crossing the corpus callosum with surrounding T2
b Anaplastic astrocytoma (WHO grade III).
hyperintensity. The mass itself demonstrates relatively low T2 signal (Fig. 5.1.1) b GBM (WHO grade IV).
and diffusion restriction (Fig. 5.1.2, DWI), suggestive of high cellularity. Coronal
post-contrast T1 shows avid enhancement (Fig. 5.1.3). Note the ependymal
r GBM is the most common primary brain malignancy in
enhancement along the septum pellucidum (Fig. 5.1.3, arrow), left lateral adults. Peak incidence in sixth decade.
ventricle (curved arrow) and anterior third ventricle (arrow) on the axial r Can occur de novo or from degeneration of low-grade
post-contrast T1 (Fig. 5.1.4). Susceptibility is noted within the mass, consistent
with intratumoral hemorrhage (Fig. 5.1.5, arrow). Note a synchronous lesion in astrocytoma.
the right parietal lobe with necrosis and hemorrhage (∗ ) (Figs. 5.1.1, 5.1.2, 5.1.5). r Imaging:
b Hallmark: irregularly shaped mass with central
necrosis, hemorrhage, and hypervascularity. Tends to
Patient 2
spread along the white matter tracts such as corpus
Figures 5.1.6–5.1.8 A heterogeneous mass (∗ ) in the paramedian left frontal callosum, anterior and posterior commissures.
lobe with adjacent T2 hyperintensity (Fig. 5.1.6). The abnormal signal crosses
the genu of the callosum and reaches the right frontal lobe. The mass Subependymal spread is frequently seen.
demonstrates avid enhancement on the axial post-contrast T1 (Fig. 5.1.7), and b Mostly solitary, but multicentric lesions can occur.
markedly increased relative cerebral blood volume (rCBV) on the dynamic b T2/FLAIR: heterogeneous mass with surrounding T2
susceptibility contrast perfusion image (Fig. 5.1.8).
hyperintensity, which represents infiltrative tumor
and/or peritumoral vasogenic edema. The mass itself
can be relatively T2 hypointense due to high cellularity.
Diagnosis b Post-contrast T1: nearly all GBMs at least partially
Glioblastoma multiforme (GBM). enhance. Typically thick, irregular, and nodular
ring-like enhancement with central necrosis.
b DWI: may show diffusion restriction due to high
Differential diagnosis cellularity.
r Metastasis. b GRE: susceptibility due to hemorrhage.
r CNS lymphoma. b MR perfusion: increased rCBV, which can be used to
r Radiation necrosis. guide surgical biopsy.
r Infection. b MR spectroscopy: elevated choline, lactate/lipid peaks,
r Subacute infarction. low NAA.
r Tumefactive demyelination. r Treatment: surgery, chemotherapy, radiation therapy.
Further reading
Wilkins; 2009.
131
Section 1: Brain
Case 5.2
132

r Demyelinating or other white matter diseases, such as
progressive multifocal leukoencephalopathy (PML) or
posterior reversible encephalopathy (PRES).
History Key points
Patient 1: 61-year-old man presents with lethargy and
r GC is characterized by diffuse overgrowth of glial elements
cognitive slowing for a few months.
with infiltration of at least three contiguous areas of brain
Patient 2: 67-year-old man presents with personality change but relative preservation of underlying neuronal
and new-onset seizure-like activity. architecture.
r Patients typically present with nonspecific and nonfocal
Findings neurological symptoms. Personality and mental status
changes are most common. Symptoms are subacute or
Patient 1
chronic onset, disproportionately mild in relation to the
Figures 5.2.1–5.2.4 Axial FLAIR images demonstrate hyperintensity in the
bilateral centrum semiovale (Fig. 5.2.1) extending along the posterior limbs of
extent of disease.
internal capsules (Fig. 5.2.2) to the pons (Fig. 5.2.3) and the middle cerebellar r Imaging:
peduncles (Fig. 5.2.4). b CT: normal or diffuse hypoattenuation.
b T2/FLAIR: infiltrative hyperintense signal, with mild
Patient 2 sulcal effacement/mass effect. Expansion of brain
Figures 5.2.5, 5.2.6 Axial FLAIR images show hyperintensity and associated parenchyma but with relative preservation of
cortical expansion involving the bilateral inferior frontal lobes, right insula underlying architecture.
and thalamus (Fig. 5.2.5), and bilateral temporal lobes, right greater than left b T1: hypointense.
(Fig. 5.2.6). b Post-contrast T1: usually no or minimal enhancement.
Avid enhancement suggests malignant
dedifferentiation into GBM.
Diagnosis b No hemorrhage, necrosis, or restricted diffusion.
Gliomatosis cerebri (GC). b MR spectroscopy: increased choline/creatine and
choline/NAA ratio.
Differential diagnosis b MR perfusion: low or normal rCBV compared to
r Diffusely infiltrative astrocytoma. normal white matter.
r Viral encephalitis. r Treatment: chemotherapy, radiation therapy, steroid.
133
Section 1: Brain
Further reading
Felsberg GJ, Silver SA, Brown MT, Tien RD. Yang S, Wetzel S, Law M, Zagzag D, Cha S.
Radiologic–pathologic correlation. Dynamic contrast-enhanced
gliomatosis cerebri. AJNR Am J T2∗ -weighted MR imaging of gliomatosis
Neuroradiol 1994; 15(9): 1745–1753. cerebri. AJNR Am J Neuroradiol 2002;
23(3): 350–355.
134
Case 5.3
History
35-year-old female presents with seizure.
Findings
Figure 5.3.1 Axial NECT demonstrates a low-attenuation mass in the left
frontal lobe.
Figure 5.3.2 Axial T2 MRI shows an expansile, infiltrative hyperintense mass

involving the left frontal cortex and subcortical white matter.
Figure 5.3.3 Post-contrast axial MPRAGE does not show enhancement.
Diagnosis
Oligodendroglioma.
r Low-grade astrocytoma.
r Ganglioglioma.
r Dysembryoplastic neuroepithelial tumor (DNET).
Figure 5.3.3 r Ischemic infarction.
135
Section 1: Brain
b MRI:
Key points
r Oligodendrogliomas are slow-growing, infiltrative WHO Heterogeneous T1 hypointense and T2/FLAIR
hyperintense mass.
grade II tumors. Anaplastic oligodendrogliomas are WHO
Infiltrative but well demarcated and without
grade III.
r Most often occur supratentorially, especially frontal lobes significant associated vasogenic edema.
T2 heterogeneity reflects calcium (signal
(50–65%). Frequently calcified.
r Peak incidence at 40–50 years of age. loss/dropout), cystic change, and, less often, blood
r Clinical presentation includes seizure (most common), products.
50% of lesions enhance.
headache, mental status change or focal neurological
New enhancement in follow-up studies is suggestive
deficits.
r Imaging: of malignant degeneration.
No diffusion restriction on DWI.
b CT: Perfusion/spectroscopy may help differentiate from
Low-attenuation mass involving the cortex and anaplastic subtype.
subcortical white matter, frequently with associated r Treatment: surgical resection ± adjuvant
clumped, coarse calcifications.
radiotherapy/chemotherapy. 1p/19q co-deletion is
Bone windows may reveal expansion, remodeling,
associated with better chemotherapy response and
and thinning of the calvarium.
favorable prognosis.
Further reading
Koeller KK, Rushing EJ. From the archives
of the AFIP: Oligodendroglioma and its
variants: radiologic–pathologic
1669–1688.
136
Case 5.4
137
Section 1: Brain
138
139
Section 1: Brain

Four patients present with the same diagnosis. r Immunocompetent: GBM, metastasis, neurosarcoidosis,
systemic lymphoma with secondary CNS involvement.
Findings r Immunocompromised: toxoplasmosis, tuberculosis, fungal
or pyogenic abscess.
Patient 1
Figures 5.4.1–5.4.4 Mass lesion (∗ ) in the left parieto-occipital region, which
is T2 isointense to the gray matter with adjacent T2 hyperintense vasogenic
edema (Fig. 5.4.1, arrow), with restricted diffusion (Fig. 5.4.2 DWI,
Fig. 5.4.3 ADC), and demonstrates homogeneous enhancement on Key points
post-contrast T1 (Fig. 5.4.4). The signal characteristics are typical of primary CNS r PCNSL accounts for 4% of newly diagnosed primary
lymphoma (PCNSL) in immunocompetent patients.
central nervous system (CNS) tumors.
r It can affect both immunocompromised (AIDS,
Patient 2 organ-transplant, congenital immunodeficiency) and
Figures 5.4.5–5.4.7 Multifocal intraparenchymal lesions in the left immunocompetent patients.
frontoparietal region (Fig. 5.4.5), left basal ganglia, and splenium of corpus r Pathology: large B-cell non-Hodgkin lymphoma.
callosum, with hyperintensity on FLAIR (Fig. 5.4.6). The left frontoparietal lesion
demonstrates avid, homogeneous enhancement on post-contrast T1 (Fig.
r Can have dramatic response to steroid. CSF analysis
5.4.7). The basal ganglia lesion also demonstrates enhancement, while the reveals malignant lymphoid cells in up to 40% of PCNSL
splenial lesion does not enhance (not shown) and has an infiltrative patients.
appearance.
r Imaging:
b CT: often hyperdense due to high cellularity.
Patient 3
b MRI: parenchymal masses, preferentially involve deep
Figures 5.4.8–5.4.10 Multifocal periventricular T2 hyperintense lesions
adjacent to the frontal horns (Fig. 5.4.8, arrows) and in the hypothalamus gray matter and periventricular regions, tend to spread
(Fig. 5.4.9, curved arrow). The lesions demonstrate patchy enhancement on along the ependymal surface and cross the corpus
post-contrast T1 (Fig. 5.4.10). The periventricular distribution is also typical for callosum. Usually iso- or hypointense to gray matter on
PCNSL.
T1 and T2 sequences due to high cellularity, with
diffusion restriction, dense and homogeneous
Patient 4 enhancement (immunocompetent patients). In
Figures 5.4.11, 5.4.12 Diffusely increased FLAIR signal in the bilateral basal immunocompromised patients, usually multifocal
ganglia and left thalamus (Fig. 5.4.11), with multiple ring-enhancing lesions on
post-contrast T1 (Fig. 5.4.12). PCNSL frequently involves the deep nuclei and ring-enhancing lesions, with necrosis and sometimes
has ring enhancement in immunocompromised patients, in contrast to hemorrhage.
homogeneous enhancement in immunocompetent patients. b MR spectroscopy: elevated choline, decreased NAA.
b MR perfusion: rCBV significantly lower than GBM.
b Rarely calcifies except for treated lesions.
Diagnosis
r Treatment: chemotherapy, radiation therapy.
Primary CNS lymphoma (PCNSL).
Further reading
Haldorsen IS, Espeland A, Larsson EM. Koeller KK, Smirniotopoulos JG, Jones RV.
Central nervous system lymphoma: Primary central nervous system
characteristic findings on traditional and lymphoma: radiologic–pathologic
advanced imaging. AJNR Am J correlation. Radiographics 1997; 17(6):
Neuroradiol 2011; 32(6): 984–992. 1497–1526.
140
Case 5.5
141
Section 1: Brain
142
History Key points

Patient 1: 5-year-old girl presents with acute ataxia, left r Brainstem gliomas are rare tumors that affect children
facial weakness, and somnolence. more often than adults. They constitute 15% of all pediatric
Patient 2: 49-year-old man presents with hoarseness. Exam brain tumors.
r It is a heterogeneous group of tumors and can be classified
reveals right vocal cord paralysis.
into three distinct subtypes:
Patient 3: 18-year-old male presents with headache and
weakness. b Diffuse infiltrative glioma – usually infiltrative fibrillary
tumor (WHO grade II) or high-grade glioma, mostly
affecting the pons, with poor prognosis.
Findings b Focal glioma – usually WHO grade I pilocytic
Patient 1 astrocytoma, mostly affecting midbrain, medulla, and
cervicomedullary junction, with good prognosis.
Figure 5.5.1 NECT shows diffuse low attenuation and expansion of the pons. b Tectal glioma: low-grade tumor, affecting the tectal
Figure 5.5.2 Axial FLAIR demonstrates a hyperintense, infiltrative, and plate, with good prognosis.
expansile mass in the pons extending to the prepontine cistern and encircling r Clinical presentations:
the basilar artery (arrow).
b Diffuse or focal glioma: cranial nerve palsies, ataxia,
Figure 5.5.3 Post-contrast sagittal MPRAGE demonstrates no perceptible
enhancement.
nystagmus, long tract signs such as hemiparesis.
b Tectal glioma: typically presents with headache due to
obstructive hydrocephalus and gaze palsy.
Patient 2 r Imaging:
Figure 5.5.4 Axial FLAIR shows a well-circumscribed, partially exophytic mass
from right dorsal medulla extending into the fourth ventricle. b Diffuse pontine glioma:
CT: hypodense or isodense, rarely calcified.
Figure 5.5.5 Post-contrast axial T1 demonstrates avid, homogeneous MRI: hypointense on T1, hyperintense on T2 and
enhancement.
FLAIR, infiltrates and expands the pons, often with
exophytic component in prepontine cistern, and
Patient 3 encases the basilar artery.
Figures 5.5.6, 5.5.7 Sagittal T1 (Fig. 5.5.6) and axial T2 (Fig. 5.5.7) images Infiltrative fibrillary tumor usually does not
demonstrate enlargement of the lateral and third ventricles. There is a T1 enhance and respects the pontomedullary junction.
isointense and T2 slightly hyperintense mass (curved arrows) in the region of
superior and inferior colliculi causing obliteration of cerebral aqueduct and High-grade glioma enhances heterogeneously and
obstructive hydrocephalus. can invade adjacent structures.
b Focal midbrain/medullary glioma: hypodense on CT,
Figure 5.5.8 This patient later underwent endoscopic third ventriculostomy. hypointense on T1, hyperintense on T2/FLAIR, with
CSF flow sequence demonstrates patent flow through the ventriculostomy (∗ ),
but no flow through the aqueduct (arrow). well-circumscribed border and frequently exophytic
component, heterogeneous or intense enhancement.
b Tectal glioma: homogeneous expansion of tectal plate
causing obstruction of cerebral aqueduct, isodense on
Diagnosis CT, iso- to slightly hypointense on T1, slightly
Brainstem glioma: hyperintense on T2, usually no enhancement.
Patient 1: diffuse pontine glioma. r Treatment:
Patient 2: focal medullary glioma. b Diffuse pontine glioma: external beam radiation is the
Patient 3: tectal glioma. only effective therapy. Poor prognosis, with death
typically occurring within 1 year of diagnosis.
b Focal glioma: surgical resection, if it can be performed
Differential diagnosis without excessive morbidity. Radiation and/or
r Brainstem encephalitis. chemotherapy in unresectable or partially resected
r ADEM. tumor.
r Osmotic demyelination syndrome. b Tectal plate glioma: CSF shunting or third
r Hamartoma in neurofibromatosis type I. ventriculostomy.
Further reading
Guillamo JS, Doz F, Delattre JY. Brain stem
gliomas. Curr Opin Neurol 2001; 14(6):
711–715.
143
Section 1: Brain
Case 5.6
144
145
Section 1: Brain
History Diagnosis
Four pediatric patients with posterior fossa mass. Patient 1: Medulloblastoma.
Patient 2: Ependymoma.
Findings Patient 3: Pilocytic astrocytoma.
Patient 1 Differential diagnosis

Figure 5.6.1 NECT demonstrates a round mass in the fourth ventricle with r The most common pediatric posterior fossa tumors are
calcification. Hydrocephalus is noted, with dilation of the temporal horns.
medulloblastoma, astrocytoma, atypical teratoid–rhabdoid
Figures 5.6.2–5.6.4 Axial T2 (Fig. 5.6.2) MRI shows a heterogeneous T2 tumor (ATRT), and ependymomas.
hyperintense mass with a few intratumoral cysts. The mass shows restricted r Other masses occurring in the posterior fossa include
diffusion (Fig. 5.6.3) and subtle enhancement. Note the tonsillar herniation.
choroid plexus papilloma, hemangioblastoma,
ganglioglioma, epidermoid, lymphoma, metastatic disease,
Patient 2 etc.
Figure 5.6.5 Axial T2 MRI shows a heterogeneous mass within the fourth
ventricle. The markedly hypointense foci may represent calcification or
hemorrhage (curved arrow). There is tumor extension to the bilateral Key points
cerebellopontine angles through the foramina of Luschka (arrows). r Medulloblastoma or infratentorial primitive
Figure 5.6.6 Axial DWI shows isointensity of the mass to the cerebellum. neuroectodermal tumor (PNET):
b WHO grade IV tumor, occurring in children less than
Figure 5.6.7 Sagittal post-contrast T1 MRI demonstrates mild heterogeneous
enhancement. Note extension of the mass through the foramen of Magendie 10 years of age. It typically arises from the superior
(arrow). Massive hydrocephalus is noted. cerebellar velum (roof of the fourth ventricle).
However, the desmoplastic subtype occurs more in
Patient 3 older children or adults, and tends to be eccentrically
located in the cerebellar hemisphere.
Figure 5.6.8 NECT shows a predominantly cystic mass in the right
cerebellum, with solid mural nodule and calcification.
b Clinical presentation: headache, nausea and vomiting,
truncal ataxia, etc. The time from symptom onset to
Figure 5.6.9 Axial T2 MRI demonstrates the cystic and solid mural diagnosis is short due to its aggressive behavior.
component, causing effacement of the fourth ventricle. b Imaging:
Figure 5.6.10 Axial post-contrast T1 shows enhancing wall and enhancing CT: well defined, hyperdense mass of cerebellar
mural nodule. vermis with surrounding edema. Hydrocephalus is
146
common. Cysts (50%) and calcifications (20%) can r Ependymoma:

occur. b The third most common pediatric posterior fossa
MRI: iso- or hypointense on T1 to normal gray tumor, typically occurring between 1 and 5 years of age.
matter. T2 signal is variable, can be isointense to It arises from the ependymal cells lining the fourth
hyperintense. FLAIR is hyperintense. Restricted ventricle. Histologically, they are classified as WHO
diffusion due to high cellularity. Heterogeneous grade II (cellular type or variants) or III (anaplastic).
enhancement. CSF dissemination/metastasis is b Clinical presentation: headache, nausea/vomiting, etc.,
common. Therefore, it is essential to obtain MRI of with a prolonged time from symptom onset to imaging
the entire neuroaxis before surgery. due to slow growth.
b Treatment: surgery, chemotherapy, radiation. b Imaging:
r Cerebellar astrocytoma: CT: isodense mass arising from the floor of the
b Nearly as common as medulloblastoma in the posterior fourth ventricle, commonly with calcification, cysts,
fossa. Typically presents in children between 5 and 15 and hemorrhage.
years old. Pilocytic astrocytoma is the most common MRI: isointense on T1, hyperintense on T2 but
histologic type. usually heterogeneous in signal due to calcification
b Clinical presentation: more indolent course of and hemorrhage, which are better seen on
headache, gait disturbance, or ataxia due to slow gradient-recalled echo (GRE) sequence. Intense but
growth. variable post-contrast enhancement. No diffusion
b Imaging: restriction due to low cellularity. Hydrocephalus is
The classic appearance of pilocytic astrocytoma is a common.
large cyst with a solid mural nodule. The cyst wall Tends to squeeze through the foramen of Luschka to
may or may not enhance. Less commonly, the tumor the cerebellopontine angle, and through the
is predominantly solid with little or no cystic foramen of Magendie to the cisterna magna.
b Treatment: surgical resection, chemotherapy, radiation
component.
On MRI, the cystic component is hypointense on T1 therapy.
and hyperintense on T2/FLAIR. No diffusion r ATRT is similar to medulloblastoma histologically and
restriction. Little or no surrounding vasogenic radiologically, but it exhibits more aggressive biological
edema. behavior and occurs in younger children (age ⬍ 3 years).
b Treatment: surgical resection is usually curative.
Further reading
Plaza MJ, Borja MJ, Altman N, Saigal G.
Conventional and advanced MRI features
of pediatric intracranial tumors: posterior
fossa and suprasellar tumors. AJR Am J
Roentgenol 2013; 200(5): 1115–1124.
147
Section 1: Brain
Case 5.7
Figure 5.7.2
Figure 5.7.1 History

44-year-old female presents with headache.
Findings
Figure 5.7.1 NECT shows a partially calcified mass in the left lateral ventricle
with resultant ventricular dilation.
Figure 5.7.2 Axial T2 MRI demonstrates a “bubbly” appearing, mixed cystic

and solid mass attached to the septum pellucidum.
Figure 5.7.3 Post-contrast axial T1 shows heterogeneous enhancement.
Diagnosis
Central neurocytoma.
r Subependymoma.
r Subependymal giant cell astrocytoma.
r Choroid plexus papilloma.
r Intraventricular meningioma.
r Ependymoma.
Key points
r Central neurocytoma is WHO grade II benign
neuroepithelial intraventricular tumor.
r It typically occurs in young and middle-aged adults. Most
Figure 5.7.3 patients are asymptomatic. If symptomatic, clinical
148
manifestations may include headache, altered mental periventricular hypodensities reflective of

status, seizure, or hydrocephalus. transependymal CSF flow.
r Imaging: b MRI: heterogeneous, generally T1 isotense, T2
b CT: lobulated mass within the lateral ventricle with hyperintense intraventricular mass attached to the
mixed solid (iso- to hyperdense) and cystic (iso- to septum pellucidum with a “bubbly” appearance.
hypodense) components. It is usually attached to the Calcifications cause susceptibility artifact and low
septum pellucidum and extends near the foramen T1/T2 signal. Intraparenchymal invasion is rare.
of Monro. Bowing of the septum pellucidum Prominent flow voids within the mass are noted, best
away from the involved ventricle is common. appreciated on T2-weighted imaging. The solid
Calcifications are seen 50–70% of the time. Obstruction component demonstrates diffuse avid enhancement.
of foramen of Monro may lead to hydrocephalus with r Treatment: surgical resection.
Further reading
Smith AB, Smirniotopoulos JG,
Horkanyne-Szakaly I. From the
radiologic pathology archives:
intraventricular neoplasms:
radiologic–pathologic correlation.
Radiographics 2013; 33(1): 21–43.
149
Section 1: Brain
Case 5.8
150
Patient 2
Figure 5.8.4 Axial T2 MRI shows a well-demarcated, hyperintense, cystic
mass in the right parietal lobe, without causing edema or mass effect.
Figure 5.8.5 Coronal FLAIR shows the wedge-shaped, cortically based mass
that points towards the ventricle. No enhancement is seen on post-contrast T1
(not shown).
Diagnosis
Dysembryoplastic neuroepithelial tumor (DNET).
r Ganglioglioma.
r Low-grade astrocytoma.
r Oligodendroglioma.
r Cortical dysplasia.
r Neuroepithelial cyst.
Key points
r DNET is a WHO grade I benign tumor composed of both
glial and neuronal elements. It can arise anywhere in the
Figure 5.8.5 (patient 2) supratentorial brain, most commonly in the temporal
lobes.
r Clinical presentation: older children or young adults with
intractable partial complex seizures.
History r Imaging:
Two young patients present with seizure. b Well-circumscribed, bubbly cystic, intracortical mass,
which may or may not involve the subcortical white
matter. Can be wedge-shaped, pointing towards the
Findings ventricle.
Patient 1 b Frequently associated with cortical dysplasia.
Figure 5.8.1 Axial T2 MRI demonstrates a hyperintense, well-circumscribed b If superficial, may cause remodeling of the adjacent
mass in the left medial temporal lobe, which involves the cortex and subjacent calvarium.
white matter. b CT: low attenuation, non-enhancing mass.
Figure 5.8.2 Coronal FLAIR shows a “bubbly” appearing mass with both
b MRI: hypointense on T1, hyperintense on T2, iso- or
cystic and solid components. hyperintense on FLAIR. Does not enhance or restrict
diffusion. No edema or mass effect.
Figure 5.8.3 Coronal post-contrast T1 shows no perceptible enhancement of
the solid component. r Treatment: surgical resection.
Further reading
Fernandez C, Girard N, Paz Paredes A,
Bouvier-Labit C, Lena G,
Figarella-Branger D. The usefulness of
MR imaging in the diagnosis of
dysembryoplastic neuroepithelial tumor
in children: a study of 14 cases. AJNR Am
J Neuroradiol 2003; 24(5): 829–834.
151
Section 1: Brain
Case 5.9
Figure 5.9.1
Figure 5.9.2
152
r Langerhans cell histiocytosis (LCH).
r Leukemia/lymphoma.
r Sarcomas (Ewing’s, osteosarcoma, rhabdomyosarcoma).
r Osteomyelitis.
r Thalassemia.
Key points
r Neuroblastoma is a poorly differentiated tumor of
primordial neural crest cells originating from the adrenal
glands or sympathetic chains.
r It is the third most common tumor in childhood, only
behind leukemia/lymphoma and primary CNS tumors.
r Ophthalmic symptoms such as “raccoon eyes” and
proptosis are common initial presentations due to orbital
metastasis. “Raccoon eyes” describes periorbital
ecchymosis, secondary to obstruction of palpebral vessels
by the tumor within the orbit.
r Cranial metastases are usually calvarial osseous lesions that
infiltrate the outer and inner tables with extradural
Figure 5.9.5 component. Periostitis with “hair-on-end” spicules is
characteristic of neuroblastoma and helps distinguish it
from LCH, which is typically a well-defined lytic lesion
History without periostitis. Brain parenchymal or leptomeningeal
3-year-old girl presents with headache, lethargy, and metastases are rare.
right-eye proptosis. r Imaging:
b CT: expansile lytic bone lesions with spiculated
Findings periositis and associated enhancing soft-tissue mass.
Figures 5.9.1, 5.9.2 NECT show permeative osseous lesions centered b MRI: heterogeneous osseous or extradural masses,
around the lateral wall of the right orbit (Fig. 5.9.1) and right frontal calvarium which are T1 hypointense and T2/FLAIR hyperintense
(Fig. 5.9.2) with spiculated periostitis (arrows).
compared to adjacent muscles, and with avid
Figure 5.9.3 Axial T2 MRI demonstrates a slightly hyperintense mass enhancement.
infiltrating the greater wing of the right sphenoid, with both extracranial b Abdominal CT/MRI or nuclear medicine studies
(arrow) and intraorbital (∗ ) components. Right-globe proptosis is noted.
should be obtained to identify the primary tumor if
Figures 5.9.4, 5.9.5 Post-contrast axial (Fig. 5.9.4) and coronal (Fig. 5.9.5) MRI neuroblastoma is suspected. Nuclear medicine MIBG
show avid enhancement of the mass. Note the extracranial (arrow), intraorbital scan is sensitive and specific for staging to evaluate for
(∗ ), and intracranial/extradural (block arrow) components. the extent, distribution, and systemic disease
involvement.
Diagnosis r Treatment: surgery, chemotherapy, radiation.
Metastatic neuroblastoma.
Further reading
D’Ambrosio N, Lyo JK, Young RJ, Haque SS,
Karimi S. Imaging of metastatic CNS
neuroblastoma. AJR Am J Roentgenol
2010; 194(5): 1223–1229.
153
Section 1: Brain
Case 5.10
History
43-year-old man with headache and multiple episodes of
syncope.
Findings
Figures 5.10.1–5.10.3 Sagittal MPRAGE (Fig. 5.10.1), axial T2 (Fig. 5.10.2), and
axial FLAIR (Fig. 5.10.3) images demonstrate a multiseptated cystic mass in the
left midbrain and pons, causing mass effect on the third ventricle
(Fig. 5.10.2, arrow) and mild dilation of lateral ventricles. This lesion is T1
hypointense (Fig. 5.10.1), T2 hyperintense (Fig. 5.10.2), and with complete
signal suppression on FLAIR (Fig. 5.10.3).
Diagnosis
Tumefactive perivascular space.
r Cystic neoplasm (such as DNET).
r Infectious/inflammatory cysts (neurocysticercosis,
cryptococcosis, etc.).
r Cystic encephalomalacia.
r Other cysts (neuroepithelial cyst, arachnoid cyst).
Figure 5.10.3 r Mucopolysaccharidosis.
154
r Clinical presentation: headache is most common. Other

Key points
r Perivascular spaces (Virchow–Robin spaces) are pial-lined symptoms include dizziness, dementia, visual changes, etc.
r Imaging: unilocular or multiloculated cystic mass
interstitial fluid-filled spaces accompanying penetrating
following CSF signal on all pulse sequences. T1
arteries and arterioles that do not communicate with
hypointense, T2 hyperintense, and completely suppressed
subarachnoid space.
r Usually asymptomatic, less than 5 mm, and most on FLAIR (some cases may have increased signal in the
adjacent brain parenchyma). No enhancement or diffusion
commonly identified in the basal ganglia along the course
restriction.
of lenticulostriate arteries. r Treatment: do not touch lesion. Follow-up imaging to
r The enlarged (giant, tumefactive) perivascular space most
document stability. Ventricular shunting may be required
commonly occurs in the mesencephalothalamic region,
for hydrocephalus caused by midbrain lesion.
and can cause mass effect or hydrocephalus.
Further reading
Salzman KL, Osborn AG, House P, Jinkins
JR, Ditchfield A, Cooper JA, et al. Giant
tumefactive perivascular spaces. AJNR
Am J Neuroradiol 2005; 26(2): 298–305.
155
Section 1: Brain
Case 5.11
156

15-year-old male presents with recurrent seizures. r Craniopharyngioma.
r Hypothalamic/chiasmatic astrocytoma.
Findings Key points

Figure 5.11.1 NECT shows a suprasellar mass (arrow), isodense to the brain
r Hamartoma of tuber cinereum is a non-neoplastic
parenchyma. gray-matter hetertopia located between mammillary body
and hypothalamic infundibulum, which can be sessile or
Figure 5.11.2 Axial T2 shows that the mass is located in the hypothalamus
eccentric to the right (black arrow). It is isointense to the gray matter. The right pedunculated.
mammillary body (block arrow) is displaced posteriorly compared to the left r Clinical presentations include epilepsy (gelastic seizure is
(curved arrow). characteristic), precocious puberty, and diabetes insipidus.
r Imaging:
Figures 5.11.3, 5.11.4 Pre- (Fig. 5.11.3) and post-contrast (Fig. 5.11.4)
sagittal T1 images show the mass (white arrow) between the infundibulum and b CT: non-enhancing suprasellar mass, isodense or
mammillary bodies. It is isointense to the gray matter and does not enhance.
slightly hypodense to brain parenchyma.
b MRI: T1 isointense, T2 isointense to slightly
hyperintense, compared to gray matter, non-enhancing
Diagnosis on post-contrast T1.
Hamartoma of tuber cinereum. r Treatment: surgery.
Further reading
Boyko OB, Curnes JT, Oakes WJ, Burger PC.
Hamartomas of the tuber cinereum: CT,
MR, and pathologic findings. AJNR Am J
Neuroradiol 1991; 12(2): 309–314.
157
Section 1: Brain
Case 5.12
158
159
Section 1: Brain

Patient 1: 41-year-old woman presents with headache, r Arachnoid cyst.
dizziness, and visual disturbance. r Dermoid cyst.
Patient 2: 55-year-old man presents with seizure. r Inflammatory cysts such as neurocysticercosis.
r Cystic neoplasms.
Key points
Findings r Epidermoids and dermoids are rare non-neoplastic
Patient 1 developmental lesions derived from ectodermal rests.
r They typically present in the third to fifth decades of age
Figure 5.12.1 Axial T2 image shows a lobulated hyperintense lesion
insulating the prepontine cistern, encasing the basilar artery and multiple and with insidious symptoms. Symptoms related to
cranial nerves. supratentorial epidermoids include seizures/headaches.
Figure 5.12.2 Sagittal post-contrast T1 shows no enhancement. Note the
Posterior fossa epidermoids may cause cranial nerve
mass effect causing posterior displacement of the brainstem. dysfunctions.
r As opposed to dermoids, which tend to be at midline, the
Figure 5.12.3 On axial FLAIR, the lesion does not completely null and is epidermoids are usually located laterally. The most
slightly hyperintense to CSF.
common location is cerebellopontine angle, followed by
Figure 5.12.4 Axial DWI shows restricted diffusion. fourth ventricle, parasellar region/middle cranial fossa,
ventral to brainstem, subfrontal, and interhemispheric
regions.
Figures 5.12.5, 5.12.6 Axial post-contrast CT (Fig. 5.12.5) and sagittal b CT: round/lobulated non-enhancing cystic mass with
post-contrast T1 MRI (Fig. 5.12.6) show a non-enhancing lobulated left
subfrontal cystic mass. CSF density.
b MRI: signal characteristics on conventional T1 and T2
Figure 5.12.7 Axial FLAIR shows soft-tissue strands within the lesion, slightly signal are similar to CSF. It may be T1 hyperintense in
hyperintense to CSF.
some cases due to high triglycerides and unsaturated
Figure 5.12.8 Axial DWI shows hyperintensity within the lesion. fatty acids, referred as “white epidermoids.” On FLAIR,
the signal usually does not completely null, and strands
of soft tissue may become apparent. DWI typically
shows characteristic hyperintensity. These features help
Diagnosis distinguish from arachnoid cysts.
Intracranial epidermoid. r Treatment: surgical excision.
Further reading
Wilkins; 2009.
160
Case 5.13
History
25 year-old female presents with headache.
Findings
Figure 5.13.1 Coronal NECT shows a hyperdense, oval mass in the superior
third ventricle.
Figures 5.13.2, 5.13.3 Axial T2 (Fig. 5.13.2) and sagittal T1 (Fig. 5.13.3)
demonstrate the mass in anterior/superior third ventricle wedged at the
foramen of Monro. It is T2 isointense and T1 hyperintense to the brain
parenchyma. Note the fornix drapes over the lesion. There is no hydrocephalus.
Diagnosis
Colloid cyst.
r Basilar tip aneurysm.
r Craniopharyngioma.
r Neurocysticercosis.
r Subependymoma.
Figure 5.13.3 r Pulsatile CSF flow artifact.
161
Section 1: Brain
Key points in the anterosuperior third ventricle is

r Colloid cyst is a mucin-containing cyst, nearly always characteristic.
b MRI: signal intensity is variable, depending on
located in the anterior/superior third ventricle.
r The most common presenting symptom is headache. Other cholesterol and water concentration. The majority
are T2 isointense and T1 hyperintense to brain
symptoms include altered mental status, rarely coma or
parenchyma. It does not restrict diffusion or enhance.
sudden death. It should be noted that the size of the lesion
Thin peripheral rim enhancement may be rarely seen.
is not a reliable predictor of outcome, as even small ones
Hydrocephalus with or without transependymal CSF
can cause sudden death. Approximately 50% of patients are
may be seen in a minority of patients.
asymptomatic and are diagnosed incidentally.
r Imaging: r Treatment: complete surgical resection via
b CT: round/oval hyperdense or isodense mass neuroendoscopic approach.
wedged into the foramen of Monro located
Further reading
Armao D, Castillo M, Chen H, Kwock L.
Colloid cyst of the third ventricle:
imaging-pathologic correlation. AJNR
Am J Neuroradiol 2000; 21(8): 1470–1477.
162
Case 5.14
Figure 5.14.2
Figure 5.14.1
163
Section 1: Brain
History Key points

5-year-old boy with progressive lethargy, nausea and r Arachnoid cysts are developmental lesions that are formed
vomiting for 1 week. due to splitting of the arachnoid layers and accumulation
of CSF within this potential space.
r More than 50% of arachnoid cysts occur in the middle
Findings cranial fossa. Other common intracranial locations include
posterior fossa (cisterna magna and cerebellopontine
Figure 5.14.1 NECT shows compression of left lateral ventricle and rightward angle), suprasellar cistern, quadrigeminal cistern, and
midline shift, likely from a left convexity extra-axial fluid collection, which is
isodense to the cortex. cerebral convexity.
r Most arachnoid cysts are asymptomatic. Occasionally,
Figures 5.14.2, 5.14.3 Axial T2 (Fig. 5.14.2) and T1 (Fig. 5.14.3) demonstrate cysts can enlarge and rupture into the adjacent subdural or
a left temporal arachnoid cyst (∗ ), which is T2 hyperintense and T1 isointense to
cortex, reflecting hemorrhagic component. Note thickening of the cyst wall
epidural spaces. Hemorrhage will occur if there is vascular
(arrow) and adjacent subdural hematoma. disruption in the cyst wall. These patients typically present
with acute headache and other neurological deficits due to
Figure 5.14.4 Axial FLAIR at the level of vertex demonstrates a subdural increased intracranial pressure and mass effect.
hematoma along the left convexity tracking along the anterior falx.
r Imaging:
b Uncomplicated arachnoid cysts:
Diagnosis Extra-axial cyst with CSF density on CT.
Follow CSF signal intensity on all MRI sequences.
Ruptured arachnoid cyst with subdural hematoma. b Hemorrhagic cysts:
CT: isodense or hyperdense to CSF.
MRI: variable T1 and T2 signal depending on the
Differential diagnosis amount of intracystic hemorrhage.
r Epidermoid. Subdural or epidural hematoma/hygroma if
r Cystic tumor. ruptured.
r Inflammatory cysts. r Treatment: surgical evacuation.
Further reading
Bilginer B, Onal MB, Oguz KK, Akalan N.
Arachnoid cyst associated with subdural
hematoma: report of three cases and
review of the literature. Childs Nerv Syst
2009; 25(1): 119–124.
164
Case 5.15
Figure 5.15.1
Figure 5.15.2
165
Section 1: Brain
Diagnosis
Ruptured intracranial dermoid cyst.
r Lipoma.
r Teratoma.
r Craniopharyngioma.
r Air embolism.
Key points
r An intracranial dermoid cyst is a benign extra-axial lesion
that develops from sequestration of ectoderm cells and that
usually grows slowly secondary to the production of hair
and oil from internal dermal components. Histologically,
dermoid cysts have an outer wall composed of squamous
epithelium, with inner contents of sebaceous and apocrine
glands, fat and hair follicles.
r Dermoid cysts are typically asymptomatic until rupture,
which may be spontaneous or induced by trauma. Upon
rupturing, the internal cholesterol debris incites aseptic
chemical meningitis as it disseminates throughout the
Figure 5.15.5
subarachnoid spaces.
r Clinically, patients may present with altered mental status,
seizure, coma, focal neurological deficits secondary to
infarctions due to vasospasm, or even death.
History r Imaging:
20-year-old male presents with headache, altered mental b CT: extra-axial hypodense mass with internal fat
status, and seizure. contents, usually occurs at midline. Ruptured dermoid
cysts present with disseminated fat droplets in the
cortical sulci and basilar cisterns. Fat–fluid levels may
Findings be seen in the ventricles.
b MRI: usually T1 and T2 hyperintense due to fat
Figures 5.15.1, 5.15.2 NECT reveals a hypodense midline mass content. Chemical shift artifact on T2 sequence due to
(Fig. 5.15.1, ∗ ) and numerous tiny droplets (Fig. 5.15.2, black arrows)
disseminated throughout the ventricles and cortical sulci. The attenuation of fat–fluid interface results in a hypointense rim. Fat
these lesions measures negative Hounsfield units, consistent with fat. suppression sequences are also helpful to confirm the
presence of fat. Ruptured dermoids can produce
Figure 5.15.3 Axial T1 MRI again demonstrates the midline mass (∗ ) that is
heterogeneously T1 hyperintense. There are scattered T1 hyperintense fat
hyperintense signal on FLAIR and susceptibility
droplets within the subarachnoid space (black arrows). artifact on T2∗ GRE within the cortical sulci. On
post-contrast imaging, leptomeningeal enhancement
Figures 5.15.4, 5.15.5 The midline mass is T2 hyperintense with a may be seen with chemical meningitis.
hypointense rim from chemical shift artifact (Fig. 5.15.4). The fat saturates out
on the coronal STIR image (Fig. 5.15.5). r Treatment: complete total resection.
Further reading
Smirniotopoulos JG, Chiechi MV.
Teratomas, dermoids, and epidermoids of
the head and neck. Radiographics 1995;
15(6): 1437–1455.
166
Case 5.16
Figure 5.16.2
History
Figure 5.16.1
5-month-old girl presents with vomiting and seizure.
Findings
Figure 5.16.1 Axial T2 shows a lobulated mass in the left lateral ventricle
(arrow). The mass is predominantly iso- to hyperintense to the brain
parenchyma but demonstrates small areas of hypointense signal, which may
represent calcification or hemorrhage. There is no invasion to the adjacent
brain parenchyma.
Figure 5.16.2 Post-contrast axial T1 demonstrates avid enhancement of the

intraventricular mass. Incidentally noted is an enlarged perivascular space in
the left basal ganglia (∗ ).
Figure 5.16.3 A second enhancing lesion is noted at the pineal region

(arrow), representing CSF drop metastasis.
Diagnosis
Choroid plexus papilloma.
r Choroid plexus carcinoma.
Key points
r WHO grade I intraventricular tumor, most commonly
Figure 5.16.3 affecting children under 5 years of age.
167
Section 1: Brain
r Approximately 50% are located in the atrium of the lateral r Imaging:

ventricle, 40% in the fourth ventricle, and 5% in the third b CT: lobular, high attenuation, intraventricular mass
ventricular roof. Multiple sites of involvement in 5% of with associated hydrocephalus. Calcifications are seen
cases. 25% of the time.
r Though rare, CSF seeding must be evaluated for and b CTA or conventional angiography may show
excluded. enlargement of anterior or posterior choroidal artery
r Hydrocephalus is usually secondary to CSF
for masses in the lateral ventricles.
overproduction but may also occur secondary to b MRI: lobular T1 isotense/T2 hyperintense
mechanical obstruction or impaired resorption. intraventricular mass with papillary and frond-like
r Histologically, the tumor consists of fibrovascular
projections. Internal vascular flow voids on T2.
connective tissue fronds with similar appearance to normal Periventricular FLAIR hyperintensity from
choroid plexus. transependymal CSF flow. Intense, homogeneous
r Choroid plexus carcinoma is more likely to invade brain
post-contrast enhancement.
parenchyma, whereas choroid plexus papilloma remains r Treatment: total surgical resection with rare recurrence.
intraventricular. However, imaging studies cannot reliably
distinguish these two entities.
Further reading
Naeini RM, Yoo JH, Hunter JV. Spectrum of
choroid plexus lesions in children. AJR
Am J Roentgenol 2009; 192(1): 32–40.
168
Case 5.17
169
Section 1: Brain
Figures 5.17.4, 5.17.5 Axial (Fig. 5.17.4) and sagittal (Fig. 5.17.5)
post-contrast T1 again reveal the suprasellar mass. The solid nodular
component (Fig. 5.17.4, black arrow) demonstrates enhancement.
Diagnosis
Craniopharyngioma.
r Rathke cleft cyst.
r Hypothalamic/chiasmatic glioma.
r Pituitary adenoma.
r Dermoid/epidermoid.
Key points
r Craniopharyngioma is a benign (WHO grade I) tumor,
arising from remnants of Rathke’s pouch.
r Bimodal age distribution, with peaks in childhood (5–10
years old) and adulthood (50–60 years old).
r Typically suprasellar (75%), although 20–25% of lesions
have an intrasellar component.
r Clinical presentations: visual disturbances, headache, and
Figure 5.17.5 endocrine deficiencies such as growth failure, delayed
puberty, and diabetes insipidus.
r Two histological subtypes: adamantinomatous (90%),
History papillary (10%).
7-year-old boy presents with visual disturbance and r Imaging:
headache. b Adamantinomatous subtype: typically mixed solid and
cystic tumor with calcification (90%) and peripheral
Findings capsular/nodular enhancing components.
b Papillary subtype: usually solid enhancing mass
Figure 5.17.1 Axial NECT demonstrates a mixed cystic and solid suprasellar
mass with a low-attenuation cystic portion and a calcified solid component without calcification.
(curved arrow). Hydrocephalus is noted. b MRI: cystic component shows variable T1 and T2
signal due to proteinaceous content, classically T1
Figure 5.17.2 Axial T2 MRI shows a hyperintense suprasellar mass with a
peripheral hypointense nodular component (curved arrow). Areas of signal
hyperintense and T2/FLAIR hyperintense. Edema may
void within the nodular component represent calcifications seen on CT. involve the optic chiasm/tract. The peripheral capsule
of the cyst demonstrates thin, rim-like enhancement.
Figure 5.17.3 Axial FLAIR demonstrates a multilobulated cystic mass in the Solid tumor components demonstrate enhancement.
suprasellar region. The posterior cystic component (∗ ) is hyperintense and
occupies the third ventricle, resulting in obstructive hydrocephalus with r Treatment: gross total resection, radiation, cystic
transependymal CSF flow. instillation with sclerosing agents.
Further reading
Saleem SN, Said AH, Lee DH. Lesions of the
hypothalamus: MR imaging diagnostic
features. Radiographics 2007; 27(4):
1087–1108.
170
Case 5.18
171
Section 1: Brain
History Patient 4
Figures 5.18.4–5.18.6 Axial post-contrast T1 images with fat saturation
Patient 1: 58-year-old male with history of lung cancer demonstrate enhancement of the prechiasmatic right optic nerve ( Fig. 5.18.4,
presents with headache. arrow), bilateral internal acoustic canals (Fig. 5.18.5, arrows), pineal gland
(Fig. 5.18.6, arrow) and choroid plexus (Fig. 5.18.6, block arrow).
Patient 2: 46-year-old male presents with new-onset seizure.
The patient is later found to have renal cell carcinoma.
Patient 3: 75-year-old male presents with “subdural Diagnosis
hemorrhage.”
Intracranial metastasis.
Patient 4: 61-year-old female with breast cancer presents
with right visual loss.
r Parenchymal metastasis: primary brain tumors, abscess,
radiation necrosis, tumefactive demyelination, subacute
Findings infarction, evolving hematoma.
Patient 1 r Leptomeningeal metastasis: lymphoma, sarcoidosis,
Figure 5.18.1 NECT shows a mass lesion in the inferior right frontal lobe with infectious meningitis.
a hematocrit level. An additional hyperdense, hemorrhagic mass is noted in the r Dural metastasis: meningioma, subdural hematoma.
right temporal lobe. Both lesions have a large amount of associated vasogenic
edema.
Key points
r Metastases are the most common intracranial tumors in
Patient 2 adults. In adults, the most common primary malignancies
Figure 5.18.2 Post-contrast axial T1 MRI shows multiple enhancing lesions in include lung cancer, breast cancer, melanoma, colorectal
bilateral basal ganglia and right insular region.
cancer, and renal cell carcinoma. In children, the most
common sources include sarcomas, neuroblastoma, and
Patient 3 germ-cell tumors.
r Intracranial metastases can occur to the brain parenchyma,
Figure 5.18.3 NECT demonstrates hyperdense material along the bilateral
cerebral convexities (arrows), which is surgically proven to be dural metastasis dura, leptomeninges, as well as some uncommon locations
from prostate cancer, mimicking subdural hematoma. such as cranial nerves, pituitary gland, pineal gland,
172
choroid plexus. Parenchymal metastases are most necrosis, calcification, etc. Usually with a large amount
common. of vasogenic edema compared to the size of the lesion
r The most common mechanism is hematogenous spread. (abscess can have similar finding).
r Clinical symptoms: variable, and depends on the location b Post-contrast T1: Essentially all metastatic lesions
of the lesions. Common symptoms include headache, enhance on MRI because of the defective blood–brain
focal neurological deficits, seizure, cognitive dysfunction, barrier. Enhancement can be solid, nodular, or
etc. ring-like. A high dose of contrast may increase the
r Imaging: conspicuity of subtle lesions.
b Contrast-enhanced MRI is the modality of choice and b DWI: usually no restriction, but tumors with high
more sensitive than CT. cellularity or mucinous content can restrict diffusion
b Solitary (50%) or multiple lesions, typically at the and mimic pyogenic abscess.
b GRE: bloom if hemorrhage or calcifications are present.
gray–white matter interface.
b T1 and T2/FLAIR signals are variable, depending on r Treatment: surgical resection, radiation (stereotactic
multiple factors such as tumor cellularity, hemorrhage, radiosurgery or whole-brain radiation), chemotherapy.
Further reading
Atlas SW, editor. Magnetic Resonance
Imaging of the Brain and Spine, 4th edn.
Philadelphia, PA: Lippincott Williams &
Wilkins; 2009.
173
Section 1 Brain
Chapter
Miscellaneous cerebral emergencies
6 Yang Tang, Matthew R. Parry, Sugoto Mukherjee, and Max Wintermark
Case 6.1
174
Chapter 6: Miscellaneous cerebral emergencies
175
Section 1: Brain
History include encephalitis, ADEM, cerebral infarction,

hypoxic–ischemic injury, hypoglycemic injury,
Patient 1: 9-year-old girl with acute leukemia on
osmotic demyelination, progressive multifocal
chemotherapy develops altered mental status.
leukoencephalopathy, etc.
Patient 2: 52-year-old man status post cardiac transplant,
presents with headache, confusion, and visual disturbance.
Key points
r PRES describes a unique pattern of typically symmetric,
Findings reversible cerebral vasogenic edema associated with a
Patient 1 number of neurotoxic states, including but not limited to
Figure 6.1.1 NECT shows subcortical hypodensity in the left parietal lobe. hypertensive encephalopathy, preeclampsia/eclampsia,
organ transplantation, immunosuppressive/chemotherapy
Figures 6.1.2, 6.1.3 Axial FLAIR images reveal cortical and subcortical agents, and autoimmune diseases.
hyperintensity in the bilateral parieto-occipital regions. r The symptoms are nonspecific, ranging from headache,
Figure 6.1.4 DWI shows mild diffusion restriction in left parietal lobe. altered mental status, and seizure to visual disturbances
and loss of consciousness.
Figure 6.1.5 GRE sequence shows punctate microhemorrhages in the left r Although the exact mechanism is uncertain, it is thought
parietal lobe.
to be related to altered brain perfusion with break-through
of the blood–brain barrier and leakage of fluid causing
Patient 2 edema.
Figures 6.1.6–6.1.8 Axial FLAIR images demonstrate hyperintensity in the r PRES most frequently affects the parieto-occipital regions,
posterior limb of internal capsules, splenium of corpus callosum (Fig. 6.1.6), followed by the posterior frontal and temporal regions,
pons (Fig. 6.1.7), cerebellum and medulla (Fig. 6.1.8). There is no associated
diffusion restriction, hemorrhage, or enhancement.
with symmetric and reversible cortical/subcortical
vasogenic edema.
r Usual locations include cerebellum, brainstem, deep white
Diagnosis matter (corpus callosum, internal/external capsules,
Posterior reversible encephalopathy syndrome (PRES). corona radiata), deep gray matter (thalamus and basal
ganglia). Unilateral hemispheric and spinal cord
involvement also rarely occur.
Differential diagnosis r Atypical imaging presentations are frequently encountered,
r MRI is usually diagnostic for typical PRES in the including diffusion restriction (indicating tissue injury
appropriate clinical setting. from cytotoxic edema), parenchymal/subarachnoid
r For PRES affecting unusual locations or with atypical hemorrhage, and post-contrast parenchymal/
imaging characteristics, differential diagnoses would leptomeningeal enhancement.
Further reading
Bartynski WS. Posterior reversible McKinney AM, Short J, Truwit CL,
encephalopathy syndrome, part 1: McKinney ZJ, Kozak OS, SantaCruz KS,
fundamental imaging and clinical et al. Posterior reversible encephalopathy
features. AJNR Am J Neuroradiol 2008; syndrome: incidence of atypical regions
29(6): 1036–1042. of involvement and imaging findings. AJR
Am J Roentgenol 2007; 189(4): 904–912.
176
Case 6.2
History
Patient 1: 59-year-old male with hepatitis C presents with
acute altered mental status.
Patient 2: 67-year-old female with history of alcoholic
cirrhosis presents with altered mental status.
Findings
Patient 1
Figure 6.2.1 Axial FLAIR shows symmetric cortical edema and FLAIR
hyperintensity in the insula, cingulate, and frontal cortices.
Figure 6.2.2 Axial DWI demonstrates restricted diffusion in the

corresponding regions.
Patient 2
Figure 6.2.3 NECT shows diffuse cerebral edema with sulcal effacement and
loss of gray–white matter differentiation. Note the poor definition of basal
ganglia.
Diagnosis
Figure 6.2.3 (patient 2) Acute hepatic (hyperammonemic) encephalopathy.
177
Section 1: Brain
Differential diagnosis the patient’s symptoms, although CT can show

r Hypoxic–ischemic encephalopathy. generalized edema in severe cases.
b MRI:
r Hypoglycemia.
r Toxic encephalopathy (carbon monoxide, ethelyene glycol, Chronic hepatic encephalopathy is typically
manifested by T1 hyperintensity in the globus
illicit drugs).
r Extrapontine myelinolysis. pallidi, subthalamic regions, and midbrain due to
r Creutzfeldt–Jakob disease. manganese deposition.
Acute hepatic encephalopathy may show diffuse
cortical/subcortical edema (T2/FLAIR
Key points hyperintensity and restricted diffusion) with
r Hepatic encephalopathy describes a spectrum of relative sparing of the perirolandic and occipital
neuropsychiatric abnormalities seen in patients with liver regions. It has been reported that symmetric
dysfunction and/or portosystemic shunting. involvement of the cingulate gyrus and insular
r The clinical signs and symptoms are variable, ranging from cortex is a more specific imaging feature
subtle behavioral changes and confusion to overt coma and for acute hepatic encephalopathy, while
death. additional cortical involvement is variable and
r Imaging: asymmetric.
b CT is less sensitive and is usually obtained to exclude r Treatment: treat underlying causes, lactulose.
acute intracranial abnormalities that may contribute to
Further reading
U-King-Im JM, Yu E, Bartlett E, Soobrah R,
Kucharczyk W. Acute hyperammonemic
encephalopathy in adults: imaging
findings. AJNR Am J Neuroradiol 2011;
32(2): 413–418.
178
Case 6.3
History
59-year-old female with history of poor oral intake presents
with subacute onset of altered mental status.
Findings
Figures 6.3.1–6.3.3 Axial FLAIR images demonstrate symmetric FLAIR
hyperintensity in the bilateral medial thalami (Fig. 6.3.1), hypothalami (Fig. 6.3.2,
arrows), tectal plate (Fig. 6.3.2, curved arrows), and periaqueductal gray matter
(Fig. 6.3.3).
Diagnosis
Wernicke’s encephalopathy.
r Encephalitis: certain viral infections such as Japanese
encephalitis and West Nile fever can cause symmetric
involvement of the thalami, basal ganglia, and brainstem.
r Other metabolic diseases such as Leigh syndrome, Wilson’s
Figure 6.3.3 disease, osmotic demyelination.
179
Section 1: Brain
r Toxic encephalopathy, such as metronidazole-induced r Clinical presentation: variable. Only a minority of patients
encephalopathy. present with the classic triad of ataxia, altered mentation,
r Arterial or venous occlusion: symmetric involvement of and oculomotor dysfunction. If untreated, amnesia,
thalami can also be caused by occlusion of rostral basilar psychosis, coma, and even death can occur.
artery or rarely the artery of Percheron. Thrombosis of r Imaging:
deep venous system (internal cerebral vein, vein of Galen, b On MRI, increased T2/FLAIR signal are typically seen
straight sinus) can cause venous infarction of the thalamus in the periventricular regions of the third ventricle
and basal ganglia without cerebral lobar involvement. including medial thalami, hypothalami, tectal plates,
r Variant Creutzfeldt–Jakob disease.
and periaqueductal gray matter.
r Primary CNS lymphoma: commonly involves the deep b Atypical areas of involvement include cerebellum,
hemispheric periventricular white matter, corpus callosum, cranial nerve nuclei, and cerebral cortex. These are
and basal ganglia, especially in immunocompromised probably more commonly seen in nonalcoholic
patients. patients, although this is debatable.
b DWI may show restricted diffusion in acute Wernicke’s
Key points encephalopathy.
r Wernicke’s encephalopathy is a potentially lethal b Enhancement of the affected areas, more frequently
emergency caused by thiamine deficiency. It is most seen in alcoholic patients.
frequently seen in chronic alcohol abuse, although it has r Treatment: supplementation of thiamine by intravenous or
also been described in nonalcoholic patients with intramuscular routes, followed by oral administration.
malnutrition.
Further reading
Zuccoli G, Santa Cruz D, Bertolini M,
Rovira A, Gallucci M, Carollo C, et al.
MR imaging findings in 56 patients with
Wernicke encephalopathy: nonalcoholics
may differ from alcoholics. AJNR Am J
Neuroradiol 2009; 30(1): 171–176.
180
Case 6.4
Figure 6.4.2
Figure 6.4.1
181
Section 1: Brain
r Hypertensive encephalopathy or posterior reversible

History
encephalopathy (PRES).
31-year-old male was found unresponsive with severe r Progressive multifocal leukoencephalopathy.
respiratory distress due to opioid overdose. He was initially r Acute demyelinating encephalomyelitis (ADEM).
resuscitated, but developed progressive agitation, with
hallucination after a few days, eventually leading to coma
and death. Key points
r Although gray matter is more vulnerable to
hypoxic–ischemic injury than white matter, cases of
Findings isolated white-matter injury have been well documented.
In many patients, acute onset of neuropsychiatric
Figures 6.4.1, 6.4.2 Axial FLAIR images demonstrate diffuse symmetric
FLAIR hyperintensity involving the subcortical and periventricular white matter symptoms may happen days to weeks following apparent
of bilateral frontal, parietal (Fig. 6.4.1), temporal, and occipital lobes (Fig. 6.4.2). recovery of cerebral hypo-oxygenation, therefore termed
delayed post-hypoxic leukoencephalopathy.
Figures 6.4.3, 6.4.4 DWI (Fig. 6.4.3) and ADC (Fig. 6.4.4) show restricted r The etiology of hypoxia varies: cardiac arrest, drug
diffusion in the corresponding regions.
overdose, carbon monoxide intoxication, etc. Clinical
symptoms typically include disorientation, hallucination,
Diagnosis amnesia, parkinsonism, and psychosis.
r MRI typically shows diffuse T2/FLAIR hyperintensity and
Delayed post-hypoxic leukoencephalopathy.
restricted diffusion in the white matter of bilateral cerebral
hemispheres, usually sparing the posterior fossa, in
Differential diagnosis keeping with acute demyelination and cytotoxic
r Heroin inhalation-induced leukoencephalopathy: typically edema.
involves the cerebellar, posterior cerebral white matter, and r Treatment: supportive care, rehabilitation. Although
posterior limbs of internal capsule without restricted outcome is usually bad, complete clinical recovery has
diffusion (“chasing the dragon”). been reported in some patients.
Further reading
Chalela JA, Wolf RL, Maldjian JA, Kasner Molloy S, Soh C, Williams TL. Reversible
SE. MRI identification of early white delayed posthypoxic leukoencephalo-
matter injury in anoxic-ischemic pathy. AJNR Am J Neuroradiol 2006;
encephalopathy. Neurology 2001; 56(4): 27(8): 1763–1765.
481–485.
182
Case 6.5
History
27-year-old man with history of type I diabetes mellitus and
recent hospitalization for diabetic ketoacidosis, presents
with ataxia and slurred speech.
Findings
Figure 6.5.1 Axial T2 shows diffuse hyperintensity of the pons with sparing of
the periphery and corticospinal tracts.
Figure 6.5.2 Axial DWI demonstrates restricted diffusion of the pons (ADC
map not shown).
Figure 6.5.3 Axial post-contrast T1 shows no perceptible enhancement.
Diagnosis
Central pontine myelinolysis (CPM).
r Ischemic infarct: usually (although not always) asymmetric
Figure 6.5.3 and respecting the midline due to the distribution of
183
Section 1: Brain
pontine perforators, and typically involves both central and r Clinical presentations include altered mental status,
peripheral fibers. pseudobulbar symptoms, spastic tetraparesis, or even
r Demyelinating disease. “locked in” syndrome.
r Rhomboencephalitis. r MRI is the diagnostic modality of choice.
r Infiltrating neoplasm. b The imaging findings in the acute phase include
confluent FLAIR/T2 hyperintensity in the pons (CPM),
Key points and in basal ganglia and cerebral white matter (EPM).
b Sparing of peripheral pontine fibers and corticospinal
r Osmotic demyelination syndrome consists of central
pontine myelinolysis (CPM) and extrapontine myelinolysis tracts are characteristic for CPM.
b DWI typically shows restricted diffusion in the acute or
(EPM).
r Etiology is likely demyelination due to rapid shifts of subacute phase. Diffusion abnormality normalizes in
serum osmolality, classically secondary to rapid correction chronic phase.
b Lesions usually do not enhance.
of hyponatremia, but can occur in normonatremic patients
as well. r Treatment is mostly supportive.
Further reading
Howard SA, Barletta JA, Klufas RA, Saad A,
De Girolami U. Best cases from the AFIP:
osmotic demyelination syndrome.
Radiographics 2009; 29(3): 933–938.
184
Case 6.6
185
Section 1: Brain
r The differential diagnosis of lesions involving the splenium

History
of the corpus callosum includes viral encephalitis, seizure
60-year-old woman presents with altered mental status and
or antiepileptic medications, demyelinating lesions such as
ataxia. She was recently treated for infectious colitis.
osmotic myelinolysis and Marchiafava–Bignami disease,
and intoxication from methotrexate, 5-fluorouracil, etc.
Findings
Figure 6.6.1 Axial FLAIR shows symmetric hyperintensity in the bilateral
cerebellar dentate nuclei (curved white arrows), dorsal pons involving the
Key points
vestibular nuclei (white arrows), and central tegmental tracts (black arrows).
r Metronidazole is a commonly used antibiotic to treat
anaerobic infections. A number of neurological side effects
Figure 6.6.2 Axial DWI reveals mild diffusion restriction of dentate nuclei have been reported including cerebellar symptoms,
(ADC not shown), while the pontine lesions do not show diffusion abnormality.
encephalopathy, seizures, altered mentation, and
Figures 6.6.3, 6.6.4 Axial FLAIR demonstrates hyperintensity of splenium of peripheral neuropathy.
corpus callosum (Fig. 6.6.3, arrow) and midbrain (Fig. 6.6.4, curved arrows), r MRI features: the most frequently involved brain
without diffusion restriction (not shown). structures include cerebellar dentate nuclei, midbrain,
corpus callosum, pons, medulla, hemispheric subcortical
Diagnosis white matter, and basal ganglia.
r Most lesions demonstrate vasogenic edema with T2/FLAIR
Metronidazole-induced toxic encephalopathy.
hyperintensity, while sometimes lesions in dentate nuclei
or splenium of corpus callosum may have associated
Differential diagnosis diffusion restriction in keeping with cytotoxic edema. No
r Acute Wernicke’s encephalopathy. enhancement is seen.
r A few other rare conditions such as methyl bromide r Lesions should be bilateral and symmetric, which is a
intoxication, maple-syrup disease, and cerebellitis may typical feature for metabolic or toxic encephalopathy. Most
cause symmetric signal abnormalities in the cerebellar lesions are reversible after cessation of metronidazole.
dentate nuclei. r Treatment: cessation of metronidazole, supportive.
Further reading
Kim E, Na DG, Kim EY, Kim JH, Son KR,
Chang KH. MR imaging of
metronidazole-induced encephalopathy:
lesion distribution and diffusion-
weighted imaging findings. AJNR Am J
Neuroradiol 2007; 28(9): 1652–1658.
186
Case 6.7
187
Section 1: Brain
188
189
Section 1: Brain
190
History Diagnosis
Patient 1: 39-year-old man with history of Radiation necrosis.
craniopharyngioma status post trans-sphenoidal tumor
resection and radiation, presents with headache, lethargy,
and frequent falls. Differential diagnosis
r Recurrent, residual, or metastatic tumor.
Patient 2: 46-year-old man with history of nasopharyngeal
carcinoma status post radiation develops a left temporal
lobe lesion on the routine post-treatment MRI. Key points
Patient 3: 67-year-old woman with history of metastatic r There are three types of brain radiation changes:
lung cancer status post whole-brain radiation, presents with b Acute: occurs during radiation.
worsening headache and weakness. b Subacute: occurs up to 3 months after radiation ends.
Patient 4: 52-year-old man with history of glioblastoma b Delayed: occurs months to years after radiation,
multiforme (GBM) status post biopsy, temozolomide commonly referred to as radiation necrosis.
chemotherapy, and radiation, develops a new enhancing r There are two typical clinical scenarios of radiation
lesion in the right frontal lobe. necrosis:
b Radiation therapy for head/neck malignancy,
Findings extra-axial brain tumor, or brain arteriovenous
malformation: the diagnosis is usually not difficult,
Patient 1 since the location of radiation-induced injury
Figure 6.7.1 Sagittal post-contrast T1 demonstrates a geographic area of characteristically occurs along the path of the radiation
peripheral enhancement in the body of corpus callosum. An additional enhanc-
ing area is also seen in the pons. port and should not be confused with recurrent or
metastatic tumor. For example, temporal lobe necrosis
Figures 6.7.2, 6.7.3 Axial T2 images show associated vasogenic edema in the frequently occurs in irradiated patients with
bilateral frontal lobes and pons.
nasopharyngeal cancer due to the incorporation of the
temporal lobes in the radiation field, as demonstrated
in patient 2. In patient 1, the enhancing lesions in the
Patient 2
corpus callosum and pons are determined to be
Figures 6.7.4, 6.7.5 Coronal post-contrast T1 (Fig. 6.7.4) demonstrates an
enhancing focus in the left temporal lobe with associated vasogenic edema on
radiation necrosis after reviewing the radiation
coronal T2 (Fig. 6.7.5). plan.
b Radiation therapy for primary or metastatic brain
tumors: difficult to differentiate from recurrent/
Patient 3 residual disease. Currently, the only method of
Figures 6.7.6, 6.7.7 Axial post-contrast T1 (Fig. 6.7.6) demonstrates enhanc- distinguishing radiation injury (or, broadly speaking,
ing lesions in the left frontal and posterior right frontal lobe. Axial FLAIR (Fig. 6.7.7) pseudoprogression as a combined treatment effect of
shows extensive subcortical and periventricular hyperintensity, likely reflecting a radiation and chemotherapy) from recurrent/residual
combination of radiation-induced leukoencephalopathy and vasogenic edema.
The enhancing lesions may represent radiation necrosis or recurrent tumor, tumor is to perform surgical biopsy or serial MRI
which are difficult to differentiate based on conventional imaging. follow-up.
Figure 6.7.8 Single-voxel spectroscopy of the right frontal lesion demon-
strates a prominent lipid/lactate peak at 1.3 ppm, suggestive of necrosis, Table 6.7.1 Advanced imaging techniques to distinguish radiation
although a high choline peak at 3.2 ppm is also observed with choline/NAA ratio necrosis and recurrent/residual tumor
⬎ 2, consistent with recurrent/residual tumor. Radiation necrosis and recurrent
tumor frequently coexist. Recurrent/residual
Radiation necrosis tumor
Proton Low choline, low NAA, High choline, low NAA,
Patient 4 spectroscopy elevated lipid/lactate elevated lipid/lactate
(necrotic tumor)
Figures 6.7.9–6.7.11 Axial post-contrast T1 (Fig. 6.7.9) demonstrates a ring-
enhancing lesion in the inferior right frontal lobe. There is adjacent FLAIR hyper- MRI perfusion Decreased relative Elevated relative
intense signal in the bilateral inferior frontal lobes (Fig. 6.7.10). Multivoxel spec- cerebral blood volume cerebral blood volume
troscopy at the region of enhancement (Fig. 6.7.11) shows markedly elevated (rCBV) (rCBV)
choline peak at 3.2 ppm and diminished NAA at 2.0 ppm, consistent with recur- Diffusion-weighted Normal or facilitated Restricted diffusion
rent/residual tumor. imaging diffusion favors due to tumor
radiation changes hypercellularity
Figure 6.7.12 The spectrum of a slightly posterior voxel at the region of non-
FDG-PET Decreased metabolic Increased metabolic
enhancing FLAIR hyperintensity also shows markedly elevated choline and mod-
activity activity
erately diminished NAA, which is frequently observed in the infiltrative glioma.
191
Section 1: Brain
r Imaging: b T2/FLAIR: Hyperintense vasogenic edema and/or

b Post-contrast T1 shows enhancing lesions in the leukoencephalopathy, but difficult to distinguish from
radiation field. Certain lesion morphologies infiltrative tumor.
b Advanced imaging techniques (Table 6.7.1) can be
(such as “cut green pepper,” “soap bubble,”
“Swiss cheese,” or “spreading wavefront” helpful in making the distinction in some cases,
enhancement patterns) are frequently seen in although these have not been validated in prospective
radiation necrosis but can be seen in recurrent/ clinical trials.
residual tumor as well. r Treatment: steroid, hyperbaric oxygen, bevacizumab.
Further reading
Fink JR, Carr RB, Matsusue E, Iyer RS, Hygino da Cruz LC, Jr, Rodriguez I, Shah R, Vattoth S, Jacob R, Manzil FF,
Rockhill JK, Haynor DR, et al. Domingues RC, Gasparetto EL, O’Malley JP, Borghei P, et al. Radiation
Comparison of 3 Tesla proton MR Sorensen AG. Pseudoprogression and necrosis in the brain: imaging features
spectroscopy, MR perfusion and MR pseudoresponse: imaging challenges in and differentiation from tumor
diffusion for distinguishing glioma the assessment of posttreatment glioma. recurrence. Radiographics 2012; 32(5):
recurrence from posttreatment effects. J AJNR Am J Neuroradiol 2011; 32(11): 1343–1359.
Magn Reson Imaging 2012; 35(1): 56–63. 1978–1985.
192
Case 6.8
Figure 6.8.1
Figure 6.8.2
193
Section 1: Brain
Figure 6.8.6
Figure 6.8.5
History genetic syndrome caused by mutations of various

13-month-old boy presents with hypotonia and nystagmus. mitochondrial or nuclear DNAs involved in the respiratory
chain complex and oxidative metabolism.
r The majority of patients present during infancy or early
Findings childhood, before 2 years of age. Late childhood and adult
Figures 6.8.1, 6.8.2 Axial T2 MRI images demonstrate symmetrically
onset has rarely been reported as well.
increased signal intensity in the bilateral basal ganglia (Fig. 6.8.1, arrows) and
r Clinical presentation is variable, including developmental
dorsomedial thalami (Fig. 6.8.1, block arrows), substantia nigra (Fig. 6.8.2, delay, psychomotor regression, weakness, dystonia, and
curved arrows) and periaqueductal gray matter (Fig. 6.8.2, dashed arrows).
brainstem dysfunction such as ataxia, ophthalmoplegia,
Figures 6.8.3, 6.8.4 Axial DWI shows restricted diffusion in the swallowing, and breathing difficulties.
corresponding regions. r Neuroimaging:
b The pattern of involvement varies depending on the
Figures 6.8.5, 6.8.6 Single-voxel proton spectroscopy images show a
prominent lactate doublet at 1.3 ppm (arrow) using a short echo time of 30 underlying genetic cause. The typical findings are
(Fig. 6.8.5), which inverts when using an intermediate echo time of 135 symmetric involvement of the bilateral basal ganglia,
(Fig. 6.8.6).
thalami, and brainstem structures such as subthalamic
nuclei, cerebral peduncles, periaqueductal gray matter,
Diagnosis central tegmental tracts, inferior olivary nuclei, etc.
Leigh syndrome. b In patients with SURF1 mutation, involvement of lower
brainstem with sparing of basal ganglia is characteristic.
b Cerebellar nuclei, cerebellar and cerebral white matter,
Differential diagnosis and spinal cord can also be affected. Isolated
r Other mitochondrial diseases. involvement of cerebral white matter is rare in
r Hypoxic–ischemic injury. Leigh syndrome, which distinguishes it from
r Toxic and metabolic encephalopathy (such as Wernicke’s). leukoencephalopathy.
r Wilson’s disease. b The areas of involvement demonstrate low attenuation
r Viral encephalitis. on CT, T1 hypointensity, and T2/FLAIR hyperintensity
on MRI.
b DWI shows restricted diffusion in acute disease and
Key points facilitated diffusion in chronic disease.
r Leigh syndrome, aka subacute necrotizing b Proton spectroscopy may show a large lactate peak and
encephalomyelopathy, is a progressive neurodegenerative decreased NAA in acute disease. However, the lactate
194
peak may be absent in chronic disease and is a r Treatment: supportive therapy, cofactor supplementation.
nonspecific finding as it can be present in other Poor prognosis.
conditions such as stroke and neoplasms.
Further reading
Rossi A, Biancheri R, Bruno C, Di Rocco M,
Calvi A, Pessagno A, et al. Leigh
syndrome with COX deficiency and
SURF1 gene mutations: MR imaging
24(6): 1188–1191.
195
Section 1: Brain
Case 6.9
Figure 6.9.1
Figure 6.9.2
196
Diagnosis
Maple-syrup urine disease (MSUD).
r Other inherited metabolic disease.
r Hypoxic–ischemic encephalopathy.
Key points
r MSUD is an autosomal recessive genetic disorder due to a
defect in the oxidative decarboxylation of the branched-
chain ␣-keto acids. The defect causes accumulation of
leucine, isoleucine, valine, and their corresponding keto
acids in serum, cerebrospinal fluid, and urine, which lead
to severe brain damage if untreated.
r Clinical presentation: typically normal at birth and
becomes symptomatic during the first week of life, with
poor feeding, vomiting, dystonia, lethargy, failure to
thrive, seizure, etc. A urine odor of maple syrup is
characteristic.
r Imaging:
Figure 6.9.5
b Profound edema in the cerebellar white matter, dorsal
brainstem, cerebral peduncles, posterior limb of
internal capsule, globi pallidi, and perirolandic regions.
History CT: low attenution. FLAIR/T2: hyperintense. T1:
10-day-old infant presents with vomiting and seizure. hypointense.
b Infratentorial ⬎ supratentorial.
b Characteristic involvement of corticospinal tracts and
Findings
central tegmental tracts in the pons is diagnostic.
Figure 6.9.1 NECT shows diffuse edema in the supratentorial and b DWI: restricted diffusion due to intramyelinic edema.
infratentorial white matter.
b MR spectroscopy: branched-chain ␣-keto acids peak at
Figures 6.9.2–6.9.4 Axial DWI images demonstrate diffusion restriction in 0.9 ppm.
the bilateral perirolandic regions (Fig. 6.9.2), basal ganglia and posterior limbs r Treatment:
of internal capsules (Fig. 6.9.3), pontine and cerebellar white matter (Fig. 6.9.4).
Note the characteristic involvement of both corticospinal tracts (white arrows) b Treatment of acute metabolic crisis with dialysis.
and tegmental tracts (black curved arrow) in the pons (Fig. 6.9.4). b Life-long dietary restriction and monitoring of
Figure 6.9.5 Axial T2 demonstrates hyperintensity of pontine and cerebellar branched-chain amino acids to avoid brain injury.
white matter.
Further reading
Parmar H, Sitoh YY, Ho L. Maple syrup
urine disease: diffusion-weighted and
diffusion-tensor magnetic resonance
imaging findings. J Comput Assist Tomogr
2004; 28(1): 93–97.
197
Section 1: Brain
Case 6.10
198
r Approximately 60% patients with paraneoplastic or

History
nonparaneoplastic autoimmune encephalitis have
65-year-old man with history of small-cell lung cancer
identifiable serum antigens. The neuronal cell surface
develops hallucination and multiple episodes of seizure.
antigens include anti-NMDAR, anti-
AMPAR, anti-GABABR, and anti-VGKC-associated, and
examples of the intracellular antigens include anti-Hu,
Findings anti-Yo, anti-CV2, and anti-Ma2.
Figures 6.10.1, 6.10.2 Axial FLAIR images demonstrate hyperintensity in r Patients with limbic encephalitis typically present with
the bilateral medial temporal lobes (Fig. 6.10.1) and subinsular regions (Fig.
6.10.2).
subacute onset of seizures, memory loss, confusion, and
psychiatric symptoms.
Figure 6.10.3 Post-contrast axial T1 shows no enhancement. r Imaging:
b The majority of patients with autoimmune encephalitis
Figure 6.10.4 Axial DWI shows no diffusion restriction.
have normal brain MRIs.
b Abnormal MRI patterns include:
Diagnosis Limbic encephalitis (most common).
Paraneoplastic limbic encephalitis. Cerebellar degeneration.
Brainstem encephalitis.
Striatal encephalitis.
Differential diagnosis Leukoencephalopathy.
b Limbic encephalitis:
r Viral encephalitis such as herpes encephalitis, human
herpesvirus 6 encephalitis, etc.
It is characterized by T2/FLAIR hyperintensity in
r Infiltrating neoplasm. the medial temporal lobes, typically involving the
r Status epilepticus. amygdala and hippocampus.
This pattern can be unilateral or bilateral,
symmetric or asymmetric.
Key points Extrahippocampal limbic structures such as insula
r Paraneoplastic neurological syndrome is a heterogeneous or cingulate cortex can also be involved.
Diffusion restriction and enhancement are rare.
disorder associated with a number of systemic cancers,
most commonly including lung, breast, ovarian, and r Treatment: treating the underlying neoplasm and
lymphoma. Sometimes the primary malignancy cannot be immunomodulatory therapy. In general, patients with
identified despite extensive workup. cell-surface antigens tend to be more responsive to
r This syndrome is believed to be caused by autoantibodies treatment than those whose condition is associated with
and T-cell responses against either neuronal cell-surface intracellular antigens.
antigens or intracellular antigens, which cross-react with
the tumor antigens.
Further reading
Bataller L, Kleopa KA, Wu GF, Rossi JE, Vernino S, Geschwind M, Boeve B.
Rosenfeld MR, Dalmau J. Autoimmune Autoimmune encephalopathies.
limbic encephalitis in 39 patients: Neurologist 2007; 13(3): 140–147.
immunophenotypes and outcomes. J
Neurol Neurosurg Psychiatry 2007; 78(4):
381–385.
199
Section 1: Brain
Case 6.11
200
Diagnosis
Idiopathic intracranial hypertension (IIH) (pseudotumor
cerebri).
r Other causes of increased intracranial pressure (ICP) such
as tumor, hemorrhage, infection, hydrocephalus, and dural
sinus thrombosis.
Key points
r Normal ICP ranges between 8 and 20 cm H2 O in adults
and older children, 1–10 cm H2 O in younger children and
can be up to 25 cm H2 O in obese patients.
r IIH is due to chronically increased ICP without a known
cause.
r It classically affects obese women of childbearing age, with
typical clinical presentations consisting of headache, vision
change, pulsatile tinnitus, photophobia, eye pain, etc.
Diminished visual acuity, visual field loss, and papilledema
can be observed on ophthalmologic exams.
r The diagnosis is usually made based on clinical history,
Figure 6.11.5
examination, and increased CSF opening pressure.
r Imaging:
b Neuroimaging studies can be unremarkable even in
History
patients with markedly increased ICP, and are
15-year-old girl presents with severe headache and acute
primarily performed to exclude secondary causes of
onset of bilateral vision loss. CSF opening pressure
increased ICP.
measures 58 cm H2 O. b Several neuroimaging findings have been identified to
support the diagnosis of IIH, including:
Findings Dilation of optic nerve sheath.
Figure 6.11.1 Coronal STIR shows widening of bilateral optic nerve sheaths Flattening of posterior globe/sclera.
with expanded CSF surrounding the optic nerves. Increased tortuosity of optic nerves.
Intraocular protrusion of optic disc.
Figure 6.11.2 Axial T2 again shows the widened right optic nerve sheath and
flattening of posterior sclera (arrow). Also note intraocular protrusion of left
Empty sella.
optic nerve head (block arrow). Similar findings are observed in both globes on Cerebellar tonsil ectopia.
different slices, corresponding to papilledema on the fundoscopic exam. Slit-like ventricles.
Stenosis of dural sinuses can be identified in the
Figure 6.11.3 Axial post-contrast T1 with fat saturation reveals enhancement
of the bilateral optic nerve sheaths and left optic nerve head, reflecting venous majority of patients with IIH, although it is
congestion from increased intracranial pressure. controversial whether this finding represents the
cause or simply a consequence of chronically
Figure 6.11.4 2D TOF MRV demonstrates severe focal stenosis of left
transverse sinus (block arrow). The right transverse/sigmoid sinuses are elevated ICP.
diffusely small in caliber (arrows), which is congenital. r Treatment: conservative treatments such as weight
Figure 6.11.5 Sagittal T1 shows downward displacement of cerebellar
reduction and acetazolamide etc. Optic nerve sheath
tonsils. Also note empty sella, due to chronic compression and bony fenestration and CSF shunting are reserved for patients
remodeling of CSF pulsation. failing the conservative treatments.
Further reading
Degnan AJ, Levy LM. Pseudotumor cerebri: Farb RI, Vanek I, Scott JN, Mikulis DJ, Yuh EL, Dillon WP. Intracranial
brief review of clinical syndrome and Willinsky RA, Tomlinson G, et al. hypotension and intracranial
imaging findings. AJNR Am J Neuroradiol Idiopathic intracranial hypertension: the hypertension. Neuroimaging Clin N Am
2011; 32(11): 1986–1993. prevalence and morphology of 2010; 20(4): 597–617.
sinovenous stenosis. Neurology 2003;
60(9): 1418–1424.
201
Section 1: Brain
Case 6.12
202
203
Section 1: Brain
Figure 6.12.6 Coronal post-contrast T1 reveals diffuse, smooth dural

enhancement.
Figures 6.12.7, 6.12.8 Sagittal (Fig. 6.12.7) and axial T2 (Fig. 6.12.8) MRI of
the thoracic spine demonstrate ventral and dorsal epidural fluid collections in
the cervical and thoracic spine (arrows).
Figure 6.12.9 Post-myelogram CT reveals contrast material within the ventral

epidural fluid collection (∗ ), confirming CSF leak.
Diagnosis
Intracranial hypotension due to CSF leak.
r Intracranial hypotension is frequently misdiagnosed,
especially if MRI is not performed. It can be mistaken for
migraine, meningitis, subdural hematoma, Chiari I
malformation, etc.
Key points
Figure 6.12.9 (patient 2) r Intracranial hypotension is due to CSF leakage through a
dural defect. Spontaneous intracranial hypotension can
History be caused by rupture of meningeal diverticula or dural
Patient 1: 57-year-old woman with recent lumbar surgery perforation from disc-osteophytes, although no cause
develops severe headache, vomiting, and altered mental can be identified in many patients. Secondary causes
status. include lumbar puncture and brain/spinal surgery or
Patient 2: 40-year-old woman presents with orthostatic trauma.
r The symptoms include orthostatic headache, neck stiffness,
headache over a few months.
tinnitus, nausea/vomiting, and photophobia. Downward
brain herniation with progressive mental status decline,
Findings coma, and death has been reported in untreated severe
Patient 1 cases.
r The clinical diagnosis is typically made based on history,
Figure 6.12.1 NECT demonstrates subdural collections along the bilateral
convexities (arrows), hypodense on the right and hyperdense on the left symptoms, and low CSF opening pressure (⬍ 6 cm H2 O).
compared to brain parenchyma, suggestive of subdural hematomas of various r Imaging:
ages.
b Brain sagging is the most specific MRI feature, as a
Figure 6.12.2 NECT shows effacement of basilar cistern and anterior direct result of low CSF pressure.
displacement of the pons against the clivus. b According to the Monro–Kellie doctrine, intracranial
Figure 6.12.3 NECT shows low-lying cerebellar tonsils (arrows). blood volume increases to compensate for the loss
of CSF to keep intracranial volume constant, which
Figure 6.12.4 Axial CT of the abdomen reveals lumbar laminectomy is manifested on MRI as engorgement of dural
changes, as well as postsurgical fluid collections within the dorsal soft tissue
(arrows) and the right psoas muscle (∗ ). venous sinuses/epidural venous plexus and dural
enhancement.
b Subdural fluid collections can occur as a result of
Patient 2 transudation of fluid from the venous structures.
Figure 6.12.5 Sagittal post-contrast T1 demonstrates brain sagging, with b CT or MR myelogram can be used to identify the
obliteration of prepontine cistern and flattening of the pons against the clivus,
obliteration of suprasellar cistern and draping of optic chiasm over the
source of CSF leakage.
pituitary, as well as descent of cerebellar tonsils. Engorgement of dural venous r Intracranial hypotension can be managed by conservative
sinuses and epidural venous plexus in the skull base and upper cervical spine
are also noted.
treatment, epidural blood patch, or surgery.
204
Further reading
Schievink WI. Misdiagnosis of spontaneous Yuh EL, Dillon WP. Intracranial
intracranial hypotension. Arch Neurol hypotension and intracranial
2003; 60(12): 1713–1718. hypertension. Neuroimaging Clin N Am
2010; 20(4): 597–617.
205
Section 1: Brain
Case 6.13
206
History Diagnosis
Patient 1: 60-year-old man presents with status epilepticus. Peri-ictal signal changes.
Patient 2: 50-year-old women with known history of left
parietal meningioma develops complex partial seizure with Differential diagnosis
secondary generalization. r Ischemic infarction.
r Infiltrative tumor.
r Viral or limbic encephalitis.
Findings
r Generalized tonic–clonic seizure or status epilepticus
Figure 6.13.1 Axial FLAIR shows asymmetric swelling and hyperintensity of
left hippocampus and uncus (arrow). can induce transient MRI signal changes in the cortex,
subcortical white matter, hippocampus, corpus callosum,
Figure 6.13.2 Coronal T2 confirms swelling and hyperintensity of left
hippocampus (arrow). No evidence of hippocampal atrophy.
basal ganglia, or cerebellum, manifested by swelling,
T2/FLAIR hyperintensity, diffusion restriction, and
Figure 6.13.3 Axial DWI reveals hyperintensity of left hippocampal tail parenchymal/leptomeningeal enhancement.
(arrow). Corresponding ADC map (not shown) shows mild restricted diffusion. r This phenomenon is thought to reflect seizure-related
cytotoxic edema, and is typically reversible on the
follow-up MRI after seizure stops.
Patient 2 r Although mostly transient in nature, prolonged or
Figure 6.13.4 Axial T2 demonstrates the known left parietal meningioma (∗ ) repeated seizure activities can lead to laminar necrosis
with vasogenic edema anterior to it (black arrow). There are additional areas of
cortical swelling and hyperintensity in the left parietal and frontal regions and permanent brain damage, and the edema of the
(white arrows), which was not present on the prior studies. hippocampus may be the initial step in the development of
hippocampal sclerosis.
Figure 6.13.5, 6.13.6 DWI and ADC reveal restricted diffusion in r Treatment: antiepileptics. Follow-up MRI may be needed
the corresponding cortical regions (white arrows), while the vasogenic
edema anterior to the meningioma shows facilitated diffusion (black to confirm resolution and exclude other more ominous
arrow). processes.
207
Section 1: Brain
Further reading
Cianfoni A, Caulo M, Cerase A, Della Marca Donaire A, Carreno M, Gomez B, Fossas P, Kim JA, Chung JI, Yoon PH, Kim DI, Chung
G, Falcone C, Di Lella GM, et al. Bargallo N, Agudo R, et al. Cortical TS, Kim EJ, et al. Transient MR signal
Seizure-induced brain lesions: a wide laminar necrosis related to prolonged changes in patients with generalized
spectrum of variably reversible MRI focal status epilepticus. J Neurol tonicoclonic seizure or status epilepticus:
abnormalities. Eur J Radiol 2013; 82(11): Neurosurg Psychiatry 2006; 77(1): periictal diffusion-weighted imaging.
1964–1972. 104–106. AJNR Am J Neuroradiol 2001; 22(6):
1149–1160.
208
Case 6.14
r Dentate gyrus, CA1, and CA4 regions are most commonly

History affected, with inhibitory interneuronal loss and gliosis
31-year-old male presents with seizure.
characterizing the pathology.
r Imaging:
Findings b Temporal lobe-specific MRI protocol is necessary to
Figures 6.14.1, 6.14.2 Coronal T2 (Fig. 6.14.1) and FLAIR (Fig. 6.14.2) ensure high sensitivity and specificity.
demonstrate atrophy and hyperintensity of right hippocampus. b Primary abnormalities include T2/FLAIR
hyperintensity, atrophy, and obscuration of internal
Diagnosis architecture of the hippocampus.
Mesial temporal sclerosis (MTS). b Secondary abnormalities include atrophy of ipsilateral
limbic structures including fornix and mammillary
body, enlargement of ipsilateral ventricular temporal
Differential diagnosis horn, and choroidal fissure.
r Cortical dysplasia (often concomitant). b DWI shows increased signal (T2 shine-through) but no
r Low-grade neoplasm (astrocytoma, DNET, etc.).
diffusion restriction. No post-contrast enhancement.
r Status epilepticus. b Quantitative volumetric analysis can be instructive in
less clear cases.
Key points b MTS is found bilaterally in approximately 10% of
r MTS is the most common cause of partial complex patients – using symmetry as a marker of normality can
epilepsy in adults. lead to misdiagnosis.
r It is controversial whether MTS is acquired, congenital, or r Treatment: Medical management with anti-epileptic drugs
a combination of both. It may be secondary to childhood is first-line therapy. If intractable, anterior temporal
febrile seizures. lobectomy produces a 70–95% success rate.
Further reading
Bote RP, Blazquez-Llorca L, Fernandez-Gil
MA, Alonso-Nanclares L, Munoz A,
De Felipe J. Hippocampal sclerosis:
histopathology substrate and magnetic
resonance imaging. Semin Ultrasound CT
MR 2008; 29(1): 2–14.
209
Section 1: Brain
Case 6.15
210
History Key points

44-year-old woman with history of prior pontine stroke r WD is the antegrade disintegration of axon and its myelin
presents with progressive ataxia. sheath following injury to the proximal axon or neuron cell
bodies.
r In CNS, the most common WD involves the corticospinal
Findings tracts after infarction of the motor strip, although other
white-matter tracts such as corpus callosum, middle
Figure 6.15.1 Axial T2 shows left paramedian pontine T2 hyperintensity, cerebellar peduncles, and optic radiation can also be
consistent with chronic infarction from the occlusion of a pontine perforating
branch. involved.
r In pons, the pontine nuclei receive input from the
Figure 6.15.2 Axial FLAIR at a slightly more caudal level demonstrates ipsilateral cerebral cortex and project pontocerebellar
hyperintensity of bilateral middle cerebellar peduncles (arrows), more
prominent on the left. Also note the hyperintensity of the left corticospinal
fibers to the contralateral cerebellum via the middle
tract (block arrow). cerebellar peduncles. Unilateral pontine lesions can cause
WD in bilateral middle cerebellar peduncles, because of
Figure 6.15.3 Axial FLAIR at the level of the medulla shows hyperintensity of the crossed distribution of the pontocerebellar fibers. In
the left pyramid along the corticospinal tract (block arrow).
addition, the corticospinal tract can also be affected, giving
Figure 6.15.4 Axial T2 of the cervical spine demonstrates hyperintensity in rise to the characteristic imaging appearance presented in
the right hemicord (block arrow), consistent with Wallerian degeneration of left this case.
corticospinal tract, which has crossed the medullary decussation. r MRI signal changes related to WD largely depend on the
time of imaging after injury:
b Stage 1: usually no T1/T2 signal change is observed.
Diagnosis However, diffusion restriction may occur, allowing
Wallerian degeneration (WD) of pontocerebellar and early detection.
corticospinal tracts. b Stage 2: the involved white matter tracts show T1
hyperintensity and T2 hypointensity due to increased
lipid content from myelin breakdown.
Differential diagnosis b Stage 3 is characterized by T1 hypointensity and T2
r Neurodegenerative disease: WD in spinal cord may mimic hyperintensity due to gliosis.
amyotrophic lateral sclerosis. WD in middle cerebellar b Stage 4 is characterized by chronic atrophy of the
peduncles may mimic multisystem atrophy. affected white matter tract. There is usually no
r Demyelinating disease. associated enhancement.
r Toxic and metabolic encephalopathy. r Treatment: none.
Further reading
De Simone T, Regna-Gladin C, Carriero
MR, Farina L, Savoiardo M. Wallerian
degeneration of the pontocerebellar
fibers. AJNR Am J Neuroradiol 2005;
26(5): 1062–1065.
211
Section 1: Brain
Case 6.16
212
History Key points

53-year-old female presenting with worsening weakness, r ALS is the most common motor neuron degenerative
dysphagia, and dysarthria. disease. Most cases are sporadic, affecting patients 50 years
or older and with a male predilection.
r It affects both upper motor neurons (hyperreflexia) and
Findings lower motor neurons (fasciculation and atrophy). Bulbar
involvement leads to dysarthria, dysphagia, tongue
Figures 6.16.1–6.16.4 Axial (Figs. 6.16.1–6.16.3) and sagittal (Fig. 6.16.4)
FLAIR images show symmetric hyperintensity in the subcortical white matter of fasciculation, etc. Respiratory failure is typically the cause
precentral gyri (Fig. 6.16.1, arrow), posterior limbs of the internal capsules of death.
(Fig. 6.16.2), and cerebral peduncles (Fig. 6.16.3), tracking along the r Imaging:
corticospinal tracts (Fig. 6.16.4).
b The primary role of imaging is to exclude alternative
diagnoses such as cervical cord compression or
Diagnosis multiple sclerosis.
Amyotrophic lateral sclerosis (ALS). b Characteristic T2/FLAIR hyperintense signal along the
bilateral corticospinal tracts, extending from the
subcortical white matter of the precentral gyri, corona
Differential diagnosis radiata, posterior limb of the internal capsules, cerebral
r Primary lateral sclerosis. peduncles, and to the anterolateral column of the spinal
r Demyelinating and inflammatory disease (multiple cord, reflecting demyelination and gliosis.
b In some patients, hypointense T2/FLAIR signal can be
sclerosis, ADEM, Behçet’s disease, etc.).
r Wallerian degeneration. seen in motor cortex, likely due to iron deposition.
r Infiltrative neoplasms. r Treatment: neuroprotective agents (e.g., glutamine-release
r Other neurodegenerative disease (vitamin B12 deficiency inhibitors) and antispasmodics (baclofen, diazepam). ALS
and Friedreich ataxia, etc.). is a progressive disease, usually leading to death within 10
r Artifacts. years of symptom onset.
Further reading
Agosta F, Chio A, Cosottini M, De Stefano
N, Falini A, Mascalchi M, et al. The
present and the future of neuroimaging in
amyotrophic lateral sclerosis. AJNR Am J
Neuroradiol 2010; 31(10): 1769–1777.
213
Section 2 Head and neck
Chapter
Facial trauma
7 David Chiao, Yang Tang, Max Wintermark, and Sugoto Mukherjee
Case 7.1
Figure 7.1.1
Figure 7.1.2
History Key points

38-year-old male presents with diplopia after being punched
r Typically due to blunt trauma to the orbit from motor
in the eye during a bar fight.
vehicle collision, assault, sporting accident, or fall.
r Characterized by fractures of the medial wall and floor
Findings (which are thinner than the lateral wall and roof).
Figure 7.1.1 Axial NECT demonstrates a left medial orbital wall fracture. Note b Pure form: medial wall and floor fractures only.
the medial deviation of the left medial rectus muscle, which appears thickened b Impure form: medial wall and floor fractures + orbital
(white curved arrow).
rim.
Figure 7.1.2 Coronal NECT demonstrates a left medial orbital wall fracture b “Blow-in” fracture: depression of the orbital roof into
(white curved arrow). There is also a left orbital floor fracture which involves the
infraorbital canal (white arrow); hemorrhage is noted in the left maxillary sinus.
the orbit; associated with globe injury.
b “Blow-up” fracture: elevation of the orbital roof into
the cranial vault; associated with intracranial
Diagnosis injury.
Orbital blow-out fractures. r Imaging: diagnosis with thin-slice multiplanar NECT.
r Types:
Differential diagnosis b Open door: large, displaced fracture fragment.
r Pseudofractures (nutrient canal, cranial suture, infraorbital b Trapdoor: small, minimally displaced fracture
canal). fragment.
214
Chapter 7: Facial trauma
The small fracture fragment snaps back into place, r Clinical presentation: patients can present with extraocular
trapping the inferior rectus muscle between bones. movement restriction, diplopia, enophthalmos,
Most pediatric orbital blow-out fractures are hypoglobus, and cheek hypesthesia.
trapdoor type. r Treatment:
r Complicating features: b Uncomplicated fractures are managed with high-dose
b Extraocular muscle injury: can result in restriction in steroids.
b Complicated fractures are managed with orbital floor
extraocular movement and diplopia. (Note: extraocular
muscle entrapment is a clinical diagnosis!) reconstruction after the initial swelling has resolved but
b Herniation of orbital contents into the maxillary or before significant scarring has formed (⬍ 2 weeks).
b Immediate surgery is indicated for:
ethmoid sinuses: can result in enophthalmos (posterior
displacement of the globe) or hypoglobus (inferior Bony fragments impinging on the optic nerve.
displacement of the globe). Presence of an oculocardiac reflex (bradycardia due
b Involvement of the infraorbital canal: can result in to traction of the extraocular muscles and/or
cheek hypesthesia (decreased sensation) due to injury compression on the globe).
to the infraorbital nerve. “White-eyed” blow-out fractures (significant
b Optic nerve injury: can result in vision loss. Often seen extraocular movement restriction with little
in orbital apex fractures overlying ecchymosis or edema) in young patients.
b Globe injury: can result in vision loss. Early enophthalmos or hypoglobus.
Further reading
Caranci F, Cicala D, Cappabianca S, Briganti Ellis E, 3rd. Orbital trauma. Oral Maxillofac
F, Brunese L, Fonio P. Orbital fractures: Surg Clin North Am 2012; 24(4): 629–648.
role of imaging. Semin Ultrasound CT Winters R, Chastant R, Graham HD, 3rd.
MR 2012; 33(5): 385–391. When is immediate surgical intervention
required for isolated orbital blowout
fractures? Laryngoscope 2014; 124(3):
585–586.
215
Section 2: Head and neck
Case 7.2
r Imaging: CT is the imaging modality of choice in

History
evaluating orbital trauma. Ultrasound scan is
19-year-old (patient 1) and 46-year-old (patient 2) with
contraindicated when open globe injury is suspected. MRI
trauma.
should be considered when a clinically suspected globe
rupture is not identified at CT.
Findings r Ruptured globe/open globe injury:
b CT findings: altered globe contour, loss of volume of
Patient 1
globe, scleral discontinuity, intraocular air, intraocular
Figure 7.2.1 Axial NECT shows rupture of the right globe, which appears
small with loss of normal contour, associated with intraocular (curved arrow), foreign bodies, increased or decreased anterior
intraconal (long arrow), and extraconal hemorrhage (short arrow). chamber depth.
b Nontraumatic causes of altered globe contour
(coloboma and staphyloma) and post-traumatic orbital
Patient 2 hematoma deforming the globe can mimic open globe
Figure 7.2.2 Axial NECT shows dislocated left lens (arrow). injury.
r Lens injuries (partial or complete dislocation):
b Posterior dislocations are more common, anterior
Diagnosis dislocation being prevented by iris.
Traumatic globe rupture, traumatic dislocation of the lens. b Complete posterior dislocation: lens lies in dependent
position.
Differential diagnosis b Partial posterior dislocation: lens is angled posteriorly
r Mimics of globe rupture and nontraumatic dislocation of on the side of detachment.
b Nontraumatic lens dislocation: systemic connective
lens.
tissue disorders, such as Marfan syndrome,
Ehlers–Danlos syndrome, and homocystinuria, usually
Key points bilateral.
r In acute orbital trauma, clinical evaluation may be difficult r Other injuries:
because of periorbital soft-tissue swelling and poor patient b Anterior segment injuries:
cooperation due to altered mentation or sedation. Hemorrhage: hyperattenuating fluid.
216
Corneal laceration: may not be evident in imaging if to anchoring effect of short ciliary vessels and
closed by iris prolapse. Decreased volume of the nerves.
anterior chamber indicates corneal laceration. Vitreous hemorrhage: hyperattenuating fluid.
Anterior subluxation of the lens can mimic corneal b Ophthalmic veins and optic nerve complex injuries,
laceration, differentiated by the position of the lens. especially at the orbital apex.
b Posterior segment injuries: b Bony orbit fractures, with or without herniation of
Retinal detachment: V-shaped appearance due to orbital contents.
firm retinal attachment at optic disc. b Foreign bodies.
Choroidal detachment: lentiform shape with r Treatment: depends on the type of injury.
sparing of the posterior portion of the globe due
Further reading
Kubal WS. Imaging of orbital trauma. Sung EK, Nadgir RN, Fujita A, Siegel C,
Radiographics 2008; 28(6): 1729– Ghafouri RH, Traband A, et al. Injuries of
1739. the globe: what can the radiologist offer?
Radiographics 2014; 34(3): 764–776.
217
Case 7.3
218
History Diagnosis
Patient 1: 36-year-old male presents after being punched in Patient 1: Simple naso-orbital-ethmoidal (NOE) fractures.
the nose during a boxing match. Patient 2: Complicated naso-orbital-ethmoidal (NOE)
Patient 2: 33-year-old male status post dashboard injury fractures.
from head-on motor vehicle collision.
r Isolated nasal bone fracture.
Findings r Isolated orbital blow-out fracture.
Patient 1
Figure 7.3.1 Axial NECT demonstrates bilateral nasal bone fractures (white
arrows).
Key points
r Typically caused by direct forceful impact to the midface
Figure 7.3.2 Axial NECT demonstrates bilateral ethmoid bone fractures from motor vehicle collision, assault, sporting accident, or
(white arrows) with hemorrhage in the bilateral ethmoid sinuses. Left preseptal fall.
gas is noted (white curved arrow).
r Characterized by disruption of the medial vertical
Figure 7.3.3 Coronal NECT demonstrates cortical irregularity of the medial maxillary buttress with fractures of the nasal bones, frontal
wall of the right orbit (white arrow). Hemorrhage is noted in the frontal sinuses, processes of the maxillary bones, and ethmoid bones.
with punctate foci of pneumocephalus (white curved arrow). r Imaging:
b Diagnosis with thin-slice multiplanar CT.
Patient 2 b Often highly comminuted fractures: the NOE
Figure 7.3.4 Axial NECT demonstrates highly comminuted fractures of the “crumples” on direct impact.
nasal bones, frontal processes of the maxilla, and ethmoid bones (white b Classified by the degree of injury to the medial canthal
arrows). There is mild telecanthus due to lateral displacement of small osseous
fragments by the medial canthus ligaments.
ligament (Manson classification):
Type I: medial canthal ligament attached to a large
Figure 7.3.5 Axial NECT at the level of the maxillary sinuses demonstrates fracture fragment.
involvement of the left nasolacrimal duct (white arrow). Fracture fragments and Type II: medial canthal ligament attached to a small
hemorrhage are noted in the right maxillary sinus (white curved arrow).
fracture fragment.
Figure 7.3.6 Coronal NECT demonstrates left greater than right medial Type III: medial canthal ligament avulsed from its
orbital wall and floor fractures (arrows). The nasofrontal ducts are involved. insertion (clinical diagnosis).
219
r Complicating features: r Treatment:

b Nasofrontal duct involvement: can result in disruption b Surgery is performed after the initial swelling has
of the frontal sinus drainage pathway. Surgical resolved but before significant scarring has formed
obliteration of the frontal sinuses is required to prevent (⬍ 2 weeks).
secondary mucocele formation. b Open reduction is superior to closed reduction (better
b Nasolacrimal duct involvement: can result in epiphora aesthetic outcomes).
(overflow of tears onto the face). b Type I fractures: plate fixation.
b Cribiform plate involvement: can result in CSF leak. b Type II and III fractures: difficult to repair, often
b Globe injury: can result in vision loss. require microplate fixation with wiring and nasal
r Clinical presentation: patients can present with nasal pain, dorsum grafting.
retracted nasal bridge, telecanthus, enophthalmos, visual
abnormalities, and CSF rhinorrhea.
Further reading
Hopper RA, Salemy S, Sze RW. Diagnosis of Mehta N, Butala P, Bernstein MP. The
midface fractures with CT: what the imaging of maxillofacial trauma and its
surgeon needs to know. Radiographics pertinence to surgical intervention.
2006; 26(3): 783–793. Radiol Clin North Am 2012; 50(1): 43–57.
220
Case 7.4
Figure 7.4.1
Figure 7.4.2
221
r Pseudofractures (nutrient canal, zygoticofrontal suture,
zygomaticomaxillary suture, zygomaticotemporal suture,
zygomaticosphenoid suture, zygomaticofacial canal).
Key points
r ZMC fractures are typically caused by a blow to the cheek
which separates the zygoma from its sutural attachments.
b The zygoma is a strong bone; however, the surrounding
sutures are weak.
b Four sutures: zygomaticofrontal, zygomaticomaxillary,
zygomaticotemporal, zygomaticosphenoid.
b Note: ZMC fractures are known as “tripod” or
“trimalar” fractures. Technically, however, they could
be called “quadripod” or “quadrimalar” fractures.
Figure 7.4.5 r Isolated zygomatic arch fractures are uncommon (10%).
r Imaging:
b Diagnosis with thin-slice multiplanar NECT.
History b Characterized by disruption of the zygomatic arch,
37-year-old male presents with loss of the left malar lateral orbital wall, orbital floor, and anterior and
eminence after being kicked in the face during a kickboxing lateral walls of maxillary sinus.
tournament. The masseter muscle pulls the zygoma
posterolaterally.
Findings r Complicating features:
b Displaced ZMC fractures can increase orbital volume,
Figure 7.4.1 Axial NECT demonstrates comminuted fractures of the left
zygomatic arch (white arrows). leading to enophthalmos.
b Fracture along the zygomaticomaxillary suture often
Figure 7.4.2 Axial NECT demonstrates fractures of the anterior and lateral involves the infraorbital canal, resulting in cheek
walls of the left maxillary sinus (white arrows).
hypesthesia.
Figure 7.4.3 Axial NECT demonstrates cortical buckling of the lateral wall of r Clinical presentation: patients can present with loss of
the orbit (white arrow).
cheek projection, increased facial width, cheek
Figure 7.4.4 Coronal NECT demonstrates a fracture line along the left hypsesthesia, and enophthalmos.
zygomaticofrontal suture (white arrow). r Treatment:
Figure 7.4.5 Coronal NECT demonstrates a fracture line along the left
b Nondisplaced/minimally displaced fractures are
zygomaticomaxillary suture (white arrow) with associated fracture through the managed conservatively.
infraorbital canal. A displaced fracture is seen inferior to the suture with b Displaced or comminuted fractures are managed
opacification of the maxillary sinus with hemorrhage (white curved arrow).
surgically.
b The zygomatic arch establishes facial width and profile.
Diagnosis It is repaired if severely angulated or displaced.
b Cosmetic outcomes are excellent.
Zygomaticomaxillary complex (ZMC) fractures.
Further reading
Hopper RA, Salemy S, Sze RW. Diagnosis of Mehta N, Butala P, Bernstein MP. The Patel R, Reid RR, Poon CS. Multidetector
midface fractures with CT: what the imaging of maxillofacial trauma and its computed tomography of maxillofacial
surgeon needs to know. Radiographics pertinence to surgical intervention. fractures: the key to high-impact
2006; 26(3): 783–793. Radiol Clin North Am 2012; 50(1): 43–57. radiological reporting. Semin Ultrasound
CT MR 2012; 33(5): 410–417.
222
Case 7.5
223
History Key points

42-year-old male presents after motor vehicle collision. r Typically due to motor vehicle collision, assault, or fall.
r Le Fort fractures are complex, unstable transfacial
fractures.
Findings
b The Le Fort model gives a framework for describing
Figure 7.5.1 Coronal NECT demonstrates displaced fractures of the bilateral
pterygoid plates (white arrows), the sine qua non of Le Fort fractures. complex facial fractures. However, few complex facial
fractures are pure Le Fort fractures.
Figure 7.5.2 Coronal NECT demonstrates fractures of the lateral walls of the b Fractures occur along weaknesses in the facial skeleton.
piriform aperture bilaterally (white arrows), unique to the Le Fort I fracture b The sine qua non of Le Fort fractures is disruption of
pattern.
the pterygoid plates.
Figure 7.5.3 Coronal NECT demonstrates fractures along the nasofrontal r Buttress concept: facial buttresses are strong facial bones
sutures bilaterally (white arrows). This can be seen in Le Fort II or III fractures.
that support the framework of the face.
Figure 7.5.4 Coronal NECT demonstrates multiple facial fractures, including b Horizontal buttresses:
bilateral orbital floor fractures (white arrows), which are unique to the Le Fort II
fracture pattern.
Upper and lower maxillary buttresses.
Upper and lower mandibular buttresses.
Figure 7.5.5 Axial NECT demonstrates multiple facial fractures, including a b Vertical buttresses:
left zygomatic arch fracture (white arrow), which is unique to the Le Fort III Medial, lateral, and posterior maxillary
fracture pattern.
buttresses.
Figure 7.5.6 Coronal NECT demonstrates complex facial fractures. This Mandibular buttress.
patient has features of Le Fort I, II, and III fractures. Note the foci of gas along r Imaging: diagnosis with thin-slice multiplanar NECT.
the crista galli (white arrows), signifying an associated skull base fracture
through the anterior fossa. This places the patient at risk of a CSF leak. r Types:
b Type I: “floating palate.” Separates palate from
remainder of skull. Disruption of the medial
Diagnosis (inferiorly) and lateral (inferiorly) maxillary vertical
Le Fort fractures. buttresses.
Unique fracture: lateral margin of the piriform
aperture.
Differential diagnosis b Type II: pyramidal fracture (most common Le Fort
r Simple facial fractures, ZMC fracture, NOE fracture. fracture). Separates midface from remainder of skull.
224
Disruption of the medial (superiorly) and lateral r Clinical presentation: patients can present with midface
(inferiorly) maxillary vertical buttresses. depression, mobile facial skeleton, enophthalmos, diplopia,
Unique fracture: inferior orbital rim. malocclusion.
b Type III: craniofacial disassociation (least common). r Treatment: surgical reduction.
Separates entire face from remainder of skull. b Normal occlusion must be achieved prior to midface
Disruption of the medial (superiorly) and lateral fixation.
(superiorly) maxillary vertical buttresses + disruption b Fixation is generally performed in a “top-to-bottom”
of the zygomatic arch. manner, starting at the frontal bar and working
Unique fracture: zygomatic arch. down.
r Complicating features:
b Injury to associated structures (including the globe,
frontal sinus, infraorbital canal, carotid canal, etc.).
b Dentoalveolar involvement.
Further reading
Hopper RA, Salemy S, Sze RW. Diagnosis of Mehta N, Butala P, Bernstein MP. The Patel R, Reid RR, Poon CS. Multidetector
midface fractures with CT: what the imaging of maxillofacial trauma and its computed tomography of maxillofacial
surgeon needs to know. Radiographics pertinence to surgical intervention. fractures: the key to high-impact
2006; 26(3): 783–793. Radiol Clin North Am 2012; 50(1): 43–57. radiological reporting. Semin Ultrasound
CT MR 2012; 33(5): 410–417.
225
Case 7.6
226
Diagnosis
Mandibular fractures (coronoid process fracture,
subcondylar fracture, parasymphyseal fracture).
r Pseudofractures (nutrient canal, mandibular canal).
Key points
r Typically due to motor vehicle collision, assault, sporting
accident, or fall.
b Second most common facial fracture.
b Often associated with TMJ dislocation.
r Mandible is a “ring structure”: two fractures are common
(50%).
b “Flail mandible”: symphyseal fracture with bilateral
subcondylar fractures. Requires surgery for fixation.
r Imaging:
Figure 7.6.5 Fractures through the tooth socket are considered
open fractures. Require antibiotic prophylaxis.
Common: condylar, angle, body, parasymphyseal.
History
– Subcondylar fracture: condylar head is typically

23-year-old male presents with malocclusion after being hit pulled medially by the lateral pterygoid muscle.
in the jaw with a baseball bat. – Parasymphyseal fracture: typically follows the
long axis of the teeth.
Findings Uncommon: ramus, coronoid process, symphyseal.
b Panorex radiographs are useful to evaluate for
Figure 7.6.1 Axial NECT demonstrates a minimally displaced left coronoid
process fracture (white curved arrow). High-attenuation fluid is noted in the associated dental root fractures.
right maxillary sinus, likely representing acute hemorrhage (white arrow). b MRI can be used to assess TMJ disc morphology and
position in selected cases.
Figure 7.6.2 Axial NECT demonstrates a left subcondylar fracture (white
curved arrow). Hemorrhage is noted in the bilateral maxillary sinuses (white r Complicating features:
arrows).
b Involvement of the mandibular canal: can result in chin
Figure 7.6.3 Coronal NECT again demonstrates the left subcondylar fracture hypesthesia (decreased sensation) due to injury to the
(white curved arrow). mental nerve.
Figure 7.6.4 Axial NECT demonstrates a displaced left parasymphyseal r Clinical presentation: patients can present with jaw pain,
fracture (white curved arrow). ecchymosis, bony deformity, malocclusion, trismus,
Figure 7.6.5 3D reformat demonstrates multiple facial fractures, including
sublingual hematoma, and chin hypesthesia.
left coronoid process fracture (white block arrow), left subcondylar fracture
r Treatment: internal or external fixation to restore normal
(white curved arrow), and left parasymphyseal fracture (white arrow). occlusion and encourage bony union.
Further reading
Mehta N, Butala P, Bernstein MP. The Schuknecht B, Graetz K. Radiologic
imaging of maxillofacial trauma and its assessment of maxillofacial, mandibular,
pertinence to surgical intervention. and skull base trauma. Eur Radiol 2005;
Radiol Clin North Am 2012; 50(1): 43–57. 15(3): 560–568.
227
Case 7.7

r Pseudofractures (nutrient canal, cranial suture, internal
Patient 1: 48-year-old female with persistent mechanical
hearing loss 8 weeks after motor vehicle collision. auditory canal, cochlear aqueduct, vestibular aqueduct,
canaliculi).
Patient 2: 56-year-old male with sensorineuronal hearing r Mastoid air cells effusion, mastoiditis.
loss after motor vehicle collision.
Key points
r Typically due to motor vehicle accident, assault, sporting
Findings accident, or fall.
Figure 7.7.1 Axial NECT demonstrates incudomalleolar dislocation, with the b Diagnosis with thin-slice multiplanar NECT; vascular
“ice cream” (malleus head, white arrow) falling off the “cone” (incus body, white
curved arrow). injury is evaluated with CTA/CTV.
b Secondary signs include mastoid air cell effusion,
middle ear effusion, pneumolabyrinth, hemorrhage, or
pneumocephalus adjacent to the mastoid air cells, and
Patient 2 parapharyngeal space air.
Figure 7.7.2 Axial NECT demonstrates a transverse temporal bone fracture r Types:
which violates the otic capsule (white arrow). Note the associated
pneumolabyrinth. b Longitudinal fracture: along the long axis of the
petrous bone.
Typically due to lateral impact.
Frequently involves the external auditory canal,
Diagnosis tympanic membrane, and middle ear.
Patient 1: longitudinal temporal bone fracture with Patients present with persistent (⬎ 8 weeks)
ossicular dislocation. mechanical hearing loss. (Transient hearing loss can
Patient 2: transverse temporal bone fracture with violation be due to debris in the external or middle ear.)
of the otic capsule. Associated with ossicular dislocation.
228
– Incus is most commonly involved: b Tegmen tympani fracture:

incudostapedial > incudomalleolar > complete Can result in CSF leak and cephalocele.
incus dislocation > others. b CSF drains into the middle ear (via a tegmen defect)
– Incudostapedial dislocation: difficult to visualize and into the nasopharynx (via the eustachian tube) or
on NECT; incus lenticular process anterior or into the external auditory canal (via a ruptured
posterior to stapes head. tympanic membrane).
– Incudomalleolar dislocation: “ice cream” b Facial nerve canal involvement:
(malleus head) sliding off “cone” (incus body) in Typically occurs at the genu.
the epitympanum. Can result in facial nerve palsy.
b Transverse fracture: along the short axis of the petrous b Carotid canal involvement: can result in carotid artery
bone. injury (rare).
Typically due to frontal or occipital impact. b Jugular bulb or dural venous sinus involvement: can
Frequently involves the inner ear and otic result in venous thrombosis (rare).
capsule. r Clinical presentation: patients can present with
Extends from the foramen magnum or jugular periauricular ecchymosis (Battle’s sign), hearing loss, CSF
foramen to the middle cranial fossa. otorrhea, and facial nerve palsy.
Associated with sensorineuronal hearing loss, r Treatment:
perilymphatic fistula, CSF leak, and facial nerve b Ossicle dislocation:
injury.
b Oblique fracture: mixed features. Ossicular reconstruction ± placement of graft
b Note: classification according to otic capsule injury material.
b CSF leaks:
(otic capsule violating vs. otic capsule sparing) is more
Most CSF leaks resolve spontaneously within 7 days;
clinically relevant as it better predicts clinical
a few patients require surgical correction.
outcomes.
Antibiotics are administered to prevent retrograde
r Complicating features:
infection.
b Intracranial abnormalities: seen in up to 90% of b Facial nerve palsy:
patients. Immediate facial nerve palsy is usually permanent;
b Perilymphatic fistula: delayed facial nerve palsy is usually transient.
Due to disruption of the round or oval windows. Usually managed conservatively with steroids;
Can result in vertigo and sensorineuronal hearing surgical decompression can be attempted in selected
loss. cases.
Further reading
Collins JM, Krishnamoorthy AK, Kubal WS, Little SC, Kesser BW. Radiographic Yetiser S, Hidir Y, Birkent H, Satar B,
Johnson MH, Poon CS. Multidetector CT classification of temporal bone fractures: Durmaz A. Traumatic ossicular
of temporal bone fractures. Semin clinical predictability using a new system. dislocations: etiology and management.
Ultrasound CT MR 2012; 33(5): 418–431. Arch Otolaryngol Head Neck Surg 2006; Am J Otolaryngol 2008; 29(1): 31–36.
132(12): 1300–1304.
229
Case 7.8
the styloid process. Eagle syndrome is characterized by

History compression of these structures.
52-year-old female with chronic throat pain and trouble r Note: an elongated styloid process (or stylohyoid ligament
swallowing. ossification) is seen in 5% of patients and is usually
asymptomatic; only a small fraction of patients develop
Findings Eagle syndrome.
Figure 7.8.1 Oblique sagittal NECT demonstrates an elongated styloid r Most common in women in the fifth to sixth decade of life.
process (white arrow), measuring ⬎ 30 mm. Most patients with elongated r Imaging:
styloid processes are asymptomatic.
Figure 7.8.2 Axial NECT demonstrates compression of the right internal b Styloid process (or stylohyoid ligament ossification) >
jugular vein (white curved arrow) by the right styloid process (white arrow). 30 mm and in close association with a neurovascular
structure.
Diagnosis b Dynamic imaging with head rotation can be
Eagle syndrome. performed.
r Clinical presentation:
Differential diagnosis b Classic type: throat pain, globus sensation,
r Asymptomatic elongated styloid process. odynophagia, dysphagia. Classically seen status post
tonsillectomy with scarring near the styloid apex which
Key points compresses the glossopharyngeal and vagus nerve.
b Carotid type: pain over the parietal skull or in the
r Rare syndrome characterized by neuropathic or
superior periorbital region.
vasoocclusive symptoms caused by elongation of the
styloid process or stylohyoid ligament ossification. r Treatment: typically treated with conservative
r The internal carotid artery, internal jugular vein, management. Surgical resection can be pursued in selected
glossopharyngeal nerve, and vagus nerve travel medial to cases.
230
Further reading
Fusco DJ, Asteraki S, Spetzler RF. Eagle’s Kim E, Hansen K, Frizzi J. Eagle syndrome:
syndrome: embryology, anatomy, and case report and review of the literature.
clinical management. Acta Neurochir Ear Nose Throat J 2008; 87(11): 631–633.
(Wien) 2012; 154(7): 1119–1126.
231
Case 7.9
Figure 7.9.1
Figure 7.9.2
History b Diagnosis with thin-slice multiplanar NECT and

38-year-old female status post strangulation during
domestic altercation. endoscopy; vascular injuries are evaluated with
CTA.
b Characterized by laryngeal cartilage irregularity ±
Findings soft-tissue edema or hematoma.
Figure 7.9.1 Axial NECT demonstrates angular deformity of the left thyroid b Findings can be subtle!
cartilage (white arrow), concerning for thyroid cartilage fracture. Note the b Horizontal fractures are commonly missed; coronal
extensive subcutaneous emphysema.
reformats are helpful.
Figure 7.9.2 Axial NECT demonstrates periglottic edema with moderate
Incomplete cartilage ossification can mimic
narrowing of the airway (white arrow). cartilage fracture.
Arytenoid dislocation can mimic vocal cord
paralysis.
Diagnosis r Schaefer–Fuhrman classification:
Laryngeal injury.
b Group I: mild edema/hematoma. Observation.
b Group II: moderate edema/hematoma. Risk of airway
Differential diagnosis compromise.
Edema, cellulitis, incomplete cartilage ossification. b Group III: severe edema/hematoma, exposed cartilage.
Surgical exploration.
b Group IV: severe edema/hematoma, exposed cartilage,
Key points
r Typically caused by blunt trauma to the neck from motor > 2 fracture lines. Surgical exploration.
vehicle collision or strangulation, penetrating injury from r Clinical presentation: patients can present with neck pain,
knife or gunshot, or iatrogenic injury from intubation. hoarseness, crepitus, cough, hemoptysis, stridor,
b Uncommon injury. respiratory distress, and loss of the laryngeal protuberance
b Less common in children (high-riding larynx protected (“Adam’s apple”).
r Treatment:
by mandible).
b Conservative management: single nondisplaced
r Thyroid > cricoid > arytenoids.
thyroid cartilage fracture.
b Associated hyoid bone fracture in 1/4. b Early surgical repair (⬍ 24 hours): displaced,
r Imaging: comminuted thyroid cartilage fractures.
232
b Early surgery ensures optimal outcomes in voice and b Tracheostomy may be required for group II+ injuries.
airway. b Airway stenting may be required for group IV injuries.
Further reading
Becker M, Duboe PO, Platon A, Kohler R, Bell RB, Verschueren DS, Dierks EJ.
Tasu JP, Becker CD, et al. MDCT in the Management of laryngeal trauma. Oral
assessment of laryngeal trauma: value of Maxillofac Surg Clin North Am 2008;
2D multiplanar and 3D reconstructions. 20(3): 415–430.
AJR Am J Roentgenol 2013; 201(4):
W639–W647.
233
Chapter
Head and neck infections
8 Jason DeBerry, Max Wintermark, Sugoto Mukherjee, and Yang Tang
Case 8.1
History
Three different patients, all presenting with fever, sore
throat, and dysphagia.
Findings
Figure 8.1.1 CECT shows enlargement of bilateral palatine tonsils (∗ ) with
linear striated enhancement pattern consistent with acute tonsillitis.
Figure 8.1.2 CECT demonstrates enlargement of bilateral palatine tonsils,

with a left peritonsillar abscess (block arrow) surrounded by inflamed tonsillar
tissue without extension to the parapharyngeal, masticator, or submandibular
spaces.
Figure 8.1.3 CECT shows an abscess within the right palatine tonsil (curved
arrow). The abscess extends laterally to the right parapharyngeal space (arrow),
consistent with a peritonsillar abscess.
Diagnosis
Figure 8.1.3 Acute tonsillitis, tonsillar and peritonsillar abscess.
234
Chapter 8: Head and neck infections
r CT may show enlargement of tonsils, with “kissing tonsils”

r Tonsillar mass. appearance with or without airway obstruction. Linear
striated pattern of enhancement (“tiger stripe” sign) is
relatively specific for tonsillitis.
Key points r Infected tonsils may suppurate. If the abscess is contained
r Tonsillitis is the most common oropharyngeal infection in within the tonsil, it is called tonsillar abscess. If the
children and young adults. infection extends through the tonsillar capsule and
r Common pathogens include ␤-hemolytic Streptococcus, involves the space between the capsule and superior
Staphylococcus aureus, pneumococcus, and Haemophilus constrictor muscles, it is called peritonsillar abscess (PTA),
influenzae. also referred to as quincy. PTA may spread to the adjacent
r Symptoms include sore throat, fever, dysphagia, otalgia, parapharyngeal, masticator, and submandibular spaces.
and trismus. r Treatment: antibiotics, drainage of abscess.
r Usually a clinical diagnosis. If diagnosis is uncertain and
abscess is suspected, CECT is the imaging modality of
choice.
Further reading
Hardingham M. Peritonsillar infections.
Otolaryngol Clin North Am 1987; 20(2):
273–278.
235
Case 8.2
Figure 8.2.4
Findings
Figure 8.2.3 Figures 8.2.1–8.2.4 CECT images of neck (Figs. 8.2.1, 8.2.2, 8.2.4 axial,
Fig. 8.2.3 coronal reformat) demonstrate left palatine tonsil edema with a small
tonsillar abscess (Fig. 8.2.1, curved arrow), thrombosis of the left internal jugular
vein (Figs. 8.2.2, 8.2.3, arrows), and its tributary veins (Figs. 8.2.1, 8.2.2, block
History arrow). Note enhancing vessel wall suggestive of thrombophlebitis. Nodule
and airspace disease are noted in the right upper lobe, with bilateral pleural
24-year-old man with sore throat, chest pain, and fever. effusion (Fig. 8.2.4).
236
r Typically affects healthy young adults between the ages of

Diagnosis
16 and 25 years.
Lemierre’s syndrome. r Often referred to as “forgotten disease” or postanginal
septicemia.
Differential diagnosis r Common in the preantibiotic era, now with rising
r Nonseptic thrombosis of internal jugular vein. incidence due to antibiotic resistance.
r Primary pulmonary infections. r Most commonly caused by Fusobacterium necrophorum, a
r Pulmonary metastasis. normal oropharyngeal flora.
r Symptoms: sore throat, fever, trismus, chest pain, dyspnea,
Key points etc.
r Characterized by recent oropharyngeal infection, with r Treatment: antibiotics, anticoagulation, pharyngeal abscess
septic thrombophlebitis of internal jugular vein and its drainage.
tributaries and septic pulmonary emboli.
Further reading
Screaton NJ, Ravenel JG, Lehner PJ,
Heitzman ER, Flower CD. Lemierre
syndrome: forgotten but not extinct –
report of four cases. Radiology 1999;
213(2): 369–374.
237
Case 8.3
238
Patient 3
Figure 8.3.5 CECT shows an empty tooth socket of the third left maxillary
molar (tooth number 16) from recent extraction. There are ill-defined
phlegmonous changes involving the left masticator space including the
pterygoid (black arrow) and masseter (white arrow) muscles. The inflammation
extends to the left parapharyngeal space (∗ ) and posterior soft palate (block
arrow). The left internal carotid artery is slightly displaced posteriorly (curved
arrow).
Diagnosis
Odontogenic abscess.
r None.
Key points
r Dental infection is the most common cause of oral cavity
infections. Odontogenic abscesses may arise from dental
infections or after dental procedures. The most common
symptoms include tooth pain, facial pain and swelling,
Figure 8.3.5 (patient 3) dysphagia or dysphonia.
r Infections of maxillary teeth tend to spread contiguously to
the buccal and masticator spaces.
History r For the mandibular teeth, infection of the second or
Three patients present with facial swelling and pain. third mandibular molar teeth tends to involve the
submandibular space, because the roots of these teeth
Findings extend below the mylohyoid muscle. The infection of
more anterior mandibular teeth is usually confined to the
Patient 1 sublingual space because the roots of these teeth are above
Figure 8.3.1 Panorex shows periapical lucency of the second right the mylohyoid muscle.
mandibular molar (tooth number 31) (black arrow). Note scattered gas locules r Occasionally, dental infection can spread to the orbits or
in the right submandibular soft tissue (white arrows).
paranasal sinuses, or even intracranially.
Figures 8.3.2, 8.3.3 CECT in soft tissue algorithm (Fig. 8.3.2) shows a r CECT is the preferred modality to evaluate suspected
peripherally enhancing gas-containing right submandibular abscess (block
arrow). Slightly more superior image in bone window (Fig. 8.3.3) demonstrates
odontogenic abscess.
the dehiscence of the lingual cortex associated with the infected molar (curved r An odontogenic abscess typically demonstrates
arrow). peripherally enhancing fluid collections with associated
cellulitis and myositis. Images in bone windows may
Patient 2 reveal the periapical lucency, cortical dehiscence, and
osteomyelitis.
Figure 8.3.4 CECT shows a gas-containing abscess in the left sublingual
space (block arrow) medial to the mylohyoid muscule. Patient was intubated
r Treatment: tooth extraction, abscess drainage, and
for airway protection. intravenous antibiotic therapy.
Further reading
Rana RS, Moonis G. Head and neck Yonetsu K, Izumi M, Nakamura T. Deep
infection and inflammation. Radiol Clin facial infections of odontogenic origin:
North Am 2011; 49(1): 165–182. CT assessment of pathways of space
involvement. AJNR Am J Neuroradiol
1998; 19(1): 123–128.
239
Case 8.4
Findings
Figures 8.4.1, 8.4.2 Axial CECT of the neck demonstrates a collection of
fluid and air locules within the left tonsillar fossa, consistent with a peritonsillar
abscess (Fig. 8.4.1). Phlegmonous changes are noted tracking to the sublingual
space and floor of the mouth (block arrows), extending posteriorly to the
pre-epiglottic space (∗ ).
Figure 8.4.3 Sagittal reformat shows thickening of the epiglottis (∗ ) and

aryepiglottic folds (curved arrow).
Diagnosis
Ludwig’s angina.
r Oral cavity dermoid.
r Simple ranula.
r Venolymphatic malformation.
Key points
r Ludwig’s angina is an aggressive, rapidly spreading
Figure 8.4.3 infection of the floor of the mouth that may involve the
sublingual, submandibular, and submental spaces. It can
spread posteriorly to the epiglottis, leading to airway
compromise, and requires careful monitoring and rapid
History airway intervention.
52-year-old woman with 5-day history of progressive sore r Most cases are caused by infection of the second or third
throat, presents with submental swelling and inability to mandibular molar teeth. Other sources of infection include
open mouth. peritonsillar abscess or suppurative parotitis. Infection
240
usually takes the form of cellulitis without abscess r CECT is the imaging modality of choice to define the
formation. extent of infection and identify drainable abscess.
r Patients typically present with fever, chills, mouth pain, r Treatment: intravenous antibiotics, airway management,
stiff neck, drooling, dysphagia, muffled voice, and trismus. surgical drainage if abscess is identified.
r Submental/submandibular “woody” induration is a typical
finding on physical exam, sometimes with palpable
crepitus.
Further reading
Bou-Assaly W, McKellop J, Mukherji S.
Computed tomography imaging of acute
neck inflammatory processes. World J
Radiol 2010; 2(3): 91–96.
241
Case 8.5
242
Figure 8.5.4 AP neck radiograph confirms the thickening of the aryepiglottic

fold on the left (white arrow).
Figure 8.5.5 Axial CT image of neck with contrast shows severe edema and
thickening of left aryepiglottic fold (white arrows), which obliterates the left
piriform sinus. The supraglottic airway is patent. This patient did not require
intubation and improved with intravenous antibiotics and steroids.
Diagnosis
Adult supraglottitis.
r Other upper respiratory infections such as pharyngitis,
tonsillitis, peritonsillar abscess, Ludwig’s angina, and
laryngitis.
r Noninfectious conditions include angioedema, chemical
ingestion injury, tumor, or trauma.
Key points
r The term supraglottitis or epiglottitis describes severe
inflammation of the epiglottis and adjacent supraglottic
structures, which can be a potentially fatal condition due to
sudden obstruction of upper airway. While the incidence
of childhood epiglottitis has significantly declined due to
History Haemophilus influenzae type B vaccination, the incidence
Patient 1: 66-year-old man presents with sore throat, in adults has increased and is about 2.5 times that in
difficulty swallowing, and change in voice. children.
r The symptoms of adult supraglottitis are nonspecific and
Patient 2: 50-year-old woman presents with worsening
hoarseness, odynophagia, and sore throat. include sore throat, odynophagia, muffled voice, drooling,
dyspnea, stridor, and cough. The diagnosis is generally
made on the basis of history and direct endoscopy.
Findings r Radiographic signs of supraglottitis include thickening of
the epiglottis (thumb sign), swelling of arytenoids,
Patient 1 aryepiglottic folds, base of tongue, and prevertebral soft
Figure 8.5.1 Lateral neck radiograph shows marked thickening of the tissue, ballooning of hypopharynx, and poorly defined
epiglottis (black arrow), arytenoids (∗ ), and aryepiglottic folds (white arrow).
Note the pharyngeal ballooning (double-head arrow) reflecting vallecula.
supraglottic/glottic airway obstruction. This patient was subsequently r CT can be used to confirm the diagnosis, to evaluate its
intubated for airway protection. complications, and to differentiate from other diagnostic
entities. The most common CT findings are thickening of
Figure 8.5.2 Normal lateral neck radiograph for comparison, with normal
appearance of epiglottis (black arrow), arytenoids (∗ ), aryepiglottic folds (white the epiglottis, aryepiglottic folds, and false and true vocal
arrow), and normal caliber of pharyngeal airway (double-head arrow). cords, obliteration of the pre-epiglottic fat, thickening of
the platysma muscle, and reticulation of the subcutaneous
Patient 2 fat.
r Treatment consists of antibiotics and steroids. If there is
Figure 8.5.3 Lateral neck radiograph demonstrates mild thickening of
epiglottis (black arrow) and marked lobulated thickening of the aryepiglottic potential airway obstruction on laryngoscopic exam,
fold (white arrow). emergent intubation is necessary to protect the airway.
Further reading
Ducic Y, Hebert PC, MacLachlan L, Neufeld Nemzek WR, Katzberg RW, Van Slyke MA, Smith MM, Mukherji SK, Thompson JE,
K, Lamothe A. Description and Bickley LS. A reappraisal of the radiologic Castillo M. CT in adult supraglottitis.
evaluation of the vallecula sign: a new findings of acute inflammation of the AJNR Am J Neuroradiol 1996; 17(7):
radiologic sign in the diagnosis of adult epiglottis and supraglottic structures in 1355–1358.
epiglottitis. Ann Emerg Med 1997; 30(1): adults. AJNR Am J Neuroradiol 1995;
1–6. 16(3): 495–502.
243
Case 8.6
Key points
History r Acute retropharyngeal calcific tendinitis is also known as
Neck pain and headache with slight fever. acute calcific prevertebral tendonitis or longus colli
tendonitis.
r Retropharyngeal calcific tendinitis is due to inflammation
of the longus colli muscle, which is precipitated by
Findings deposition of calcium hydroxyapatite crystals on its tendon
from C1 to C3.
Figure 8.6.1 Plain lateral radiograph of the neck demonstrates amorphous r Affects patients in the third to sixth decade of life, without
calcifications anterior to C2 vertebral body (curved arrow) with mild
prevertebral soft-tissue swelling (∗ ). gender predilection.
r Signs and symptoms include neck pain, odynophagia,
Figure 8.6.2 Sagittal CT of the neck better demonstrates prevertebral dysphagia, and low-grade fever.
calcifications anterior to C2 vertebral body (curved arrow) and mild r Plain radiography may show amorphous calcification and
prevertebral soft-tissue swelling (∗ ).
swelling anterior to C1–C3.
r CT is the imaging modality of choice to demonstrate
prevertebral calcification at C1–C3 with adjacent swelling.
Diagnosis Enhancement should not be seen.
r MRI will show inflammation in the prevertebral muscles as
Acute retropharyngeal calcific tendinitis.
hyperintensity on T2-weighted imaging. Enhancement
mimicking retropharyngeal abscess can be seen on
post-contrast imaging. Attention should be paid to actively
Differential diagnosis look for T1 and T2 hypointense structures (corresponding
r Retropharyngeal space abscess. to the calcifications) within the effusion to make the
r Retropharyngeal space effusion. correct diagnosis.
r Perivertebral space infection. r Treatment: self-limiting, analgesics.
244
Further reading
Lee S, Joo KB, Lee KH, Uhm WS. Acute Offiah CE, Hall E. Acute calcific tendinitis of
retropharyngeal calcific tendinitis in an the longus colli muscle: spectrum of CT
unusual location: a case report in a appearances and anatomical correlation.
patient with rheumatoid arthritis and Br J Radiol 2009; 82(978): e117–e121.
atlantoaxial subluxation. Korean J Radiol
2011; 12(4): 504–509.
245
Case 8.7
Findings
Figures 8.7.1, 8.7.2 Axial (Fig. 8.7.1) and sagittal (Fig. 8.7.2) CECT of the neck
show an irregular peripheral enhancing fluid collection in the retropharyngeal
space, consistent with an abscess. Fluid is also noted tracking along the right
paravertebral muscle. Extensive phlegmonous changes are also noted in the
right neck involving the parapharyngeal space and carotid space, as well as the
sternocleidomastoid muscle, with myositis and overlying superficial cellulitis.
Note a suppurative right retropharyngeal lymph node (black arrow) and an
inflamed nonsuppurative left retropharyngeal lymph node (white arrow).
Figure 8.7.3 Axial CECT of the chest shows edema and inflammatory
changes of the mediastinum consistent with mediastinitis. Loculated
peripheral enhancing abscesses in the anterior and posterior mediastinum on
the right (arrows).
Diagnosis
Retropharyngeal abscess and descending necrotizing
mediastinitis (DNM).
r For retropharyngeal fluid:
Figure 8.7.3 b Retropharyngeal edema, which can be reactive to
adjacent neck infection, radiation therapy, or calcific
tendinitis of longus colli.
b Suppurative retropharyngeal lymph node.
History b Retropharyngeal hematoma from surgery, trauma, or
7-month-old boy with fever, stridor, and asymmetric anticoagulation.
right-sided neck swelling. b Retropharyngeal abscess.
246
Key points components: the true retropharyngeal space and the

r DNM is a rare condition caused by downward spread of danger space. The anterior true retropharyngeal space
terminates at variable level from T1 to T6 vertebrae. The
neck infections into the mediastinum.
r Previously with high mortality rate, mostly because of posteriorly located danger space extends further down to
the posterior mediastinum and terminates at the level of
delayed diagnosis.
r Infection most commonly originates from the oral cavity the diaphragm.
r CECT is the imaging modality of choice.
or oropharynx. r The findings of mediastinitis include inflammatory
r The most common route of spread is through the
stranding of mediastinal fat, mediastinal fluid
retropharyngeal/danger space, and rarely through the
collection, pleural and/or pericardial fluid collections,
anterior visceral space.
r The retropharyngeal space is posterior to the lymphadenopathy, and vascular thromobosis.
r Treatment: antibiotics and surgical drainage.
pharynx/esophagus and anterior to the prevertebral
muscles. It is divided by the alar fascia into two
Further reading
Scaglione M, Pinto A, Giovine S, Di Nuzzo
L, Giuliano V, Romano L. CT features of
descending necrotizing mediastinitis: a
pictorial essay. Emerg Radiol 2007; 14(2):
77–81.
247
Case 8.8
History Key points

31-year-old man with 1-week history of nasal congestion, r The orbital septum is a layer of connective tissue extending
left eye swelling, pain, and proptosis. from the peripheral orbital rim to the eyelid, which acts as
the anterior boundary of the orbit.
Findings r Orbital infection can be divided into preseptal and
Figure 8.8.1 Axial CECT image of the orbits demonstrates opacification of left
postseptal (orbital) cellulitis. Preseptal cellulitis is a mild
ethmoid air cells. Left periorbital soft-tissue edema is noted, as well as disease and usually secondary to trauma, insect or animal
retrobulbar fat stranding (arrow) and proptosis. bites, foreign body, or sinusitis. Postseptal (orbital)
cellulitis has a higher morbidity, and is mostly secondary
Figure 8.8.2 Axial CECT through the superior aspect of the orbits reveals a
rim-enhancing fluid collection (black arrows) in the superomedial extraconal to sinusitis.
space of the left orbit, consistent with a subperiosteal abscess. Note the lack of r Clinical presentations of these two conditions are similar
enhancement of the left superior ophthalmic vein (white block arrow) and include orbital pain, erythema, and swelling.
compared to the normal contralateral side, consistent with thrombosis.
Patients with postseptal cellulitis may present with
ophthalmoplegia, pain with ocular movement, and
Diagnosis proptosis.
Orbital cellulitis. r CECT is the imaging modality of choice.
b Preseptal cellulitis: periorbital soft-tissue thickening
Differential diagnosis and enhancement.
r Orbital inflammatory diseases including systemic b Postseptal cellulitis: retrobulbar fat infiltration,
connective disease (lupus, sarcoidosis, Wegener thickening and enhancement of extraocular muscles
granulomatosis, etc.) and idiopathic inflammatory (myositis), subperiosteal abscess, rarely
pseudotumor. thrombophlebitis of the superior ophthalmic vein and
r Orbital lymphoproliferative disorder. cavernous sinus.
248
r MRI is superior to demonstrate soft-tissue involvement but b Postseptal cellulitis: intravenous antibiotics and close
is reserved for complicated cases. monitoring. Large subperiosteal abscess requires
r Treatment: surgical drainage.
b Preseptal cellulitis: oral antibiotics as outpatient.
Further reading
Eustis HS, Mafee MF, Walton C, Mondonca
J. MR imaging and CT of orbital
infections and complications in acute
rhinosinusitis. Radiol Clin North Am
1998; 36(6): 1165–1183, xi.
249
Case 8.9
250
Diagnosis
Acute invasive fungal sinusitis with cavernous sinus
thrombosis.
r Complicated acute nonfungal sinusitis.
r Sinonasal malignancy.
r Sinonasal granulomatous disease (Wegener
granulomatosis, sarcoidosis, inflammatory pseudotumor).
Key points
r Fungal sinusitis is broadly classified into noninvasive and
invasive forms. Noninvasive disease consists of chronic
allergic fungal sinusitis and mycetoma, while invasive
disease is subdivided into acute invasive fungal sinusitis,
granulomatous invasive fungal sinusitis, and chronic
invasive fungal sinusitis. Each of these subtypes has
distinct clinical and radiological features.
r Acute invasive fungal sinusitis is the most lethal form. It
affects two groups of patients:
Figure 8.9.5
b Poorly controlled diabetics. Zygomycetes such as
Rhizopus and Mucor are responsible for most cases in
this group (zygomycosis).
History b Immunocompromised patients with severe
45-year-old woman with lymphoma on chemotherapy neutropenia, such as patients with hematological
develops acute onset of fever and right-sided malignancy, systemic chemotherapy, or bone-marrow
ophthalmoplegia. transplant. This group is mostly affected by Aspergillus.
r Clinical presentations: fever, sinusitis symptoms,
Findings characteristic painless necrotic nasoseptal ulcer (eschar)
Figure 8.9.1 Axial NECT of the paranasal sinuses reveals opacification of on physical exam, frequently rapid orbital and intracranial
bilateral sphenoid sinuses, with osseous destruction of the sphenoid septum extension leading to death.
and the lateral wall of the left sphenoid sinus (arrows). r Imaging:
Figure 8.9.2 Axial T2-weighted MRI demonstrates opacification of bilateral b CT may show findings of acute sinusitis including
sphenoid sinuses and posterior ethmoid air cells. Note the hypointense
material within the sphenoid sinuses, which extends into the left cavernous
mucosal thickening and fluid, as well as bony
sinus (arrow) through the osseous defect. erosion/destruction. Bony changes and mucosal disease
can be very subtle or insignificant in some cases, as the
Figure 8.9.3 Axial T1-weighted MRI shows heterogeneous signal within the fungi spread along the vasculature. Infiltration of
sphenoid sinuses and posterior ethmoid air cells with scattered areas of
intrinsic hyperintensity. Isointense soft tissue extends into the left cavernous adjacent structures can be seen on CT but is best
sinus (arrows), which markedly narrows the left cavernous ICA flow void demonstrated by MRI.
(curved arrow). b T1- and T2-weighted MRI sequences typically show
Figure 8.9.4 Contrast-enhanced fat-suppressed axial T1-weighted MRI heterogeneous signal of sinus secretions due to
demonstrates enhancing material within the affected paranasal sinuses and left proteinaceous contents. The fungal hyphae may cause
cavernous sinus, with narrowing of the left cavernous ICA flow void (curved hyperintense T1 and hypointense T2 signal, and
arrow).
enhance on the T1 post-contrast sequence. MRI is best
Figure 8.9.5 CT angiogram of the head confirms the narrowing of the left to reveal disease extension into the adjacent soft tissue
cavernous ICA (curved arrow). (premaxillary and retroantral fat, pterygopalatine fossa,
251
masticator spaces), orbits, cavernous sinuses, and brain r Treatment: aggressive surgical debridement and systemic
parenchyma. antifungal therapy.
b CT and MR angiogram can be used to confirm vascular
complications such as narrowing, thrombosis, or
pseudoaneurysm.
Further reading
Aribandi M, McCoy VA, Bazan C, 3rd.
Imaging features of invasive and
noninvasive fungal sinusitis: a review.
Radiographics 2007; 27(5): 1283–1296.
252
Case 8.10
253
r Metastasis.
History r Langerhan’s cell histiocytosis.
15-year-old presents with acute onset of fever, headache,
and left ophthalmoplegia.
Key points
Findings r Petrous apicitis, also known as apical petrositis, is an
infectious process involving a pneumatized petrous apex,
Figure 8.10.1 Axial CECT demonstrates fluid opacification of the left petrous
apex (black arrow) and rim-enhancing fluid within the left middle cranial fossa which lies at the anterior superior portion of the temporal
(white arrow). bone, due to extension of acute otitis media or mastoiditis.
r There are numerous important structures bounding or
Figure 8.10.2 Axial CECT in bone windows demonstrates cortical erosion of contained by the petrous apex, including inner ear
the anterolateral wall of the left opacified petrous apex (curved white arrow),
compared to the contralateral pneumatized petrous apex. structures, internal carotid artery, jugular bulb, inferior
petrosal sinus, and internal auditory canal.
Figure 8.10.3 Post-contrast T1-weighted MRI demonstrates rim-enhancing r Petrous apicitis is less common now with the widespread
fluid within the left middle cranial fossa (white arrow) and left petrous apex
(black arrow). and early use of antibiotics for acute otomastoiditis.
r The most common organisms include Streptococcus
Figure 8.10.4 T2-weighted MRI demonstrates fluid opacification of the left pneumoniae, Haemophilus influenzae, and Staphylococcus
middle cranial fossa (white arrow) and left petrous apex (black arrow).
aureus.
r Children typically present acutely ill, and adults are more
Figure 8.10.5 Post-contrast T1-weighted MRI demonstrates cavernous sinus
thrombosis (∗ ). indolent.
r Typical presentation includes fever and a few or all of
Figure 8.10.6 MRA demonstrates stenosis at the left internal carotid artery
(curved arrow).
Gradenigo’s triad symptoms: otorrhea, cranial nerve VI
palsy, and facial pain (trigeminal nerve involvement).
r Extension of infection can result in meningitis, cerebral
Diagnosis abscess formation, cranial nerve involvement (CN V and
Acute petrous apicitis. VI), and venous sinus thrombosis.
r CT will show cortical breakdown in opacified apex air cells
and peripherally enhancing fluid.
Differential diagnosis r MRI imaging demonstrates T2 hyperintensity and T1
r Osteomyelitis. hypointensity within the affected apex, with post-contrast
r Inflammatory pseudotumor. peripheral rim enhancement fluid.
254
r Gallium SPECT can be useful to evaluate treatment r Treatment: primarily antibiotics; however, if symptoms are
response. severe, mastoidectomy tracking to petrous apex air cells.
Further reading
Radhakrishnan R, Son HJ, Koch BL. Petrous Razek AA, Huang BY. Lesions of the petrous
apex lesions in the pediatric population. apex: classification and findings at CT
Pediatr Radiol 2014; 44(3): 325–339; quiz and MR imaging. Radiographics 2012;
323–324. 32(1): 151–173.
255
Case 8.11
Findings
Figure 8.11.1 CECT in bone window through the skull base demonstrates
osseous erosion of the clivus (white arrow) with adjacent mild right sphenoid
mucosal thickening.
Figure 8.11.2 Post-contrast axial T1 with fat saturation demonstrates a

peripherally enhancing extra-axial hypointense fluid collection in the right
temporal region, representing a subdural empyema (white arrow). The left
superior ophthalmic vein is dilated with internal filling defects consistent with
thrombosis (black arrow).
Figure 8.11.3 Post-contrast axial T1 with fat saturation shows enhancing

bone marrow signal in the clivus, consistent with osteomyelitis (∗ ).
Diagnosis
Skull base osteomyelitis (SBO), complicated by subdural
empyema and left superior ophthalmic vein thrombosis.
r Clival chordoma, chondrosarcoma, clival metastasis, clival
invasion by nasopharyngeal carcinoma.
Figure 8.11.3
Key points
History r SBO is a rare entity that commonly occurs in diabetics,
15-year-old female presents with fever and headache. typically middle-aged male patients, as a result of
256
extension of otitis externa or direct extension from (common CN VII, given its close proximity to the external
sphenoidal sinus disease. Immunocompromised patients auditory canal).
are also susceptible. r CT may demonstrate soft-tissue mass with bony erosion at
r Infection typically begins in the external auditory canal the central skull base.
and progresses through the fissures of Santorini and the r MRI is the imaging modality of choice, demonstrating
tympanomastoid suture to the Haversian system of the focal or diffuse clival hypointensity on T1 relative to
compact bone and then to the skull base. normal fatty marrow and post-contrast enhancement. Pre-
r SBO carries serious neurologic morbidity (31%) and and paraclival soft-tissue infiltration with obliteration of
mortality (10%). normal fat planes can also be seen, with variable extension
r Pseudomonas aeruginosa and Staphylococcus aureus are the into the cavernous sinus.
most common causative agents, less common being fungal r Gallium-67 scintigraphy is helpful to monitor progress as
or mixed bacterial infections. the findings normalize after early resolution of infection.
r Commonly presents with headaches. Other symptoms r Treatment: long-term systemic antibiotics, rarely surgical
include atypical facial pain and cranial nerve palsies debridement in recalcitrant cases.
Further reading
Chang PC, Fischbein NJ, Holliday RA. Johnson AK, Batra PS. Central skull base
Central skull base osteomyelitis in osteomyelitis: an emerging clinical entity.
patients without otitis externa: imaging Laryngoscope 2014; 124(5): 1083–1087.
24(7): 1310–1316.
257
Case 8.12
258
r Langerhans cell histiocytosis.

History r Chronic otitis media.
59-year-old female with fever, left otalgia/otorrhea, and
confusion.
Findings Key points

r Coalescent mastoiditis represents the end stage of
Figure 8.12.1 Axial NECT shows complete opacification of the left mastoid
air cells with erosion of the bony septae (arrow).
acute mastoiditis after the continued blockage of the
aditus ad antrum, pressure build-up, and eventually
Figure 8.12.2 Axial T2-weighted image shows a low signal area with demineralization of the mastoid septae/cortex.
prominent surrounding edema in the left temporal lobe (∗ ). Notice the r Extracranial complications include the formation of a
extracranial involvement (arrow).
subperiosteal abscess. An extracranial abscess originating
Figure 8.12.3 Axial diffusion-weighted image shows restricted diffusion from the mastoid tip can track inferiorly behind the
(confirmed by ADC, not shown) in the left temporal lobe confirming the sternocleidomastoid muscle, better known by the eponym
presence of an abscess.
Bezold’s abscess.
Figure 8.12.4 Axial T1 post-contrast demonstrates enhancement of the
r Intracranial complications include epidural/subdural
fluid-filled left mastoid air cells (arrow). Notice the filling defect in the adjacent abscess, meningitis, cerebritis/cerebral abscess, and dural
sigmoid and transverse sinus (curved arrow). sinus thrombosis.
r Cerebritis/cerebral abscess most commonly occurs in the
Diagnosis adjacent temporal lobe. Sinus thrombosis most commonly
occurs in the sigmoid sinus, and close scrutiny of the
Coalescent mastoiditis, complicated by abscess in the left
sigmoid plate on CT is critical.
temporal lobe, dural sinus thrombosis, and extracranial r CT shows opacification of the mastoid air cells with
subperiosteal abscess.
erosion of the mastoid septae/cortex. MRI with
MRA/MRV is essential to demonstrate the potential
Differential diagnosis intracranial complications, which can be clinically
r Temporal bone metastatic disease. occult.
Further reading
Lemmerling MM, De Foer B, Verbist BM, Minks DP, Porte M, Jenkins N. Acute
VandeVyver V. Imaging of inflammatory mastoiditis – the role of radiology. Clin
and infectious diseases in the temporal Radiol 2013; 68(4): 397–405.
bone. Neuroimaging Clin N Am 2009;
19(3): 321–337.
259
Chapter
Orbits
9 Thomas J. E. Muttikal, Yang Tang, Max Wintermark, and Sugoto Mukherjee
Case 9.1
Figure 9.1.2
Figure 9.1.1
260
Chapter 9: Orbits
History Key points

26-year-old patient presents with blurred vision, orbital r More common in women, temperate climate, and
pain. and tingling sensation. Caucasians.
r Typical age of initial presentation is 20–45 years.
r Rapidly developing visual impairment.
Findings r Dyschromatopsia (change in color perception).
Figure 9.1.1 Contrast-enhanced axial T1-weighted fat-saturated MRI shows r Retro-orbital or ocular pain, exacerbated by eye movement.
abnormal enhancement of the optic nerves bilaterally (arrows).
r Can be the initial presenting feature of multiple sclerosis.
Figure 9.1.2 Axial FLAIR shows hyperintense periventricular white matter r Imaging:
lesions. b MRI: Enlarged nerve, hyperintense in T2 fat-saturated
Figure 9.1.3 Sagittal T2-weighted MRI shows hyperintense lesions in the images, with abnormal contrast enhancement.
corpus callosum involving the calloso-septal margin. Characteristic white-matter lesions when present
suggest the underlying cause as multiple sclerosis.
Figure 9.1.4 Contrast-enhanced sagittal T1-weighted MRI shows enhancing
periventricular white matter lesion (arrow).
b Differentiation from neuromyelitis optica (NMO) is by
the distribution of spinal lesions and brain lesions.
NMO has long-segment involvement of the spinal cord,
Diagnosis unlike multiple sclerosis.
Optic neuritis due to multiple sclerosis. r In most cases, visual function begins to improve 1 week to
several weeks after onset, even without any treatment.
r Residual deficits in color vision, contrast and brightness
Differential diagnosis sensitivity, and visual acuity are common.
r Infectious, demyelinating (neuromyelitis optica) and r Treatment: intravenous methyl prednisolone and
inflammatory causes of optic neuritis, including immunomodulators may be used for treatment in the acute
post-radiation optic neuritis. phase.
Further reading
Aiken AH, Mukherjee P, Green AJ, Jacobs DA, Galetta SL. Petzold A, Plant GT. Diagnosis and
Glastonbury CM. MR imaging of optic Neuro-ophthalmology for classification of autoimmune optic
neuropathy with extended echo-train neuroradiologists. AJNR Am J neuropathy. Autoimmun Rev 2014;
acquisition fluid-attenuated inversion Neuroradiol 2007; 28(1): 3–8. 13(4–5): 539–545.
recovery. AJNR Am J Neuroradiol 2011;
32(2): 301–305.
261
Case 9.2
History
58-year-old patient presenting with headache.
Findings
Figure 9.2.1 Axial T2 image shows a small rounded low signal intensity lesion
representing abnormal flow void (arrow).
Figure 9.2.2 Coronal T2 fat-saturated image demonstrates a small aneurysm

directed superiorly from the ophthalmic segment of the left internal carotid
artery (arrow).
Figure 9.2.3 Axial image from CT angiography confirms the small aneurysm
(arrow).
Diagnosis
Ophthalmic artery aneurysm.
r Superior hypophyseal artery aneurysm.
Figure 9.2.3 r Artifact from pneumatized clinoid process.
262
Chapter 9: Orbits
b Ophthalmic artery aneurysm arises from the

Key points
r Common in females. ophthalmic segment adjacent to the origin of
r Peak incidence in sixth decade. ophthalmic artery.
b Superior hypophyseal artery aneurysm projects
r Incidental imaging finding, headache, subarachnoid
medially from more distal portion of the ophthalmic
hemorrhage, visual symptoms (large aneurysms), TIA.
r Imaging: although MRI may show incidental aneurysm as segment of internal carotid artery, related to the origin
of superior hypophyseal artery.
flow void in abnormal location, MR angiography or
preferably CT angiography is helpful for detailed r Treatment: endovascular coiling.
evaluation of the aneurysm.
Further reading
Day AL. Aneurysms of the ophthalmic Shapiro M, Becske T, Riina HA, Raz E,
segment. A clinical and anatomical Zumofen D, Jafar JJ, et al. Toward an
analysis. J Neurosurg 1990; 72(5): endovascular internal carotid artery
677–691. classification system. AJNR Am J
Neuroradiol 2014; 35(2): 230–236.
263
Case 9.3
264
Chapter 9: Orbits
History Key points

24-year-old patient presents with severe right eye pain. r Typical age of initial presentation is later childhood or
Patient has history of venous varix with recurrent proptosis early adulthood.
and diplopia. r Intermittent diplopia or proptosis during episodes of
straining, prone or stooping.
Findings r Intraorbital hemorrhage.
r Thrombosis can result in acute retro-orbital pain,
Figure 9.3.1 Non-contrast fat-saturated T1-weighted image of the orbit
shows heterogeneous hyperintense intraconal lesion in the right orbit (arrow). proptosis, and decreased visual acuity.
r Imaging:
Figure 9.3.2 Contrast-enhanced fat-saturated T1-weighted image of the
orbit shows absence of enhancement of the intraconal lesion in the right orbit b Contrast-enhanced CT or MRI in prone position, or
(arrow). contrast-enhanced CT with Valsalva maneuver, best
Figure 9.3.3 Prior contrast-enhanced CT of the same patient with Valsalva
demonstrates the lesion. The decrease in size or
maneuver shows enhancing intraconal lesion (arrow). disappearance of the lesion on supine images without
Valsalva maneuver is pathognomonic.
Figure 9.3.4 Prior non-contrast CT of the same patient, without Valsalva b Thrombosed varix appears hyperdense in CT,
maneuver. The lesion is not evident, as it is collapsed.
hyperintense in T1 fat-saturated MRI, without contrast
enhancement.
Diagnosis r Treatment: asymptomatic lesions are generally not
Thrombosed orbital venous varix. treated. Surgical excision with or without endovascular
treatment may be considered in patients with severe
Differential diagnosis symptoms. Spontaneous resolution can occur following
r Orbital venous malformation. thrombosis.
r Cavernous hemangioma.
Further reading
Smoker WR, Gentry LR, Yee NK, Reede DL,
Nerad JA. Vascular lesions of the orbit:
more than meets the eye. Radiographics
2008; 28(1): 185–204.
265
Case 9.4
266
Chapter 9: Orbits
Figure 9.4.4 Post-contrast axial T1 fat-saturated MRI shows prominent

central enhancement of the lesion (arrow).
Figure 9.4.5 Post-contrast axial T1 MRI done later shows increasing

enhancement, which now almost fills the entire lesion (arrow).
Diagnosis
Orbital cavernous hemangioma.
r Schwannoma.
r Venous varix.
Key points
r Most common benign orbital lesion in adults.
r Usually presents as a retrobulbar mass with painless
proptosis, diplopia, visual field defects.
r Commonly intraconal, but can occur in the extraconal
compartment.
r Unlike capillary hemangiomas and venolymphatic
malformations, these present in adults and are well
Figure 9.4.5 circumscribed.
r Imaging:
b CT: well circumscribed, rounded, or oval
History soft-tissue-density mass. Calcification may sometimes
42-year-old patient presents with diplopia. be present. The orbital apex is usually spared.
b MRI: isointense on T1 and hyperintense on T2 to
extraocular muscles. Thrombosis if present appears
Findings hyperintense in T1 images. May show
Figure 9.4.1 CECT shows an enhancing, well-circumscribed left intraconal low-signal-intensity internal septations and
lesion (arrow).
low-signal-intensity pseudocapsule.
b Centrifugal pattern of enhancement with delayed
Figure 9.4.2 Coronal T2 fat-saturated MRI shows a hyperintense left
intraconal lesion (arrow). filling-in of the entire lesion is pathognomonic.
r Treatment: surgical management for severe symptomatic
Figure 9.4.3 Axial T1 MRI shows the intraconal lesion (arrow), which is
isointense to the extraocular muscles. cases, otherwise conservative.
Further reading
Smoker WR, Gentry LR, Yee NK, Reede DL, Tanaka A, Mihara F, Yoshiura T, Togao O,
Nerad JA. Vascular lesions of the orbit: Kuwabara Y, Natori Y, et al.
more than meets the eye. Radiographics Differentiation of cavernous
2008; 28(1): 185–204; quiz 325. hemangioma from schwannoma of the
orbit: a dynamic MRI study. AJR Am J
Roentgenol 2004; 183(6): 1799–1804.
267
Case 9.5
History
45-year-old patient presents with worsening eye pain,
proptosis, and diplopia.
Findings
Figure 9.5.1 Coronal T1-weighted image shows a diffuse lesion isointense
with the extraocular muscles (EOMs) involving the intraconal and extraconal
space as well as EOMs of left orbit.
Figure 9.5.2 Coronal T2 fat-saturated MRI shows diffuse low-signal-intensity

lesion in the left orbit, with scattered areas of signal intensity less than EOMs.
Figure 9.5.3 Coronal T1 post-contrast fat-saturated MRI shows intense

heterogeneous enhancement of the lesion in the left orbit.
Diagnosis
Orbital pseudotumor.
r Lymphoproliferative disorders.
r Infectious and inflammatory conditions including orbital
Figure 9.5.3 sarcoidosis.
268
Chapter 9: Orbits
r Biopsy should be considered in patients with atypical

Key points
r Idiopathic orbital pseudotumor can be classified into history, poor response to steroid, or recurrent disease.
r Imaging:
several forms depending on the location, including
myositis (extraocular muscles), dacryoadenitis (lacrimal b Low signal intensity compared to EOMs in
gland), periscleritis, optic perineuritis (outer dural sheath T2-weighted fat-saturated images.
of optic nerve and adjacent fat), apical (infiltration or mass b Marked but heterogeneous contrast enhancement.
at orbital apex), mixed forms, and Tolosa–Hunt syndrome b Lack of restricted diffusion.
(superior orbital fissure and cavernous sinus involvement). b Imaging features can overlap with lymphoma, with
IgG4-related sclerosing disease is a specific subgroup. presence of pain differentiating the two.
r Orbital pain. b Tendinous involvement helps in distinguishing EOM
r Other clinical features may be present, including proptosis, involvement from Graves disease, where the tendons
restricted ocular movement, periorbital erythema, and are typically spared.
vision loss. r Treatment: steroids and immunosuppressants.
r Response to steroids.
Further reading
Kapur R, Sepahdari AR, Mafee MF,
Putterman AM, Aakalu V, Wendel LJ,
et al. MR imaging of orbital
inflammatory syndrome, orbital cellulitis,
and orbital lymphoid lesions: the role of
diffusion-weighted imaging. AJNR Am J
Neuroradiol 2009; 30(1): 64–70.
269
Case 9.6
270
Chapter 9: Orbits
History Key points

47-year-old patient presents with worsening proptosis and r Painless proptosis, diplopia, motility disturbances.
diplopia. r Firm/rubbery mass.
r Fifth to seventh decade.
r
Findings Imaging:
b Can be circumscribed or infiltrative, with involvement
Figure 9.6.1 Coronal T2 fat-saturated MRI shows intermediate-
signal-intensity lesion involving both intraconal and extraconal space of left of any segment of the globe.
orbit, predominantly involving the superolateral quadrant (arrow). b Contours the globe, often without actual invasion of
structures.
Figure 9.6.2 Coronal T1 post-contrast fat-saturated MRI shows enhancing b Lacrimal fossa/superolateral quadrant is the most
lesion involving both intraconal and extraconal space of left orbit,
predominantly involving the superolateral quadrant (arrow). common site of involvement.
b Restricted diffusion, hypo- to isointense to orbital fat
Figures 9.6.3, 9.6.4 Axial DWI (Fig. 9.6.3) demonstrates hyperintense signal
of the lesion (arrow), and ADC image (Fig. 9.6.4) shows low signal intensity of
on T2, with variable contrast enhancement, is more
the lesion (arrow), consistent with restricted diffusion. suggestive for lymphomas.
b Pain, low T2 signal intensity, and absence of restricted
diffusion points to pseudotumor, which also tends to
Diagnosis involve extraocular muscles more often and to have a
Orbital lymphoma. more heterogeneous pattern of enhancement.
r Treatment: depending on the stage, radiation and/or
Differential diagnosis systemic therapy are used.
r Benign lymphoproliferative disorders.
r Infectious and inflammatory conditions including orbital
pseudotumor, sarcoidosis, and metastasis.
Further reading
Kapur R, Sepahdari AR, Mafee MF, Priego G, Majos C, Climent F, Muntane A.
Putterman AM, Aakalu V, Wendel LJ, Orbital lymphoma: imaging features and
et al. MR imaging of orbital inflammatory differential diagnosis. Insights Imaging
syndrome, orbital cellulitis, and orbital 2012; 3(4): 337–344.
lymphoid lesions: the role of diffusion-
weighted imaging. AJNR Am J
Neuroradiol 2009; 30(1): 64–70.
271
Case 9.7
Figure 9.7.2
Figure 9.7.1
History
47-year-old patient presents with diplopia.
Findings
Figures 9.7.1, 9.7.2 Coronal T2 fat-saturated MRI (Fig. 9.7.1) and coronal T1
post-contrast fat-saturated MRI (Fig. 9.7.2) demonstrate bilateral symmetric
enlargement of extraocular muscles.
Figure 9.7.3 Axial T1-weighted post-contrast fat-saturated MRI shows

enlarged extraocular muscles with sparing of the tendon (arrow).
Diagnosis
Thyroid ophthalmopathy.
r Lympoproliferative disorders.
r Orbital pseudotumor.
r Sarcoidosis.
r Amyloidosis.
Figure 9.7.3 r Metastasis.
272
Chapter 9: Orbits
Key points In the chronic phase, the muscles may appear atrophic,
r Most common in females in fourth and fifth decades. with fatty infiltration.
b Other findings include increased retro-orbital fat and
r Commonly presents with proptosis, orbital edema, and
intraorbital fat stranding.
restricted gaze. b CT: in addition to the above-mentioned morphological
r Usually bilateral, and more often symmetrical.
r May precede, occur simultaneously with, or follow the changes, the enlarged EOMs show areas of low density
due to focal accumulation of mucopolysaccharide.
systemic manifestations of Graves disease. b MRI: involved muscle shows low signal on T1-weighted
r Imaging:
image and intermediate to high signal on T2-weighted
b Inferior rectus is most commonly involved, followed by
image.
medial rectus, superior rectus, levator palpebrae, and r Treatment: medical management. Surgery if conservative
superior oblique muscle.
b Sparing of tendons is characteristic of thyroid treatment fails, including orbital decompression if vision is
threatened.
ophthalmopathy, which differentiates it from other
conditions affecting the extraocular muscles (EOMs).
Further reading
Barrett L, Glatt HJ, Burde RM, Gado MH. Glatt HJ. Optic nerve dysfunction in thyroid
Optic nerve dysfunction in thyroid eye eye disease: a clinician’s perspective.
disease: CT. Radiology 1988; 167(2): Radiology 1996; 200(1): 26–27.
503–507.
273
Case 9.8
274
Chapter 9: Orbits
History Key points

45-year-old male with history of pituitary adenoma presents r Usually in patients with pre-existing pituitary
with acute onset of headaches, visual disturbance, and macroadenoma, resulting in hemorrhage or infarction.
altered mental status. r Headache, visual disturbances, ophthalmoplegia, altered
mental status, coma.
r Imaging:
Findings b CT: not sensitive. May show hyperdense pituitary mass
with fluid–fluid levels.
Figure 9.8.1 Axial NECT shows a pituitary lesion with fluid level (arrow).
b MRI: signal intensity in T1- and T2-weighted images
Figure 9.8.2 Axial T2-weighted MRI shows a fluid–fluid layer with varies depending on infarct or hemorrhage as well as
hypointense dependent material consistent with acute hemorrhage (short the time of presentation. Fluid–fluid levels may be seen.
arrow) and residual tumor along the left lateral aspect of the sella (long arrow). b Acute stage, if hemorrhagic, appears isointense on T1
Figure 9.8.3 Coronal T2-weighted MRI shows residual tumor along the left and hypointense on T2; if infarct, appears hypo- to
lateral sella extending to the cavernous sinus (arrow). isointense on T1 and hyperintense on T2. Post-contrast
MRI may show thin enhancing rim.
Figure 9.8.4 Coronal T1-weighted contrast-enhanced MRI demonstrates b Subacute stage, if hemorrhagic, appears hyperintense
enhancing residual tumor along the left lateral sella extending in to the
cavernous sinus (arrow). The hemorrhage does not enhance. on T1 and T2; if infarct, appears hypointense on T1 and
hyperintense on T2.
b Chronic stage appears hypointense on T1 and
Diagnosis hyperintense on T2 in infarct and hemorrhage.
b Axial T2∗ gradient imaging shows blood products as
Pituitary apoplexy.
hypointense area. Diffusion-weighted imaging may
help identify the apoplexy early in its course as
restricted diffusion.
Differential diagnosis r Treatment: medical management with or without surgical
r Necrotic pituitary macroadenoma.
removal.
Further reading
Piotin M, Tampieri D, Rufenacht DA, Mohr Rogg JM, Tung GA, Anderson G, Cortez S. Tosaka M, Sato N, Hirato J, Fujimaki H,
G, Garant M, Del Carpio R, et al. The Pituitary apoplexy: early detection with Yamaguchi R, Kohga H, et al.
various MRI patterns of pituitary diffusion-weighted MR imaging. AJNR Assessment of hemorrhage in pituitary
apoplexy. Eur Radiol 1999; 9(5): 918–923. Am J Neuroradiol 2002; 23(7): 1240–1245. macroadenoma by T2∗ -weighted
gradient-echo MR imaging. AJNR Am J
Neuroradiol 2007; 28(10): 2023–2029.
275
Case 9.9
276
Chapter 9: Orbits
History Diagnosis
32-year-old patient presents with worsening headaches and Cavernous sinus syndrome due to lymphoma.
diplopia.
r Cavernous sinus syndrome due to other causes:
Findings
b Metastases.
Figure 9.9.1 Axial CT shows an extra-axial hyperdense lesion in the region of b Sarcoidosis.
right cavernous sinus (long arrow) and hyperdense diffusely thickened left
temporalis muscle (short arrow).
b Infectious and inflammatory etiology including
pseudotumor.
Figure 9.9.2 Axial T2-weighted MRI shows the extra-axial lesion in the region
of right cavernous sinus with intermediate to low signal intensity (long arrow)
and diffusely thickened left temporalis muscle with intermediate to low signal
intensity (short arrow). Key points
r Neoplastic, inflammatory, infectious, and vascular lesions
Figure 9.9.3 Axial T1-weighted MRI shows the lesion, which is isointense to arising in the cavernous sinus or from adjacent structures
the brain parenchyma in the region of right cavernous sinus (long arrow) and
diffusely thickened left temporalis muscle with intermediate intensity (short can result in cavernous sinus syndrome.
arrow). r Manifestations depend on the contents affected (cranial
nerves III, IV, V1, V2, and VI, internal carotid artery
Figure 9.9.4 Contrast-enhanced axial T1-weighted MRI shows the enhancing with associated sympathetic fibers) and include
lesion in the region of right cavernous sinus (long arrow), diffusely thickened
enhancing left temporalis muscle (short arrow), and thickening and ophthalmoplegia, pain, sensory loss along the distribution
enhancement of the dura in the left temporal region (curved arrow). of the first and second divisions of the trigeminal nerve,
involvement of the sympathetic and parasympathetic
Figures 9.9.5, 9.9.6 Axial DWI (Fig. 9.9.5) and ADC images (Fig. 9.9.6) supply of the pupil, and Horner’s syndrome.
demonstrate restricted diffusion in the extra-axial lesion in the region of right
cavernous sinus (long arrow) and in the diffusely thickened left temporalis
r Imaging features are often nonspecific. In the presented
muscle (short arrow). case, there is multifocal involvement of cavernous sinus,
277
dura, and temporalis muscle, with mass-like lesions with meningioma, inflammatory pseudotumor, and
restricted diffusion. The T2 hypointense appearance of the sarcoidosis.
lesions, with hyperdense appearance on CT and restricted r Treatment: medical treatment, radiation or surgery
diffusion, suggests high cellularity of the lesion, as may be depending on the etiology, relationship of lesion to the
seen with lymphoma. Other lesions with overlapping neurovascular structures, and involvement of the adjacent
imaging features in this location include metastasis, structures.
Further reading
Lee JH, Lee HK, Park JK, Choi CG, Suh DC. Razek AA, Castillo M. Imaging lesions of the
Cavernous sinus syndrome: clinical cavernous sinus. AJNR Am J Neuroradiol
features and differential diagnosis with 2009; 30(3): 444–452.
MR imaging. AJR Am J Roentgenol 2003;
181(2): 583–590.
278
Chapter
Paranasal sinuses
10 Jason DeBerry, Max Wintermark, Sugoto Mukherjee, and Yang Tang
Case 10.1
279
History Key points

32-year-old male with longstanding sinus complaints r Allergic fungal sinusitis is the most common form of
presents with recent severe exacerbation of symptoms. fungal sinusitis and usually affects immunocompetent
young adults.
r Frequent association with atopic history, allergic rhinitis,
Findings
and asthma.
Figures 10.1.1, 10.1.2 Axial (Fig. 10.1.1) and coronal (Fig. 10.1.2) NECT r Patients usually present with longstanding symptoms of
images show the expansion of the paranasal sinuses with hyperdense
secretions (∗ ) and sinus wall dehiscence (arrows). nasal congestion, chronic sinusitis, and headaches.
r CT characteristically demonstrates pan sinus disease with
Figure 10.1.3 Axial T2 image shows the marked hypointensity (∗ ) within expanded sinuses with hyperdense fungal material within
expanded ethmoid and sphenoid sinuses, mimicking intrasinus air. Also note
the thin rim of peripheral T2 hyperintense secretions (white arrow). them.
r On MRI, these lesions demonstrate a complex appearance
Figure 10.1.4 Coronal post-contrast T1-weighted images show the marked with a mixed pattern of T1 and T2 signal. Characteristic
expansion of the sinuses, and mucosal enhancement (white arrows). There is
lack of enhancement of the central secretions (∗ ).
low signal is present on T2-weighted sequences, secondary
to fungal secretions containing heavy metals. The
surrounding peripheral mucosa demonstrates avid
Diagnosis enhancement.
Allergic fungal sinusitis. r Although these lesions are usually noninvasive, extra
attention should be paid to the sinus boundaries, as when
left untreated they can extend to involve the adjacent orbit
Differential diagnosis and the skull base, with intraorbital and intracranial
r Sinonasal carcinoma.
complications.
r Sinonasal lymphoma.
r Sinonasal polyposis.
Further reading
Aribandi M, McCoy VA, Bazan C, 3rd.
Imaging features of invasive and
noninvasive fungal sinusitis: a review.
Radiographics 2007; 27(5): 1283–1296.
280
Chapter 10: Paranasal sinuses
Case 10.2
Figure 10.2.1
Figure 10.2.2
History
54-year-old male with recent onset of left-sided facial pain
Key points
r Malignant tumors of the paranasal sinuses are rare,
and swelling.
accounting for 3% of all head and neck cancers; 80% arise
in the maxillary sinuses, of which 60–90% are squamous
Findings cell carcinoma (SCC).
r Associated risk factors include human papillomavirus,
Figure 10.2.1 Axial CECT demonstrates heterogeneously enhancing
soft-tissue mass centered within the left maxillary sinus, with destruction of the inhaled wood dust, metallic particles, and chemicals.
anterior, medial, and posterolateral walls. r Usually asymptomatic until advanced stage. Maxillary
sinus tumors can cause nasal blockage, trismus, epiphora,
Figure 10.2.2 Coronal CECT of the same patient demonstrates aggressive
osseous destruction of the mass, with extension into the left orbit (curved orbital pain, proptosis, trigeminal or sphenopalatine
arrow) and nasal cavity (arrow). ganglion-related deficits due to perineural tumor spread.
r CECT usually shows an enhancing mass with aggressive
osseous destruction. Unilateral opacification of sinus on
Diagnosis CT usually suggests an underlying mass lesion and requires
Squamous cell carcinoma of the maxillary sinus. workup for exclusion.
r The majority of SCC masses have intermediate signal on
T2-weighted imaging, distinguishing it from inflammatory
Differential diagnosis tissues, which have high signal on T2. Osseous erosion and
r Sinonasal adenocarcinoma. remodeling is seen in large tumors.
r Sinonasal non-Hodgkin lymphoma. r Predictors of survival include tumor size and negative
r Invasive fungal sinusitis. surgical margin. Surgery is the primary treatment
r Wegener granulomatosis. modality.
Further reading
Lubek JE, Clayman L. An update on Robbins KT, Ferlito A, Silver CE, Takes RP,
squamous carcinoma of the oral cavity, Strojan P, Snyderman CH, et al.
oropharynx, and maxillary sinus. Oral Contemporary management of sinonasal
Maxillofac Surg Clin North Am 2012; cancer. Head Neck 2011; 33(9): 1352–
24(2): 307–316, x. 1365.
281
Case 10.3
Findings
Figures 10.3.1, 10.3.2 Sagittal (Fig. 10.3.1) and coronal (Fig. 10.3.2)
contrast-enhanced T1 images demonstrate avidly enhancing mass in the nasal
cavity and ethmoid sinuses (block arrows) extending through the cribriform
plate into the anterior cranial fossa (arrow). Note a characteristic cyst at the
tumor–brain interface (curved arrow).
Figure 10.3.3 Axial T2 image shows intermediate signal of the mass relative
to the brain parenchyma. Obstructed secretion is noted in the frontal sinuses.
Diagnosis
Esthesioneuroblastoma.
r Sinonasal undifferentiated carcinoma.
r Sinonasal squamous cell carcinoma.
r Sinonasal adenocarcinoma.
r Sinonasal non-Hodgkin lymphoma.
r Melanoma.
Key points
Figure 10.3.3 r Esthesioneuroblastoma is also known as olfactory
neuroblastoma and pleomorphic olfactory neuroblastoma.
r Malignant tumor arising from olfactory neuroepithelium
in superior nasal cavity with unknown etiology.
History r Rare tumor that typically affects patients in their second
44-year-old man presents with headache and nasal and sixth decades of life and accounts for 3–6% of all nasal
stuffiness. cavity and sinonasal neoplasms.
282
r Commonly presents as unilateral nasal obstruction and r Local spread can occur throughout the paranasal sinuses,
epistaxis. skull base, orbit, cavernous sinus, and brain. Intracalvarial
r Classic appearance is a dumbbell-shaped mass with its invasion is considered a poor prognostic finding.
waist at the level of the cribriform plate. CT will r Treatment consists of craniofacial resection and
demonstrate gross bone remodeling with enlargement of radiotherapy.
the nasal cavity in the cribriform plate region. Mass is iso- r Recurrence depends on modality of treatment, but can
to slightly hyperdense with scattered areas of necrosis and occur up to a decade after diagnosis. A recurrence rate of
marginal cysts on CT, hypointense/intermediate on up to 36% has been reported.
T1-weighted imaging and intermediate/hyperintense on
T2 to brain with areas of cystic degeneration. It
demonstrates intense homogeneous enhancement.
Further reading
Gallia GL, Reh DD, Salmasi V, Blitz AM,
Koch W, Ishii M. Endonasal endoscopic
resection of esthesioneuroblastoma: the
Johns Hopkins Hospital experience and
review of the literature. Neurosurg Rev
2011; 34(4): 465–475.
283
Case 10.4
Figure 10.4.2
Figure 10.4.1
284
History Key points

35-year-old presents with recent onset of right-sided facial r Inverted papilloma is a benign epithelial tumor of nasal
pain and headaches. mucosa of which 11% degenerate into or coexist with
squamous cell carcinoma. They account for up to 4.7% of
Findings all sinonasal tumors.
r Classically occurs along the lateral nasal wall near the
Figure 10.4.1 Axial NECT demonstrates soft-tissue mass arising along the middle meatus and involves at least one sinus in 82% of
right lateral nasal wall at the middle meatus (curved arrow) with extension into
the right maxillary sinus (∗ ). cases, most commonly the maxillary sinus.
r Etiology is not well understood; however, 30% of inverted
Figure 10.4.2 Coronal NECT demonstrates bony remodeling (suggestive for papillomas have been found to be positive for HPV.
a less aggressive process) of the right lateral maxillary secondary to mass effect r Usually affects males more than females, in fifth through
(arrow).
eighth decades of life.
Figure 10.4.3 On T2 image, the right maxillary mass has a T2 heterogeneous r Most common presentation is recurrent sinusitis and nasal
appearance, with high-signal secretions surrounding the lesions and extending
within it.
obstruction. Additional symptoms include epistaxis,
rhinorrhea, anosmia, facial pain, and frontal headache.
Figure 10.4.4 The right maxillary sinus mass enhances heterogeneously on r CECT will demonstrate enhancing soft-tissue mass along
T1 post-contrast imaging, with a heterogeneous convoluted appearance, also the lateral nasal wall at the middle meatus with extension
described as a “cerebriform” pattern of enhancement.
into the sinuses and associated bone remodeling.
r MRI is best used for preoperative planning after the
Diagnosis diagnosis on CT. The mass is iso- to hyperintense to muscle
Inverted papilloma. on T1 and heterogeneously hyperintense to muscle
on T2, with “convoluted, cerebriform” appearance on
post-contrast images.
Differential diagnosis r Surgical resection is the primary treatment, given the
r Solitary sinonasal polyp.
potential for degeneration and concomitant squamous cell
r Sinonasal squamous cell carcinoma.
carcinoma.
r Sinonasal polyposis.
Further reading
Momeni AK, Roberts CC, Chew FS. Imaging Slootweg PJ, Ferlito A, Cardesa A,
of chronic and exotic sinonasal disease: Thompson LD, Hunt JL, Strojan P, et al.
review. AJR Am J Roentgenol 2007; 189(6 Sinonasal tumors: a clinicopathologic
Suppl): S35–S45. update of selected tumors. Eur Arch
Otorhinolaryngol 2013; 270(1): 5–20.
285
Case 10.5
Figure 10.5.1
Figure 10.5.2
History
45-year-old female presents with seizure and altered mental
status.
Findings
Figures 10.5.1, 10.5.2 Sagittal (Fig. 10.5.1) and coronal NECT (Fig. 10.5.2)
demonstrate an osseous defect in the right anterior skull base (block arrow),
with soft tissue protruding into the frontal and ethmoid sinuses (∗ ).
Figure 10.5.3 Axial T2 image demonstrates herniation of a portion of the

right inferior frontal brain parenchyma and meninges through the osseous
defect.
Diagnosis
Frontoethmoidal meningoencephalocele.
r Nasal dermoid.
r Nasal glioma.
Figure 10.5.3
r Nasopharyngeal neoplasm.
286
r CTA is helpful in characterizing the bony defect and

Key points
r Frontoethmoidal meningoencephalocele is herniation of vasculature for operative planning. MRI is useful in
identifying the contents of the sac and any associated
brain and meninges through a congenital defect between
parenchymal pathology. T1-weighted MRI will
the frontal and ethmoid bones.
r Etiology of frontoethmoidal meningoencephalocele is demonstrate displacement of structures through the skull
defect. T2 MRI is best for distinction of neural tissue from
unknown, with the highest incidence occurring in
CSF.
Southeast Asia. r Primary treatment is surgical correction of the cranial
r Common presentation occurs at birth with skin-covered
defect.
masses or protrusions on the face, nose, glabella, or
forehead that may enlarge with crying. Other presentations
include CSF rhinorrhea, nasal obstruction, recurrent
meningitis, and nasal purulent discharge.
Further reading
Tirumandas M, Sharma A, Gbenimacho I,
Shoja MM, Tubbs RS, Oakes WJ, et al.
Nasal encephaloceles: a review of
etiology, pathophysiology, clinical
presentations, diagnosis, treatment, and
complications. Childs Nerv Syst 2013;
29(5): 739–744.
287
Chapter
Temporal bone
11 Michael Reardon, Yang Tang, Max Wintermark, and Sugoto Mukherjee
Case 11.1
History
56-year-old male with left-sided otorrhea and ear pain.
Findings
Figures 11.1.1–11.1.3 Axial, coronal, and sagittal NECT images demonstrate
a soft-tissue mass (black arrows) localized to the external auditory canal with
adjacent osseous destruction. Figure 11.1.3 demonstrates a small bone
fragment within the soft-tissue mass (thin black arrow).
Diagnosis
External auditory canal cholesteatoma.
r Keratosis obturans.
r Squamous cell carcinoma.
r Medial canthal fibrosis.
r Malignant otitis externa.
Figure 11.1.3
288
Chapter 11: Temporal bone
r Characteristic CT appearance is that of a soft-tissue-density

Key points
r Cholesteatoma is a soft-tissue mass composed of exfoliated mass located in the external auditory canal causing
adjacent bone erosion and containing internal bone
keratin within stratified squamous epithelium.
r Cholesteatomas rarely occur in the external auditory canal fragments. The bony erosion can be both smooth and
irregular. Commonly involves the inferior wall of the
(0.1–0.5% of cases). Most cases present in the middle ear
canal.
and mastoid. r Evaluate images for involvement into the middle ear
r The lesion can arise spontaneously if there is loss of the
structures and adjacent mastoid air cells.
normal epithelial migration from the tympanic membrane r Treatment options include routine office debridement and
to the outer ear canal, or can arise post-traumatically or
surgical excision.
postsurgically if there is entrapment of squamous epithelial
debris.
Further reading
Heilbrun ME, Salzman KL, Glastonbury
CM, Harnsberger HR, Kennedy RJ,
Shelton C. External auditory canal
cholesteatoma: clinical and imaging
spectrum. AJNR Am J Neuroradiol 2003;
24(4): 751–756.
289
Case 11.2
History
55-year-old male with chronic left middle ear infections and
conductive hearing loss.
Findings
Figure 11.2.1 Coronal NECT demonstrates a soft-tissue mass centered in the
epitympanum and mastoid cavity causing erosion of the surrounding bony
structures including the scutum (curved arrow). There is dehiscence with the
lateral semicircular canal (straight arrow). No normal middle ear ossicles are
identified, consistent with complete erosion.
Figure 11.2.2 Axial NECT demonstrates the soft-tissue mass extending

though the aditus ad antrum (arrow). The surrounding temporal bone
demonstrates a sclerotic appearance (∗ ).
Figure 11.2.3 Axial diffusion-weighted image demonstrates restricted

diffusion in the left middle ear cavity (confirmed by ADC, not shown).
Diagnosis
Middle ear cholesteatoma.
Figure 11.2.3
290
Differential diagnosis tympanic membrane, referred to as pars flaccida

r Glomus tympanicum paraganglioma. cholesteatomas.
r Pars tensa cholesteatomas arise from the lower tympanic
r Cholesterol granumoma.
r Coalescent mastoiditis. membrane.
r Pars flaccida cholesteatomas are classically located in
Prussak’s space, with erosion of the adjacent scutum.
r Larger lesions erode the surrounding bony structures
Key points including the middle ear ossicles, tegmen tympani, and
r Cholesteatoma is a soft-tissue mass composed of exfoliated bony covering over the lateral semicircular canal (leading
keratin within stratified squamous epithelium. to dehiscence).
r The majority of acquired cases arise from a retraction r MRI demonstrates a non-enhancing lesion with restricted
pocket in the posterior superior portion of the diffusion.
Further reading
De Foer B, Kenis C, Vercruysse JP, Somers De Foer B, Vercruysse JP, Bernaerts A, imaging versus delayed gadolinium-
T, Pouillon M, Offeciers E, et al. Imaging Meersschaert J, Kenis C, Pouillon M, enhanced T1-weighted MR imaging –
of temporal bone tumors. Neuroimaging et al. Middle ear cholesteatoma: value in detection. Radiology 2010;
Clin N Am 2009; 19(3): 339–366. non-echo-planar diffusion-weighted MR 255(3): 866–872.
291
Case 11.3
292
r Metastatic disease.
History r Jugular foramen meningioma.
52-year-old female presents with right-sided pulsatile r Endolymphatic sac tumor.
tinnitus.
Findings Key points

r Benign highly vascular tumors which originate from the
Figure 11.3.1 Axial NECT demonstrates a permeative destructive lesion glomus bodies (extra-adrenal neural crest cells) located at
centered in the right jugular foramen (arrow). Notice its close association to the
right carotid canal (∗ ). the jugular foramen and cochlear promontory.
r The glomus tympanicum tumor is a well-defined lesion
Figure 11.3.2 Coronal NECT demonstrates the lesion extending superiorly situated along the cochlear promontory that presents as a
and laterally from the jugular foramen (∗ ) to the middle ear cavity, overlying the
cochlear promontory (arrow) and oval window. retrotympanic vascular mass.
r Glomus jugulare refers to paragangliomas arising at the
Figure 11.3.3 Axial T1 post-contrast with fat saturation, showing an avidly jugular foramen. The tumors present a more aggressive
enhancing mass centered in the jugular foramen abutting the posterior aspect appearance with surrounding permeative destructive bone
of the right internal carotid artery. Notice the flow voids within the lesion
(curved arrow). changes. The usual trajectory of growth for these tumors is
superior and lateral into the hypotympanum, at which
Figure 11.3.4 Coronal T1 post-contrast with fat saturation demonstrating the point they are referred to as glomus jugulotympanicum
enhancing lesion’s trajectory of growth (arrow) superiorly and laterally into the
middle ear cavity. paragangliomas.
r Pulsatile tinnitus is the most common presenting
symptom.
Diagnosis r Glomus jugulotympanicum tumors present on CT as a
Glomus jugulotympanicum paraganglioma. mass lesion centered in the jugular foramen extending to
the middle ear cavity with a surrounding moth-eaten
Differential diagnosis appearance of the bone. MRI shows an avidly enhancing
r Cholesteatoma. mass with internal flow voids, the classic “salt and pepper”
r Schwannoma. appearance.
Further reading
De Foer B, Kenis C, Vercruysse JP, Somers Weissman JL, Hirsch BE. Imaging of
T, Pouillon M, Offeciers E, et al. Imaging tinnitus: a review. Radiology 2000;
of temporal bone tumors. Neuroimaging 216(2): 342–349.
Clin N Am 2009; 19(3): 339–366.
293
Case 11.4
History
46-year-old male with headache.
Findings
Figure 11.4.1 Axial NECT demonstrates an expansile well-defined lesion in
the left petrous apex with thinning of the surrounding cortex (∗ ). Notice
normal-appearing pneumatized right petrous apex (black arrow).
Figure 11.4.2 Axial T1 without contrast shows a well-marginated left petrous

apex lesion that is homogeneously T1 hyperintense (∗ ).
Figure 11.4.3 Axial T2 shows the lesion to be T2 hyperintense with a

well-defined hypointense rim (∗ ).
Diagnosis
Petrous apex cholesterol granuloma.
r Congenital cholesteatoma.
r Asymmetric marrow.
r Mucocele.
r Petrous apicitis.
Figure 11.4.3 r Petrous apex effusion.
294
r Characteristic MRI appearance with both T1 and T2

Key points
r Most common petrous apex lesion in adults. Usually an hyperintensity. The lesion can also demonstrate a
well-defined peripheral ring of T2 hypointensity related to
asymptomatic incidental finding when small. Once the
hemosiderin deposition. Typically does not enhance
lesion expands, mass effect can develop and affect the
centrally but can demonstrate a peripheral rim of
surrounding cranial nerves (abducens, facial, and
enhancement.
vestibulocochlear). r CT shows a well-defined lytic expansile lesion with
r Arises from a pneumatized petrous apex. Unknown
thinning of the cortex.
etiology but classically thought to originate from negative r Symptomatic lesions can undergo surgical drainage
pressure leading to tissue breakdown, hemorrhage, and
through a variety of approaches including infracochlear,
cholesterol formation with subsequent foreign-body
infralabyrinthine, and minimally invasive endoscopic
giant-cell reaction. Chronic history of otitis media is a
endonasal approach.
major risk factor for the development of cholesterol
granuloma.
Further reading
Schmalfuss IM. Petrous apex. Neuroimaging
Clin N Am 2009; 19(3): 367–391.
295
Case 11.5
History
43-year-old male presents with continued right-sided
sensorineural hearing loss after recent middle ear infection.
Findings
Figure 11.5.1 Axial T1-weighted image without intravenous contrast
demonstrates subtle hyperintensity in the right cochlea (arrow) as compared to
the normal left side.
Figure 11.5.2 Axial T1-weighted image after intravenous contrast

administration demonstrates avid enhancement (arrow) in the right cochlea.
Figure 11.5.3 Axial high-resolution T2-weighted image demonstrates lack of

normal fluid signal (arrow) within the basal turn of the right cochlea.
Diagnosis
Labyrinthitis.
r Labyrinthine hemorrhage.
Figure 11.5.3 r Labyrinthine schwannoma.
296
r Three stages described by imaging: acute, fibrous, and

Key points
r Infection or inflammation of the inner ear involving the labyrinthitis ossificans.
r The acute stage occasionally demonstrates subtle T1
fluid-filled membranous labyrinthine structures: the
hyperintensity and avid contrast enhancement.
vestibule, semicircular canals, and cochlea. r The fibrous stage demonstrates a loss of the normal fluid
r Classically presents as the sudden onset of unilateral
intensity signal on high-resolution T2-weighted images
sensorineural hearing loss.
r Etiologies include meningitis, direct spread from middle with continued contrast enhancement.
r Labyrinthitis ossificans demonstrates ossification within
ear infections, hematogenous, and post-traumatic.
r Labyrinthitis arising from meningitis (bacterial, viral, or the membranous labyrinth, best evaluated with
thin-section temporal bone CT.
autoimmune) is the most common cause of childhood r Labyrinthitis ossificans is a relative contraindication to
sensorineural hearing loss, typically bilateral.
cochlear implant placement.
Further reading
Eshetu T, Aygun N. Imaging of the temporal Lemmerling MM, De Foer B, Verbist BM,
bone: a symptom-based approach. Semin VandeVyver V. Imaging of inflammatory
Roentgenol 2013; 48(1): 52–64. and infectious diseases in the temporal
bone. Neuroimaging Clin N Am 2009;
19(3): 321–337.
297
Case 11.6
History
44-year-old female with progressive left-sided hearing loss
and tinnitus.
Findings
Figure 11.6.1 Axial CECT demonstrates an erosive mass centered in the
posterior medial left temporal bone with internal bony spicules (arrow). There
is involvement of the adjacent middle ear, mastoid air cells, and petrous apex.
Figure 11.6.2 Axial T1 without contrast shows the expansile tumor with a
peripheral rim of intrinsic T1 shortening (arrows).
Figure 11.6.3 Axial post-contrast T1 with fat saturation demonstrates avid

enhancement throughout the lesion.
Diagnosis
Endolymphatic sac tumor (ELST).
r Glomus jugulare.
r Meningioma.
r Schwannoma.
Figure 11.6.3 r Metastatic disease.
298
r CT demonstrates a retrolabyrinthine erosive mass with

Key points
r Adenomatous neoplasm arising from the epithelium intratumoral bony spicules and a thin rim of expanded
bone.
within the endolymphatic sac. r Non-contrast T1 classically demonstrates foci of intrinsic
r Located in the posterior medial temporal bone between the
hyperintensity, with avid enhancement without flow voids
internal auditory canal and sigmoid sinus. Larger lesions
on post-contrast imaging.
can extend into the middle ear and jugular foramen, and r Angiography demonstrates a hypervascular mass. Smaller
can encroach into the cerebellopontine angle
tumors are primarily supplied by branches of the external
cistern.
r Most tumors occur sporadically, but there is an increased carotid artery; larger tumors may recruit branches of the
internal carotid.
incidence in patients with von Hippel–Lindau disease r ELSTs are treated with complete surgical resection.
(VHL). VHL patients can also have bilateral
Pre-surgical embolization can help minimize the
endolymphatic sac tumors.
intraoperative bleeding.
Further reading
De Foer B, Kenis C, Vercruysse JP, Somers Leung RS, Biswas SV, Duncan M, Rankin S.
T, Pouillon M, Offeciers E, et al. Imaging Imaging features of von Hippel–Lindau
of temporal bone tumors. Neuroimaging disease. Radiographics 2008; 28(1): 65–79;
Clin N Am 2009; 19(3): 339–366. quiz 323.
299
Case 11.7
History Key points

35-year-old male with left-sided facial palsy for 2 weeks. r The facial nerve is composed of six named segments:
intracranial, meatal (IAC), labyrinthine, tympanic,
mastoid, and extratemporal segments.
Findings r Bell’s palsy is secondary to reactivation of latent herpes
simplex virus 1 (HSV-1) within the geniculate ganglion,
Figure 11.7.1 Axial post-contrast T1 shows left facial nerve enhancement in
the internal auditory canal (IAC) fundus (arrow), and anterior tympanic which results in inflammatory edema of the facial
segment (curved arrow). The tympanic segment is also mildly thickened. nerve.
r Normal cranial nerve enhancement can be seen on
Figure 11.7.2 Axial post-contrast T1 demonstrates enhancement of the
labyrinthine segment of the left facial nerve (arrow). post-contrast imaging at the geniculate ganglion and in the
tympanic and mastoid segments of the facial nerve, due to
its peri- and epineural venous plexuses. The meatal and
Diagnosis labyrinthine segments do not normally enhance.
r Bell’s palsy is the most common cause of sudden-onset
Bell’s palsy.
unilateral facial nerve palsy.
r Imaging is not usually needed in early evaluation.
Differential diagnosis However, imaging findings are seen within the first month
r Facial nerve schwannoma. of onset of paralysis. On post-contrast T1 images there will
r Facial nerve venous malformation. be an asymmetric “tuft” of enhancement of the fundus of
r Perineural tumor spread from parotid gland. the IAC and labyrinthine segment of cranial nerve 7.
300
(Entire intratemporal CN7 may enhance.) No bony r Treatment: steroids. Surgical facial nerve decompression
abnormality should be seen. for profound denervation.
Further reading
Tien R, Dillon WP, Jackler RK.
Contrast-enhanced MR imaging of the
facial nerve in 11 patients with Bell’s
palsy. AJNR Am J Neuroradiol 1990;
11(4): 735–741.
301
Chapter
Head and neck tumors
12 David Chiao, Sugoto Mukherjee, Yang Tang, and Max Wintermark
Case 12.1

54-year-old male with new-onset hoarseness. r Laryngeal chondrosarcoma.
r Laryngeal sarcoidosis.
Findings r Gastroesophageal reflux disease.
Figure 12.1.1 Axial CECT shows thickened and enhancing right true vocal
cord (block arrow), with extralaryngeal extension into the right strap muscles
across the right thyroid cartilage (arrow). The lesion involves the anterior
commissure (∗ ). Also note the sclerotic appearance of the right aspect of the Key points
cricoid cartilage (curved arrow).
r Laryngeal carcinomas usually arise in older males, who
Figure 12.1.2 Coronal CECT shows the transglottic extension of the lesion typically present with hoarseness and change in voice.
involving both the right true and false vocal cords (∗ ) across the laryngeal r Due to early clinical presentation, most of the glottis
ventricle (curved arrow) and the inferior right paraglottic space.
carcinomas present early, resulting in good prognosis.
Metastatic lymph nodes at presentation in these early-stage
Diagnosis tumors are rare.
Laryngeal carcinoma.
302
Chapter 12: Head and neck tumors
r Imaging: b Arytenoid cartilage sclerosis on CT is a nonspecific

b On CECT, these appear as enhancing, infiltrative, or finding. MRI is helpful for cartilage invasion.
exophytic masses. r Treatment:
b The role of imaging is to map out the extent of the b For smaller T1 lesions (limited to unilateral vocal
lesion, specifically involvement outside the cord and cord), laser surgery or radiotherapy is usually curative.
sub-/supraglottic extension, as well as extralaryngeal b For larger lesions or with nodal disease, the treatment
extension and cartilage invasion. Involvement of the usually includes surgery ± radiotherapy.
contralateral cord across the anterior commissure
should be actively looked for.
Further reading
Hermans R. Staging of laryngeal and
hypopharyngeal cancer: value of imaging
studies. Eur Radiol 2006; 16(11):
2386–2400.
303
Case 12.2
History r 70–80% of tonsillar carcinomas have lymphadenopathy on

45-year-old female with recent onset of right sided neck presentation, and nodal metastasis is considered as the
swelling. single most important prognostic factor for head and neck
SCCs.
r Level II nodal disease is seen with SCCs arising from all
Findings subsites, including nasopharynx, oral cavity/oropharynx,
and larynx/hypopharynx.
Figure 12.2.1 Axial CECT of the neck shows a well-defined rim-enhancing
cystic lesion (block arrow) anterior to the right carotid sheath and
r Unlike tobacco- and alcohol-associated head and neck
sternocleidodomastoid muscle. SCCs, human papillomas virus (HPV)-associated head and
neck cancers can be seen with a small base-of-tongue/
Figure 12.2.2 Follow-up CECT in the same patient shows a thicker irregular
rim of enhancement of the same lesion (block arrows), with an enhancing mass tonsillar primary cancer and a more impressive bulky
now seen within the right tonsillar fossa (curved arrow). cystic adenopathy in a younger subset of patients. Given
the above imaging appearance, these cancers can be easily
missed and need careful interpretation by head and
Diagnosis neck radiologists. Sometimes the primary cancer is
Metastatic level II adenopathy secondary to a primary unidentifiable, and these are then staged as carcinoma with
tonsillar squamous cell carcinoma (SCC). unknown primary.
r Imaging:
b On CT, malignant nodes appear enlarged ( 15 mm for
Differential diagnosis level I and IIA nodes), with a rounded configuration
r Type 2 branchial cleft cyst.
and central necrosis. Extracapsular spread of disease is
r Nerve sheath tumor.
manifested by indistinct nodal margins and perinodal
soft-tissue infiltration. As seen above, a level IIA cystic
nodal metastasis can easily mimic a type 2 branchial
Key points cleft cyst.
r New neck mass in an adult should be considered to be b Type II branchial cleft cysts appear as thin-walled cystic
malignant until proven otherwise. lesion in a young patient with a classic clinical
304
presentation of recurrent mass in the angle of the r Treatment: primary radiotherapy, surgery ± radiotherapy,
mandible. Given the increasing frequency of or chemotherapy ± radiotherapy, depending on additional
HPV-associated cancers, this should be a diagnosis of findings.
exclusion.
Further reading
Hudgins PA, Gillison M. Second branchial
cleft cyst: not!! AJNR Am J Neuroradiol
2009; 30(9): 1628–1629.
305
Case 12.3
306
Key points
r Typically due to injury to the vagus nerve or recurrent
laryngeal nerve.
b Can be due to trauma, surgery, carotid artery
dissection, Ortner’s syndrome (cardiovocal syndrome,
usually from left atrial enlargement), or neoplasm.
b Can be idiopathic: possibly toxic, infectious,
inflammatory, or ischemic.
r Course of the vagus nerve (CN X)
b Arises from the medulla, exits the cranial vault via the
jugular foramen, and travels in the carotid sheath
between the internal carotid artery and internal jugular
vein.
Right recurrent laryngeal nerve: arises from CN X at
the subclavian artery.
Left recurrent laryngeal nerve: arises from CN X at
the aortopulmonary window.
Note: recurrent laryngeal nerves ascend to the
larynx in the tracheoesophageal grooves.
r Imaging:
Figure 12.3.5 b Diagnosis can usually be suggested with thin-slice
multiplanar CECT and confirmed with laryngoscopy.
CECT should be performed from the skull base to
History the carina to evaluate the entire course of the vagus
68-year-old male presents with new onset of hoarseness. nerves and recurrent laryngeal nerves.
MRI shows similar findings as CECT; the affected
vocal cord can demonstrate T2 hyperintensity and
Findings enhancement in the setting of acute denervation.
Figure 12.3.1 Axial CECT demonstrates asymmetric thickening of the left
PET demonstrates absent FDG uptake in the
aryepiglottic fold with mild enlargement of the left pyriform sinus (white affected vocal cord.
curved arrow), which is partially filled with fluid. b Asymmetry of the larynx, with the affected side pulled
medially:
Figure 12.3.2 Axial CECT demonstrates anteromedial rotation of the left
arytenoid cartilage (white arrow). Paramedian position of the affected vocal cord.
Medially displaced, thickened aryepiglottic fold.
Figure 12.3.3 Axial CECT demonstrates the paramedian position of the left Anteromedial rotation of the affected arytenoid
vocal cord (white arrow).
cartilage.
Figure 12.3.4 Axial CECT demonstrates a heterogeneously enhancing Enlarged pyriform sinus.
subcarinal mass which circumferentially surrounds the esophagus (white Ballooning of the laryngeal ventricle.
arrow).
r Clinical presentation: patients can present with hoarseness,
Figure 12.3.5 Esophagogram demonstrates significant narrowing of the dysphonia, weak cough, and aspiration.
midthoracic esophagus with pronounced shouldering (white arrows), r Treatment:
consistent with esophageal malignancy.
b Prognosis depends on the etiology. Idiopathic causes
are usually self-limiting.
Diagnosis b Conservative: voice therapy.
Vocal cord paralysis (from esophageal cancer). b Surgical:
Vocal cord augmentation: material is injected in the
paraglottic space.
Differential diagnosis Thyroplasty: a small piece of thyroid cartilage is
r Status post thyroid surgery, thyroplasty, laryngocele, glottis replaced with silastic, which pushes the vocal cord
cancer. to the midline and improves vocal cord closure.
307
Further reading
Chin SC, Edelstein S, Chen CY, Som PM. Paquette CM, Manos DC, Psooy BJ.
Using CT to localize side and level of Unilateral vocal cord paralysis: a review
vocal cord paralysis. AJR Am J Roentgenol of CT findings, mediastinal causes, and
2003; 180(4): 1165–1170. the course of the recurrent laryngeal
nerves. Radiographics 2012; 32(3):
721–740.
308
Case 12.4
Findings
Figure 12.4.1 Axial NECT demonstrates an expansile mixed lytic sclerotic
mass centered in the clivus with amorphous intratumoral calcifications (white
arrow).
Figure 12.4.2 Axial T2-weighted image demonstrates a mildly T2

hyperintense mass with dark internal septations (white arrow); focal nodular
excrescences displace and deform the surface of the pons.
Figure 12.4.3 Sagittal T1-weighted image post-contrast demonstrates

relatively homogeneous enhancement of the mass (white arrow).
Diagnosis
Skull base tumor (chordoma of the clivus).
r Chondrosarcoma.
r Nasopharyngeal carcinoma.
r Giant cell tumor.
r Plasmacytoma.
r Lymphoma.
r Metastasis.
Figure 12.4.3 r Fibrous dysplasia.
r Paget’s disease.
History Key points

56-year-old male with several months of diplopia and r Chordoma is a malignant tumor which arises from
headaches. notochord remnants.
309
b Location: sarcococcygeal > clivus > rest of the T1 hypo- to isointense mass.
spine. T2 hyperintense mass with multiple septations;
r Typically occurs in older patients (5–6th decade of life) internal hemorrhage is common.
with M:F ratio of 2:1. r Clinical presentation: patients can present with diplopia,
r Slow-growing; local recurrence is common. headaches, and facial pain.
r Imaging: r Treatment: surgical resection with adjuvant radiation
b Lobulated soft-tissue mass with underlying osseous therapy.
r Local recurrence or seeding along the operative tract is
destruction and amorphous intratumoral calcification.
b Variable enhancement. common.
b MRI: r Prognosis: 5-year survival 50–75%, 10-year survival
25–50%.
Further reading
Maclean FM, Soo MY, Ng T. Chordoma: Weber AL, Liebsch NJ, Sanchez R, Sweriduk
radiological-pathological correlation. ST, Jr. Chordomas of the skull base:
Australas Radiol 2005; 49(4): 261–268. radiologic and clinical evaluation.
515–527.
310
Chapter
Pediatric head and neck conditions
13 Michael Reardon, Yang Tang, Max Wintermark, and Sugoto Mukherjee
Case 13.1
History
1-year-old male with left sided leukokoria.
Findings
Figures 13.1.1–13.1.3 Axial T1 pre-contrast (Fig. 13.1.1), axial T1
post-contrast with fat saturation (Fig. 13.1.2), and axial T2 (Fig. 13.1.3)
demonstrate cone-shaped enhancing retrolental soft tissue (Fig. 13.1.1, black
arrow). The left globe is small compared to the right. T1 images demonstrate
the marked hyperintensity in the surrounding vitreous due to retinal
detachment and hemorrhage.
Diagnosis
Persistent hyperplastic primary vitreous.
r Retinoblastoma.
r Coats’ disease.
Figure 13.1.3
311
r Imaging demonstrates a small globe with retrolental

Key points
r Developmental ocular lesion resulting from failure of enhancing soft tissue extending from the lens to the optic
nerve head.
regression of the embryonic hyaloid artery traversing the r Associated with microphthalmia and retinal detachment.
vitreous from the lens to the optic nerve head and the r Most cases are unilateral. The presence of bilateral lesions
surrounding fibrovascular tissue of the primary
suggests an underlying congenital condition such as
vitreous.
Warburg syndrome.
Further reading
Gujar SK, Gandhi D. Congenital
malformations of the orbit. Neuroimaging
Clin N Am 2011; 21(3): 585–602, viii.
312
Chapter 13: Pediatric head and neck conditions
Case 13.2
Findings
Patient 1
Figure 13.2.1 Axial NECT shows a thickened posterior vomer (arrow) and
medial deviation of the posterior wall of the left nasal cavity (curved arrow).
Layering secretions are present in the left nasal cavity, while the right nasal
cavity is patent.
Patient 2
Figure 13.2.2 Axial NECT shows narrowing of the pyriform aperture,
measuring 5 mm between the nasal processes of the anterior maxilla (arrows).
Figure 13.2.3 Axial NECT at a more inferior level demonstrates a solitary

maxillary central incisor (arrow).
Diagnosis
Patient 1: unilateral choanal atresia.
Patient 2: pyriform aperture stenosis associated with a
solitary maxillary central incisor.
r Nasal foreign body.
History r Nasal cephalocele.
Patient 1: 5-day-old female with difficulty breathing. r Nasolacrimal duct cyst.
Patient 2: 3-day-old male with respiratory distress. r Nasopharyngeal atresia.
313
b Choanal atresia is associated with many congenital

Key points
r Respiratory distress in a newborn with concern for nasal abnormalities, in particular CHARGE syndrome.
obstruction should be evaluated with thin-section NECT. r Pyriform aperture stenosis is defined as a pyriform
r Choanal atresia can be unilateral or bilateral, and can be aperture measuring less than 11 mm in term
composed of osseous elements, membranous elements, or infants.
both. b CT in pyriform aperture stenosis demonstrates
b CT in choanal atresia demonstrates a thickened prominent/overgrown nasal processes of the maxilla.
posterior vomer and medial position of the b Pyriform aperture stenosis is commonly associated
posterior wall of the nasal cavity. Secretions can be with a solitary maxillary central incisor. This finding is
present in the obstructed nasal cavity, hence the associated with holoprosencephaly.
importance of suctioning just prior to starting the scan.
Further reading
Ginat DT, Robson CD. Diagnostic imaging
features of congenital nose and nasal
cavity lesions. Clin Neuroradiol 2015;
25(1): 3–11.
314
Case 13.3
History
14-year-old male presents with epistaxis.
Findings
Figure 13.3.1 Axial CECT demonstrates an avidly enhancing mass centered
in and widening the sphenopalatine foramen (arrows).
Figures 13.3.2, 13.3.3 Axial T2 (Fig. 13.3.2) and axial post-contrast T1 (Fig.
13.3.3) demonstrate anterior deviation of the posterior wall of the left maxillary
sinus (arrow). The lesion extends laterally through the pterygopalatine fossa
into the infratemporal fossa (curved arrow). There is prominent medial
extension to the nasal cavity with deviation of the nasal septum to the right.
Note the obstructed secretions in the left maxillary sinus (∗ ).
Diagnosis
Juvenile nasopharyngeal angiofibroma (JNA).
r Rhabdomyosarcoma.
r Ethesioneuroblastoma.
r Vascular malformation.
Figure 13.3.3
315
r CT and MRI demonstrate a well-defined mass with

Key points
r Benign fibrovascular tumor found nearly exclusively in avid contrast enhancement. Angiography during
preoperative embolization typically demonstrates
young males.
r Clinical presentation is with epistaxis and unilateral nasal vascular supply to the lesion through the ascending
pharyngeal and internal maxillary arteries from the
obstruction.
r Originates at the sphenopalatine foramen, and usually external carotid. Occasionally internal carotid supply is
also demonstrated.
extends throughout the numerous surrounding structures r Total surgical resection following preoperative
including the pterygopalatine fossa, infratemporal fossa,
embolization is the standard of treatment. Radiation
nasal cavity, paranasal sinuses, nasopharynx, cavernous
therapy is reserved for cases without gross total
sinus, and middle cranial fossa.
r Classically causes anterior deviation of the posterior wall of resection.
the adjacent maxillary sinus.
Further reading
Robson CD. Cysts and tumors of the oral
cavity, oropharynx, and nasopharynx in
children. Neuroimaging Clin N Am 2003;
13(3): 427–442, ix.
316
Case 13.4
317
b Multifocal multisystem LCH (Letterer–Siwe disease):

History Age ⬍ 2.
5-year-old boy with diabetes insipidus and left-sided
Presents with a rapidly progressive systemic disease
hearing loss.
(often fatal).
r Pathology: Langerhans cells, Birbeck granules, S100 and
Findings CD1a positive.
r Imaging:
Figure 13.4.1 Sagittal pre-contrast T1 demonstrates an isointense lesion of
the hypothalamus (white arrow). There is also absence of the T1 bright spot of b Osseous lesions of the skull, skull base, and temporal
neurohypophysis. bone:
Figure 13.4.2 Sagittal post-contrast T1 demonstrates homogeneous
Skull: well-defined lytic lesion with “beveled edges.”
enhancement of the hypothalamic lesion (white arrow). “Scooped out” appearance, “floating teeth”
appearance when involving the mandible/maxilla.
Figure 13.4.3 Coronal T2-weighted imaging demonstrates that the Skull base and temporal bone: geographic
hypothalamic lesion is relatively T2 isointense (white arrow) with surrounding
edema. destructive lesion with permeative infiltration.
Can involve the ossicles and otic capsule, resulting
Figure 13.4.4 Axial post-contrast T1 demonstrates another homogeneously in hearing loss.
enhancing lesion of the left temporal bone (white arrow). b Soft-tissue mass:
Often involves the basisphenoid.
Can involve the orbit, resulting in proptosis.
Diagnosis
May have broad dural involvement.

Langerhans cell histiocytosis (LCH). b Intracranial abnormalities:
Most common manifestation: thick enhancing
infundibulum.
Differential diagnosis – Absent posterior pituitary bright spot on
r Hypothalamic–pituitary axis lesion: lymphocytic T1-weighted imaging.
hypophysitis, primary pituitary tumor, germ cell tumor, – Associated hypothalamic lesion.
glioma, meningioma, lymphoma, metastasis, sarcoidosis. – Classic clinical syndrome: diabetes insipidus.
r Temporal bone lesion: facial nerve schwannoma, Cerebellar white matter demyelination
meningioma, sarcoid, fibrous dysplasia, metastasis, Paget’s (autoimmune-mediated).
disease. Rare: choroid plexus nodules, leptomeningeal
nodules, basal ganglia lesions.
b MRI: defines extent of dural and intracranial
Key points involvement.
r Idiopathic disease characterized by proliferation of b PET: variable uptake.
Langerhans histiocytes. r Clinical presentation:
b aka eosinophilic granuloma, histiocytosis X, b Patients can present with proptosis, ophthalmoplegia,
Hand–Schuller–Christian syndrome, Letterer–Siwe hearing loss, vertigo, postauricular swelling, and scalp
disease. mass.
r Typically seen in boys. b Associated with hypothalamic–pituitary axis
r Classification: abnormalities, classically diabetes insipidus.
b Unifocal LCH (eosinophilic granuloma): r Treatment:
Age 5–20. b Depends on symptoms and extent of disease.
Presents with bone lesions. b Treatment options include observation, chemotherapy,
b Multifocal unisystem LCH (Hand–Schuller–Christian
radiation therapy, and/or surgical resection.
disease): b Surgery is indicated for disease limited to the temporal
Age 1–4. bone.
Presents with bone lesions, skin lesions, pituitary b Vasopressin is prescribed for patients with diabetes
lesions, and fever. insipidus.
318
Further reading
D’Ambrosio N, Soohoo S, Warshall C, Kilborn TN, Teh J, Goodman TR. Paediatric Prayer D, Grois N, Prosch H, Gadner H,
Johnson A, Karimi S. Craniofacial and manifestations of Langerhans cell Barkovich AJ. MR imaging presentation
intracranial manifestations of Langerhans histiocytosis: a review of the clinical and of intracranial disease associated with
cell histiocytosis: report of findings in radiological findings. Clin Radiol 2003; Langerhans cell histiocytosis. AJNR Am J
100 patients. AJR Am J Roentgenol 2008; 58(4): 269–278. Neuroradiol 2004; 25(5): 880–891.
191(2): 589–597.
319
Case 13.5
320
History Key points

Patient 1: enlarging neck mass in a 10-month-old male. r Vascular anomalies are categorized as either tumors or
Patient 2: 5-year-old presents with a facial tumor diagnosed malformations.
r Vascular tumors are true neoplasms, which may not be
at an outside institution.
present at birth and demonstrate high endothelial cell
turnover. Examples include infantile and congenital
Findings hemangiomas, tufted angioma, and kaposiform
hemangioendothelioma.
Patient 1 r Vascular malformations are present at birth and
Figure 13.5.1 Coronal T2 with fat saturation demonstrates a large cystic demonstrate normal cell turnover into adulthood. These
lesion with internal septations centered in the submandibular space. lesions are classified as venous, lymphatic, arterial,
Figure 13.5.2 Coronal T1 post-contrast with fat saturation shows that only
capillary, and mixed malformations.
the thin wall and internal septations demonstrate enhancement (arrow). No r Venous malformations are low-flow lesions and are the
solid internal components are demonstrated. most common form of the vascular malformations. A
pathognomonic feature is the presence of phleboliths,
which are more frequently seen with venous malformations
Patient 2 as compared to deep infantile hemangiomas.
Figure 13.5.3 Coronal T1 without contrast shows a large left-sided transfacial r MRI in venous malformations demonstrates a trans-spatial
soft-tissue mass, which is isointense to the adjacent musculature.
lesion with is T1 isointense and T2 hyperintense to
Figure 13.5.4 Coronal T1 post-contrast with fat saturation shows avid adjacent muscle. Phleboliths, when present, image as focal
enhancement throughout the solid lesion. areas of signal void. The lesions typically demonstrate
heterogeneous delayed enhancement after contrast
administration.
Diagnosis r Lymphatic malformations are low-flow lesions, which can
Patient 1: macrocystic lymphatic malformation. be microcystic, macrocystic, or a combination of the two.
These lesions are also known as cystic hygromas and
Patient 2: venous malformation. lymphangiomas.
r Microcystic lesions can image as a solid tumor, leading to
misdiagnosis.
Differential diagnosis r Macrocystic lesions appear as a trans-spatial septated mass.
r Rhabdomyosarcoma. Post-contrast imaging demonstrates enhancement of the
r Hemangioma. wall and internal septa. Commonly there are fluid–fluid
r Dermoid/epidermoid. levels related to internal blood products.
Further reading
Baer AH, Parmar HA, DiPietro MA, Kasten
SJ, Mukherji SK. Hemangiomas and
vascular malformations of the head
and neck: a simplified approach.
641–658, viii.
321
Section 3 Spine
Chapter
Spinal vascular diseases
14 Carlos Leiva-Salinas, Yang Tang, Sugoto Mukherjee, and Max Wintermark
Case 14.1
322
Chapter 14: Spinal vascular diseases
History Key points

65-year-old female presents with sudden bilateral lower r Spinal cord infarction is a rare cause of acute myelopathy.
extremity weakness. The lower frequency of medullary infarcts as opposed to
cerebral ischemia is probably due to the good collateral
medullary arterial supply.
Findings r Acute onset of weakness and loss of sensation in a subject
Figure 14.1.1 Sagittal T2-weighted image shows hyperintensity involving older than 50 years is the most common clinical
the medullary conus (arrows). presentation. Spinal cord ischemia can be encountered in
patients after aortic surgery. In the majority of spontaneous
Figure 14.1.2 Axial T2-weighted image shows central cord hyperintensity
(block arrow), with the classic butterfly appearance, due to the involvement of cases, no clear cause is evident.
the gray matter of the anterior horns. r Ischemia usually involves the thoracic cord, since it is a
border zone between the artery of Adamkiewicz and the
Figure 14.1.3 Axial DWI image reveals restricted diffusion (curved arrow).
arteries supplying the cervical spine. The cervical cord at
Figure 14.1.4 Axial ADC map shows corresponding low ADC values (curved C2–C3 is also particularly vulnerable.
arrow). r The MRI hallmark of spinal cord infarction is the presence
of T2 hyperintensity involving the central cord, due to the
involvement of the central gray matter. T2-weighted
Diagnosis images are usually normal in the first 3 hours after
Acute spinal cord infarct. symptom onset. DWI may be useful, although technically
challenging due to magnetic susceptibility and CSF flow
artifacts, and respiratory motion.
Differential diagnosis r Prognosis is poor, with 20% in-hospital mortality, although
r Demyelinating lesion. 50% of patients may be able to ambulate independently
r Intramedullary neoplasm. and preserve normal urinary function.
Further reading
Weidauer S, Nichtweiss M, Hattingen E, Weidauer S, Nichtweiss M, Lanfermann H,
Berkefeld J. Spinal cord ischemia: Zanella FE. Spinal cord infarction: MR
aetiology, clinical syndromes and imaging and clinical features in 16 cases.
imaging features. Neuroradiology 2015; Neuroradiology 2002; 44(10): 851–857.
57(3): 241–257.
323
Section 3: Spine
Case 14.2
324
History Key points

54 year-old female presents with progressive bilateral lower r 80% of spinal arteriovenous malformations are
extremity weakness. extramedullary.
r In type IV spinal AVMs or perimedullary AV fistulas, there
Findings is a direct communication from the anterior or posterior
spinal artery to a draining vein. In type I spinal AVMs or
Figure 14.2.1 Sagittal T2 image shows multiple intradural flow voids dural radiculomeningeal arteriovenous fistulas, the
surrounding the distal spinal cord and medullary conus. No syrinx or edema is
evident in the conus (∗ ). abnormal communication occurs at a nerve root sleeve.
r Both types of spinal AVMs present as abnormally enlarged
Figure 14.2.2 Parasagittal T2 image better delineates the serpinginous, perimedullary veins, and they can be indistinguishable by
dilated intradural veins (white arrows).
MRI/MRA. Digital subtraction angiography is the
Figure 14.2.3 Maximum-intensity projection image from a time-resolved modality of choice to distinguish between these lesions and
MRA in arterial phase again demonstrates multiple abnormal tortuous vessels for treatment planning.
along the cord (black arrows) extending beyond the cauda equina, as well as a r Increased pressure on the perimedullary veins may be
prominent radicular vein (curved arrow) at the level of T12.
transmitted to intrinsic cord veins, causing cord edema
Figure 14.2.4 Sagittal post-contrast T1 image again shows the prominent and even ischemia.
perimedullary veins (thick arrows). r Perimedullary AV fistulas usually occur in middle-aged
patients, who typically present with progressive
Diagnosis myelopathy or cauda equina syndrome, due to venous
hypertension/cord edema. Sudden thunderclap back pain
Perimedullary arteriovenous fistula, type IV spinal
due to spinal subarachnoid hemorrhage can also occur.
arteriovenous malformation. r Surgical resection or catheter embolization is the treatment
of choice, depending on the location, the size of the feeding
Differential diagnosis vessel, and the flow state of the fistula.
r Dural arteriovenous fistula, type I spinal arteriovenous
malformation.
r Normal CSF flow voids.
r Thickened tortuous nerve roots.
Further reading
Krings T, Geibprasert S. Spinal dural Minami S, Sagoh T, Nishimura K, Yamashita
arteriovenous fistulas. AJNR Am J K, Fujisawa I, Noma S, et al. Spinal
Neuroradiol 2009; 30(4): 639–648. arteriovenous malformation: MR
imaging. Radiology 1988; 169(1):
109–115.
325
Section 3: Spine
Case 14.3
326
History Key points

49-year-old female presents with sudden left-arm weakness. r Spinal cord cavernous malformations (cavernomas) are
rare, representing approximately 3–5% of intramedullary
lesions in adult patients.
Findings r Approximately one-half of intramedullary cavernomas are
Figure 14.3.1 Sagittal T2-weighted image shows a T2 hyperintense located in the thoracic cord, followed by the cervical cord
well-defined lesion surrounded by a ring of hemosiderin (arrow) within the (40%) and the medullary conus (10%).
central and left aspect of the cervical spinal cord. r Acute myelopathy in a young adult female patient is the
Figure 14.3.2 Sagittal T1-weighted image shows T1 shortening involving the most common clinical presentation. An episode of acute
central aspect of the lesion. hemorrhage is probably the mechanism underlying such
clinical events. Progressive symptoms or multiple episodes
Figure 14.3.3 Axial gradient-recalled echo (GRE) image reveals significant
magnetic susceptibility or “blooming” involving the aforementioned lesion (∗ ).
of neurological deterioration may be due to repeated
microhemorrhages and gliosis.
Figure 14.3.4 Axial T2-weighted image suggests there is a thin rim of normal r The annual hemorrhage rate for spinal cavernomas is 2.8%,
cord between the lesion and the surface of the cord (curved arrow). similar to cerebellar or cerebral malformations (3.1%).
r The MRI hallmark of spinal cavernoma is a heterogeneous,
slightly expansile, well-defined, mixed hyperintense lesion
Diagnosis surrounded by a rim of hemosiderin. Subtle contrast
Intramedullary cavernous malformation. enhancement can be seen, sometimes difficult to evaluate
because of intrinsic T1 shortening.
r Surgical resection is recommended for symptomatic
Differential diagnosis cavernomas. This may be difficult or impossible if the
r Intramedullary neoplasm. malformation involves the central cord and there is a rim
r Intramedullary AVM. of normal cord between the lesion and the cord surface.
Further reading
Labauge P, Bouly S, Parker F, Gallas S, Tong X, Deng X, Li H, Fu Z, Xu Y. Clinical
Emery E, Loiseau H, et al. Outcome in 53 presentation and surgical outcome of
patients with spinal cord cavernomas. intramedullary spinal cord cavernous
Surg Neurol 2008; 70(2): 176–181. malformations. J Neurosurg Spine 2012;
16(3): 308–314.
327
Section 3 Spine
Chapter
Spinal trauma
15 David T. Powell, Max Wintermark, Sugoto Mukherjee, and Yang Tang
Case 15.1
328
Chapter 15: Spinal trauma
History Key points

Trauma. r AOD is secondary to osteoligamentous injuries at the
craniocervical junction, usually after high-speed motor
vehicle accidents.
Findings r It typically results from forceful hyperflexion or
hyperextension with rotational shear force, commonly with
Figure 15.1.1 Sagittal CT shows the occipital condyle (block arrow) displaced
anteriorly and superiorly relative to the C1 lateral mass (arrow), consistent with coexisting brainstem, cranial nerve, and vascular injuries.
anterior atlanto-occipital dislocation. Concomitant fractures of C1 and occipital condyles are
also common.
Figure 15.1.2 Coronal CT demonstrates asymmetric widening of bilateral r Prompt and accurate radiologic diagnosis is crucial, given
occiput–C1 articulations (arrows), right greater than left.
the morbidity and mortality associated with these injuries.
Figure 15.1.3 Sagittal STIR MRI demonstrates tearing of apical ligament and r Radiographs and CT demonstrate asymmetric incongruity
anterior atlanto-occiptal membrane (black arrow), as well as partial disruption
of tectorial membrane (white arrow). Note large amount of CSF leak (∗ ) in the
of the occiput–C1 articulation, including:
prevertebral soft tissue. b Increased distance between occipital condyles and C1
lateral masses ⬎ 4 mm on open-mouth view or CT.
Figure 15.1.4 Coronal STIR MRI shows fluid within the bilateral occiput–C1
facet joints (arrows).
b Widening of the basion–dens interval ⬎ 12 mm.
b Widening of the basion–posterior axial line interval
(normal ⬍ 12 mm posterior to basion and ⬍ 4 mm
Diagnosis anterior to basion).
Atlanto-occipital dislocation (AOD). r MRI with STIR sequence best demonstrates injuries of the
stabilizing ligaments, including disruption of the tectorial
membrane, atlanto-occipital membrane, alar and apical
Differential diagnosis ligaments, etc., as well as fluid signal within the occiput–C1
r Nontraumatic atlanto-occipital instability, most frequently joints.
secondary to Down syndrome or rheumatoid arthritis. r Treatment: spine immobilization and occiput-to-C2 fusion.
Further reading
Deliganis AV, Baxter AB, Hanson JA, Fisher
DJ, Cohen WA, Wilson AJ, et al.
Radiologic spectrum of craniocervical
distraction injuries. Radiographics 2000;
20(Spec No.): S237–S250.
329
Section 3: Spine
Case 15.2
330
Diagnosis
Occipital condylar fracture.
r Accessory ossicles.
Key points
r Occipital condyle fractures are typically secondary to
forceful trauma, especially in high-speed motor vehicle
accidents.
r Neurologic impairment is related to concomitant
intracranial or cervical cord injuries.
r Imaging:
b Radiography is limited in detection of occipital condyle
fractures.
b CT is the modality of choice and can also demonstrate
associated injuries including skull base fractures,
atlanto-occipital and C1–C2 misalignment and other
C-spine fractures.
b Anderson–Montesano classification of occipital
condyle fractures:
Type I (least common): impaction fracture due to
axial loading. Ipsilateral alar ligament may be
History injured, but stability is usually maintained by intact
Three patients after motor vehicle accidents. tectorial membrane and contralateral alar
ligament.
Findings Type II: fracture of skull base extending into
occipital condyle and often foramen magnum.
Patient 1 Intact tectorial membrane and alar ligaments
Figure 15.2.1 Axial CT of the cervical spine shows a nondisplaced fracture of maintain stability.
right occipital condyle (arrow), consistent with type I Anderson–Montesano
fracture.
Type III (most common): avulsion fracture of
inferomedial occipital condyle, often with medial
Figure 15.2.2 Coronal CT of the cervical spine reveals extension of the displacement of fragment. Alar ligament and
fracture to the right hypoglossal canal (arrow).
tectorial membrane injuries may result in
instability.
Patient 2 b Newer and combined classification schemes
Figure 15.2.3 Axial CT of the cervical spine demonstrates a nondisplaced incorporating the degree of displacement have also
fracture of right occipital bone (white arrow) extending into the occipital been described (Tuli and Hanson classifications).
condyle (black arrow), consistent with type II Anderson–Montesano fracture. b MRI is crucial in evaluating the integrity of the tectorial
membrane (determinant of stability at the
Patient 3 craniocervical junction) and alar ligaments, and in
Figures 15.2.4, 15.2.5 Axial (Fig. 15.2.4) and coronal (Fig. 15.2.5) CT of the detecting other soft-tissue injuries.
cervical spine demonstrate avulsion fracture of the inferomedial right occipital r Treatment: collar or halo immobilization, or
condyle with medial displacement of the fragment, representative of type III
Anderson–Montesano fracture. atlanto-occipital fusion, depending on degree of stability.
Further reading
Hanson JA, Deliganis AV, Baxter AB, Cohen
WA, Linnau KF, Wilson AJ, et al.
Radiologic and clinical spectrum of
occipital condyle fractures: retrospective
review of 107 consecutive fractures in 95
patients. AJR Am J Roentgenol 2002;
178(5): 1261–1268.
331
Section 3: Spine
Case 15.3
History
Trauma.
Findings
Figure 15.3.1 Open-mouth radiograph shows widening of the C1 arch and
offset of C1 lateral margin relative to C2 (arrows).
Figure 15.3.2 Axial CT of the cervical spine demonstrates displaced fracture

of both anterior (white arrow) and posterior (black arrow) C1 arches. Note an
avulsed fracture fragment of left inner C1 pillar (block arrow), which is indicative
of injury to the transverse ligament.
Figure 15.3.3 Coronal CT of the cervical spine shows offset of C1–C2 lateral
margins (arrows), avulsed fragment of left inner C1 pillar (block arrow), and
misalignment between left C1 lateral mass and occipital condyle (curved
arrow).
Diagnosis
Figure 15.3.3 C1 Jefferson fracture.
332
Differential diagnosis open-mouth odontoid views and coronal CT. A

r Congenital fusion anomalies of C1 (should be well variable number of fracture planes extend through the
anterior and posterior arches of C1.
corticated). b Signs of instability and transverse ligament injury
r Artifact from C1–C2 rotation.
r Pseudospread of C1 in young children. include:
Combined transverse separation of C1 lateral
masses greater than 7 mm.
Key points Atlantodental interval (ADI) greater than 3 mm in
r Jefferson fractures are burst fractures of C1 rings, adults and 5 mm in children.
secondary to traumatic axial loading of the occipital Two anterior ring fractures.
condyles on the C1 lateral masses. Avulsion of the C1 tubercle at the insertion site of
r Neurologic impairment is uncommon, although transverse ligament.
retropulsion may result in spinal cord injury. Associated b MRI is the modality of choice in evaluating the
vascular injury to vertebral arteries and PICA can occur. integrity of the transverse ligament and other
r Jefferson fracture is classically described as a four-part soft-tissue or cord injury.
fracture of the anterior and posterior arches of C1, r Concomitant injuries may include C2 or lower cervical
although different fracture patterns occur with a variable fractures, vertebral artery injury, and spinal cord injury.
number of fragments. r Treatment: collar or halo immobilization or internal
r Imaging:
fixation, depending on degree of stability.
b Radiography or CT demonstrates lateral displacement
of the lateral masses of C1 relative to C2 on
Further reading
Chen YF, Liu HM. Imaging of Lustrin ES, Karakas SP, Ortiz AO,
craniovertebral junction. Neuroimaging Cinnamon J, Castillo M, Vaheesan K,
Clin N Am 2009; 19(3): 483–510. et al. Pediatric cervical spine: normal
anatomy, variants, and trauma.
Radiographics 2003; 23(3): 539–560.
333
Section 3: Spine
Case 15.4
334
History Key points

Two trauma patients. r Odontoid C2 fractures are primarily the result of trauma,
accounting for 10–20% of all cervical spine fractures.
r Occur in bimodal age population: young, physically active
Findings patients and elderly patients with various comorbidities.
r Associated spinal cord and neurologic injury is uncommon
Patient 1 (⬍ 25%) in patients surviving an isolated odontoid
Figure 15.4.1 Open-mouth radiograph of the cervical spine shows an fracture.
irregular fracture through the base of odontoid (arrows). r Radiographs and CT demonstrate fracture of the odontoid
Figure 15.4.2 Coronal CT of the cervical spine confirms the fracture through and/or body of C2, according to the commonly used
the base of odontoid (arrow), consistent with type II odontoid fracture. The Anderson–D’Alonzo classification:
odontoid is not displaced.
b Type I (least common): occur at the tip of the odontoid,
above the transverse ligament. Considered stable as an
Patient 2 isolated injury. Associated with alar ligament avulsion
Figure 15.4.3 Coronal CT of the cervical spine demonstrates a fracture and other craniocervical injuries.
through the base of odontoid (white arrow) extending to the body and right b Type II (most common): occur at the base of the
lateral mass (curved arrow). This is consistent with a type III odontoid fracture.
odontoid. High rates of nonunion due to
Figure 15.4.4 Sagittal CT of the cervical spine shows anterior displacement biomechanical stress and limited cancellous bony
of odontoid relative to C2 vertebral body (arrow). Note the marked prevertebral architecture. Can be unstable.
soft-tissue swelling (∗ ). b Type III: occur below the odontoid–C2 junction and
extend into the body of C2. High rates of nonunion.
Considered a stable fracture.
Diagnosis r Modified classification schemes have been described
Odontoid C2 fracture. depending on fracture orientation and treatment
outcomes.
r MRI is useful in assessing concomitant injuries,
Differential diagnosis particularly ligamentous disruption.
r Congenital variants (os odontoideum and ossiculum r Treatment: immobilization and/or fixation depending on
terminale). fracture extent and concomitant injuries.
Further reading
Anderson LD, D’Alonzo RT. Fractures of the
odontoid process of the axis. 1974. J Bone
Joint Surg Am 2004; 86-A(9): 2081.
335
Section 3: Spine
Case 15.5
336
Diagnosis
Hangman fracture.
r Pseudosubluxation in children.
r Congenital spondylolysis of C2 (rare).
Key points
r Second most common C2 fracture after odontoid fractures.
r Historically seen with judicial hanging, it is now usually
secondary to forceful trauma after high-speed motor
vehicle accidents.
r It typically results from forceful hyperextension and
distraction causing bilateral pars interarticularis fractures
of C2, also termed traumatic spondylolisthesis.
r Neurological complication is rare, since the fracture
widens the spinal canal.
r Imaging:
b Radiography and CT show fracture lucencies
through the pars interarticularis of C2, which are
sometimes associated with anterior subluxation of
skull, C1, and C2 relative to C3 but preserved
History alignment of the posterior elements and the
Two patients present after motor vehicle crash. spinolaminar line.
b Effendi classification:
Type I: nondisplaced or minimally displaced
Findings
fractures without angulation and with less than

Patient 1 3 mm anterior subluxation of C2. Stable.
Figure 15.5.1 Sagittal CT shows slightly distracted fracture of C2 left pars Type II: more angulated or displaced fracture with
interarticularis (curved arrow). No C2 subluxation. ⬎ 3 mm anterior subluxation of C2, often with
posterior longitudinal ligament and C2–C3 disc
Figure 15.5.2 Axial CT shows minimally displaced fractures of bilateral C2
pars interarticularis (arrows). injury. Unstable.
Type III: displaced fracture with unilateral or
bilateral facet subluxation or dislocation.
Patient 2 Unstable.
Figure 15.5.3 Lateral radiograph reveals a slightly displaced fracture of C2 b MRI with STIR sequence best demonstrates injuries to
pars interarticularis (black arrow). There is subtle disruption of the C2
spinolaminar line (curved black arrow) relative to C1 and C3 (white arrows), as
the anterior and posterior longitudinal ligaments, other
well as C2–C3 anterolisthesis (white block arrow). cervical stabilizing ligaments, cervical spinal cord, and
prevertebral soft tissue.
Figure 15.5.4 Parasagittal STIR MRI image demonstrates a distracted C2 pars b Consider CT angiogram if fracture extends into the
interarticularis fracture (black curved arrow) with adjacent edema.
transverse foramen, to exclude vertebral artery
Figure 15.5.5 Sagittal STIR MRI demonstrates edema within the C2–C3 disc, injury.
grade I anterolisthesis, disruption of posterior longitudinal ligament (black r Treatment: collar or halo immobilization, or surgical
arrow) and interspinous ligament (black curved arrow), and prevertebral
soft-tissue edema (white block arrow). fusion, depending on severity.
Further reading
Levine AM, Edwards CC. The management Mirvis SE, Young JW, Lim C, Greenberg J.
of traumatic spondylolisthesis of the axis. Hangman’s fracture: radiologic
J Bone Joint Surg Am 1985; 67(2): assessment in 27 cases. Radiology 1987;
217–226. 163(3): 713–717.
337
Section 3: Spine
Case 15.6
338
339
Section 3: Spine
History Key points

Trauma. r Hyperflexion sprain and fracture/subluxation injuries of
the cervical spine are most commonly secondary to
forceful trauma such as a motor vehicle accident, fall, or
Findings diving injury.
r The injury initially begins with the posterior ligament
Patient 1 complex and extends anteriorly. The posterior ligament
Figure 15.6.1 Lateral radiograph of the cervical spine shows reversal of complex includes: supraspinous ligament, interspinous
lordosis at C5–C6 with mild narrowing of anterior disc space (arrow). There is
distraction of the facet joint (curved arrow) and fanning of the spinous ligaments, capsular ligaments, and ligamentum flavum.
processes (double-head arrow). r The spectrum of hyperflexion injuries includes
hyperflexion ligamentous sprain, anterior wedge fractures
Figure 15.6.2 Parasagittal CT of the cervical spine shows subluxation of the with or without disruption of the posterior ligament
C5–C6 facet joint (curved arrow).
complex, unilateral/bilateral interfacetal dislocation, and
Figure 15.6.3 Sagittal STIR MRI demonstrates disruption of the ligamentum flexion tear-drop fracture.
flavum at C5–C6 (curved arrow). r The stability of the spine and associated cord injury
depends on the extent of osteoligamentous disruption and
Patient 2 malalignment. Unstable hyperflexion injuries include:
Figure 15.6.4 Sagittal CT of the cervical spine demonstrates grade II C5–C6
flexion tear-drop fracture, bilateral interfacetal dislocation,
anterolisthesis (arrow), narrowing of disc space, and widened interspinous and anterior wedge fracture with posterior ligament
distance (double-head arrow). complex disruption.
r Prompt and accurate diagnosis of hyperflexion injury is
Figure 15.6.5 Parasagittal CT of the cervical spine shows “jumped” C5–C6
facet, with the inferior C5 facet (white arrow) anterior to the superior C6 facet critical to reduce morbidity, given the possibility of
(black arrow). significant spinal cord injury.
r Imaging:
Figure 15.6.6 Axial CT of the cervical spine shows bilateral C5–C6 interfacetal
dislocation, with the inferior C5 facets (white arrows) anterior to the superior C6 b Radiographs and CT demonstrate anterior subluxation,
facets (black arrows). This is called the “inverted hamburger sign.” widening or fanning of the spinous processes, widening
Figure 15.6.7 Sagittal STIR MRI reveals cord compression and edema (∗ ) due
of the posterior disc space, dislocation or uncovering of
to grade II C5–C6 anterolisthesis. There is disruption of all stabilizing ligaments the facets, anterior wedge fracture usually involving the
at this level, as well as prevertebral and posterior paraspinal soft-tissue edema. superior endplate, and flexion tear-drop fracture of the
vertebral body.
b Close evaluation of facet alignment and widening of
Patient 3
the posterior disc space can help differentiate actual
Figure 15.6.8 Sagittal CT of the cervical spine demonstrates grade II C5–C6
retrolisthesis, wedge-shaped compression deformity of C5, and anterior anterior subluxation from straightening of cervical
“tear-drop” fracture fragment. Also note prevertebral edema. lordosis, often due to muscle spasm.
b MRI demonstrates associated soft-tissue and
ligamentous injury, often indicated by edematous
Diagnosis hyperintense T2 signal. It can also assess for spinal cord
Hyperflexion injury of cervical spine. edema and hemorrhage.
b Evaluate for concomitant vascular injury, particularly
involving the vertebral artery, with CTA or MRA.
Differential diagnosis r Treatment: reduction, immobilization, fixation, depending
r None. on the severity of the injury.
Further reading
Dreizin D, Letzing M, Sliker CW, Chokshi
FH, Bodanapally U, Mirvis SE, et al.
Multidetector CT of blunt cervical spine
trauma in adults. Radiographics 2014;
34(7): 1842–1865.
340
Case 15.7
r Neurologic impairment and spinal cord injury are

History
common, sometimes presenting with central cord
Trauma.
syndrome (disproportionate motor deficits in upper
extremities compared to lower extremities).
Findings r Ligamentous injury results in significant instability,
Figure 15.7.1 Sagittal CT of the cervical spine shows mild widening of the particularly during extension.
C3–C4 disc space anteriorly with a small “tear-drop” fracture fragment at the C3 r Imaging:
anterior/inferior corner from avulsion injury of anterior longitudinal ligament.
There is minimal C3–C4 retrolisthesis. b Widening of the anterior disc space with prevertebral
soft-tissue swelling.
Figure 15.7.2 Sagittal STIR MRI demonstrates tearing of anterior (white b Anterior longitudinal ligament injury results in
arrow) and posterior (curved arrow) longitudinal ligaments. There is
prevertebral edema (block arrow). Cord edema (∗ ) is also noted. avulsion fracture of the anterior-inferior corner of the
vertebral body (“extension tear-drop fracture”).
Extension tear-drop fractures are triangular or oriented
Diagnosis more horizontally than “flexion tear-drop fractures,”
Hyperextension cervical spine injury. which are usually larger and oriented more vertically.
b Posterior subluxation of the vertebral body may
Differential diagnosis indicate posterior longitudinal ligament injury.
b Facet and posterior element fractures may be also
r Hyperflexion injury.
present.
b MRI with STIR sequence best demonstrates
Key points ligamentous and cervical cord injury as well as
r Hyperextension injuries of the cervical spine are secondary prevertebral edema/hematoma. Integrity of the disc,
to direct impact to the frontal skull or whiplash from a and of the anterior longitudinal and posterior
motor vehicle accident. longitudinal ligaments, should be assessed.
341
Section 3: Spine
b Consider CT or MR angiography to evaluate for b Less severe trauma results in ligamentous strain, such
vascular injury. as with a whiplash injury.
b Hyperextension injury is more common in the mid to r Treatment: immobilization and possibly surgical
lower cervical spine. decompression and steroids if spinal cord injury.
Further reading
Rao SK, Wasyliw C, Nunez DB, Jr. Spectrum
of imaging findings in hyperextension
injuries of the neck. Radiographics 2005;
25(5): 1239–1254.
342
Case 15.8
r More common in younger patients with severe trauma or

History elderly patients with underlying canal stenosis from spinal
71-year-old man presents with bilateral weakness of upper
degenerative changes. Occurs in males more frequently
and lower extremities after ground-level fall. Cervical spine
than females. C5–C6 is the commonest level.
CT shows no acute fractures. r Upper extremities are affected more than lower extremities
because of the medial location of corticospinal tracts
Findings within the cord for upper extremities. Can be associated
Figures 15.8.1, 15.8.2 Sagittal (Fig. 15.8.1) and axial (Fig. 15.8.2) T2 MRI with bladder dysfunction and sensory loss. Injury graded
demonstrates hyperintensity in the central cord at C3 and C4 level (Fig. 15.8.1, clinically by American Spinal Injury Association (ASIA)
arrows). There is multilevel cervical spondylosis with disc-osteophyte
formation, which is most severe at C3–C4 level causing cord compression. classification.
r Imaging:
b MRI demonstrates abnormal cord signal, cord
Diagnosis expansion, traumatic osseous and ligamentous injury
Central cord syndrome.
to the spine (often hyperextension injury).
b Cord edema, indicated by hyperintense T2-weighted
Differential diagnosis signal, is associated with incomplete injury and
r Myelomalacia from chronic cord compression. portends a better neurologic outcome.
r Transverse myelitis from demyelinating, inflammatory, or b Cord contusion, indicated by central hypointense
infectious etiology. T2-weighted signal mixed with peripheral hyperintense
r Cord infarction. signal, also has favorable neurologic outcome.
b Cord hemorrhage is demonstrated by hypointense
Key points T2-weighted signal with a thin rim of hyperintense
r Central cord syndrome is the most common incomplete signal and low signal intensity on GRE; more
spinal cord syndrome, most frequently due to commonly seen in complete spinal injury with severe
hyperextension injury. neurologic impairment.
343
Section 3: Spine
b Evaluate for disc herniation, pre-existing degenerative r Treatment: spinal stabilization, steroids, surgical
changes, and congenitally narrow spinal canal, which decompression if necessary.
predispose the patient to spinal cord injury.
Further reading
Miyanji F, Furlan JC, Aarabi B, Arnold PM,
Fehlings MG. Acute cervical traumatic
spinal cord injury: MR imaging findings
correlated with neurologic outcome –
prospective study with 100 consecutive
patients. Radiology 2007; 243(3):
820–827.
344
Case 15.9
345
Section 3: Spine
History Diagnosis
Two patients with trauma. Thoracolumbar fractures.
Patient 1: burst fracture.
Patient 2: Chance fracture.
Findings
Patient 1 Differential diagnosis
Figure 15.9.1 Axial CT of the lumbar spine demonstrates a severely r None.
comminuted burst fracture of T12 vertebral body with multiple retropulsed
bony fragments in the spinal canal.
Figure 15.9.2 Sagittal CT of the lumbar spine demonstrates a perched

Key points
T11–T12 facet joint (curved arrow).
r A Chance fracture is a hyperflexion–distraction injury
which is usually the result of a motor vehicle accident or
Figure 15.9.3 Sagittal STIR MRI of the lumbar spine shows bone-marrow
edema of T12 vertebral body (∗ ). There is discontinuity of the cord at this level fall.
(white arrow), consistent with cord transection. r Chance-type fractures were first recognized after seatbelt
use became more prevalent. The seatbelt and anterior
aspect of the abdomen act as a fulcrum as the torso flexes
during the deceleration associated with a motor vehicle
Patient 2
accident, resulting in distraction of the posterior elements.
Figure 15.9.4 Sagittal CT of the lumbar spine demonstrates widening of r Vast majority occur at the thoracolumbar junction (T12 to
L1–L2 posterior disc space and widening of interspinous distance L2).
(double-head arrow). There is a linear fracture fragment (block arrow) from the
superior endplate of L1. Note multiple intraluminal filling defects in the distal
r Associated with intra-abdominal trauma, most frequently
abdominal aorta (arrows) from acute aortic injury. involving the bowel/mesentery, spleen, liver, and pancreas.
r Despite severe mechanism of trauma, neurologic deficit
Figure 15.9.5 Coronal CT of the lumbar spine shows dislocation of bilateral
L1–L2 facet joints (arrows). and cord injury are relatively uncommon.
r Burst fracture is a subtype of a Chance fracture with
Figure 15.9.6 Sagittal STIR MRI of the lumbar spine reveals bone-marrow fracture planes extending to the posterior cortex of the
edema of L2 and L3 vertebrae (∗ ). At L1–L2 level, the ligamentum flavum and vertebral body with possible retropulsion of fracture
interspinous ligament are torn and replaced by a hematoma (black block
arrows). The conus is herniated posteriorly through the dura/ligament defect fragments. Higher rate of associated cord injury and
(white arrow). There is diffusely increased signal in the distal thoracic cord. neurologic deficits.
346
r Imaging: b Lucency and loss of definition of the pedicles due to

b Transverse-oriented fracture and distraction of the horizontal fracture.
b Evaluate for extension to the posterior cortex of
posterior elements extending anteriorly to involve the
pedicles and portions of the vertebral body. The vertebral body and retropulsion.
b MRI important to assess for associated ligamentous
posterior, middle, and sometimes the anterior column
are involved, resulting in an unstable injury. injury, disc herniation, epidural hematoma, and spinal
b Sagittal CT/MRI and lateral radiography best depict the cord injury. Hemorrhage demonstrated by hypointense
horizontal fracture plane through the posterior T2 signal along the fracture plane, with surrounding
elements. hyperintense T2 signal indicative of edema.
b Empty vertebral body sign: AP radiograph sign b Abdomen/pelvis CT is recommended to assess for
demonstrating radiolucency of the affected vertebral associated intra-abdominal injury.
body due to fracture and fanning of the spinous r Treatment: thoracolumbosacral orthosis (TLSO)
process. brace or posterior fusion depending on degree of
b Naked facet sign: distraction of the superior and instability.
inferior articular facets.
Further reading
Bernstein MP, Mirvis SE, Shanmuganathan
K. Chance-type fractures of the
thoracolumbar spine: imaging analysis in
53 patients. AJR Am J Roentgenol 2006;
187(4): 859–868.
347
Section 3: Spine
Case 15.10
Findings
Patient 1
Figure 15.10.1 Sagittal CT of the cervical spine demonstrates ankylosis of the
cervical spine with syndesmophytes bridging multiple vertebral bodies
(arrows) and facets (curved arrows), consistent with ankylosing spondylitis.
There is a subtle, nondisplaced fracture through the superior endplate of C6
(block arrow).
Figure 15.10.2 AP radiograph of the pelvis demonstrates ankylosis of

bilateral sacroiliac joints with complete loss of joint space (arrows). Also note
the fusion of lower lumbar vertebrae with ossification of annulus fibrosus
(“bamboo spine”).
Patient 2
Figure 15.10.3 Sagittal CT of the cervical spine demonstrates marked
widening of T7–T8 disc space (block arrow) and a fracture through the facet
(curved arrow), consistent with hyperextension injury. There is ankylosis of
thoracic spine at multiple levels with bridging syndesmophytes (white
arrows).
Diagnosis
Figure 15.10.3 (patient 2) Ankylosing spondylitis with fractures.
History r Other seronegative spondyloarthropathies (psoriatic
Patient 1: 55-year-old man presents with neck pain after arthritis, inflammatory bowel disease, reactive arthropathy,
ground-level fall. etc.).
Patient 2: 40-year-old man presents after motor vehicle r Juvenile rheumatoid arthritis (JRA).
crash. r Diffuse idiopathic skeletal hyperostosis (DISH).
348
Key points tearing of the anterior/posterior longitudinal ligaments,

r Ankylosing spondylitis (AS) is a chronic inflammatory cord injury, cervical/thoracic spinous process fractures,
posterior column fracture/ligament injury.
condition of the spine. Approximately 90% are HLA-B27 b Fracture planes may extend transversely through the
positive. More common in males than in females.
r AS results in altered biomechanics of the spine, which vertebral body and disc elements, mimicking a Chance
fracture. Injury may affect all three spinal columns
predisposes patients to spinal trauma. Only minor
resulting in an unstable osseous/ligamentous insult.
traumatic forces are needed to fracture the ankylosed spine
Continued motion at the fracture site can lead to
compared to a normal spine.
r Cervical spine is the most common site of spinal fracture, pseudoarthrosis, often resembling an infectious
spondylitis.
although thoracolumbar injury occurs frequently as well. b Radiographs and CT reveal the fracture lucency,
r Treat a patient with AS presenting with symptoms of new
anterior/posterior longitudinal ligament tear if ossified,
neck or back pain as an unstable spinal fracture until
and malalignment.
proven otherwise. Spinal cord injury is variable depending b MRI better delineates occult fractures (linear
on location and severity of fracture.
r Imaging: hypointense T1 and surrounding edematous
hyperintense T2 signal), avascular necrosis and
b AS is characterized by: symmetric syndesmophyte
associated soft-tissue injury. Evaluate for ligamentous
formation (bamboo spine), apophyseal and injury, which is often indicated by hypointense T1
costovertebral joint ankylosis, ligamentous ossification, signal along the ligament tear with surrounding
reactive sclerosis at the corners of vertebral bodies edema.
(shiny corners), osteopenia, sacroiliitis. r Treatment: immobilization, fixation, posterior
b Spinal injury in patients with AS is often characterized
decompression depending on fracture extent and
by: osseous fracture (which may be occult
concomitant injuries.
radiographically), avascular necrosis, pseudoarthrosis,
Further reading
Wang YF, Teng MM, Chang CY, Wu HT,
Wang ST. Imaging manifestations of
spinal fractures in ankylosing spondylitis.
AJNR Am J Neuroradiol 2005; 26(8):
2067–2076.
349
Section 3: Spine
Case 15.11
350
r Brachial plexus injuries typically result from forceful

History
traction to the arm, most commonly from high-speed
Two patients after motor vehicle crash.
motor vehicle accidents in adults or obstetric injuries in
infants (C5–C6 Erb–Duchenne type, and C8–T1 Klumpke
Findings type).
Patient 1 r Pathologically, it consists of a full spectrum from minor
Figure 15.11.1 Axial T2 MRI demonstrates an extradural CSF collection
stretch injury/neuropraxia to complete nerve root avulsion.
slightly displacing the cord to the left. The right neural foramen is empty with Based on the level of injury, it can be classified into
the absence of nerve root (arrow), in contrast to the normal left foramen. preganglionic (central to the dorsal root ganglia, worse
prognosis) or postganglionic (better prognosis) injuries.
Figure 15.11.2 Coronal STIR MRI demonstrates a hyperintense outpouching
at C6–C7 right neural foramen consistent with a pseudomeningocele due to
r Imaging:
preganglionic C7 root avulsion. b Noncontrast CT is insensitive and only shows
associated fractures and paraspinal hematoma.
Patient 2 b CT myelography would demonstrate deformity or
Figure 15.11.3 Axial T2 MRI shows an extradural CSF collection displacing absence of nerve roots and sleeves, spinal cord
the cord to the right. The left neural foramen is empty without neural element displacement, traumatic pseudomeningocele.
(arrow). T2 hyperintensity of cord reflects edema (block arrow) from acute b MRI or MR neurography is the modality of choice:
nerve root avulsion.
Stretch injury: thickening and T2/STIR
Figure 15.11.4 Coronal STIR MRI reveals diffuse thickening and edema of the hyperintense signal of the brachial plexus
left brachial plexus roots and trunks (arrows). There is discontinuity of left C7 elements.
nerve root distal to the left foramen without a pseudomeningocele (block
arrow). Nerve root avulsions: absence of neural elements at
the neural foramen without or with formation of
pseudomeningocele (CSF intensity thecal sac
Diagnosis outpouching).
Brachial plexus injury. Cord edema at the site of root avulsion.
Edema and enhancement of paraspinal muscles
Differential diagnosis (especially multifidus) is an indirect sign of root
r Nerve root sleeve cyst or nerve sheath tumor can mimic avulsion injury.
pseudomeningocele. r Management of brachial plexus injuries depends on the
r Brachial neuritis can mimic traction injury. location and severity of the insult:
b Preganglionic lesions, indicating nerve root avulsion,
Key points are treated with nerve root transfers to restore function
r The brachial plexus is formed by the ventrial rami of C5 to and muscle innervation.
T1. Anatomically, it can be divided into supraclavicular b Postganglionic lesions are distal to the sensory
(roots, trunks), retroclavicular (divisions), and ganglion and are treated conservatively or with a nerve
infraclavicular (cords and terminal branches) segments. autograft.
Further reading
Aralasmak A, Karaali K, Cevikol C, Uysal H, Yoshikawa T, Hayashi N, Yamamoto S, Tajiri
Senol U. MR imaging findings in brachial Y, Yoshioka N, Masumoto T, et al.
plexopathy with thoracic outlet Brachial plexus injury: clinical
syndrome. AJNR Am J Neuroradiol 2010; manifestations, conventional imaging
31(3): 410–417. findings, and the latest imaging
techniques. Radiographics 2006; 26(Suppl
1): S133–S143.
351
Section 3 Spine
Chapter
Spinal infectious and inflammatory diseases
16 Thomas J. E. Muttikal, Max Wintermark, Sugoto Mukherjee, and Yang Tang
Case 16.1
History
42-year-old woman presents with a few days’ history of
progressive bilateral upper and lower extremity weakness
and altered sensations.
Findings
Figure 16.1.1 Sagittal T2-weighted image (a) shows diffuse T2 hyperintense
signal abnormality and cord swelling extending from cervicomedullary
junction to C7. Sagittal T1-weighted image (b) shows diffuse cord swelling
extending from cervicomedullary junction to C7, with slight heterogeneous
signal.
Figure 16.1.2 Axial T2-weighted image shows signal abnormality diffusely

affecting the cord, with some sparing of the left lateral, anterior, and posterior
aspects of the cord.
Figure 16.1.3 Sagittal post-contrast T1-weighted image shows contrast

enhancement extending from C2 to C4 level.
Diagnosis
Figure 16.1.3
Neuromyelitis optica.
352
Chapter 16: Spinal infectious and inflammatory diseases
b Diffuse cord swelling can be seen on T1- and

r Multiple sclerosis. T2-weighted images.
b Contrast enhancement is typically present in the acute
r Acute disseminated encephalomyelitis (ADEM).
r Spinal cord neoplasm. stage.
b Brain MRI may be normal, may show nonspecific
T2/FLAIR hyperintense white-matter lesions, or may
Key points show characteristic lesions in the region of high
r Optic neuritis. concentration of AQP4. Also see Case 3.4.
r Lhermitte sign on clinical examination. b Shorter segment involvement of the spinal cord with
r The cervical spinal cord lesion may extend toward the characteristic appearance of white-matter lesions in the
brainstem and cause hiccups, intractable nausea, vomiting, brain favors multiple sclerosis.
vertigo, hearing loss, facial weakness, trigeminal neuralgia, b ADEM shows multifocal white-matter lesions with
diplopia, ptosis, nystagmus, and respiratory failure. variable enhancement, and is almost always associated
r Encephalopathy may be present. with brain lesions on MRI.
r NMO-IgG antibody against aquaporin 4 (AQP4). In the b Spinal cord tumors have more insidious clinical
brain, the hypothalamus and regions adjacent to the third presentation. MRI shows cord expansion with diffuse
and fourth ventricles are rich in AQP4. or nodular contrast enhancement, heterogeneous
r Imaging: appearance with cystic or hemorrhagic areas, and
b MRI shows diffuse T2 signal intensity extending three peritumoral edema.
or more vertebral levels. r Treatment: steroids, plasmapheresis.
Further reading
Kitley JL, Leite MI, George JS, Palace JA. The Sahraian MA, Radue EW, Minagar A.
differential diagnosis of longitudinally Neuromyelitis optica: clinical
extensive transverse myelitis. Mult Scler manifestations and neuroimaging
2012; 18(3): 271–285. features. Neurol Clin 2013; 31(1):
139–152.
353
Section 3: Spine
Case 16.2
354
History Key points

35-year-old HIV-positive woman with worsening bilateral r The most common cause of spinal cord disease in AIDS
lower extremity weakness and numbness. patients is HIV myelopathy, which most often occurs in
late stages of AIDS.
r The typical clinical findings are insidious onset of spastic
Findings
paraparesis, loss of vibration and position sense, and
Figures 16.2.1, 16.2.2 Sagittal T2 MRI of cervical (Fig. 16.2.1) and thoracic urinary frequency, urgency, and incontinence.
spine (Fig. 16.2.2) demonstrate a long segment of diffuse T2 hyperintensity
within the cord (arrows).
r More than half of these patients have concomitant AIDS
dementia complex (such as the current case) as well as
Figure 16.2.3 Axial T2 of the thoracic cord shows that the signal abnormality other HIV complications, which may obscure myelopathy
mainly involves the lateral (arrows) and dorsal columns (block arrow). No
associated enhancement on the post-contrast T1 images (not shown). symptoms.
r HIV myelopathy usually starts in the mid to low thoracic
Figure 16.2.4 Axial FLAIR reveals confluent hyperintensity in the cord, with increasing rostral involvement as the disease
periventricular white matter (curved arrows), compatible with HIV
encephalopathy.
progresses.
r HIV myelopathy is a diagnosis of exclusion.
r Pathologically, it is characterized by intramyelin and
Diagnosis periaxonal vacuolation involving the lateral and posterior
HIV-associated vacuolar myelopathy. columns of the cervical and thoracic cord, similar to the
changes in subacute combined degeneration.
r Imaging: although frequently normal, MRI plays an
Differential diagnosis essential role to exclude other extrinsic or intrinsic cord
r Opportunistic infection (toxoplasmosis, CMV,
disease. MRI abnormalities are more frequent in patients
tuberculosis). with advanced myelopathy. The most common finding is
r Subacute combined degeneration (B12 or copper
cord atrophy. The cord signal abnormalities are observed in
deficiency). a minority of these patients and consist of diffuse
r Demyelinating disease.
nonspecific T2 hyperintensity, without or with patchy
r Transverse myelitis.
enhancement on post-contrast T1.
Further reading
Chong J, Di Rocco A, Tagliati M, Danisi F,
Simpson DM, Atlas SW. MR findings in
AIDS-associated myelopathy. AJNR Am J
Neuroradiol 1999; 20(8): 1412–1416.
355
Section 3: Spine
Case 16.3
Findings
Figure 16.3.1 Sagittal T2-weighted image shows diffuse T2 signal
abnormality and cord swelling extending from cervicomedullary junction to
the upper thoracic region (arrows).
Figure 16.3.2 Axial T2-weighted image shows signal abnormality diffusely

affecting the cord.
Figure 16.3.3 Sagittal post-contrast T1-weighted image demonstrates

patchy contrast enhancement (arrow).
Diagnosis
Transverse myelitis.
r Neuromyelitis optica (NMO).
r Multiple sclerosis.
r Acute disseminated encephalomyelitis (ADEM).
r Spinal arteriovenous malformation (AVM).
r Infective myelitis in immunocompromised patients.
Figure 16.3.3 r Spinal cord infarct.
r Spinal cord neoplasm.
History Key points

19-year-old woman presents with a few days’ history of r Bilateral weakness, sensory disturbance, and autonomic
progressive upper and bilateral lower extremity weakness dysfunction typically evolving over hours or days.
and altered sensations. r Could be disease-associated or idiopathic.
356
r Idiopathic transverse myelitis is a diagnosis of exclusion b Spinal vascular malformations show flow voids
after the clinical, imaging, and laboratory analysis do not associated with cord signal abnormality. Spinal cord
reveal specific underlying etiology. infarct presents acutely (minutes) compared to
r Imaging: transverse myelitis, has predominant motor
b MRI shows diffuse T2 signal intensity extending three component, and the distribution of the lesion, with
or more vertebral levels, and sometimes diffuse cord sparing of the dorsal cord, may also help to
swelling on T1- and T2-weighted images. Contrast differentiate.
b Spinal cord tumors have more insidious clinical
enhancement is variable, with no enhancement to
patchy enhancement. Area of enhancement is less than presentation. MRI shows cord expansion with diffuse
T2 signal abnormality. Meningeal enhancement may be or nodular contrast enhancement, heterogeneous
seen occasionally. Fractional anisotropy is decreased in appearance with cystic or hemorrhagic areas, and
the lesion and in the distal normal-appearing cord. peritumoral edema.
b Combination of long segment spinal cord lesion with b Clinical history and meningeal enhancement help to
optic nerve involvement suggests NMO, while shorter differentiate infective myelitis in immunocompromised
segment involvement of the spinal cord with patients.
characteristic appearance of white-matter lesions in the r Treatment: treatment of idiopathic transverse myelitis
brain point to multiple sclerosis. includes high-dose steroids with or without
b ADEM shows multifocal white-matter lesions with plasmapheresis/immunoglobulins. If the underlying cause
variable enhancement, and is almost always associated is found, specific treatment can be initiated in addition to
with brain lesions on MRI. the steroids. Prognosis varies with the underlying cause.
Further reading
Goh C, Phal PM, Desmond PM. Kitley JL, Leite MI, George JS, Palace JA. The
Neuroimaging in acute transverse differential diagnosis of longitudinally
myelitis. Neuroimaging Clin N Am 2011; extensive transverse myelitis. Mult Scler
21(4): 951–973, x. 2012; 18(3): 271–285.
357
Section 3: Spine
Case 16.4
Figure 16.4.2
History
66-year-old woman with a 2-week history of progressive
Figure 16.4.1 bilateral lower extremity weakness and areflexia on exam.
Findings
Figure 16.4.1 Sagittal T2-weighted image shows mild thickening of the
cauda equina nerve roots (arrows).
Figures 16.4.2, 16.4.3 Sagittal (Fig. 16.4.2) and axial (Fig. 16.4.3)
post-contrast T1-weighted images show smooth enhancement of lumbosacral
nerve roots (arrows).
Diagnosis
Guillain–Barré syndrome (GBS).
r Imaging differentials include infections, metastasis,
lymphoma, leukemia, sarcoidosis, prior radiation,
intrathecal chemotherapy or lumbar surgery, subacute
inflammatory demyelinating polyneuropathy (SIDP),
chronic inflammatory demyelinating polyneuropathy
(CIDP), hereditary polyneuropathies.
Key points
r aka acute inflammatory demyelinating polyneuropathy
Figure 16.4.3 (AIDP).
358
r Monophasic disease entity characterized by acute feature for GBS. Enhancement of the pial surface of the
ascending paralysis and areflexia, which typically reaches distal cord and conus can also be seen.
its peak within 3–4 weeks. b Enhancement of cauda equina nerve roots is a
r A variant form of GBS is Miller Fisher syndrome, which nonspecific finding and can be seen in numerous other
involves cranial nerves and is characterized by conditions, including infectious spinal meningitis
ophthalmoplegia, ataxia, and areflexia. (including bacterial, tuberculosis, viral, fungal, Lyme
r Many cases of GBS are preceded by upper respiratory tract disease); neoplastic conditions including
infection or diarrhea. leptomeningeal metastasis, lymphoma and leukemia;
r The typical finding of cerebrospinal fluid (CSF) analysis is inflammatory etiologies such as sarcoidosis; iatrogenic
an elevated CSF protein with a normal white blood cell conditions including prior radiation, intrathecal
count (albuminocytologic dissociation). Clinical chemotherapy, systemic chemotherapy with vincristine,
neurophysiology studies (electromyography and nerve and lumbar surgery; subacute and chronic
conduction studies) show evidence of an acute inflammatory demyelinating polyneuropathies and
polyneuropathy with predominantly demyelinating hereditary polyneuropathies. The thickening of the
features in GBS. cauda equina is more nodular in neoplastic conditions
r Imaging: and sarcoidosis. Subacute and chronic demyelinating
b On MRI, cauda equina nerve roots shows slight polyneuropathies and hereditary polyneuropathies
uniform thickening on T2, and smooth, non-nodular have a different clinical time course.
enhancement on post-contrast T1 images. The r Treatment: plasma exchange, intravenous
enhancement may preferentially affect the ventral immunoglobulins.
versus the dorsal roots, which is a distinguishing MRI
Further reading
Byun WM, Park WK, Park BH, Ahn SH, Yuki N, Hartung HP. Guillain–Barré
Hwang MS, Chang JC. Guillain–Barré syndrome. N Engl J Med 2012; 366(24):
syndrome: MR imaging findings of the 2294–2304.
spine in eight patients. Radiology 1998;
208(1): 137–141.
359
Section 3: Spine
Case 16.5
360
Diagnosis
Neurosarcoidosis of the spine.
r Leptomeningeal tumor spread.
r Lymphoma.
r Infection.
Key points
r Spinal sarcoidosis usually occurs in patients with known
systemic sarcoidosis, and it is very rarely the first
manifestation of the disease.
r Lesions can be seen within the spinal canal
(intramedullary, extramedullary–intradural, and
extradural) or involve the spine as vertebral and disc space
lesions. Intramedullary sarcoidosis occurs in fewer than
1% of sarcoidosis cases. Extramedullary–intradural lesions
are most common, occurring in up to 60% of spinal
sarcoidosis cases. Extradural lesions are exceedingly rare.
About half of the patients with spinal lesions have
concomitant intracranial lesions.
Figure 16.5.5
r Imaging:
b MRI appearance of intramedullary lesions is
nonspecific, with low T1, high T2 signal and patchy
History enhancement after contrast administration. It may
mimic other intramedullary lesions such as tumor,
24-year-old man with progressive weakness and malaise. infection, demyelinating disease (MS or NMO), or
other types of transverse myelitis.
b Leptomeningeal involvement appears as smooth or
Findings nodular leptomeningeal enhancement on the
post-contrast T1. Differential diagnoses for
Figure 16.5.1 Sagittal T2 of cervical spine shows fusiform expansion and T2 leptomeningeal sarcoidosis include infections (TB,
hyperintensity of the cervical cord (short arrows). There is also T2 hyperintense
signal abnormality in the brainstem (long arrow) and cerebellum (curved fungal, bacterial infections, etc.), metastasis, and
arrow). lymphoma.
b Extradural lesions appear as T1 hypointense and T2
Figure 16.5.2 Axial T2 of cervical spine shows intramedullary hyperintensity
mostly within the central gray matter (arrow). hyperintense lesions with marked contrast
enhancement, sometimes with dural tails, mimicking
Figure 16.5.3 Post-contrast sagittal T1 image of cervical spine shows patchy meningiomas or nerve sheath tumors.
nodular leptomeningeal enhancement along the surface of the cord (short b Vertebral lesions usually occur in the lower thoracic
arrow). Leptomeningeal enhancement is also seen along the brainstem (long
arrow) and cerebellum (curved arrow). and upper lumbar spine. The lesions appear as multiple
lytic lesions with sclerotic margins, sclerotic lesions, or
Figure 16.5.4 Post-contrast axial T1 image of cervical spine shows patchy mixed lytic and sclerotic involvement in CT scan. MRI
nodular leptomeningeal enhancement (arrows) along the surface of the cord.
appearance varies accordingly.
Figure 16.5.5 Post-contrast sagittal T1 of lumbar spine shows smooth (long b See Case 3.3 for discussion of intracranial
arrow) and nodular (short arrows) enhancement of cauda equina nerve roots. neurosarcoidosis.
Further reading
Smith JK, Matheus MG, Castillo M. Imaging
manifestations of neurosarcoidosis. AJR
Am J Roentgenol 2004; 182(2): 289–295.
361
Section 3: Spine
Case 16.6
362
r In adults the infection is thought to begin at the vertebral

History
body endplates and extend to the intervertebral disc space.
56-year-old male with severe focal back pain and fever. r Risk factors include spinal instrumentation, intravenous
drug use, immunosuppression, long-term steroid use, and
Findings diabetes mellitus.
Figures 16.6.1 , 16.6.2 Lateral (Fig. 16.6.1) and AP (Fig. 16.6.2) radiographs r Patients may demonstrate an elevated CRP and/or ESR.
of the thoracic spine demonstrate loss of the intervertebral disc space at T8–T9 r Most common causative organism is Staphyloccoccus
(curved arrow) with associated vertebral body height loss and focal kyphosis.
aureus, with Streptococcus viridans occurring with
Figure 16.6.3 Sagittal T1-weighted image of the thoracic spine intravenous drug use and immunosuppression.
demonstrates decreased signal intensity of T8 and T9 vertebral bodies (block r Mycobacterium tuberculosis involvement is called Pott’s
arrow) surrounding the intervertebral disc space (curved arrow). There is mild disease.
retropulsion into the central canal (arrow) as well as a low-signal-intensity
soft-tissue mass anterior to the T7, T8, and T9 vertebral bodies (∗ ).
r Most commonly involves the lumbar spine; neurologic
deficit most commonly seen with cervical spine
Figure 16.6.4 Sagittal T2-weighted image of the thoracic spine involvement.
demonstrates increased signal intensity within the T8 and T9 vertebral bodies r Plain films are insensitive in the early stages and may show
(block arrow) as well as the intervertebral disc space (curved arrow). Again seen
is retropulsion of high-signal material into the central canal (arrow) with at least disc space narrowing with ill-defined vertebral body
mild central canal stenosis. Abnormal prevertebral soft tissue is again seen (∗ ). endplates. Sclerosis is seen after 10 weeks.
r MRI is the imaging modality of choice, with a high
sensitivity and specificity, and can usually uncover an
Diagnosis underlying cause if present, such as a neoplastic process.
Spondylodiscitis.
b T1: will show low signal in the disc space and adjacent
vertebral body endplates.
Differential diagnosis b T2: will show high signal in the disc space and adjacent
r Langerhans cell histiocytosis. vertebral body endplates secondary to bone-marrow
r Schmorl node, modic type I degenerative changes. edema. Adjacent soft tissues will also show increased
r Charcot joint. signal.
b T1 with contrast: will show enhancement of the
Key points vertebral body endplates and perivertebral soft tissues.
r Bimodal age distribution: pediatric patients and patients Peripheral enhancement and central low signal indicate
over 50 years old. abscess formation.
r In the pediatric age group, infection begins in the b DWI: high signal acutely and low signal when chronic.
intervertebral disc, as blood supply in this region is still
present.
Further reading
Dunbar JA, Sandoe JA, Rao AS, Crimmins Leone A, Dell’Atti C, Magarelli N, Colelli P,
DW, Baig W, Rankine JJ. The MRI Balanika A, Casale R, et al. Imaging of
appearances of early vertebral spondylodiscitis. Eur Rev Med Pharmacol
osteomyelitis and discitis. Clin Radiol Sci 2012; 16 (Suppl 2): 8–19.
2010; 65(12): 974–981.
363
Section 3 Spine
Chapter
Spinal tumors
17 Catherine Shaeffer, Max Wintermark, Sugoto Mukherjee, and Yang Tang
Case 17.1
Figure 17.1.1
Figure 17.1.2

29-year-old male presents with slowly progressive arm and r Ependymoma.
leg weakness. r Other intramedullary cord neoplasms.
Findings Key points

r Cord astrocytomas represent the second most common
Figure 17.1.1 Sagittal T1-weighted contrast-enhanced MRI demonstrates a spinal cord tumor, and are most commonly located in the
heterogeneously enhancing expansile lesion of the thoracic spinal cord
involving segments T5 through T12 (arrows). cervical and thoracic spines.
r Most commonly occur in the third decade; typically males
Figure 17.1.2 Sagittal T2-weighted MRI in a different patient with the same are more affected than females.
diagnosis demonstrates a hyperintense expansile mixed cystic/solid lesion in r Distinguishing features from cord ependymomas, which
the cervical and thoracic cord (curved arrow).
are more common, include younger age of the patients and
a less heterogeneous appearance. These are rarely
Diagnosis associated with hemorrhage, and are also more
Astrocytoma of the spinal cord. eccentrically located.
364
Chapter 17: Spinal tumors
r Imaging: intramedullary masses, with heterogeneous

b Plain radiographs and CT: may see bony changes such enhancement. They are usually eccentric to the cord,
as cortical scalloping/thinning and scoliosis. and may have exophytic versus extramedullary
b MRI: astrocytomas usually involve a long segment, and appearance.
they are expansile, sometimes holocordic r Treatment: surgical resection.
Further reading
Koeller KK, Rosenblum RS, Morrison AL.
Neoplasms of the spinal cord and filum
terminale: radiologic–pathologic
1721–1749.
365
Section 3: Spine
Case 17.2
366
r Myxopapillary ependymomas almost always occur in the

History
conus or cauda equina, and account for roughly 50% of
42-year-old male patient presents with progressive back
adult spinal ependymomas. Rare in pediatrics.
pain, weakness, and paresthesias. r Grade 2 ependymomas are the most common type in
pediatric populations.
Findings r Grade 3 anaplastic ependymomas are more likely to have
Figure 17.2.1 Sagittal T2-weighted image demonstrates a T2 hyperintense CSF dissemination at the time of diagnosis, with a worse
elongated ill-defined region within the spinal cord extending from C2 to C7, prognosis.
consistent with cord edema (block arrow). A slightly expansile lesion is noted at r Imaging:
C4–C5, with T2 hypointensity (arrow) likely due to hemorrhage and a cystic
component. b CT: may show canal widening, with a slightly
hyperdense mass demonstrating contrast
Figure 17.2.2 Axial T2-weighted image demonstrates an expansive, T2
hyperintense lesion (arrow) within the right to central cord causing narrowing enhancement. May see adjacent bony changes with
of the epidural space at this level. No extension into the neural foramen. scalloping and expansion of the neural foramen.
b MRI demonstrates an expansive, well-circumscribed
Figure 17.2.3 Sagittal T1-weighted image with contrast demonstrates a
homogeneously enhancing, well-circumscribed, rounded mass (arrow) within mass arising from the central aspect of the cord. Iso- to
the cord at the level of C4–C5. No other areas of abnormal enhancement. No hypointense signal intensity on T1-weighted images,
extension of the mass beyond the spinal cord. enhancing either homogeneously or heterogeneously
Figure 17.2.4 Axial T1-weighted image with contrast demonstrates a
with contrast. Ependymomas demonstrate high T2
well-circumscribed lesion in the right central cord, which enhances signal intensity with surrounding spinal cord edema. A
homogeneously (arrow). No extension into the neural foramen. rim of low signal intensity, the “cap sign,” (seen in
approximately 20% of cases) may be due to
hemosiderin from previous hemorrhage.
Diagnosis b Can be differentiated from astrocytomas based on the
Ependymoma of the cervical spinal cord. central location in the cord, more heterogeneous
appearance with cysts and hemorrhage, more
Differential diagnosis delineated borders with contrast, and appearance of the
r Astrocytoma. “cap sign.”
b Leptomeningeal spread of disease in the spine can be
Key points detected with MRI, showing smooth enhancement
r Most common spinal cord tumor seen in adults. The peak along the surface of the spinal cord; extramedullary
intradural enhancing foci; nerve root irregularity with
incidence is at 40 years of age. Second most common spinal
thickening, nodularity, or clumping; and thecal sac
cord tumor in pediatric patients (30% of intramedullary
irregularity.
spinal lesions).
r Arises from ependymal cells lining the central canal. r Treatment: surgical resection with or without irradiation.
r Four types of ependymal tumors: ependymoma (grade 2),
anaplastic ependymoma (grade 3), subependymoma
(grade 1), and myxopapillary ependymoma (grade 2).
Further reading
1721–1749.
367
Section 3: Spine
Case 17.3
History Key points

r Ependymomas are the most common intramedullary
45-year-old male presents with progressive paraparesis and
bladder and bowel symptoms. spinal cord tumor in adults. 13% of all spinal
ependymomas are myxopapillary ependymomas, which
almost always occur in the cauda equina or conus and arise
Findings from the filum glia. Typically intradural in location but can
Figure 17.3.1 Sagittal T2-weighted image demonstrates a well-defined, be seen in the extradural space.
expansile, predominantly hyperintense lesion (∗ ) arising from the central cord r Increased incidence in men compared to women, with a
of the thoracolumbar spine at the level of T12 to L2. Scattered internal T2
hypointensities likely reflect hemorrhage. There is distortion of the conus. No peak incidence at 35 years of age. Typical presenting
adjacent bony erosion or scalloping is noted. Previous surgical changes are symptoms are low back pain. Neurologic deficits are rare.
noted at L5–S1 level. r Imaging:
Figure 17.3.2 Sagittal T1-weighted post-contrast image demonstrates a b Plain film: may see widened spinal canal or bony
fairly well-circumscribed heterogeneously enhancing expansive elongated remodeling but is usually normal.
lesion (∗ ) arising from the central thoracolumbar spinal cord spanning three b CT: homogeneously enhancing mass within the spinal
vertebral levels.
canal.
b MRI: isointense to the cord on T1 and hyperintense on
Diagnosis T2-weighted images. Avid enhancement after contrast,
Myxopapillary ependymoma. usually homogeneous but may be heterogeneous if
hemorrhage or mucinous degeneration is present. May
Differential diagnosis encase or displace the nerve roots.
r Astrocytoma. r Treatment: surgical resection.
r Subependymoma.
r Schwannoma.
r Intraspinal metastasis.
368
Further reading
Koeller KK, Rosenblum RS, Morrison AL. Shors SM, Jones TA, Jhaveri MD, Huckman
Neoplasms of the spinal cord and filum MS. Best cases from the AFIP:
terminale: radiologic–pathologic myxopapillary ependymoma of the
correlation. Radiographics 2000; 20(6): sacrum. Radiographics 2006; 26(Suppl 1):
1721–1749. S111–6.
369
Section 3: Spine
Case 17.4
History
39-year-old male with upper extremity weakness.
Findings
Figure 17.4.1 Sagittal T2-weighted MRI demonstrates edema extending
throughout the entire length of the cervical cord and upper thoracic cord
(block arrows), with prominent flow voids posterior to the cord (arrow). No
discrete mass is seen.
Figure 17.4.2 Sagittal T1-weighted contrast-enhanced MRI demonstrates a

small enhancing nodule at the pial surface of the posterior cord at the level of
C3 (arrow).
Figure 17.4.3 Axial T1-weighted contrast-enhanced MRI demonstrates

multiple bilateral cerebellar lesions, which have intensely enhancing nodular
components (arrows) and non-enhancing cystic components (∗ ), consistent
with multiple hemangioblastomas.
Diagnosis
Hemangioblastoma of the cervical cord in von
Hippel–Lindau disease (VHL).
Figure 17.4.3
370
r Imaging:
r Ependymoma. b CT: enhancing nodule with hypodense cyst-like
r Meningioma. component on post-contrast images.
r Schwannoma. b MRI: typically hypo- to isointense on T1 and iso- to
r Metastasis. hyperintense on T2, with avid enhancement of the
r AVM. nodular component on post-contrast imaging,
secondary to the pial blood supply. Roughly 40% are
mostly solid with ill-defined margins and intense
Key points enhancement on post-contrast imaging. Associated
r Benign neoplasm most commonly seen in young and
with cord edema or syrinx. Flow voids may be seen
middle-aged adults. Most common location is the with larger lesions.
cerebellar hemispheres; spinal involvement can also be b Angiography: avidly enhancing nodule with dilated
seen. arteries and draining veins.
r Approximately 4–20% are seen as part of VHL, in which
r Treatment: resection. However, with no capsule present,
case they are often multiple.
r Patients present with pain and weakness. recurrence is common if resection is incomplete.
Further reading
Baker KB, Moran CJ, Wippold FJ, 2nd,
Smirniotopoulos JG, Rodriguez FJ,
Meyers SP, et al. MR imaging of spinal
hemangioblastoma. AJR Am J Roentgenol
2000; 174(2): 377–382.
371
Section 3: Spine
Case 17.5
History
47-year-old male presents with progressive upper back pain.
Findings
Figure 17.5.1 Axial T2-weighted image demonstrates an expansile lobular
mildly hyperintense dumbbell-shaped lesion. The lesion fills the right neural
foramen of the thoracic spine and expands laterally to involve the right
posterior hemithorax. There is extension from the neural foramen medially into
the epidural space with mass effect on the thecal sac (curved arrow). No
evidence of cord compression.
Figure 17.5.2 Coronal T2-weighted image demonstrates a well-rounded

heterogeneously hyperintense lesion extending from the T3–T4 right neural
foramen which expands the neural foramen and extends into the epidural
space medially, slightly compressing and deforming the thecal sac (curved
arrow).
Figure 17.5.3 Axial T1-weighted image post-contrast demonstrates a

well-circumscribed lesion expanding the right upper thoracic neural foramen
and extending into the posterior thorax. There is anterior displacement of the
trachea and lateral displacement of the thecal sac, without invasion. The lesion
enhances peripherally with a central area of non-enhancement (∗ ).
Diagnosis
Figure 17.5.3 Thoracic spinal paraganglioma.
372
r May have associated syringohydromyelia.

Differential diagnosis r Imaging:
r Spinal schwannoma.
r Spinal neurofibroma. b CT and plain radiographs: may demonstrate vertebral
r Malignant peripheral nerve sheath tumor. scalloping.
r Pleural-based tumors. b MRI demonstrates a well-circumscribed mass, which is
isointense to the cord on T1-weighted images and iso-
to hyperintense to the cord on T2-weighted images.
Key points T2-weighted images may demonstrate a “cap sign,”
r Paragangliomas are neuroendocrine tumors, which
which signifies hemorrhage. Avid enhancement due to
typically arise from the adrenal gland, jugular foramen, or the vascular nature of these lesions, with flow voids
carotid body. Spinal paragangliomas typically involve the either peripherally and/or centrally within the tumor
cauda equina or filum terminale and are located in the nodule.
intradural extramedullary compartment.
r More common in middle-aged male patients, with r Treatment: surgical resection.
insidious onset of symptoms such as back pain and
weakness.
Further reading
Soderlund KA, Smith AB, Rushing EJ,
Smirniotopolous JG. Radiologic–
pathologic correlation of pediatric and
adolescent spinal neoplasms: Part 2,
Intradural extramedullary spinal
neoplasms. AJR Am J Roentgenol 2012;
198(1): 44–51.
373
Section 3: Spine
Case 17.6
History
Patient presents with headache.
Findings
Figure 17.6.1 Sagittal T2-weighted image demonstrates a mixed-signal
well-circumscribed lesion within the anterior foramen magnum, with focus of
increased signal centrally (∗ ). Note the posterior displacement, compression,
and abnormal T2 signal within the medulla (arrow).
Figure 17.6.2 Axial T2-weighted image demonstrates an anterior foramen

magnum lesion (∗ ) with heterogeneous high signal centrally and lower signal
peripherally, which exhibits mass effect on the medulla (arrow).
Figure 17.6.3 Sagittal T1-weighted image with contrast demonstrates

intense homogeneous enhancement of the anterior foramen magnum lesion
(∗ ).
Diagnosis
Meningioma of the foramen magnum.
r Schwannoma.
r Epidural metastasis.
Figure 17.6.3 r Clival chordoma.
374
b MRI: iso- to hypointense to the cord on T1-weighted

Key points
r 2–3% of meningiomas are located within the spine. Spinal images. May be slightly hyperintense on T2-weighted
images. Contrast-enhanced images will demonstrate
meningiomas make up 25% of spinal tumors and are
avid homogeneous enhancement. Typically do not
mostly seen in middle-aged females. Most commonly
extend into the neural foramen. May be heterogeneous
occur in the thoracic spine, and less likely in the cervical or
due to presence of cysts, or calcifications and vessels.
lumbar spine. b “Dural tail sign,” which is adjacent dural thickening, is
r Arise from arachnoid cap cells. Mostly benign, with 1% of
seen in 60% of cases. However, this is not specific to
meningiomas demonstrating malignancy.
r Typically intradural. Extradural tumors are mostly seen in meningiomas.
b Angiography: early dense tumor blush which persists
pediatric populations.
r Imaging findings: into the venous phase with radial array of vessels.
r Treatment: resection ± preoperative embolization.
b CT: hyperdense well-defined mass, with homogeneous
enhancement on post-contrast images.
Further reading
Soderlund KA, Smith AB, Rushing EJ,
Smirniotopolous JG. Radiologic–
pathologic correlation of pediatric and
adolescent spinal neoplasms: Part 2,
Intradural extramedullary spinal
neoplasms. AJR Am J Roentgenol 2012;
198(1): 44–51.
375
Section 3: Spine
Case 17.7
History
56-year-old female with history of renal cell carcinoma
presents with back pain and lower extremity weakness.
Findings
Figure 17.7.1 Sagittal T2-weighted MRI demonstrates a focus of
intramedullary heterogeneous signal (arrow) at T11 with surrounding cord
edema (block arrow).
Figure 17.7.2 Sagittal T1-weighted contrast-enhanced MRI demonstrates a

well-defined rounded focus of enhancement within the central cord at T11
(arrow).
Figure 17.7.3 Axial T1-weighted contrast-enhanced MRI demonstrates a

well-defined rounded focus of enhancement within the central cord with
expansion of the cord (arrow).
Diagnosis
Figure 17.7.3 Spinal cord metastasis from renal cell carcinoma.
376
r Most commonly seen in the cervical cord, then thoracic,

r Intramedullary abscess. then lumbar.
r Patients typically experience symptoms for less than 1
r Inflammatory lesions (transverse myelitis, multiple
month.
sclerosis). r Mean age is greater than 55 years.
r Primary spinal cord tumors (ependymoma, astrocytoma,
r Imaging:
hemangioblastoma).
b Plain radiographs are typically normal.
b CT typically normal, but hypervascular tumors may
Key points show enhancement within the cord.
r Fewer than 1% of spinal metastases are intramedullary in b MRI: typically lesions are hypo- to isointense on
location. Typically vertebral metastases are seen (94%), and T1-weighted imaging, and hyperintense on
less commonly intradural extramedullary metastases (5%). T2-weighted imaging with surrounding edema. Masses
r May arise from hematogenous spread (including
usually demonstrate avid enhancement, either
retrograde venous spread), lymphatic spread, or direct homogeneous or heterogeneous, on post-contrast
contiguous spread from adjacent structures. images. May see findings of cord expansion,
r Lung cancer is the most common primary cancer to have
hemorrhage, and necrotic changes.
intramedullary metastasis; other primaries include breast
r Treatment: radiation therapy and corticosteroids. Poor
cancer, lymphoma, leukemia, melanoma, renal cell
carcinoma, and colorectal cancers. prognosis.
Further reading
Rykken JB, Diehn FE, Hunt CH, Schwartz
KM, Eckel LJ, Wood CP, et al.
Intramedullary spinal cord metastases:
MRI and relevant clinical features from a
13-year institutional case series. AJNR
Am J Neuroradiol 2013; 34(10):
2043–2049.
377
Section 3: Spine
Case 17.8
History
Patient presents with lower extremity weakness.
Findings
Figure 17.8.1 Sagittal T2-weighted MRI demonstrates thickening of the
cauda equina nerve roots (block arrows).
Figure 17.8.2 Sagittal T1-weighted contrast-enhanced MRI demonstrates

thickened and enhancing cauda equina nerve roots (arrows).
Figure 17.8.3 Axial T2-weighted MRI demonstrates thickened nerve roots

(curved arrow), right hydronephrosis (∗ ), and retroperitoneal lymphadenopathy
(block arrows).
Diagnosis
Leptomeningeal spread of lymphoma.
r Arachnoiditis.
r Leptomeningeal infection.
r Leptomeningeal carcinomatosis.
Figure 17.8.3
r Charcot Marie Tooth disease.
378
r Imaging findings:
Key points
r Leptomeningeal metastasis can occur with both primary b CT: frequently normal.
intracranial malignancies and widespread metastatic b MRI: thickened nerve roots on T2-weighted imaging.
disease (most commonly breast and lung cancer). Post-contrast images will demonstrate irregular
r Presentation mirrors that of the primary malignancy, nodular thickening of the leptomeninges.
although patients can present with radiculopathy and/or r Treatment: radiation and intrathecal chemotherapy. Poor
weakness. prognosis.
r Leptomeningeal lymphoma represents two-thirds of
secondary CNS lymphoma.
Further reading
1721–1749.
379
Section 3: Spine
Case 17.9
380
History Key points

56-year-old male with a history of lung cancer develops r Adults: most commonly result from prostate, breast, or
progressive lower extremity weakness. lung primary cancers.
r Children: most commonly result from Ewing’s sarcoma,
Findings neuroblastoma, germ cell tumors, or Hodgkin’s disease.
r Most commonly located in the thoracic spine, followed by
Figure 17.9.1 Sagittal T1 image demonstrates hypointense lesions involving the lumbar spine, then the cervical spine.
T8 and T12 vertebrae (arrows), consistent with osseous metastases. Epidural
tumor is seen at T7–T8 (curved arrow), causing cord compression.
r Cord compression is the first manifestation of disease in
lung cancer 20% of the time.
Figure 17.9.2 Sagittal STIR image demonstrates corresponding r MRI is the gold standard of diagnosis, with a reported
hyperintensity of T8 and T12 vertebral lesions (arrows) and T7–T8 dorsal
epidural tumor (curved arrow). sensitivity of 93% and specificity of 97%.
r Intramedullary or leptomeningeal metastases are much
Figure 17.9.3 Sagittal post-contrast T1 shows enhancement of epidural less frequent than epidural metastases.
tumor, with both ventral and dorsal components (curved arrows). r Treatment: corticosteroid is the common first-line
Figure 17.9.4 Axial T2 shows circumferential epidural tumor (curved arrows) treatment to decrease local inflammation and edema, and
with effacement of CSF, cord compression, and deformity (∗ ). it can prevent neurologic deterioration in the short term.
External beam radiotherapy has been the standard of
treatment in the long term.
Diagnosis
Cord compression from epidural metastatic disease.
r Epidural abscess, hematoma, or lipoma.
r Primary tumors of the spinal cord.
Further reading
Husband DJ, Grant KA, Romaniuk CS. MRI
in the diagnosis and treatment of
suspected malignant spinal cord
compression. Br J Radiol 2001; 74(877):
15–23.
381
Section 3 Spine
Chapter
Miscellaneous spine emergencies
18 Thomas J. E. Muttikal, David Clifton, Yang Tang, Sugoto Mukherjee, and Max Wintermark
Case 18.1
History
73-year-old patient presents with a few days’ history of
severe worsening of chronic back pain with left sciatica and
foot weakness.
Findings
Figure 18.1.1 Sagittal T2-weighted (a) and T1-weighted images (b)
demonstrate intermediate-signal-intensity lesion (arrows) superior to the disc
in the epidural space impinging on the traversing nerve.
Figure 18.1.2 Axial T2-weighted MRI shows intermediate-signal lesion

(arrow) causing left lateral recess and left neural foraminal stenosis.
Figure 18.1.3 Axial T1-weighted MRI shows intermediate-signal lesion

(arrow) causing left lateral recess and left neural foraminal stenosis.
Diagnosis
Disc extrusion.
Figure 18.1.3
382
Chapter 18: Miscellaneous spine emergencies
b Usually epidural in location. Rarely intradural.

Differential diagnosis b Peripheral contrast enhancement. Delayed
r Nerve sheath tumor.
post-contrast imaging may show diffuse enhancement.
Peripheral contrast enhancement helps in diagnosis in
Key points cases of intradural sequestered disc, as well as when the
r Usual presentations include back pain and radiculopathy. disc is sequestered in an unusual location such as the
r Motor weakness can occur due to compression of the posterior epidural space. Usually nerve sheath tumors
nerves. show higher T2 signal intensity than disc herniation.
r Cauda equina syndrome can occur if the herniated disc Peripheral contrast enhancement helps to differentiate
causes severe thecal compression. disc herniation from nerve sheath tumor in difficult
r Imaging: cases.
b Isointense to parent disc on T1-weighted image. r Treatment: conservative management, image-guided
b Iso- to hyperintense on T2-weighted image. injection, or surgery.
Further reading
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(11):
2525–2545.
383
Section 3: Spine
Case 18.2
384
Diagnosis
Epidural abscess.
r Epidural hematoma.
r Extradural metastasis.
Key points
r Up to one-third of cases are caused by Staphylococcus
aureus, followed by Mycobacterium tuberculosis.
r Risk factors include intravenous drug use,
immunocompromised status, recent instrumentation, and
diabetes mellitus.
r Abscess location provides clue to etiology:
b Anterior abscess generally spreads from adjacent
discitis/osteomyelitis. Less common.
b Posterior abscess is generally a result of hematogenous
spread from the gut, lungs, heart, or skin. More
common.
Figure 18.2.5 r Most common in males in the sixth or seventh decade.
r Neurologic deficits occur secondary to cord compression
and ischemic effects of compromised epidural venous
History plexus.
74-year-old male presents with fever and diffuse tenderness r CT findings: enhancing epidural mass with/without mass
along the spine. effect on the spinal cord.
r MRI findings:
Findings b T1-weighted imaging demonstrates iso- to hypointense
(to the cord) extra-axial mass lesion.
Figures 18.2.1–18.2.3 Sagittal T2-weighted images of the cervical, thoracic, b T1-weighted imaging with contrast will show
and lumbar spine demonstrate homogeneously hyperintense epidural
collection causing mass effect upon the spinal cord (arrows). ring-enhancing abscess as well as heterogeneously
enhancing phlegmon. Dural enhancement also
Figure 18.2.4 Axial T2-weighted image of the cervical spine demonstrates
the hyperintense dorsal epidural fluid collection (arrows) causing severe
seen.
compression and deformity of the cervical cord (curved arrow). b T2-weighted imaging demonstrates hyperintense
extra-axial mass lesion.
Figure 18.2.5 Sagittal post-contrast T1-weighted image of the lumbar spine
demonstrates leptomeningeal enhancement in the region of the conus and
r Nuclear medicine gallium scan will show increased
the filum terminale (arrows). uptake.
Further reading
Diehn FE. Imaging of spine infection. Radiol
Clin North Am 2012; 50(4): 777–798.
Rigamonti D, Liem L, Sampath P, Knoller N,
Namaguchi Y, Schreibman DL, et al.
Spinal epidural abscess: contemporary
trends in etiology, evaluation, and
management. Surg Neurol 1999; 52(2):
189–196; discussion 197.
385
Section 3: Spine
Case 18.3
History Key points

r Most spontaneous epidural hematomas are idiopathic.
28-year-old female with acute-onset neck pain and sensory
deficit in bilateral upper extremities. Vascular anomalies, anticoagulation, and coagulopathies
can also represent causes.
r Bimodal distribution: childhood, and again in the
Findings fifth and sixth decade, with a 4:1 male-to-female
Figures 18.3.1, 18.3.2 Sagittal T1 (Fig. 18.3.1) and T2 (Fig. 18.3.2) MRI distribution.
images of the cervical spine demonstrate an elongated fluid collection within r Thoracic and lumbar spines are more often involved than
the ventral epidural space from C2 to C7 causing mass effect on the spinal
cord. This collection is slightly T1 hyperintense, and T2 hyperintense with a the cervical spine in adults. In children, the cervicothoracic
hematocrit level. region is more commonly involved.
r CT findings: hyperdense epidural mass with or without
Diagnosis cord compression.
r MRI findings:
Spontaneous epidural hematoma.
b T1-weighted imaging: variable signal intensity
Differential diagnosis depending on hematoma age.
r Epidural abscess.
Acute (⬍ 48 hours): isointense.
r Epidural lipomatosis.
Subacute and chronic: hyperintense.
r Epidural metastasis.
Fat suppression will differentiate fat from blood
r Lymphoma. products.
386
b T1-weighted imaging with contrast: focal Can display hypointense foci secondary to
enhancement may represent active extravasation. deoxyhemoglobin in blood products.
Peripheral enhancement is usually related to dural r Treatment: may resolve spontaneously, but may require
hyperemia. surgery in cases of severe neurologic deficit. Duration of
b T2-weighted imaging: usually heterogeneously
neurologic symptoms correlates with degree of return of
hyperintense. function.
Further reading
Holtas S, Heiling M, Lonntoft M.
Spontaneous spinal epidural hematoma:
findings at MR imaging and clinical
correlation. Radiology 1996; 199(2):
409–413.
387
Section 3: Spine
Case 18.4
388
History progressive quadriparesis, dysphagia, vertigo, convulsions,

dysarthria, nystagmus, respiratory dysfunction, drop
54-year-old patient presents with progressive arm and leg
attacks, and sudden death.
weakness and paresthesia. r Lhermitte’s phenomenon is sometimes observed.
r Radiculopathy due to narrowing of neural foramina from
Findings subaxial subluxations.
Figure 18.4.1 Sagittal reformat of the CT scan shows erosion of dens r Imaging:
(a, arrow), increased distance between atlas and dens (b, star), thick soft tissue b Conventional radiography: atlantoaxial subuxation,
(b, double arrows) compressing the cervicomedullary junction.
demonstrated by increased distance between atlas and
Figure 18.4.2 Sagittal T2-weighted image shows hypointense tissue (short anterior dens of ⬎ 3 mm and dynamic instability in
arrow) causing cord compression and myelomalacia (long arrow). flexion and extension views. In symptomatic patients,
Figure 18.4.3 Axial T2-weighted MRI demonstrates hypointense tissue (short the films in flexion should be taken only after the
arrow) causing cord compression and myelomalacia (long arrow). The dens is other radiographs have excluded an odontoid fracture
eccentric to the right (curved arrow). or severe atlantoaxial subluxation. Posterior
atlantodental interval (PADI), which reflects the space
Figure 18.4.4 Post-contrast sagittal T1-weighted image shows lack of
enhancement of the hypointense tissue (arrow). available for the spinal cord, is a better predictor of
neurologic compromise than anterior atlantodental
interval.
Diagnosis b CT scan: compared to plain radiographs, CT scan
Compressive myelopathy due to rheumatoid arthritis. demonstrates bony changes better as well as soft-tissue
changes including narrowing of the spinal canal and
Differential diagnosis cord compression.
r Calcium pyrophosphate deposition (CPPD). b MRI: spinal cord/cervicomedullary compression, cord
r Gout. edema, and myelomalacia are evaluated with MRI.
r Infectious arthritis. Atlantoaxial ligament should be evaluated for integrity
versus loosening. Chronic fibrous pannus appear
Key points hypointense in T1- and T2-weighted images without
r Compression of neuraxis due to atlantoaxial subluxation significant contrast enhancement, differentiating it
and pannus could result in painless sensory loss in the from infective pathologies.
extremities, peripheral paresthesias, paresthesias and pain r Treatment: atlantoaxial subluxation with spinal
in the back of neck in C2 distribution, decreased sensation cord/cervicomedullary compression is treated with
and pain in the face, loss of sphincter control, slowly surgical fusion and decompression.
Further reading
Iizuka H, Iizuka Y, Kobayashi R, Nishinome Joaquim AF, Appenzeller S. Cervical spine
M, Sorimachi Y, Takagishi K. The involvement in rheumatoid arthritis: a
relationship between an intramedullary systematic review. Autoimmun Rev 2014;
high signal intensity and the clinical 13(12): 1195–1202.
outcome in atlanto-axial subluxation
owing to rheumatoid arthritis. Spine J
2014; 14(6): 938–943.
389
Section 3: Spine
Case 18.5
Findings
Figure 18.5.1 Sagittal T2-weighted image demonstrates
heterogeneous-signal-intensity tissue posterior to the dens (long arrow)
compressing the cervicomedullary junction (short arrow).
Figure 18.5.2 Sagittal T1-weighted image (a) shows

intermediate-signal-intensity tissue (short arrow) compressing the
cervicomedullary junction (long arrow). Post-contrast sagittal T1-weighted
image (b) shows minimal enhancement of the lesion and enhancing dura
(double arrows).
Figure 18.5.3 CT scan shows calcification of the lesion (arrow).
Diagnosis
Cervicomedullary compression and myelopathy due to
calcium pyrophosphate deposition (CPPD) – periodontoid
pseudotumor.
r Gout.
r Hydroxyapatite deposition disease.
r Rheumatoid arthritis.
Figure 18.5.3
r Osteomyelitis.
Key points
r Elderly patients.
History r CPPD can occur as isolated involvement of cervical spine.
72-year-old patient presents with progressive upper and r Crowned dens syndrome: fever, cervico-occipital pain,
lower extremity weakness and neck pain. neck stiffness, retro-odontoid soft-tissue calcification.
390
r Features secondary to compression of cervicomedullary patchy enhancement of the lesion. MRI shows the
junction and cervical nerves. cervicomedullary compression and signal changes
r Imaging: associated with myelopathy.
b CT scan: calcification of transverse ligament and b Hydroxyapatite deposition disease can appear very
retro-odontoid soft tissue. Calcification can also be similar in imaging. Hydroxyapatite deposition disease
seen in other cervical spine ligaments, disc space, is seen in a younger age group. Although rare, the
capsules of facet joints. Longstanding disease can result involvement of C1–C2 by gout can appear very similar
in erosions and cysts in dens, loss of disc height with to CPPD, which may be differentiated by laboratory
erosive endplate changes, subchondral cysts, and evaluation and involvement of other joints.
osteophyte formation. Calcification of transverse ligament and retro-odontoid
b MRI: retro-odontoid soft tissue appears as iso- to soft tissues differentiates from rheumatoid arthritis.
low-intensity mass on T1-weighted images and Cranial settling is seen with rheumatoid arthritis, not
low-intensity/heterogeneous mass on T2-weighted with CPPD.
images. Post-contrast images may show peripheral and r Treatment: conservative management in the initial stage,
surgery in the advanced stage.
Further reading
Fenoy AJ, Menezes AH, Donovan KA, Kakitsubata Y, Boutin RD, Theodorou DJ,
Kralik SF. Calcium pyrophosphate Kerr RM, Steinbach LS, Chan KK, et al.
dihydrate crystal deposition in the Calcium pyrophosphate dihydrate crystal
craniovertebral junction. J Neurosurg deposition in and around the atlantoaxial
Spine 2008; 8(1): 22–29. joint: association with type 2 odontoid
fractures in nine patients. Radiology
2000; 216(1): 213–219.
391
Section 3: Spine
Case 18.6
History Key points

8-year-old patient presents with back pain. r Pediatric patients.
r Clinical presentation includes back pain, fever, and
Findings neurological symptoms due to spinal canal and neural
Figure 18.6.1 Lateral radiograph of the spine shows vertebra plana (arrow). foraminal stenosis, including myelopathy and
radiculopathy.
Figure 18.6.2 Sagittal T2-weighted image (a) shows severe compression of r Can be asymptomatic incidental finding.
L2 vertebra (arrow), with convex bowing of the posterior cortex into the spinal r Imaging:
canal. No soft-tissue component is present. Sagittal T2-weighted image with fat
saturation (b) shows severe compression of L2 vertebra (arrow), with posterior b Radiograph: lytic lesion, vertebral collapse – vertebra
cortex projecting into the spinal canal. The vertebra shows diffuse high signal.
No soft-tissue component is present. plana. Disc space is generally spared.
b CT scan: lytic lesion, vertebral collapse – vertebra
plana. Disc space is generally spared. Large soft-tissue
Diagnosis mass is absent.
Vertebral plana (eosinophilic granuloma). b MRI: affected vertebra shows hypointense signal in
T1-weighted image, hyperintense signal in T2
Differential diagnosis fat-saturated image, with homogeneous enhancement
r Ewing’s sarcoma. on post-contrast T1-weighted image. Disc space is
r Leukemia. generally spared. Large soft-tissue mass is absent.
r Lymphoma. b Ewing’s sarcoma generally has larger soft-tissue mass
r Metastases. compared to eosinophilic granuloma. Leukemia is
r Osteomyelitis. diffuse/multifocal with abnormal marrow signal in
r Trauma. other vertebrae. Lymphomatous involvment is also
392
usually diffuse/multifocal with abnormal marrow r Treatment: conservative management in the initial stage,
signal in other vertebrae. Metastases can appear similar especially for an isolated lesion. For multifocal disease, or
to eosinophilic granulomas, while the presence of a in cases with systemic involvement, multiple treatment
primary site differentiates the two. Osteomyelitis modalities are used including chemotherapy, radiation,
usually involves the disc space. and surgery.
Further reading
David R, Oria RA, Kumar R, Singleton EB, DiCaprio MR, Roberts TT. Diagnosis and
Lindell MM, Shirkhoda A, et al. Management of Langerhans Cell
Radiologic features of eosinophilic Histiocytosis. J Am Acad Orthop Surg
granuloma of bone. AJR Am J Roentgenol 2014; 22(10): 643–652.
1989; 153(5): 1021–1026.
393
Section 3: Spine
Case 18.7
History
77-year-old woman presents with worsening of chronic Key points
bilateral lower extremity weakness and band-like pain r Lower extremity weakness.
around the chest. r Sensory symptoms.
r Chronic course, sometimes with periods of waxing and
Findings waning.
r Uncertain etiology, which may be due to incomplete or
Figure 18.7.1 Sagittal T2-weighted image demonstrates T2 hyperintense
signal abnormality and mild cord swelling extending from T2 to T3 level (short disrupted formation of an arachnoid cyst or represent walls
arrows), with compression and ventral displacement of the cord inferior to this of collapsed arachnoid cyst.
at T3–T4 level (long arrow). r Imaging:
Figure 18.7.2 Axial T2-weighted image shows signal abnormality diffusely b MRI shows focal cord compression, with diffuse T2
affecting the cord, with some sparing of the peripheral rim. signal intensity superior or inferior to focal cord
compression.
b Web may sometimes be demonstrated by
Diagnosis high-resolution T2 imaging.
Arachnoid web. b Usually occurs in the upper thoracic region.
b Focal deformity along the ventral aspect of the cord
Differential diagnosis differentiates cord herniation through ventral dural
r Arachnoid cyst.
r Cord herniation.
394
defect. If the ventral cord is closely opposed to ventral usually fill more slowly than the remainder of the
dura, differentiation may be difficult. subarachnoid space on myelography.
b Arachnoid cysts often demonstrate well-defined walls r Treatment: surgical resection of web.
and smooth wide scalloping of the cord surface, and
Further reading
Paramore CG. Dorsal arachnoid web with Reardon MA, Raghavan P, Carpenter-Bailey
spinal cord compression: variant of an K, Mukherjee S, Smith JS, Matsumoto JA,
arachnoid cyst? Report of two cases. J et al. Dorsal thoracic arachnoid web and
Neurosurg 2000; 93(2 Suppl): 287–290. the “scalpel sign”: a distinct clinical-
Petridis AK, Doukas A, Barth H, Mehdorn radiologic entity. AJNR Am J Neuroradiol
HM. Spinal cord compression caused by 2013; 34(5): 1104–1110.
idiopathic intradural arachnoid cysts of
the spine: review of the literature and
illustrated case. Eur Spine J 2010;
19(Suppl 2): S124–S129.
395
Index
abscess aneurysm atlantoaxial subluxation 389 differential diagnosis 9

Bezold’s 259 arteriovenous fistula related treatment 389 extracranial 74
brain 93, 126 54, 56 atlanto-occipital dislocation imaging 9, 10, 19, 74
imaging 94, 126 cerebral see cerebral aneurysm 328–329 reperfusion injury following
see also pyogenic abscess mycotic 101 differential diagnosis 329 stenting 17–19
mastoiditis and 259 ankylosing spondylitis 348–349 imaging 329 carotid stenosis 19
odontogenic 238–239 differential diagnosis 348 treatment 329 cavernous malformation 39
imaging 239 imaging 348, 349 classification 41
peritonsillar 234, 235, 240 spinal injury with 349 bacterial meningitis see differential diagnosis 40
retropharyngeal 246–247 treatment 349 meningitis imaging 40, 327
imaging 246 anterior cerebral artery 56 bamboo spine 348 spinal 326–327
spinal epidural 384–385 apical petrositis see petrous basilar meningitis see meningitis clinical presentation 327
etiology 385 apicitis Bell’s palsy 300–301 differential diagnosis 327
imaging 385 arachnoid cyst 164, 395 differential diagnosis 300 treatment 327
submandibular 239 imaging 164 imaging 300 treatment 41
subperiosteal 248, 259 ruptured 163–164 treatment 301 cavernous sinus lymphoma see
tonsillar 234, 235, 236 differential diagnosis 164 benign oligemia 4 lymphoma
acute disseminated arachnoid web 394–395 Bezold’s abscess 259 cavernous sinus thrombosis 251,
encephalomyelitis differential diagnosis 394 blood-blister aneurysm 45 254
(ADAM) 77–78 imaging 394 Borrelia burgdorferi 108 central cord syndrome 343–344
differential diagnosis 77, arachnoiditis 96, 126 see also Lyme neuroborreliosis differential diagnosis 343
110 arterial thrombosis 9 brachial plexus injuries 350–351 imaging 343
imaging 77, 78 distal basilar 6–7 differential diagnosis 351 treatment 344
predisposing factors 78 arteriovenous fistula imaging 351 central pontine myelinolysis
treatment 78 cerebral 56 management 351 183–184
acute hepatic encephalopathy imaging 56 brain abscess see abscess differential diagnosis 183
177–178 dural 53–55 branchial cleft cyst 304 imaging 183, 184
differential diagnosis 178 imaging 54 cerebellitis 109–111
imaging 177, 178 perimedullary 324–325 calcium pyrophosphate differential diagnosis 110
acute retropharyngeal calcific clinical presentation 325 deposition (CPPD) imaging 110
tendinitis 244 differential diagnosis 325 390–391 cerebral amyloid angiopathy
differential diagnosis 244 imaging 325 differential diagnosis 390 37–38
imaging 244 treatment 325 imaging 390, 391 differential diagnosis 38
AIDS patients arteriovenous malformation carcinoma imaging 38
cytomegalovirus infection 96, (AVM) 50–52 laryngeal 302–303 treatment 38
127–128 imaging 51 maxillary sinus 281 cerebral aneurysm 42–46
HIV myelitis 355 Spetzler–Martin grading tonsillar 304 classification 45
Alberta Stroke Program Early system 51 carotid blow-out syndrome clinical presentation 45
CT Score (ASPECTS) 5 artery of Percheron 26 62–63 differential diagnosis 44
amyotrophic lateral sclerosis infarction 25–26 classification 63 fusiform 45
212–213 differential diagnosis 26 imaging 63 giant 45
differential diagnosis 213 imaging 26 treatment 63 imaging 44, 45
imaging 213 aspergillosis 101 carotid–cavernous fistula 60–61 treatments 45
treatment 213 astrocytoma 146, 147 imaging 61, 74 cerebral edema 13
Anderson–Montesano fracture imaging 146, 147, 364, 365 traumatic 73–74 cerebral herniation syndrome
331 spinal cord 364–365 treatment 74 68–70
type I 331 differential diagnosis 364 types 61, 74 imaging 69
type II 331 subtypes 131 carotid dissection 8–10, 19 treatment 70
type III 331 atherosclerosis 9 clinical presentation 10–41 types of herniation 69
396
Index
cerebral venous thrombosis delayed post-hypoxic encephalitis see meningoencepha-

30–33, 36 leukoencephalopathy clinical presentations 105 locele
differential diagnosis 32 181–182 definition 105 fungal infection 98–101, 128
imaging 32, 33 differential diagnosis 182 differential diagnosis 103, 105, aspergillosis 101
predisposing factors 32 imaging 182 179 imaging 101
treatment 33 treatment 182 etiology 105 see also sinusitis
cerebritis 91, 94, 126, 259 dental infection 239 herpes 102–103 fusiform aneurysm 45
cerebrospinal fluid leak see CSF see also odontogenic abscess imaging 103 see also cerebral aneurysm
leak dermoid 160, 166 nonherpetic viral 104–106
Chance fracture 346 ruptured 165–166 imaging 105 gelatinous pseudocysts 123, 124
choanal atresia 313–314 differential diagnosis 166 treatment 106 germinal matrix hemorrhage 15
imaging 313 imaging 166 encephalopathy see acute hepatic giant aneurysm 45
cholesteatoma 291 diffuse axonal injury 71–72 encephalopathy; glioblastoma multiforme
external ear canal 288–289 imaging 72 posterior reversible 129–131
differential diagnosis 288 disc herniation 344 encephalopathy differential diagnosis 131
imaging 288 imaging 382, 383 syndrome; Wernicke’s imaging 131
middle ear 290–291 treatment 383 encephalopathy glioma, brainstem 141–143
differential diagnosis 291 dissecting aneurysm 45 endolymphatic sac tumor clinical presentations 143
imaging 290 distal basilar thrombosis 6–7 298–299 differential diagnosis 143
chordoma 309 differential diagnosis 7 differential diagnosis 298 imaging 143
choroidal detachment 217 imaging 7 imaging 298, 299 subtypes 143
colloid cyst 161–162 dural sinus thrombosis 33–36 treatment 299 treatment 143
clinical presentation 162 imaging 36 eosinophilic granuloma 392–393 gliomatosis cerebri 132–133
differential diagnosis 161 lobar hemorrhage 36 clinical presentation 392 differential diagnosis 133
imaging 161, 162 dural tail sign 375 differential diagnosis 392 imaging 133
compressive myelopathy dysembryoplastic imaging 392 globe injury 216–217
calcium pyrophosphate neuroepithelial tumor treatment 393 imaging 216
deposition 390–391 150–151 ependymal tumor types 367 lens dislocation 216
rheumatoid arthritis related differential diagnosis 151 ependymoma 146, 147 rupture 216
389 imaging 151 anaplastic 367 glomus jugulotympanicum
vertebral metastases and clinical presentation 147 292–293
380–381 Eagle syndrome 230 imaging 146, 147, 367, 368 differential diagnosis 293
cord infarction see spinal cord clinical presentation 230 myxopapillary 367, 368 imaging 293
core infarction 4 imaging 230 differential diagnosis 368 granuloma see eosinophilic
corneal laceration 217 treatment 230 spinal cord 366–367 granuloma; petrous apex
craniopharyngioma 169–170 edema 13 epidermoid 158–160 cholesterol granuloma
differential diagnosis 170 brachial plexus injury 351 differential diagnosis 160 Guillain–Barré syndrome
imaging 170 central cord syndrome 343 imaging 160 358–359
treatment 170 cryptococcosis 123 epidural abscess see abscess differential diagnosis 358
crescent sign 10 cytomegalovirus infection 96, epidural hematoma 66–67 imaging 358, 359
Creutzfeldt–Jakob disease 128 arterial 67 treatment 359
116–117 cytotoxic 110, 123 imaging 67
clinical presentation 117 hangman fracture 337 venous 67 hamartoma of tuber cinereum
differential diagnosis 117 hemangioblastoma 370 epiglottitis 243 156–157
imaging 117 hyperextension injury 341 see also supraglottitis imaging 157
crowned dens syndrome 390 hyperflexion injury 340 esophageal cancer 307 hangman fracture 336–337
cryptococcosis 122–124 Langerhans cell histiocytosis esthesioneuroblastoma 282–283 differential diagnosis 337
differential diagnosis 123 318 differential diagnosis 282 Effendi classification 337
imaging 123, 124 maple syrup urine disease 197 imaging 282, 283 imaging 337
CSF leak 204 mastoiditis 259 recurrence 283 treatment 337
intracranial hypotension and mediastinitis 246 ethmoid bone fracture 219 hemangioblastoma, spinal
202–204 orbital cellulitis 248 see also naso-orbital- 370–371
spinal trauma and 329 palatine tonsil 236 ethmoidal fractures differential diagnosis 371
cytomegalovirus (CMV) 96, periglottic 232 Ewing’s sarcoma 392 imaging 370, 371
127–128 spinal cord metastases 376 treatment 371
clinical manifestations 96, spinal ependymoma 367 facial buttress concept 224 hematoma
128 supraglottitis 243 facial nerve 300 carotid dissection 9, 10, 19
differential diagnosis 96, 128 thoracolumbar fracture 346 palsy see Bell’s palsy epidural 66–67
imaging 96, 98–101, 128 toxoplasmosis 121 fibromuscular dysplasia 9 differential diagnosis 386
vasogenic 110, 113, 207 frontal calvarial fracture 67 imaging 386
delayed cerebral ischemia (DCI), emphysema, laryngeal injury 232 frontal sinus disruption, spinal 386–387
after subarachnoid empty delta sign 33 naso-orbital-ethmoidal intraparenchymal 38, 44
hemorrhage 47–49 empty vertebral body sign 347 fractures 220 subdural 164, 204
imaging 48 empyema 91 frontoethmoidal thoracolumbar fracture and
treatment 49 subdural 256 meningoencephalocele 346
397
Index
hepatic encephalopathy 178 internal cerebral vein Leigh syndrome 193–195 subcondylar 227
chronic 178, 179–180 thrombosis 21 clinical presentation 194 treatment 227
see also acute hepatic intracranial pressure (ICP) 201 differential diagnosis 194 maple-syrup urine disease
encephalopathy increased 201 imaging 194 196–197
heroin-induced cerebral herniation and Lemierre’s syndrome 236–237 clinical presentation 197
leukoencephalopathy 182 68–70 imaging 236 imaging 197
herpes simplex virus 103 epidural hematoma and 67 treatment 237 treatment 197
see also encephalitis idiopathic intracranial lens dislocation 216 mastoiditis 258–259
HIV-positive patients 95–97, 126 hypertension 200–201 lenticulostriate collaterals 58 complications 259
cytomegalovirus infection 96, intraparenchymal hemorrhage leptomeningeal metastases see differential diagnosis 259
127–128 cerebral amyloid angiopathy metastases imaging 259
myelopathy 354–355 38 leukoencephalopathy see delayed maxillary sinus
differential diagnosis 355 cerebral aneurysm 44 post-hypoxic inverted papilloma 284–285
imaging 355 cerebral areteriovenous leukoencephalopathy; squamous cell carcinoma 281
progressive multifocal malformation 51 progressive multifocal differential diagnosis 281
leukoencephalopathy 93, diffuse axonal injury 72 leukoencephalopathy imaging 281
125–126 dural arteriovenous fistula 54 (PML) wall fracture 222
hydrocephalus 58, 69 reperfusion hemorrhage 19 ligamentous injury, spinal mediastinitis 246
bacterial meningitis 91 thrombotic microangiopathy interspinous ligament injury medulloblastoma 146
brainstem glioma 143 21 346 clinical presentation 146
choroid plexus papilloma 168 intraventricular hemorrhage ligamentum flavum injury 340, imaging 146
craniopharyngioma 170 moyamoya disease 58 346 meningioma 207
cryptococcosis 123, 124 neonate 15 transverse ligament injury 332, differential diagnosis 374
neurocysticercosis 115 inverted hamburger sign 340 333 foramen magnum 374–375
neurocytoma 149 limbic encephalitis 198–199 imaging 374, 375
pediatric posterior fossa JC polyomavirus 96, 126 differential diagnosis 199 meningitis
tumors 146 Jefferson fracture 332–333 imaging 199 bacterial 89–91
tuberculosis 96, 126 differential diagnosis 333 treatment 199 complications 91
hydroxyapatite deposition 391 imaging 332, 333 lobar hemorrhage differential diagnosis 90
hyperextension injury, cervical transverse ligament injury 332, cerebral amyloid angiopathy imaging 90, 91
spine 341–342 333 related 37–38 basilar 80, 96, 128
imaging 341 treatment 333 differential diagnosis 36 meningoencephalitis 124
treatment 342 jugular vein thrombosis 236 dural sinus thrombosis and meningoencephalocele,
hyperflexion injury, cervical juvenile nasopharyngeal 33–36 frontoethmoidal 286–287
spine 338–340 angiofibroma 315–316 Ludwig’s angina 240–241 clinical presentation 287
imaging 340 differential diagnosis 315 clinical presentation 241 differential diagnosis 286
treatment 340 imaging 315, 316 differential diagnosis 240 imaging 286
hyperperfusion syndrome 19 treatment 316 imaging 240 mesial temporal sclerosis 209
hypertension, idiopathic treatment 241 differential diagnosis 209
intracranial 200–201 labyrinthitis 296–297 lupus cerebritis 85–86 imaging 209
imaging 201 differential diagnosis 296 differential diagnosis 86 treatment 209
treatment 201 imaging 296 imaging 86 metastases
hypotension, intracranial, due to stages 297 Lyme neuroborreliosis 107–108 intracranial 153, 171–173
CSF leak 202–204 Langerhans cell histiocytosis differential diagnosis 108 differential diagnosis 172
differential diagnosis 204 317–318 imaging 108 imaging 172, 173
imaging 204 classification 318 lymphatic malformations 321 treatment 173
hypoxic–ischemic injury clinical presentation 318 lymphoma 121 nodal 304–305
adult 11–13 differential diagnosis 318 cavernous sinus 276–278 differential diagnosis 304
differential diagnosis 13 imaging 318 clinical manifestations 277 imaging 304
imaging 13 treatment 318 differential diagnosis 277 spinal cord 376–377
neonatal 14–16 laryngeal cancer 302–303 treatment 278 differential diagnosis 377
differential diagnosis 15 differential diagnosis 302 differential diagnosis 140 imaging 376, 377
imaging 15 imaging 302, 303 imaging 140, 271, 277 treatment 377
partial injuries 15 treatment 303 orbital 270–271 spinal leptomeningeal 378–379
profound injuries 15 laryngeal injury 232–233 differential diagnosis 271 differential diagnosis 378
clinical presentation 232 primary CNS lymphoma imaging 378, 379
incudomalleolar dislocation 228, imaging 232 137–140 vertebral, with cord
229 treatment 232 compression 380–381
incudostapedial dislocation 229 Le Fort fractures 223–225 macrocystic lymphatic differential diagnosis 381
infarction clinical presentation 225 malformation 321 imaging 381
artery of Percheron 25–26 imaging 224 mandibular fracture 226–227 treatment 381
core 4, 5 treatment 225 clinical presentation 227 metronidazole 186
spinal cord 322–323 type I 224 coronoid process 227 toxicity 185–186
internal carotid artery type II 224 imaging 7, 59, 227 differential diagnosis 186
thrombosis 4 type III 224, 225 parasymphyseal 227 imaging 186
398
Index
middle cerebral artery (MCA) laboratory tests 119 inverted 284–285 differential diagnosis 95–97,
aneurysm 44 subtypes 119 clinical presentation 285 126
thrombus 4 differential diagnosis 285 imaging 93, 94, 96, 126
Miller Fisher syndrome 359 occipital bone fracture 67 imaging 285 inflammatory PML 96, 126
moyamoya disease 57–59 occipital condylar fracture paraganglioma, spinal 372–373 pseudoaneurysm, ICA 9, 63
clinical manifestations 59 330–331 differential diagnosis 373 pseudocysts, gelatinous 123, 124
differential diagnosis 59 imaging 331 imaging 372, 373 pseudomeningocele 351
imaging 58, 59 treatment 331 paraneoplastic neurological pyogenic abscess 92–94
treatment 59 odontogenic abscess 238–239 syndrome 199 differential diagnosis 93,
multiple sclerosis 75–76 imaging 239 pediatric posterior fossa tumors 125–126
differential diagnosis 76, 77 odontoid fracture 334–335 144–147 imaging 93, 94, 126
imaging 76 classification 335 differential diagnosis 146 pyriform aperture stenosis
mycetoma 101 type I 335 imaging 146 313–314
mycotic aneurysm 45 type II 335 penumbra 4 imaging 313
myelomalacia 389 type III 335 imaging 5
myxopapillary ependymoma see imaging 335 peri-ictal signal changes raccoon eyes 153
ependymoma treatment 335 206–207 radiation necrosis 187–192
olfactory neuroblastoma see differential diagnosis 207 clinical scenarios 191
naked facet sign 347 esthesioneuroblastoma imaging 207 imaging 191, 192
naso-orbital-ethmoidal fractures oligodendroglioma 135–136 perimedullary arteriovenous treatment 192
218–220 differential diagnosis 135 fistula see arteriovenous types of brain radiation
classification 219 imaging 135, 136 fistula changes 191
clinical presentation 220 treatment 136 peritonsillar abscess 234, 235, recurrent laryngeal nerve 307
complicated fractures 219 ophthalmic artery aneurysm 240 reperfusion hemorrhage after
imaging 219 262–263 perivascular space 155 carotid stenting 17–19
simple fractures 219 imaging 262, 263, 267 see also tumefactive differential diagnosis 19
treatment 220 ophthalmic vein thrombosis 256 perivascular space imaging 19
neonate optic neuritis 260–261 persistent hyperplastic primary retinal detachment 217
hypoxic–ischemic injury differential diagnosis 261 vitreous 311–312 retropharyngeal abscess 246–247
14–16 imaging 261 imaging 311, 312 differential diagnosis 246
imaging 15 treatment 261 petrous apex cholesterol imaging 246
intraventricular hemorrhage 15 orbital blow-out fracture granuloma 294–295 retropharyngeal calcific
neuro-Behçet’s syndrome 87–88 214–215 differential diagnosis 294 tendinitis 244
differential diagnosis 88 clinical presentation 215 imaging 294, 295 acute 244
imaging 88 imaging 214 treatment 295 reversible cerebral
treatment 88 treatment 215 petrous apicitis 253–255 vasoconstriction
neuroblastoma 153 types 214 clinical presentation 254 syndrome (RCVS) 23–24
metastatic 152–153 orbital cavernous hemangioma differential diagnosis 254 differential diagnosis 24
differential diagnosis 153 266–267 imaging 254 imaging 24
imaging 153 orbital cellulitis 248–249 treatment 255 rheumatoid arthritis, spinal
neurocysticercosis clinical presentation 248 pituitary apoplexy 274–275 388–389
differential diagnosis 113, 115 differential diagnosis 248 imaging 275 cord compression 389
parenchymal 112–113 imaging 248 pneumolabyrinth 228 differential diagnosis 389
imaging 113 treatment 249 polyradiculitis 96, 128 imaging 389
stages 113 orbital injury 216–217 posterior cerebral artery (PCA) treatment 389
ventricular 114–115 imaging 216 aneurysm 44
imaging 115 lens dislocation 216 multifocal stenosis 24 saccular aneurysm 45
neurocytoma 148–149 rupture 216 occlusion 21 see also cerebral aneurysm
differential diagnosis 148 orbital lymphoma see lymphoma posterior inferior cerebellar sarcoidosis 80
imaging 148, 149 orbital pseudotumor 268–269 artery aneurysm 44 see also neurosarcoidosis
neuromyelitis optica (NMO) classification 269 posterior reversible scolex see neurocysticercosis
82–84 differential diagnosis 268 encephalopathy seizure-related signal changes
differential diagnosis 83, 353 imaging 268, 269 syndrome 86, 174–176 207
imaging 83, 352, 353 orbital varix 264–265 differential diagnosis 176 sigmoid sinus thrombosis 32
spinal 352–353 imaging 265 imaging 176 sinus pericranii 64
clinical presentation 353 treatment 265 primary angiitis of the central differential diagnosis 64
treatment 84, 353 orbital wall fracture 214 nervous system (PACNS) imaging 64
neurosarcoidosis 79–81 osmotic demyelination 27–29 sinusitis
differential diagnosis 80, 361 syndrome 184 CSF analysis 29 ethmoid 93
imaging 80, 81, 361 osteomyelitis see skull base differential diagnosis 29 fungal 250–252
spinal 360–361 osteomyelitis imaging 29 allergic 279–280
treatment 81 treatment 29 classification 251
neurosyphilis 118–119 papilloma progressive multifocal clinical presentation 251
differential diagnosis 119 choroid plexus 167–168 leukoencephalopathy differential diagnosis 251,
imaging 118, 119 imaging 167, 168 (PML) 93, 125–126 280
399
Index
sinusitis (cont.) subperiosteal abscess 248 cerebral venous vascular malformations

imaging 251, 280 superior sagittal sinus thrombosis 320–321
treatment 252 thrombosis 33, thyroid ophthalmopathy differential diagnosis 321
skull base osteomyelitis 256–257 36 272–273 imaging 321
clinical presentation 257 supraglottitis 242–243 differential diagnosis 272 vasculitis 24
differential diagnosis 256 clinical presentation 243 imaging 272, 273 neurosyphilis 118
imaging 256, 257 differential diagnosis 243 treatment 273 vasospasm, after subarachnoid
treatment 257 imaging 243 tissue at ischemic risk 4 hemorrhage 47–49
skull base tumor 309–310 treatment 243 reperfusion injury 19 imaging 48
clinical presentation 310 systemic lupus erythematosus, tonsillar abscess 234, 235 treatment 49
differential diagnosis 309 neuropsychiatric Lemierre’s syndrome 236 venous malformation 321
imaging 309, 310 symptoms 86 tonsillar herniation 67, 146 ventriculomegaly 15, 96, 128
spinal cord tonsillitis 234–235 vertebral metastases see
contusion 343 Taenia solium 113 top of basilar syndrome 7, 26 metastases
edema 343 see also neurocysticercosis toxoplasmosis 120–121 Virchow–Robin space 155
hemorrhage 343 target sign 10, 96, 128 differential diagnosis 121 vitreous hemorrhage 217
infarction 322–323 tear-drop fracture 15, 63, 340 imaging 121 vocal cord paralysis 306–307
imaging 323 temporal bone fracture 228–229 treatment 121 clinical presentation 307
transection 346 clinical presentation 229 tPA thrombolysis, acute stroke differential diagnosis 307
see also central cord syndrome; complicating features 229 treatment 5 imaging 307
compressive myelopathy imaging 228 transverse myelitis 356–357 treatment 307
spinal epidural abscess see treatment 229 differential diagnosis 356 von Hippel–Lindau syndrome
abscess types 228 imaging 356, 357 370
spinal epidural hematoma see thoracolumbar fractures treatment 357
hematoma 345–347 transverse sinus thrombosis 36 Wallerian degeneration 210–211
spondylodiscitis 362–363 burst fracture 346 Treponema pallidum 119 differential diagnosis 211
differential diagnosis 363 Chance fracture 346 see also neurosyphilis imaging 211
imaging 363 imaging 346, 347 tuberculoma 96, 127–128 Wernicke’s encephalopathy 178,
stroke, anterior circulation 1–5 treatment 347 tuberculosis 95–97, 126 179–180
imaging 4, 5 thrombophlebitis 236 differential diagnosis 96, 128 clinical presentation 180
treatment 5 thrombotic microangiopathy imaging 96, 126 differential diagnosis 179
subacute necrotizing 20–22 tumefactive multiple sclerosis see imaging 179, 180
encephalomyelopathy see differential diagnosis 21 multiple sclerosis treatment 180
Leigh’s syndrome imaging 21 tumefactive perivascular space
subarachnoid hemorrhage thrombus 154–155 yeast 101
cerebral aneurysms 44 distal basilar artery 6–7 differential diagnosis 154 see also fungal infection
differential diagnosis 45 dural sinus 33–36 imaging 154, 155
reversible cerebral internal carotid artery 4 zygomaticomaxillary complex
vasoconstriction internal cerebral vein 21 ultrasound imaging, fractures 221–222
syndrome 24 middle cerebral artery 4 intraventricular clinical presentation 222
vasospasm and delayed sigmoid sinus 32 hemorrhage 15 complicating features 222
cerebral ischemia 47–49 superior sagittal sinus 33, 36 differential diagnosis 222
imaging 48 transverse sinus 36 vagus nerve 307 imaging 222
treatment 49 see also arterial thrombosis; injury 307 treatment 222
400

(A Case-Based Approach) Yang Tang, Sugoto Mukherjee, Max Wintermark - Emergency Neuroradiology - A Case-Based Approach-Cambridge University Press (2015)

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(A Case-Based Approach) Yang Tang, Sugoto Mukherjee, Max Wintermark - Emergency Neuroradiology - A Case-Based Approach-Cambridge University Press (2015)

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Emergency Neuroradiology

Max Wintermark MD MAS MBA

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7. Facial trauma 214 11. Temporal bone 288

David Chiao MD MPH University of Virginia Health System, Charlottesville,

ACA anterior cerebral artery DWI diffusion-weighted imaging

NECT non-enhanced computed tomography RCVS reversible cerebral vasoconstriction syndrome

1 Yang Tang, Xinli Du, Sugoto Mukherjee, and Max Wintermark

Figure 1.1.1 (patient 1) Figure 1.1.2 (patient 1)

Figure 1.1.3 (patient 1) Figure 1.1.4 (patient 1)

Figure 1.1.5 (patient 1)

Figure 1.1.7 (patient 2) Figure 1.1.8 (patient 2)

Figure 1.1.9 (patient 2) Figure 1.1.10 (patient 3)

Figure 1.1.11 (patient 3) Figure 1.1.12 (patient 3)

Figures 1.1.7–1.1.9 CT perfusion images show moderately prolonged TTD

Figures 1.1.11–1.1.13 CT perfusion images show markedly prolonged TTD

Figure 1.2.1 Figure 1.2.2

Figure 1.2.3 Figure 1.2.4

Figure 1.3.1 Figure 1.3.2

Figure 1.3.3 Figure 1.3.4

Figure 1.3.5 Figure 1.3.6

r Atherosclerosis: commonly at carotid bifurcation or of

Figure 1.4.1 (patient 1) Figure 1.4.2 (patient 1)

Figure 1.4.3 (patient 1) Figure 1.4.4 (patient 1)

Figure 1.4.5 (patient 1) Figure 1.4.6 (patient 1)

Figure 1.4.7 (patient 2) Figure 1.4.8 (patient 2)

Figure 1.5.1 Figure 1.5.2

Figure 1.5.3 Figure 1.5.4

Figure 1.6.1 Figure 1.6.2

Figure 1.6.3 Figure 1.6.4

Figure 1.6.5 Figure 1.6.6

Figure 1.6.7 Figure 1.6.8

r Other causes of intraparenchymal hemorrhage such as

Findings Key points

Figure 1.7.1 Figure 1.7.2

Figure 1.7.3 Figure 1.7.4

Figure 1.7.5 Figure 1.7.6

Figure 1.8.1 Figure 1.8.2

Figure 1.8.3 Figure 1.8.4

History neurological symptoms, and transient, multifocal

Figure 1.9.1 Figure 1.9.2

Figure 1.9.3 Figure 1.9.4

Figure 1.9.5 Axial FLAIR image demonstrates corresponding FLAIR

Figure 1.10.1 (patient 1) Figure 1.10.2 (patient 1)

Figure 1.10.3 (patient 1) Figure 1.10.4 (patient 2)

Figure 1.10.5 (patient 2) Figure 1.10.6 (patient 2)

Figure 1.10.7 (patient 2) Figure 1.10.8 (patient 2)

History septic emboli, and vasospasm from subarachnoid

Figure 1.11.1 (patient 1) Figure 1.11.2 (patient 1)

Figure 1.11.3 (patient 1) Figure 1.11.4 (patient 1)

Figure 1.11.5 (patient 1) Figure 1.11.6 (patient 1)

Figure 1.11.7 (patient 2) Figure 1.11.8 (patient 2)

Figure 1.11.9 (patient 2) Figure 1.11.10 (patient 2)

Figure 1.11.10 CT venogram confirms right sigmoid sinus thrombosis (black

Findings Differential diagnosis

well as hypercoagulability secondary to systemic processes – Subacute stage: hyperintense on T1,

Figure 1.12.1 (patient 1) Figure 1.12.2 (patient 1)

Figure 1.12.3 (patient 1) Figure 1.12.4 (patient 1)

Figure 1.12.5 (patient 2) Figure 1.12.6 (patient 2)

Figure 1.12.7 (patient 2) Figure 1.12.8 (patient 2)

Figure 1.13.1 Figure 1.13.2

Figure 1.13.3 Figure 1.13.4