Learning Neuroimaging - 100 Essential Cases

Learning Imaging
Series Editors:
R. Ribes · A. Luna · P.R. Ros
Francisco de Asís Bravo-Rodríguez
Rocío Díaz-Aguilera
Luiz Celso Hygino da Cruz Jr.
(Editors)
Learning
Neuroimaging
100 Essential Cases
FRANCISCO de ASÍS BRAVO-RODRÍGUEZ LUIZ CELSO HYGINO da CRUZ JR.
Reina Sofía University Hospital CDPI and IRM Ressonancia Magnetica
Diagnostic and Therapeutics Neuroradiolgy Universidade Federal do Rio de Janeiro
Menéndez Pidal Rua Capitao Salomao 44 - Botafogo
14004 Córdoba Rio de Janeiro
Spain 22171-040
fasisbravo@gmail.com Brazil
celsohygino@hotmail.com
ROCÍO DÍAZ-AGUILERA
Department of Radiology
Alto Guadalquivir Hospital
Av. Blas Infante s/n
23740 Andújar, Jaén
Spain
rdiazaguilera@gmail.com
ISBN: 978-3-642-22998-5 e-ISBN: 978-3-642-22999-2

DOI: 10.1007/978-3-642-22999-2
Springer Heidelberg Dordrecht London New York
Library of Congress Control Number: 2011940861

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To my sons Paco and Rafa,
everything and forever
To my family, especially to Juan,

for his love and endless support
To my parents, Luiz Celso and Leonice

by example and dedication.
To Simone, incomparable woman,

synonymous of love and tenderness.
Luiz Celso Hygino da Cruz jr.
Acknowledgments
The authors wish to thank the radiology residents and the staff in the CT, MRI and
Emergency Radiology units of the Radiology Department of Reina Sofía Universitary
Hospital for their support and contribution to the preparation of this book.

To the clinical staff of the department of Radiology of CDPI and IRM, as well as to Romeu
Domingues for his support and stimulus.
Luiz Celso Hygino da Cruz jr.

Contents
1 Brain Tumors
Francisco de Asís Bravo-Rodríguez and Rocío Díaz-Aguilera . . . . . . . 1
Case 1 Brain Parenchymal Metastases. . . . . . . . . . . . . . . . . . . . . . . . . . . . 2
Case 2 Anaplastic Astrocytoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4
Case 3 Ganglioglioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6
Case 4 Oligodendroglioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8
Case 5 Dysembryoplastic Neuroepithelial Tumor (DNET) . . . . . . . . . . 10
Case 6 Intracranial Ependymoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12
Case 7 Hemangioblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14
Case 8 Medulloblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16
Case 9 Pleomorphic Xanthoastrocytoma . . . . . . . . . . . . . . . . . . . . . . . . . 18
Case 10 Meningioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 20
Further Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 22
2 Orbit and Sellar Region

Case 1 Choroidal Melanoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 24
Case 2 Grave’s Ophthalmopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26
Case 3 Inflammatory Pseudotumor of the Orbit . . . . . . . . . . . . . . . . . . . 28
Case 4 Orbital Hemangioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30
Case 5 Orbital Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32
Case 6 Optic Nerve Sheath Meningioma. . . . . . . . . . . . . . . . . . . . . . . . . . 34
Case 7 Craniopharyngioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 36
Case 8 Hypothalamic Hamartoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38
Case 9 Pituitary Adenoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40
Case 10 Rathke’s Cleft Cyst . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42
Further Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 44
3 Ear, Nasal, and Paranasal Sinuses

Case 1 Antrochoanal Polyp . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 46
Case 2 Inverted Papilloma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 48
Case 3 Nasosinusal Polyposis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 50
Case 4 Sinus Mucocele . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 52
Case 5 Osteoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 54
Case 6 Rhinocerebral Mucormycosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . 56
Case 7 Sinonasal Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 58
Case 8 Acoustic Neuroma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 60
X Contents
Case 9 Cholesteatoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 62
Case 10 Jugulotympanic Glomus Tumor . . . . . . . . . . . . . . . . . . . . . . . . . . 64
Further Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 66
4 Orocervical Region
Case 1 Adenoid Cystic Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 68
Case 2 Branchial Cleft Cyst . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 70
Case 3 Carotid Body Paraganglioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 72
Case 4 Cervical Abscess . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 74
Case 5 Juvenile Nasopharyngeal Angiofibroma . . . . . . . . . . . . . . . . . . . . 76
Case 6 Laryngeal Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 78
Case 7 Nasopharyngeal Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 80
Case 8 Pleomorphic Adenoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 82
Case 9 Warthin’s Tumor . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 84
Case 10 Sialolithiasis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 86
Further Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 88
5 Spine
Case 1 Spondylodiscitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 90
Case 2 Spinal Metastatic Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 92
Case 3 Spinal Hemangioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 94
Case 4 Spinal Neurinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 96
Case 5 Spinal Meningioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 98
Case 6 Spinal Epidural Hematoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 100
Case 7 Spinal Cord Astrocytoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 102
Case 8 Myxopapillary Ependymoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 104
Case 9 Transverse Myelitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 106
Case 10 Spinal Cord Vascular Malformation . . . . . . . . . . . . . . . . . . . . . . . 108
Further Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 110
6 Neuropediatric
Luiz Celso Hygino da Cruz Jr., Raquel Ribeiro Batista, Taísa Davaus
Gasparetto, and Cláudio de Carvalho Rangel . . . . . . . . . . . . . . . . . . . . . . 111
Case 1 X-Linked Adrenoleukodystrophy . . . . . . . . . . . . . . . . . . . . . . . . . 112
Case 2 Alexander Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 114
Case 3 Septo-Optic Dysplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 116
Case 4 Pilocytic Astrocytoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 118
Case 5 Lissencephaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 120
Case 6 Acute Disseminated Encephalomyelitis (ADEM) . . . . . . . . . . . . 122
Case 7 Chiari I Malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 124
Case 8 Congenital Cytomegalovirus Infection . . . . . . . . . . . . . . . . . . . . . 126
Case 9 Dandy-Walker Malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 128
Case 10 Neurofibromatosis Type 1 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 130
Further Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 132
Contents XI
7 Vascular Diseases
Case 1 Carotid Artery Dissection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 134
Case 2 Cerebral Venous Thrombosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 136
Case 3 Cavernous Angioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 138
Case 4 Cerebral Dural Arteriovenous Fistula . . . . . . . . . . . . . . . . . . . . . . 140
Case 5 Subarachnoid Hemorrhage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 142
Case 6 Moyamoya Disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 144
Case 7 Posterior Reversible Encephalopathy Syndrome. . . . . . . . . . . . . 146
Case 8 Intraparenchymal Haematoma . . . . . . . . . . . . . . . . . . . . . . . . . . . 148
Case 9 Vein of Galen Malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 150
Case 10 Cranial Nerve Neurovascular Compression . . . . . . . . . . . . . . . . . 152
Further Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 154
8 Diffusion and Spectroscopy

Luiz Celso Hygino da Cruz Jr. and Rafael Ferracini Cabral . . . . . . . . . 155
Case 1 Acute Ischemic Stroke . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 158
Case 2 Bipolar Affective Disorder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 160
Case 3 Canavan Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 162
Case 4 Epidermoid Tumor . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 164
Case 5 Epidural Empyema. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 166
Case 6 Hepatic Encephalopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 168
Case 7 HIV Encephalitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 170
Case 8 Leigh Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 172
Case 9 Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 174
Case 10 Pyogenic Brain Abscess . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 176
Further Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 178
9 DTI and Bold MR Imaging

Luiz Celso Hygino da Cruz Jr., Isabela Garcia Vieira,
and Tatiana Chinem Takayassu . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 179
Case 1 Semilobar Holoprosencephaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . 182
Case 2 Low-Grade Glioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 184
Case 3 Gliomatosis Cerebri . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 186
Case 4 Alzheimer Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 188
Case 5 Arteriovenous Malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 190
Case 6 Amyotrophic Lateral Sclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . 192
Case 7 Diffuse Axonal Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 194
Case 8 Multiple Sclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 196
Case 9 Focal Cortical Dysplasia and Seizure . . . . . . . . . . . . . . . . . . . . . . 198
Case 10 High-Grade Tumor . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 200
Further Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 202
XII Contents
10 Miscellaneous
Case 1 Arachnoid Cyst . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 206
Case 2 Colloid Cyst . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 208
Case 3 Cerebral Toxoplasmosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 210
Case 4 Herpes Simplex Encephalitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 212
Case 5 Meningitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 214
Case 6 Neuro-Behçet’s Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 216
Case 7 Progressive Multifocal Leukoencephalopathy . . . . . . . . . . . . . . . 218
Case 8 Intracranial Hypotension Syndrome. . . . . . . . . . . . . . . . . . . . . . . 220
Case 9 Acute Head Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 222
Case 10 Ranula . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 224
Further Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 226
Contributing Authors
Antonio Cano Sanchez Isabela Garcia Vieira

Diagnostic and Therapeutical Neuroradiology Unit Hospital Unimed Chapecó
Department of Radiology Chapecó, Santa Catarina
Reina Sofía University Hospital Brazil
Córdoba
Spain Elvira Jimenez Gomez
Claudio de Carvalho Rangel Diagnostic and Therapeutical Neuroradiology Unit
Departamento de Clínica de Diagnóstico por Imagem Department of Radiology
(CDPI) e IRM Reina Sofía University Hospital
Radiologia do Hospital Central da Polícia Militar do Córdoba
Estado do Rio de Janeiro Spain
Rio de Janeiro
Brazil Claudia S. Linares Gonzalez
Tatiana Chinem Takayassu Alto Guadalquivir Hospital
Clínica de Diagnóstico por Imagem (CDPI) Andújar, Jaén
e Multimagem Spain
Santa Casa de Misericórdia do Rio de Janeiro
Rio de Janeiro
Rafael Oteros Fernandez
Brazil
Diagnostic and Therapeutical Neuroradiology Unit
Taísa Davaus Gasparetto Department of Radiology
Clínica de Diagnóstico por Imagem (CDPI) Reina Sofía University Hospital
Universidade Federal do Rio de Janeiro Córdoba
Rio de Janeiro Spain
Brazil
Fernando Delgado Acosta Manuel J. Ramos Gómez

Diagnostic and Therapeutical Neuroradiology Unit Diagnostic and Therapeutical Neuroradiology Unit
Department of Radiology Department of Radiology
Reina Sofía University Hospital Reina Sofía University Hospital
Córdoba Córdoba
Spain Spain
Rafael Ferracini Cabral Raquel Ribeiro Batista

Clínica de Diagnóstico Clínica de Diagnóstico por Imagem
por Imagem (CDPI) (CDPI) e Multimagem
Universidade Federal do Rio de Janeiro Universidade Federal do Rio de Janeiro
Rio de Janeiro Rio de Janeiro
Brazil Brazil
XIV Contributing Authors
Elisa Roldan Romero

Diagnostic and Therapeutical Neuroradiology Unit
Reina Sofía University Hospital
Córdoba
Spain
Brain Tumors 1
Francisco de Asís Bravo-Rodríguez and Rocío Díaz-Aguilera
Contents
Case 1 Brain Parenchymal Metastases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2
Case 2 Anaplastic Astrocytoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4
Case 3 Ganglioglioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6
Case 4 Oligodendroglioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8
Case 5 Dysembryoplastic Neuroepithelial Tumor (DNET). . . . . . . . . . . . . . . 10
Case 6 Intracranial Ependymoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12
Case 7 Hemangioblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14
Case 8 Medulloblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16
Case 9 Pleomorphic Xanthoastrocytoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 18
Case 10 Meningioma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 20
F. de Asís Bravo-Rodríguez et al. (eds.), Learning Neuroimaging, Learning Imaging,

DOI 10.1007/978-3-642-22999-2_1, © Springer-Verlag Berlin Heidelberg 2012
2 Francisco de Asís Bravo-Rodríguez and Rocío Díaz-Aguilera
History A 75-year-old man presented with acute onset of left hemiparesis accompanied by
Case 1 central facial palsy. The patient’s chest X-ray showed a mass in the left lower lobe
consistent with bronchogenic carcinoma.
Brain Parenchymal
Metastases
Comments Metastases to the brain are by far the most common intracranial tumors in adults,
approximately ten times more common than primary tumors. The most frequent
malignancies to metastasize to brain are, in descending order, lung, breast, and
malignant melanoma. These metastases result from hematogenous spread and usually
a b
c d
Fig. 1.1
Brain Tumors 3
localize at the gray-white interface where the caliber of blood vessels changes, thereby
trapping tumor emboli. Nevertheless, they can be located anywhere. Eighty percent of the
brain lesions occur in the cerebral hemispheres, 15% in the cerebellum, and 5% in the
brainstem. Cerebral metastases typically appear as well-defined nodules of varying sizes that
can be solid or partially cystic, surrounded by extensive perifocal edema frequently
disproportionated to the size of the metastasis.
Clinical presentation varies from asymptomatic patients, whose cerebral lesions are
detected incidentally during workup evaluation, to patients with severe neurologic impair-
ment. Headache, related to mass effect, is the most common presenting symptom and
occurs in approximately 35% of the patients. The new onset of headaches in a patient who
has not previously suffered from headaches is most characteristic.
The detection and characterization of brain metastases is crucial because the presence of
even a single brain lesion dramatically changes patient prognosis and maybe therapy as well.
Most patients with a known primary tumor undergo imaging studies when neurologic signs
and symptoms develop. Currently, magnetic resonance imaging (MRI) with contrast enhance-
ment is the technique of choice, because MRI is more sensitive and specific compared to other
imaging modalities in determining the presence, location, and number of metastases. Contrast-
enhanced computed tomography (CT) scan is used widely because of its accessibility and low
cost. On nonenhanced CT scan, the majority of metastases are isodense to the adjacent brain
parenchyma and present extensive associated edema. In some cases, edema is the only abnor-
mality detected on un-enhanced CT. Hyperdense metastases are seen with some neoplasms as
small round cell tumors. Sometimes, cerebral metastases may bleed. Hemorrhage occurs most
commonly in metastases from renal and breast carcinomas, melanoma, and choroicarcinoma.
After intravenous contrast administration, most brain metastases present intense enhance-
ment showing both solid and ring-like patterns. With regard to MRI, most lesions are slightly
hypointense to brain on T1-weighted images, except hemorrhagic and malignant melanoma
metastases that usually appear hyperintense due to the presence of blood and melanin respec-
tively. On T2-weighted images, metastases are commonly hyperintense. Surrounding edema is
relatively hypointense on fluid-attenuated inversion recovery (FLAIR) and T1-weighted
images and hyperintense on T2-weighted images. The majority of lesions enhance strongly
after contrast administration, showing the same patterns than on CT. The usefulness of diffu-
sion-weighted and perfusion-weighted imaging and proton-MR spectroscopy in the initial
diagnosis of brain metastases has not been established.
The prognosis for patients with brain metastases typically is poor.
Urgent noncontrast CT-scan (Fig. 1.1a) demonstrates a right frontal lobe mass with a low- Imaging Findings
density center and an isohyperdense peripheral component. The lesion is surrounded by
extensive edema. Cerebral MRI was performed 24 h later. On FLAIR images (Fig. 1.1b), the
digitiform edema is better depicted. After gadolinium administration (Fig. 1.1c, d), another
small lesion is detected in the left parietal lobe, at the gray-white junction (arrow in
Fig. 1.1c). Contrast-enhanced images demonstrate that the larger lesion extends into the
dura (white arrow in Fig. 1.1d). Note the adjacent dural thickening and enhancement (black
arrow in Fig. 1.1d) related to metastatic meningeal infiltration.
History A 56-year-old man was referred to a neurologist for

Case 2 progressive headache that had become more severe in
the last weeks. On physical examination, bilateral
Anaplastic Astrocytoma papilledema was found.
a b
c d
Fig. 1.2
Brain Tumors 5
Approximately half of all primary brain neoplasms are glial cell tumors and more than 75% Comments
of all gliomas are astrocytomas. The most common supratentorial tumor in all age groups
is astrocytoma. The World Health Organization (WHO) classification for infiltrating
astrocytoma considers three grades of ascending malignancy: grade II, low-grade
astrocytoma; grade III, anaplastic astrocytoma; and grade IV, glioblastoma multiforme.
Hence, anaplastic or malignant astrocytomas occupy an intermediate position between
low-grade astrocytoma and glioblastoma multiforme. WHO grade I corresponds to
pilocytic astrocytoma. This tumor can be found at any age but it is more common in older
patients. The peak incidence is in the fifth and sixth decades of life. There is slight male
gender predominance with a male-to-female ratio of 1.87:1. Anaplastic astrocytoma usually
involves the frontal and temporal lobes although it may occur throughout the cerebral
hemispheres. The tumor typically spreads through white matter tracts, ependyma,
leptomeninges, and cerebrospinal fluid (CSF).
The most frequent presenting symptoms are headache, depressed mental status, and
focal neurological deficits. Seizures are less common among patients with anaplastic astro-
cytomas compared to low-grade astrocytomas.
Imaging studies, such as CT scans and MRI (with and without contrast), are crucial in
the diagnosis, grading, and pathophysiological evaluation of astrocytomas. MRI is consid-
ered the imaging technique of choice, but a CT scan may be useful in the acute setting or
when MRI is contraindicated. New MR techniques like MR spectroscopy and perfusion-
weighted MRI have proven to yield valuable information on the initial assessment of brain
tumors. On noncontrast CT scan, anaplastic astrocytoma appears as inhomogeneous low-
density lesions surrounded by varying amounts of peripheral edema. Calcifications are
rare. After contrast material administration, the tumor shows intense and heterogeneous
enhancement. On MRI, anaplastic astrocytomas appear as poorly defined lesions with het-
erogeneous signal intensities, predominantly isointense on T1-weighted images and hyper-
intense on T2-weighted images. Most anaplastic astrocytomas enhance strongly and
irregularly following paramagnetic contrast agent administration, commonly showing a
ring-like pattern. The tumor usually presents moderate mass effect. MR spectroscopy can
help increase the ability to predict the tumor grade. As the grade of the tumor increases,
NAA and creatinine decrease and choline, lipids, and lactate increase. Perfusion-weighted
MRI shows increased relative cerebral blood volume (rCBV).
The treatment of anaplastic astrocytomas includes surgery, radiotherapy, and adjuvant
chemotherapy. Anaplastic astrocytomas are usually more responsive to chemotherapy
than glioblastomas.
MRI demonstrates a large mass located in the left frontal lobe with contralateral frontal Imaging Findings
lobe compression. The lesion has heterogeneous signal intensity on T1-weighted image
(Fig. 1.2a) and shows inhomogeneous enhancement following gadolinium administration
(Fig. 1.2b). MR spectroscopy (Fig. 1.2c) shows increased level of choline (arrow), decreased
peak of NAA (asterisk), and presence of lactate (arrowhead), which is elevated in necrotic
areas (e.g., higher grade tumors). On perfusion-weighted MR (Fig. 1.2d), the tumor shows
increased relative cerebral blood volume (rCBV) related to angiogenesis.
Case 3
Ganglioglioma
a b
c d
Fig. 1.3
Brain Tumors 7
A 16-year-old boy presented with two episodes of orofacial automatisms (masticatory History
movements) accompanied by activity interruption and bewilderment. These symptoms
were classified as complex partial seizures by the neurologist.
Gangliogliomas are relatively benign slow-growing tumors composed of mixed neuronal Comments
and glial elements. They are rare, accounting for only 0.4–1.3% of all brain tumors, and are
most often found in the temporal lobes and cerebellar hemispheres. Gangliogliomas are
usually small and well-demarcated masses. The common appearance is a cyst with a
partially calcified mural nodule. Most gangliogliomas are observed in patients younger
than 30 years with no gender predominance.
Presentation depends on patient age and tumor location. Seizures are the most common
presenting symptom, followed by signs of increased intracranial pressure. Temporal lobe
gangliogliomas usually present with temporal lobe seizures.
On CT, the classic appearance is a cyst with an iso- or hypodense mural nodule that is
often partially calcified. Nevertheless, gangliogliomas may show variable density and
enhancement patterns, with nonspecific imaging features. As the tumor is slow-growing,
when it is located peripherally, it may cause focal calvarial erosion. The MR imaging findings
in gangliogliomas are nonspecific. The lesions may be iso- to hypointense on T1-weighted
images and hyperintense on T2-weighted images. MRI is useful in differentiating the cystic
and solid components of the tumor. Cystic components occur in about 60% of ganglio-
gliomas; in the remaining cases, the lesion consists entirely of solid portions. The cystic
components may show higher signal intensity than CSF on T2-weighted images, which is
related to gelatinous material content. Contrast enhancement may have either a nodular
rim or solid enhancement pattern.
The treatment of choice is surgical resection, which is generally curative. Recurrence is
rare following gross total resection of the tumor. Metastatic spread is extremely rare.
Axial T1-weighted (Fig. 1.3a) and T2-weighted (Fig. 1.3b) MR images demonstrate a mass Imaging Findings
located in the left temporal lobe. The mass presents an anterior cystic content, which
appears hypointense on T1-WI and hyperintense on T2-WI, and a posterior solid
component, which shows signal intensity similar to brain parenchyma on T1-WI and high
signal intensity on T2-WI, but lower than the cystic portion. Following gadolinium
administration (Fig. 1.3c, d), a predominantly peripheral enhancement can be seen, related
to the solid component of the mass.
History A 57-year-old woman was referred to emergency department for two epileptic
Case 4 seizures accompanied by postcritical period. On admission, she presented with
aphasia and oral commissure displacement.
Oligodendroglioma
Comments Oligodendrogliomas (ODs) are primary glial brain tumors that are divided into
grade II and anaplastic grade III tumors (WHO criteria). It is the third most
common glioma overall, accounting for 2–5% of primary brain tumors and 5–10%
of all glial neoplasms. It shows a male predominance in gender (ratio 2:1), and the
peak incidence is the fourth and fifth decades. The vast majority of all
oligodendroglial tumors occur in the cerebral hemispheres with the frontal lobe
the most common location overall (50–65%), followed by the temporal lobe (47%),
a b
c d
Fig. 1.4
Brain Tumors 9
the parietal lobe (7–20%), and the occipital lobe (1–4%). Oligodendrogliomas are typically
unencapsulated but well-circumscribed focal white matter tumors that may extend into the
cortex and leptomeninges. They are very cellular neoplasms, with uniform nuclei. Foci of
cystic degeneration are relatively common, but hemorrhage and necrosis are rare.
Typically, oligodendrogliomas have an indolent course due to its slow growth, and
patients may survive for many years after symptom onset. Seizures are the most common
presenting manifestation, observed at diagnosis in as many as half of patients. Other
symptoms include headache, symptoms of increased intracranial pressure, or focal neuro-
logical deficits.
Diagnostic imaging studies play an integral role in tumor management. MRI is consid-
ered the technique of choice. It is very useful not only for tumor diagnosis and classifica-
tion but also for treatment planning and posttreatment surveillance. At neuroimaging,
oligodendrogliomas typically manifest as a round or oval well-defined mass involving the
cortex or subcortical white matter. On un-enhanced CT scan, about 60% are hypoattenuat-
ing while 23% are isoattenuating and about 6% are hyperattenuating. Intratumoral calcifi-
cation is common; hemorrhage and cystic degeneration are detected occasionally. When
the mass is sufficiently exophytic, calvarial erosion may be noted. After intravenous con-
trast administration, the tumor does not enhance unless it is behaving unusually aggres-
sively or has an anaplastic astrocytic component. MRI is superior to CT in defining the full
extent of tumor involvement. The neoplasm is usually hypointense compared to gray mat-
ter on T1-WI and hyperintense compared to gray matter on T2-WI. Heterogeneity of this
signal intensity is the rule. Less commonly, a large cyst-like pattern may be seen. Surrounding
vasogenic edema is not usual. “Dot-like” lacy enhancement is commonly seen following
gadolinium administration, but many tumors may not enhance at all. Advanced MR imag-
ing with the apparent diffusion coefficient (ADC) shows a characteristic but not pathogno-
monic difference between low-grade and high-grade glial neoplasms. Lower ADC values,
indicative of water restriction and likely reflective of lowered extracellular hyaluronic acid,
are noted in high-grade tumors compared to the higher ADC values seen in low-grade
tumors. The typical perfusion imaging feature for primary neoplasms of the brain is a rela-
tive tumor blood volume (rTBV) that tends to increase with neoplasm grade. Markedly
elevated rTBV has been observed in particular with low-grade oligodendrogliomas.
Therapy options vary from conservative treatment of some patients with serial imaging
studies and no intervention to aggressive multimodal treatment including surgical
resection, radiotherapy, and chemotherapy in others. Oligodendrogliomas have good
prognosis relative to other parenchymal tumors probably because of their inherently less
aggressive biological behavior.
Urgent un-enhanced CT scan (Fig. 1.4a) shows an area of hypodensity involving the left Imaging Findings
temporal and occipital lobes with coarse calcifications. Axial FLAIR images (Fig. 1.4b),
reveals ill-defined hyperintensity in the left temporal and occipital lobes with mild mass
effect but without clear tumor demarcation. T1-WI before (Fig. 1.4c) and after (Fig. 1.4d)
gadolinium administration demonstrate a 1-cm enhancing nodule and slight ill-defined
enhancement (arrow) adjacent to the nodule.
Case 5
Dysembryoplastic Neuroepithelial Tumor (DNET)
a b
c d
Fig. 1.5
Brain Tumors 11
A 36-year-old woman was referred to the neurologist for a long history of antiepileptic drug History
intake due to a brain tumor. The patient did not have more information about her treatment
process and did not provide any medical report. Neuroimaging studies were performed.
Dysembryoplastic neuroepithelial tumor (DNET) is a rare low-grade, mixed neuronal and Comments
glial tumor, usually seen in young patients and associated with pharmacologically intractable
complex partial or generalized seizures. The most common locations are the temporal or
frontal lobes; parietal lobe involvement has been also described. Other sites are very rare.
The tumor is characterized by its intracortical location, multinodular architecture, and
heterogeneous cellular composition. Usually the lesions are clinically and radiologically
stable for years. DNET are benign tumors, and malignant transformation is extremely rare.
Histologically, three types of DNET have been described.
1. The complex form is characterized by the association of a specific glioneuronal element

(SGNE) with glial nodules and a multinodular architecture. Foci of cortical dysplasia
are common.
2. The simple form demonstrates only the SGNE.
3. The third, “nonspecific” form of DNET does not show the SGNE but displays the same
clinical and neuroimaging features as complex DNET.
Clinical presentation of this tumor is stereotyped. The patient typically presents with a long
history of partial drug-resistant seizures and with absence of progressive neurologic deficit.
Neuroimaging features seem to be very useful in the diagnosis of DNET. Daumas-Duport
et al. established the clinical-radiologic criteria of DNET as follows:
1. Partial seizures, with or without secondary generalization, beginning before the age of
20 years.
2. No neurologic deficit or stable congenital deficit.
3. Cortical location of the lesion as best demonstrated by MR imaging, and
4. Absence of mass effect and peritumoral edema at imaging.
Neuroimaging studies usually show a predominantly cortical and well-defined lesion

that is not associated with peritumoral edema or mass effect. The tumor appears hypoin-
tense on T1-weighted MR images and hyperintense on T2-weighted MR images. Following
contrast material administration, most DNETs do not display contrast enhancement.
The main differential diagnoses of DNETs are oligodendrogliomas and gangliogliomas.
Complete surgical resection without any adjuvant therapies remains the treatment of
choice. Surgical excision provides the best chance to cure epilepsy and prevents hemorrhagic
complications.
Axial and coronal T1-weighted images (Fig. 1.5a, b) and axial T2-weighted images (Fig. 1.5c) Imaging Findings
demonstrate a right temporal lobe mass located in the hippocampal region. The lesion
presents multinodular aspect, which is better detected on T2-WI (Fig. 1.5c). After intravenous
gadolinium administration (Fig. 1.5d), the tumor does not show enhancement.
History A 27-year-old man presented with a 2-week history of

Case 6 progressive headache. The patient also complained of
gait disturbances, nausea, and somnolence for the past
Intracranial Ependymoma 2 days.
c d
Fig. 1.6
Brain Tumors 13
Ependymomas are glial tumors that arise from ependymal cells that line the ventricular Comments
walls and central canal of the spinal cord, so they may be found intracranially and in the
spine. Intracranial ependymomas usually present as intraventricular masses, with common
extension into the subarachnoid space, while spinal ependymomas usually present as
intramedullary masses arising from the central canal. Intracranial ependymomas account
for 2–8% of all primary intracranial brain tumors. They generally present in young
children with a mean age of diagnosis of 4 years, but there is a second smaller peak
incidence in the mid-thirties. There is no gender predominance. The most common
location of ependymoma is the fourth ventricle. The tumor has a tendency to expand this
ventricle and extrude through its outlet foramina into the cisterna magna, the
cerebellopontine angle cisterns, and through the foramen magnum into the upper cervical
spine behind the cervicomedullary junction.
The clinical manifestations associated with ependymomas vary depending on the age of
the patient and the location of the lesion. Common presenting symptoms include progres-
sive lethargy, disequilibrium, headache, nausea, and vomiting secondary to increased
intracranial pressure from obstructive hydrocephalus.
Ependymomas have some characteristic features on CT scan and MRI that help narrow
the differential diagnosis. On noncontrast CT scan, most ependymomas are isodense and
approximately one half of cases exhibit calcifications. These tumors show minimal-to-mod-
erate heterogeneous enhancement after contrast material administration. Cyst formation is
common, but overt hemorrhage is uncommon. On MRI, ependymomas demonstrate vari-
able signal intensity on both T1 and T2 WI secondary to necrosis, hemorrhage, and calcifica-
tion, but they are usually hypointense to isointense on T1-weighted images and hyperintense
compared with gray matter on T2-weighted images. Moderate inhomogeneous enhance-
ment after gadolinium intravenous injection is typical. The classic appearance of a posterior
fossa ependymoma is a lobulated soft tissue mass that appears to form a cast of the fourth
ventricle and extends out the foramina into the adjacent subarachnoid cisterns.
The differential diagnosis includes arteriovenous malformation, astrocytoma, choroid
plexus papilloma, and glioblastoma multiforme.
Treatment of intracranial ependymomas may include surgery, radiation therapy, and
chemotherapy. Surgical resection is the therapy of choice, and total resection is
recommended if possible. Radiation therapy is necessary after incomplete resections. It
seems to increase the survival period and delay recurrence for several years. Chemotherapy
has shown little benefit in improvement of survival.
Urgent nonenhanced CT scan (Fig. 1.6a) shows hydrocephalus secondary to a posterior Imaging Findings
fossa mass that compresses the fourth ventricle. The mass exhibits some calcifications
(arrow). Sagittal T2-weighted MR image (Fig. 1.6b) demonstrates a large heterogeneous
mass that arises from the floor of the fourth ventricle and protrudes through the foramen
magnum into the upper cervical spine. The tumor is almost completely filling the foramen
magnum and causes compression and anterior displacement of the bulb (arrow in Fig. 1.6c).
Axial (Fig. 1.6c) and sagittal (Fig. 1.6d) contrast-enhanced T1-WI reveal intense
heterogeneous enhancement of the tumor.
History A 37-year-old woman, with a several-month history of

Case 7 progressive headache, presented with vertigo and ataxia
for the last few days.
Hemangioblastoma
a b
c d
Fig. 1.7
Brain Tumors 15
Hemangioblastoma is a benign vascular neoplasm of unknown origin that arises almost Comments
exclusively in the central nervous system. It accounts for approximately 2% of intracranial
tumors. The cerebellar hemispheres and vermis are the most common locations, although
they can also be found in the brainstem, spinal cord, retina, and cerebrum. Most
hemangioblastomas occur sporadically; 10–20% occur as part of von Hippel-Lindau
syndrome. Histologically, the tumors are composed of multiple capillary and sinusoidal
channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. On
gross pathology, hemangioblastomas usually appear as well-defined cystic masses with a
mural nodule that abuts a pial surface. The lesion almost never calcifies. Hemangioblastomas
show male gender predominance (male:female ratio of 2:1), and are typically adult tumors
that rarely affect children; the usual age at diagnosis is between the third and fifth decades.
The clinical presentation of hemangioblastomas depends on the anatomical location
and growth patterns. Cerebellar lesions may present with signs of cerebellar dysfunction,
such as ataxia and discoordination, or with symptoms of increased intracranial pressure
due to associated hydrocephalus. Other presenting symptoms include headache, disequi-
librium, nausea/vomiting, and dizziness/vertigo.
CT may be used in the investigation of cerebellar hemangioblastoma, but MRI is the
preferred imaging study. On noncontrast CT scan, hemangioblastoma commonly pres-
ents as a large, low-density, cystic-appearing cerebellar mass. A peripheral mural nodule
may not be evident on noncontrast studies. After intravenous contrast administration, a
mural nodule that enhances intensely can be detected in the majority of cases (75%). On
MRI, the cystic component of the lesion appears hypointense compared to brain on
T1-weighted images and hyperintense on T2-WI. Solid component of the tumor has vari-
able signal intensity characteristics, but strongly enhances following contrast material
administration. Prominent serpentine “flow voids” can often be identified. A peripheral
rim of edema may also be seen in association with tumor. Hemangioblastoma has a typi-
cal angiographic appearance. It is a large avascular posterior fossa tumor with a small
highly vascular mural nodule. The tumor nidus usually shows a dense and prolonged
staining on angiography.
Differential diagnosis includes cystic astrocytoma and cystic metastases. Cystic astro-
cytomas have frequent calcifications within larger nodules, cyst wall is very thick, and they
lack angiographic blush of mural nodule.
The treatment of choice of hemangioblastoma is surgical resection. Although it is a
treatable and curable neoplasm, almost 25% of the tumors recur following surgery. Other
therapeutic modalities include endovascular embolization of the solid component of the
tumor, in order to decrease its vascularity and the blood loss during surgery, and stereotactic
radiosurgery of the tumor.
MRI reveals a large cystic mass in the right cerebellar hemisphere, hypointense on Imaging Findings
T1-weighted images (Fig. 1.7a) and hyperintense on T2-weighted images (Fig. 1.7b), with a
contrast-enhancing mural nodule (Fig. 1.7c). The lesion exerted mass effect on the
neighboring cerebellar and brainstem structures. Digital subtraction angiography
(Fig. 1.7d) shows the typical intense and prolonged staining of the tumor nidus.
History A 22-year-old woman presented with 1-month history

Case 8 of daily headache that became more severe in the
previous days, accompanied by vomiting and nausea.
Medulloblastoma On physical examination, mild ataxia and left upper
extremity dysmetria were found.
a b
c d
Fig. 1.8
Brain Tumors 17
Medulloblastoma is an infratentorial primitive neuroectodermal tumor (PNET) that occurs Comments

primarily, but not exclusively, in childhood. It accounts for approximately 7–8% of all
intracranial tumors, 30% of pediatric brain tumors, and 33% of all pediatric posterior fossa
neoplasms. The tumor shows a slight male predominance in children and males tend to
have a poorer prognosis. There is a second peak of medulloblastoma occurring in adults
24–30 years of age. Medulloblastoma most often arises in the posterior fossa, specifically in
the vermis. These neoplasms are typically midline lesions that bulge into the fourth
ventricle anteriorly and the cisterna magna posteriorly. Another less frequent location,
seen in older children and adults, is lateral cerebellum. The tumor tends to spread
throughout the CSF with spinal “drop metastases” occurring in 40% of cases. Non-central
nervous system (CNS) metastases (6%) occur in the pelvis, long bones, spine, lymph nodes,
and lung. Ninety percent of medulloblastomas cause obstructive hydrocephalus and raised
intracranial pressure as they efface the fourth ventricle and aqueduct of Sylvius.
Clinically, patients usually present with symptoms related to increased intracranial pres-
sure such as headache, especially upon awakening in the morning, and vomiting without
nausea. Other symptoms include ataxia, diplopia, as the sixth cranial nerve becomes
stretched from the hydrocephalus, and visual disturbances as a result of papilledema.
Imaging studies workup includes CT and MRI. An unenhanced cerebral CT scan is usually
performed because the majority of patients present with headache. On noncontrast CT scan, the
typical appearance of medulloblastoma is a rounded or lobulated, homogenous, midline verm-
ian mass that displaces the fourth ventricle anteriorly. The tumor is commonly hyperdense to
the brain parenchyma as a result of its high cellularity. After intravenous contrast administra-
tion, medulloblastoma shows marked enhancement. Surrounding hypodensity is indicative of
vasogenic edema. Owing to compression of the fourth ventricle and outflow of CSF, obstructive
hydrocephalus is common. MRI is the imaging technique of choice for the diagnosis and staging
of medulloblastoma and its potential subarachnoid metastases. To rule out drop metastases,
MRI of the spine is mandatory when medulloblastoma is either considered or diagnosed. Most
medulloblastomas are heterogeneously hypointense to gray matter on T1-WI and hyperintense
on T2-weighted images. Cysts are seen in 75–80% of cases and calcifications are rare. Upon
intravenous administration of gadolinium, moderate to marked enhancement occurs. In chil-
dren, homogeneous enhancement is the rule, whereas in adults, a more heterogeneous pattern
is usually seen. If the tumor extends upward into the cerebral aqueduct and third ventricle,
marked hydrocephalus with transependymal reabsorption of CSF may be detected.
Medulloblastoma is treated by surgical resection and radiotherapy to the craniospinal
axis, usually in conjunction with chemotherapy.
Urgent noncontrast cerebral CT scan (Fig. 1.8a) demonstrates a posterior fossa midline mass, Imaging Findings
located posteriorly to the fourth ventricle. The lesion shows heterogeneous appearance and a
dot-like calcification (arrow). Coronal T2-weighted MR images (Fig. 1.8b) clearly depicts the
heterogeneous mass with necrotic/cystic component (arrow).After gadolinium administration
(Fig. 1.8c, d), mild enhancement of the peripheral solid component can be seen. Sagittal
images (Fig. 1.8c) show caudal displacement of the cerebellar tonsils (arrow). Note obstructive
hydrocephalus with dilatation of the third ventricle and both lateral ventricles.
History A 43-year-old woman presented with three limited episodes of

Case 9 numbness and paresis of the right upper extremity accompanied by
disartria in the previous year.
Pleomorphic
Xanthoastrocytoma
Comments Pleomorphic xanthoastrocytoma (PXA) is a rare, usually benign,
cortical-based tumor,which represents a distinct type of supratentorial
astrocytoma that is found almost exclusively in children or young
adults. PXA constitutes less than 1% of all cerebral astrocytomas but
a b
c d
Fig. 1.9
Brain Tumors 19
they are important because they have a characteristic imaging appearance and are highly
amenable to surgical extirpation, which may be curative. PXA are thought to originate from
subpial astrocytes or multipotential neuroectodermal precursor cells. These tumors are
supratentorial in 98% of cases and typically present a superficial cortical location, most
commonly, in the temporal lobes (49%) followed by the parietal lobes (17%), frontal lobes
(10%), and occipital lobes (7%). The tumor does not show gender predominance. PXAs are
usually well-circumscribed, slow-growing, partially cystic tumors with a discrete mural
nodule. According to the new WHO classification of brain tumors, PXA is classified into the
group of “circumscribed (localized or noninfiltrative) astrocytomas,” together with pilocytic
and giant cell astrocytomas. They are cystic cortical masses with solid components abutting
the meninges in gross pathology. On histopathological examination, the tumor typically
involves both the meninges and the cortex and demonstrates pleomorphic appearance
containing fibrillary and giant multinucleated neoplastic astrocytes, large cytoplasmic lipid
deposits (xanthomatous) within the tumor cells, a dense reticulin network and lymphocytic
infiltrates.
Typically, patients have a long history of temporal lobe epilepsy and are in the first two
decades of life.
On noncontrast CT scan, PXA usually appears as a peripherally located, well-margin-
ated, intracortical hemispheric cystic tumor containing a mural nodule that is adjacent to
the peripheral leptomeninges. The tumor is often hypodense or cystic, and the mural nod-
ule enhances markedly after intravenous contrast administration. Calcifications are
unusual. The mass usually abuts the meninges. At MR imaging, PXAs are usually hypo- to
isointense compared to gray matter on T1-weighted images and hyper- to isointense rela-
tive to gray matter on T2- and FLAIR-weighted images. The solid portions of the tumor
show moderate to intense enhancement following gadolinium administration. Involvement
of the leptomeninges is highly characteristic, seen in 71% of the cases. The adjacent menin-
ges may enhance with contrast as well (similar to dural tail). Peritumoral edema may be
seen but is uncommon. They may be associated with cortical dysplasia.
Ganglioglioma should be considered in the differential diagnosis of PXA. Gangliogliomas
have a mural nodule similar to PXAs, but they are not adjacent to the meninges and do not
display a dural-tail-like reaction.
Surgical resection is the therapy of choice. The tumors are generally unresponsive to
both chemotherapy and radiation therapy. The prognosis for these tumors following
surgical resection is generally good, with an 81% survival rate at 5 years and a 70% survival
rate at 10 years. However, the tumor is also characterized by a relatively high rate of
recurrence. In addition, malignant transformation may occur occasionally.
Cerebral MRI shows a mass in the left frontal lobe involving the cortex and subcortical Imaging Findings
white matter and surrounding by edema. The lesion has a cystic component, which appears
hypointense on T1-WI (Fig. 1.9a) and hyperintense on T2-WI (Fig. 1.9b), and a lateral solid
nodule that enhances intensely following gadolinium administration (Fig. 1.9c). Perfusion-
weighted MR image (Fig. 1.9d) demonstrates decreased cerebral blood volume (CBV) in
the cystic component and increased CBV in the solid nodule.
Case 10
Meningioma
a b
c d
Fig. 1.10
Brain Tumors 21
A 43-year-old man with no relevant past medical history was admitted to the emergency History
room with a 10-min seizure. The patient presented a slight decrease in cognitive function
in the postcritical period. The clinical examination was otherwise unremarkable. Brain
MRI showed several extraaxial lesions consistent with multiple meningiomas.
Meningiomas are tumors that arise from arachnoidal cap cells, which reside in the Comments
arachnoid layer covering the surface of the brain. They account for 13–20% of all primary
intracranial tumors in adulthood. There is a female predilection (1.5:1 to 3:1) and the mean
age of presentation is over 50 years. Meningiomas are multiple in 5–40% of cases, and
multiple lesions are more frequent in patients suffering from neurofibromatosis-2 (NF-2),
an inheritable disorder with an autosomal dominant mode of transmission secondary to a
mutation of a gene located on chromosome 22.
Meningiomas are supratentorial in up to 90% of cases. The most common locations are
the parasagittal region (25%), convexity (20%), and sphenoid ridge (15%). Clinical
manifestations depend on location. Symptoms of a meningioma in the convexity or
parasagittal region are seizures, focal neurological deficits, or headaches; sphenoid
meningiomas can cause visual problems or facial numbness. Complete surgical resection is
the treatment of choice for benign meningiomas. Preoperative embolization to reduce the
blood supply to the tumor may be useful.
On CT scans, meningiomas are usually dural-based tumors that are slightly hyperattenuating Imaging Findings
in up to 70% of the cases. They enhance homogeneously and intensely after the injection of
iodinated contrast material. In nearly 25% of cases, calcifications are present. Hyperostosis
of the adjacent bone can be seen in some cases.
On T1-weighted images (Fig. 1.10a), most meningiomas have similar signal intensity to
cortical gray matter. Meningiomas present as extraaxial masses with a broad dural attach-
ment and a thin hypointense rim (open arrow) between the tumor and the brain, corre-
sponding to CSF.
On T2-weighted images (Fig. 1.10b), meningiomas may have different appearances. In
general, the tumor is hypointense to gray matter if calcium or fibrotic components are
present (open arrow). Meningiomas can show extensive perilesional edema (solid arrow).
After the injection of gadolinium, these tumors show intense and homogeneous
enhancement. In a majority of cases, enhancing of the tissue that surrounds the dural
attachment is seen; this radiological finding is known as the dural tail (Fig. 1.10c, d, open
arrows).
Digital subtraction angiography (not shown) showed meningeal arteries penetrating
into the tumor through its dural attachment, with inside branches with a characteristic
radial distribution. Homogeneous sharp tumor staining was seen in early and late phases.
Further Reading Karremann M, Pietsch T, Janssen G, Kramm CM, Wolff JE (2009)

Anaplastic ganglioglioma in children. J Neurooncol 92(2):
Abrol D, Gandotra P, Maqbool M, Shah A, Ahmad S (2007) 157–163
Dysembryoplastic neuroepithelial tumor: a rare brain tumor Kennedy B et al. Astrocytoma. Emedicine. http://emedicine.
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145–147 Koeller KK, Henry JM (2001) From the archives of the AFIP.
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Orbit and Sellar Region 2
Contents
Case 1 Choroidal Melanoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 24
Case 2 Grave’s Ophthalmopathy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26
Case 3 Inflammatory Pseudotumor of the Orbit . . . . . . . . . . . . . . . . . . . . . . . 28
Case 4 Orbital Hemangioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30
Case 5 Orbital Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32
Case 6 Optic Nerve Sheath Meningioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34
Case 7 Craniopharyngioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 36
Case 8 Hypothalamic Hamartoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38
Case 9 Pituitary Adenoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40
Case 10 Rathke’s Cleft Cyst. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42

History A 51-year-old man presented with a several-month history of progressive visual loss in
Case 1 the right eye. On ophthalmologic examination, an intraocular mass was found.
Choroidal
Melanoma Malignant melanomas are the most common primary intraocular tumors in adults.
Comments Nevertheless, these lesions are infrequent. The tumor arises from melanocytes within
the choroid. Choroidal melanoma have race predominance, and most often affect white
people while incidence among blacks is extremely rare. Median age is 55 years. Choroidal
melanomas may have variable coloration, ranging from amelanotic to darkly pigmented,
and are typically circumscribed, domed-shaped masses that grow to the vitreous cavity.
Choroidal melanoma may metastasize before diagnosis. The tumor can spread
hematogenously to the liver, lung, bone, skin, and the central nervous system (CNS).
a b
c d
Fig. 2.1
Choroidal melanomas usually remain asymptomatic for long time and the diagnosis is
made incidentally during a routine ophthalmologic examination. In general, the more
anterior their origin, the longer the delay of any symptoms. As the tumor grows, it may
become symptomatic. The most common clinical presentation is painless visual loss with
decreased visual acuity and visual field defects.
Because of their anatomic location, choroidal tumors are not accessible to biopsy with-
out intraocular surgery. Consequently, the diagnosis must be made on the basis of clinical
examination in conjunction with different diagnostic modalities such as ultrasound, com-
puted tomography (CT), magnetic resonance imaging (MRI), and angiography. Lesions
more than 3 mm in size are usually well detected on CT and MRI, whereas smaller lesions
are better evaluated with ultrasound. The accurate technique to diagnose the tumor and
to determine its size is ultrasound. On B-scan mode, intraocular melanoma appears as a
rounded or mushroom-shaped hypoechoic mass that produces excavation of underlying
uveal tissue and shadowing of subjacent soft tissues. A thin hyperechoic rim is sometimes
seen and represents a combination of elevated retina and peripheral blood vessels. These
tumors are very vascular lesions. Color Doppler ultrasound allows evaluating the tumor
vascularization both internally and in the periphery. This tumor characteristic is useful to
differentiate choroidal melanoma from other non-neoplastic entities such as subretinal
hemorrhage. Ultrasound may also detect some complications of the tumor as retinal
elevation and vitreous hemorrhage. CT scan is more useful than ultrasound to evaluate
extraocular extension and may help differentiate between choroidal or retinal detachment
and a solid tumor. After contrast material administration, choroidal melanoma shows
moderate enhancement. CT scan also is sensitive in detecting calcium, a feature of some
tumors different from uveal melanomas (characteristically choroidal osteoma). On MRI,
most choroidal melanomas appear as areas of high signal intensity on T1- and proton
density-weighted MR images, due to the paramagnetic property of melanin, and moder-
ately low signal intensity on T2-WI. The tumor demonstrates moderate enhancement
after intravenous gadolinium injection.
Documented growth of a lesion on serial examinations is the most important clinical
feature favoring the diagnosis of a choroidal melanoma. Differential diagnosis must be
made with retinal detachment, choroidal detachment, choroidal metastasis, choroidal
hemangioma, and large choroidal nevus.
Treatment depends on several factors such as visual acuity, size of tumor, age of the
patient, and presence of metastases. Small tumors can be managed by periodic observation.
In cases of tumor growth and in large and/or complicated tumors which compromise visual
function, enucleation is indicated. Nevertheless, enucleation does not prevent metastasis.
MRI study demonstrates a lobulated mass located in nasal aspect of the right ocular globe. Imaging Findings
The lesion appears slightly hyperintense to vitreous humor on axial T1-weighted image
(Fig. 2.1a) and hypointense on coronal STIR sequence (Fig. 2.1b). A hyperintense rim
surrounded the mass can be seen on T1-WI, which represents a retinal detachment (arrow
in Fig. 2.1a). Contrast-enhanced fat-saturation T1-weighted image (Fig. 2.1c) and contrast-
enhanced spin-echo T1-WI (Fig. 2.1d) show homogeneous enhancement of the lesion.
History A 57-year-old man presented with bilateral exophthalmos

Case 2 and eyelid swelling. On ophthalmologic examination,
diplopia, visual acuity loss on the right eye, and right
Grave’s Ophthalmopathy papilledema were found.
a c
Fig. 2.2
Grave’s ophthalmopathy (GO) is a thyroid-associated orbitopathy that represents a part of Comments

Grave’s disease, an autoimmune process that can affect the orbital and periorbital tissue,
the thyroid gland, and, rarely, the pretibial skin or digits (thyroid acropachy). Eye
involvement in Grave’s disease is clinically evident in 25–50% of patients. GO may precede,
coincide, or follow the systemic complications of dysthyroidism. The underlying
pathophysiology of GO is thought to be an antibody-mediated reaction against the TSH
receptors present in retrobulbar tissue. There is a lymphocytic infiltration of the orbital
tissue that causes edema in the extraocular muscles and transformation of preadipocyte
fibroblasts into adipocytes. The enlargement of extraocular muscles in conjunction with
the orbital fat accumulation produce an increase in orbital volume causing exophthalmos
and, occasionally, optic nerve compression at the narrow posterior apex of the orbit. The
edema results in tissue damage and fibrosis, with restriction in extraocular motility and
lagophthalmos. GO is the most common cause of unilateral and bilateral proptosis in adults.
GO usually occurs in patients aged 30–50 years and is much more common in females,
although severe cases occur more often in males.
The ocular manifestations of GO include eyelid swelling or retraction, chemosis, tearing,
corneal erosions or ulcerations, abnormal eye motility, exophthalmos, and periorbital
edema. Although most cases of GO do not result in visual loss, GO can cause vision-
threatening exposure keratopathy, troublesome diplopia, and compressive optic neuropathy.
In typical cases, the diagnosis can be established clinically and imaging studies are not
necessary. Orbital imaging is recommended in cases of very asymmetrical clinical picture
and in cases of clinical suspicion of optic nerve involvement. Imaging techniques include
ultrasound, CT, and MRI. Orbital ultrasound can quickly confirm if the patient has thick-
ened muscles or an enlarged superior ophthalmic vein. CT and MRI, with both axial and
coronal views, usually reveal thick muscles with tendon sparing. The inferior rectus muscle
and the medial rectus muscle are usually involved. Bilateral muscle enlargement is com-
mon; unilateral cases usually represent asymmetric involvement rather than normality of
the less involved side. The superior ophthalmic vein may be dilated. CT scan provides
excellent views of the bony anatomy of the orbit, which is important in cases that orbital
decompression is required. MRI is better to evaluate the orbital contents including the
optic nerve, orbital fat, and extraocular muscles.
Regarding treatment, the main goal is to achieve and maintain a euthyroid state. These
measures alone are sufficient in many cases, because the majority of patients with GO
present a favorable and often spontaneous self-limiting clinical course, although it may be
prolonged over one or more years. Patients who suffer from severe forms of GO can be
treated with systemic corticosteroids or orbital irradiation.
Orbital MRI study demonstrates diffuse orbital fat involvement in both sides and fusiform Imaging Findings
thickening of the extraocular muscles with tendon sparing (Fig. 2.2a). On axial and coronal
STIR sequence (Fig. 2.2b, c), the extraocular muscles present high signal intensity consistent
with inflammatory component. Note the optic nerve encasement in the apex of the orbit
due to muscles enlargement (arrow in Fig. 2.2b).
History A 28-year-old woman presented with a mass located in

Case 3 superolateral aspect of the left orbit of 6 months duration.
The patient also complained of vague symptoms of
Inflammatory Pseudotumor of the Orbit tearing, limited motility of the left eye, and episodes of
orbital inflammation since the last 2 weeks.
a b
c d
Fig. 2.3
An inflammatory pseudotumor of the orbit represents a clinical and pathologic condition Comments
of unknown etiology that is characterized by the presence of a mass within the orbit that
may mimic malignancy and that is composed of inflammatory cells and variable amounts
of fibrosis. It constitutes about 9% of all orbital mass lesions. Inflammatory pseudotumors
are most often confined to the orbit and frequently involve the lacrimal gland and the
muscular cone. The extension of the lesion beyond the orbit or even intracranially is quite
rare but it may occur in cases of extensive and chronic orbital inflammation. Orbital
pseudotumor has no gender or race predominance and can appear in individuals of any
age, although it most frequently occurs in middle-aged patients. It is usually unilateral, and
the presence of bilateral masses suggests an underlying systemic disease.
The symptoms of idiopathic pseudotumors depend on the inflammatory response
(acute, subacute, or chronic) and the location of the inflammatory tissue. Clinical manifes-
tations include exophthalmos, reduced ocular motility, diplopia, ptosis, and chemosis. In
fact, inflammatory pseudotumors are a common cause of unilateral proptosis in adults.
The radiological findings in a pseudotumor are characterized by inflammatory changes
in the different intraorbital components, such as the globe, the lacrimal glands, the extraoc-
ular muscles, the orbital fat, and the optic nerve. On CT, inflammatory pseudotumors
appear as an abnormal intraorbital mass of soft-tissue density, which varies widely in
shape, location, and size. The presence of bony changes, reflected by hyperostosis or ero-
sion and sclerotic change, indicates a long-standing benign process. Inflammatory pseudo-
tumors of the orbit are frequently accompanied by edema or fat infiltration. On MRI, the
lesions are hypointense on both T1- and T2-weighted images (possibly related to the
fibrotic changes) with strong enhancement after gadolinium administration. Fat suppres-
sion techniques clearly show intraorbital inflammation.
Orbital pseudotumor is diagnosed by exclusion, on the basis of the patient´s history,
clinical course, response to steroid therapy, laboratory test, and radiological and biopsy
findings. The diagnosis becomes even more difficult in subacute and chronic forms.
Sometimes, a chronic idiopathic inflammatory pseudotumor can simulate lymphoma, par-
ticularly when there is no history of an acute onset.
Corticosteroids are the mainstay of treatment and are administered for several months
to ensure remission. Radiotherapy may be used in patients who fail to respond to steroids
or who have a rapidly progressive course. For those patients who are refractory to both
corticosteroids and radiotherapy, anecdotal reports have suggested the use of
chemotherapeutic agents such as cyclophosphamide, methotrexate, and cyclosporine.
Orbital coronal CT scan (Fig. 2.3a) demonstrates an extraconal tumor in the theoretical Imaging Findings
location of left lacrimal gland. The tumor wraps around and displaces caudal and medially
the ocular globe. On coronal STIR MR image (Fig. 2.3b), the lesion presents high signal
intensity due to the inflammatory component. On coronal and sagittal gadolinium-
enhanced fat-suppressed T1-weighted images (Fig. 2.3c, d), the tumor shows intense
enhancement. Observe that the lesion is clearly depicted between orbital roof and superior
rectus muscle in the sagittal plane.
Case 4
Orbital Hemangioma
a b
Fig. 2.4
A 57-year-old woman presented with a soft mass located in superomedial portion of the History
left orbit that had been slowly growing for the last three years.
Cavernous hemangioma is the most common primary benign tumor of the orbit in adults. Comments
It is a slow-growing, vascular tumor, which is considered as developmental hamartoma.
The tumor is characteristically unilateral and solitary; bilateral or multiple hemangiomas
are quite rare. Most cavernous hemangiomas are located within the intraconal retrobulbar
region. These tumors usually present in the fifth decade of life with female gender
predominance. Pregnancy may accelerate the growth of the tumor.
The most common clinical presentation is a unilateral, painless, progressive proptosis.
Other manifestations such as visual acuity compromise, diplopia, and extraocular muscle
or pupillary dysfunction may occur as a result of compression of intraorbital contents by
the tumor.
The diagnosis can be made by imaging studies. Ultrasound typically shows a lesion with
good sound transmission with moderate high-echogenity on A-scan secondary to the sep-
tae within the tumor. Doppler flow study may reveal subdued blood flow within the heman-
gioma. On CT scan, the tumor appears as an oval- or round-shaped, well-defined,
homogeneous intraconal mass, which enhanced with intravenous contrast. The enhancing
pattern can be homogenous or nonhomogenous. On MRI, hemangiomas are well-defined
oval masses, homogeneous, isointense relative to muscle on T1-weighted images and
hyperintense on T2-weighted images. On dynamic MRI after gadolinium administration,
progressive and total homogeneous filling up of the mass can be seen. This enhancement
pattern is considered a typical feature of a cavernous hemangioma of the orbit, and is simi-
lar to hepatic cavernous hemangiomas. The typical contrast-enhancement spread pattern
of hemangiomas can be used to distinguish this tumor from others lesions such as
schwannomas.
Most hemangiomas remain stable throughout a patient’s life and cause no visual
impairment, therefore, the majority of patients require no interventions and can be
observed clinically. In cases of symptomatic tumors, surgical excision is the treatment of
choice.
MRI study reveals a mass located in superomedial aspect of the left orbit, hyperintense on Imaging Findings
STIR (Fig. 2.4a), and T2-weighted (Fig. 2.4b) MR images and hypointense on T1-weighted
image (Fig. 2.4c). The lesion presents well-defined lobulated margins and hypointense
round images within it consistent with calcifications (phebolites) (arrows).
Case 5
Orbital Lymphoma
a b
Fig. 2.5
A 65-year-old male presented with bilateral eyelid swelling, moderate exophthalmos, and History
orbital discomfort.
Lymphomas are the most frequent orbital malignant tumors. According to recent Comments
publications, these tumors account for 11% of all orbital masses and 55% of all malignant
tumors. Orbital lymphomas are predominantly low-grade, small, B-cell types, and are
associated with systemic disease, occurring either sequentially or concurrently, in about
one-third of the cases. Hence, a patient diagnosed with orbital lymphoma must be examined
to rule out systemic disease. Normal orbital lymphoid tissue is located on conjunctivae and
lacrimal glands, therefore, these are the most common locations of orbital lymphomas.
Lymphoid tumors of the orbit are considered a disease spectrum that includes benign
lymphoid hyperplasia, atypical lymphoid hyperplasia, and malignant lymphoma. There
are no clinical or laboratory or imaging tests that permit to distinguish between benign
and malignant lymphoid lesions.
Orbital lymphomas usually occur in older people, the average age for occurrence is the
sixth decade of life and there is a slight female predominance. Clinically, lymphoma of the
orbit generally presents with symptoms secondary to gradually increasing mass effect on
surrounding structures. Clinical features include progressive painless proptosis with or
without motility impairment, diplopia, ptosis, and, rarely, visual loss. Proptosis and visible
conjunctival mass are the most common modes of presentation. Most lesions are unilateral
although bilateral orbital involvement is seen in about 10–25% of the patients.
Ultrasound, CT, and MRI are different imaging techniques to diagnose orbital lym-
phoma. CT and MRI findings are unspecified and should be evaluated in conjunction with
clinical signs and symptoms. On CT, orbital lymphomas appear as hyperdense, homoge-
neous well-defined masses usually located on the retrobulbar region or on the superior
orbital compartment. The tumor is molded to the adjacent structures without eroding the
bone or expanding the orbit. After contrast administration, the lesion shows mild enhance-
ment. Biopsy is always mandatory for stage grouping of lymphomas. On MRI, malignant
lymphoma lesions are hyperintense compared to the extraocular muscles on both precon-
trast and postcontrast T1-weighted images. When the lacrimal duct is involved or there is
bilateral disease, it is more likely to be malignant lymphoma.
Therapeutic options for orbital lymphoma include surgical excision, radiation therapy,
and chemotherapy. The prognosis depends on the histologic type and stage of lymphoma
and therapy. In general, with modern treatment of patients with NHL, the overall survival
rate at 5 years is approximately 60%.
Coronal un-enhanced CT-scan (Fig. 2.5a) demonstrates bilateral, solid, homogeneous, and Imaging Findings
soft-tissue attenuation masses that molded to the adjacent ocular globe. Lacrimal glands
are involved and appear enlarged. On axial T1-weighted MR image (Fig. 2.5b), the lesions
present the same signal intensity than extraocular muscles. On axial gadolinium-enhanced
T1-WI (Fig. 2.5c) and sagittal contrast-enhanced fat-saturated T1-WI (Fig. 2.5d), the masses
show homogeneous and intense enhancement.
History A 40-year-old female presented with progressive visual

Case 6 acuity loss in the left eye with self-limited episodes of
mild ocular pain in this eye. On physical examination,
Optic Nerve Sheath Meningioma slight proptosis and mild unilateral papilledema were
found.
a b
c d
Fig. 2.6
Optic nerve sheath meningioma (ONSM) is a rare, benign tumor that arises from the cap Comments
cells of the arachnoid that surround the intraorbital or intracanalicular optic nerve. The
tumor can develop anywhere along the course of the optic nerve. ONSM represents about
1–2% of all meningiomas, 2% of all orbital tumors, and about 10% of optic nerve lesions.
The majority of cases occur in middle-aged females, with a 2:1 female-to-male ratio. The
tumor may be unilateral, bilateral, or multifocal with the latter two subgroups occurring
most commonly in patients with type 2 neurofibromatosis.
The clinical presentation of optic nerve sheath meningioma depends on whether they
arise from the orbit, within the optic canal or intracranially. The tumor typically grows
slowly causing gradually compression of the optic nerve with progressive visual loss in the
affected eye. The classic triad, known as the Hoyt–Spencer triad, includes loss of vision,
optic atrophy, and optociliary shunt vessels. These enlarged blood vessels indicated that the
tumor has disrupted the natural circulation through the optic nerve to the retina and chor-
oid. Other clinical manifestations are color vision disturbance, visual field defect, proptosis,
optic disc edema, and motility impairment.
The diagnosis of ONSM relies heavily on imaging findings. MRI currently remains the
method of choice for diagnosis of ONSM, although it is less sensitive than CT in the detec-
tion of calcification. ONSMs are typically isointense or slightly hypointense to brain and
optic nerve tissue on T1-weighted images and hyperintense (may also be hypointense) on
T2-weighted images. They present a homogeneous intense enhancement after gadolinium
administration often suggesting in appearance a “tram track” around the hypointense
optic nerve in axial sequences. Intracranial extension is rare and, when present, it is
restricted in a short distance along the prechiasmatic optic nerve sheath. Contrast-
enhanced CT is another useful imaging technique for evaluation of optic nerve sheath
meningioma. On CT, the tumor usually appears as a fusiform thickening of the optic nerve.
This enlargement may appear as localized or as an eccentric expansion of the optic nerve
and occurs most commonly at the orbital apex. After intravenous contrast injection, ONSMs
usually shows an intense homogeneous enhancement. Linear, diffuse, or patchy calcifica-
tions within or along an optic nerve mass are commonly detected.
The differential diagnosis includes optic glioma, orbital pseudotumor, and lymphoma.
ONSM management is variable and depends on several factors. In cases of mild or no
visual impairment with evidence of no intracranial extension, follow-up is recommended.
If loss of vision occurs and progresses, radiation therapy is the treatment of choice either
primarily or following surgery. Radiotherapy can improve vision in some patients or
preserve it in others. Chemotherapy is reserved for patients with unresectable, recurrent,
or previously irradiated meningiomas.
Axial T1-weighted MR image of the orbits (Fig. 2.6a) demonstrates a fusiform lesion that Imaging Findings
surrounds the left optic nerve, isointense to extraocular muscles. After contrast material
administration (Fig. 2.6c, d), the lesion shows intense and homogeneous enhancement. On
both T1-weighted MR image (Fig. 2.6b) and contrast-enhanced fat-suppression T1-weighted
MR images (Fig. 2.6c), the optic nerve can be clearly depicted. It appears thinner than
contralateral optic nerve and encased by the lesion (arrow in Fig. 2.6c).
Case 7
Craniopharyngioma
a b
c d
Fig. 2.7
A 61-year-old woman was referred to ophthalmologist for visual loss and progressive History
headache. On physical examination, right temporal hemianopsia was found. Neuroimaging
studies were performed next.
Craniopharyngioma is a benign, slow-growing, extra-axial, epithelial, calcified cystic tumor Comments

arising from squamous cell rests along the involuted hypophyseal Rathke’s cleft. For this
reason they occur exclusively in the region of the sella turcica and suprasellar cistern.
Craniopharyngiomas account for 3–5% of all primary intracranial brain tumors. The
prevalence of craniopharyngiomas peaks between 10 and 14 years of age, with a second
peak occurring in the fourth to sixth decades of life. There is no gender predilection.
The most common presenting symptoms are headache, visual disturbances, and endo-
crine dysfunction. Although craniopharyngiomas exhibit no hormonal activity, endocrine
symptoms can occur due to compression of the hypothalamus, pituitary stalk, and pituitary
gland.
Imaging studies strongly suggest the diagnosis. The radiologic hallmark of a cranio-
pharyngioma is the appearance of a suprasellar calcified and cystic mass. About 80–87% of
craniopharyngiomas are calcified and 70–75% are cystic. Calcifications are more common
in children (90%) than in adults (50%). CT scan is the most sensitive technique to demon-
strate calcifications. It is useful in defining both calcified and cystic components. Cyst con-
tent usually has the same attenuation as cerebrospinal fluid (CSF); contrast administration
better defines the enhancing cyst capsule. On MRI, craniopharyngiomas present a hetero-
geneous spectrum. The most common pattern is a cyst that is hypointense on T1WI and
hyperintense on T2WI. Sometimes, craniopharyngiomas are hyperintense on T1WI due to
high protein concentration, blood degradation products, or both. After gadolinium admin-
istration, the tumor enhances strongly and heterogeneously. MRI is the imaging modality
used to plan the surgical approach.
Differential diagnosis includes the following entities: Rathke’s cleft cyst, necrotic pitu-
itary adenoma, suprasellar dermoid, teratoma, thrombosed aneurysm, and cystic supra-
sellar hypothalamic and chiasmatic glioma.
Surgical removal is the treatment of choice for tumor eradication. Nevertheless,
craniopharyngiomas tend to recur after surgery. Therefore, postoperative follow-up with
gadolinium-enhanced MRI is mandatory.
Un-enhanced CT scan (Fig. 2.7a) demonstrates a sellar and suprasellar mass,hypoattenuating Imaging Findings
to brain parenchyma, with calcifications. On MRI, the lesion appears hypointense on
T1-weighted image (Fig. 2.7b) and hyperintense on T2-WI (Fig. 2.7c), which is consistent
with a predominantly cystic component. After gadolinium administration (Fig. 2.7d), the
tumor shows intense and heterogeneous enhancement and solid and necrotic or cystic
components are better differentiated. Note encasement of left internal carotid artery by
tumor (arrow in Fig. 2.7c).
Case 8
Hypothalamic Hamartoma
a b c
Fig. 2.8
A 28-year-old woman underwent an urgent unenhanced CT-scan for cranial trauma after History
traffic accident. A small lesion located in interpeduncular cistern was incidentally found on
CT. The patient referred a past medical history of epilepsy treatment long time ago.
Hypothalamic hamartoma, also called tuber cinereum hamartoma, is a relative rare Comments
congenital non-neoplastic heterotopia. It represents a midline dysraphic syndrome and
presents as an ectopic cerebral gray matter, comprising a mass of normal neuronal tissue.
Most of these lesions are small with a diameter of a few millimeters to 1.5 cm. They lie
between the infundibular stalk anteriorly and the mamillary bodies posteriorly.
These lesions have been divided into two main clinicoanatomic types: para-hypotha-
lamic hamartomas and intra-hypothalamic hamartomas. Parahypothalamic hamartomas
are pedunculated masses that are attached to the floor of the hypothalamus by a narrow
stalk. These lesions seem more likely to be associated with isosexual precocious puberty.
Intrahypothalamic hamartomas are sessile masses with a broad attachment to the hypo-
thalamus. These lesions seem to be associated more often with gelastic seizures, intellec-
tual impairment, and psychiatric disturbances than with precocious puberty. These clinical
features are noted commonly in early life and may occur as early as in the neonatal
period.
On MR imaging, hamartomas appear as well-defined pedunculated or sessile lesions at
the tuber cinereum and are isointense or mildly hypointense on T1-weighted images and
iso- to hyperintense on T2-weighted images. Hamartomas neither calcify nor exhibit
enhancement following contrast administration.
Surgery, radiosurgery, and medical treatment are the different therapeutic options. The
choice of treatment must be individualized depending on the age and clinical circum-
stances of the patient and the size and anatomic relationships of the hamartoma.
The absence of any long-term change in the size, shape, or signal intensity of the lesion
strongly supports the diagnosis of hypothalamic hamartoma.
Axial T1-weighted MR image (Fig. 2.8a) reveals a mass in interpenducular cistern (arrow), Imaging Findings
isointense to brain parenchyma. Sagittal T1-weighted MR images, before (Fig. 2.8b) and
after (Fig. 2.8c) intravenous gadolinium administration, show a lesion that lies between the
infundibular stalk anteriorly and the mamillary bodies posteriorly (arrows). The mass
presents the same signal intensity as the brain parenchyma and does not enhance with the
contrast material.
History A 18-year-old boy was referred to the emergency department with a three-day history of
Case 9 right periorbital headache, blurred vision, and diplopia. On physical examination, right
temporal hemianopsia was found.
Pituitary
Adenoma
Comments Pituitary adenomas are benign, slow-growing tumors that arise from cells in the pituitary
gland. Pituitary adenomas are relatively common, accounting for approximately 15% of
primary brain tumors. These tumors occur in individuals of 20–50 years of age and do not
show gender predilection. Based on size, pituitary tumors can be divided into microadenomas
(<1 cm in diameter) and macroadenomas (>1 cm in diameter). Microadenomas typically
present with endocrine disturbances (according to the hormone secreted by the tumor),
a b
c d
Fig. 2.9
whereas macroadenomas usually manifest with symptoms due to the local mass effect on
the optic chiasm or with pituitary failure. Lateral extension of the tumor may involve
cavernous sinus and may affect the III, IV, and VI cranial nerves causing ocular motility
impairment.
Pituitary adenomas are usually solid capsulated tumors that may contain internal areas
of necrosis, cyst formation, or hemorrhage. Calcifications are rare. According to the hor-
mone secreted by the tumor, adenomas can be divided into two categories: (1) hormone-
inactive (nonfunctional) pituitary adenomas, which typically cause problems related to
the size of the tumor compressing adjacent brain structures and (2) hormone-producing
pituitary adenomas. The three most common are prolactin-secreting pituitary adenoma
(prolactinoma), growth hormone-secreting pituitary adenoma, and ACTH-secreting
pituitary adenoma. Other hormone producing pituitary tumors are very rare. Pituitary
adenomas, with a few exceptions, are not under the control of hypothalamic releasing
factors.
Clinical manifestations are due to the local effect of the mass and distant endocrine
manifestations that can affect a variety of organ systems.
The diagnosis of a pituitary adenoma is made based on a combination of pituitary
function testing (blood hormone levels) and pituitary imaging. Conventional single-
section CT has a limited role in pituitary imaging, with a sensitivity of 17–22% in detect-
ing microadenomas. Multidetector-row CT with 64 channels may have a role, especially
in patients unable to undergo MRI. CT is best for visualizing bony detail and calcifica-
tion. On CT, microadenomas appear as a focal hypodense area within the pituitary gland.
MRI is generally preferred over CT for the diagnosis of pituitary adenomas because of
its superior definition of small lesions in the pituitary sella and its improved anatomic
definition before surgery. MRI is also preferred for postsurgical surveillance. On MRI,
microadenomas are sometimes difficult to detect unless dynamic techniques are used.
Microadenomas enhance less rapidly than normal pituitary tissue and therefore appear
relatively hypointense on rapid-sequence contrast-enhanced T1-WI. Uncomplicated
macroadenomas show the same signal intensity than gray matter on all imaging
sequences. Enhancement after gadolinium injection is typically intense but is often
heterogeneous.
The optimal treatment of a pituitary adenoma depends on multiple factors including
the hormone production by the tumor (if present), size of the tumor, how invasive the
tumor is into surrounding structures, and the age and health of the patient. Treatment
options include surgery (performed in more than 99% of cases via a transphenoidal route),
radiotherapy, and medical treatment.
On urgent noncontrast CT scan (Fig. 2.9a), a sellar and suprasellar hyperattenuating mass was Imaging Findings
found. On MRI study, the lesion is well-circumscribed and appears isointense to brain
parenchyma on T1-weighted image (Fig. 2.9b). After intravenous gadolinium administration
(Fig. 2.9c, d), the mass shows intense and homogeneous enhancement. A small cystic component
can be seen in the superior aspect of the lesion (arrow in Fig. 2.9c). The large suprasellar
component of the tumor causes optic chiasm compression. This structure cannot be identified.
Case 10
Rathke’s Cleft Cyst
a b
c d
Fig. 2.10
A 38-year-old woman presented with tension-type headache. She underwent a cerebral History
MRI study that demonstrated an “incidental” pituitary tumor.
Rathke’s cleft cysts (RCCs) are benign cystic lesions that originate from the failure of Comments
obliteration of lumen of the Rathke pouch. RCCs commonly appear as single, unilocated,
well-defined, intra/suprasellar cyst without calcification. Forty percent of these cysts are
completely intrasellar and the remaining 60% have suprasellar extension. They commonly
measure between 5 and 15 mm but can occasionally become very large. Cyst contents vary
from serous to mucoid. Most lesions are detected in middle-aged adults with female gender
predominance.
The vast majority of RCCs are asymptomatic and they are found incidentally on imag-
ing studies. Symptomatic RCCs are rare, but cysts can enlarge and produce symptoms as
pituitary dysfunction, visual disturbances, and/or headache.
On CT, RCCs appear as well-delineated, round, hypodense, intra/suprasellar mass with-
out enhancement after contrast administration. MRI findings depend on the cyst content.
On T1-weighted image, two-thirds are hyperintense to brain and one-third show low signal
intensity similar to CSF. On T2 WI, half are hyperintense, 25% are isointense, and 25% are
hypointense. RCCs typically do not show enhancement following gadolinium administra-
tion, although an enhancing rim of compressed pituitary gland surrounding the cyst is
sometimes present.
The differential diagnosis includes arachnoid cyst, cystic pituitary adenoma, cystic
craniopharyngioma, and inflammatory cyst.
Asymptomatic RCCs are treated conservatively. In symptomatic cases, drainage or
partial excision of the cyst wall (“marsupialization”) may be done. The recurrence rate
varies from 19 to 28%.
Axial and sagittal T1-weighted MR images (Fig. 2.10a, b) and fluid-attenuated inversion Imaging Findings
recovery (FLAIR) MR image (Fig. 2.10c) reveal a hyperintense nodular lesion located in the
pituitary gland. On sagittal contrast-enhanced T1-weighted MR image (Fig. 2.10d), the
lesion does not show enhancement and can be clearly differentiated from hypophysis.
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magnetic resonance imaging findings and clinical manifesta- MR imaging and CT. Neuroimaging Clin N Am 15:23–47
tions of hypothalamic hamartoma. J Neurosurg 91:212–222 Meredith TA (1998) Choroidal melanoma: diagnosis and man-
Augsburger JJ, Peyster RG, Markoe AM et al (1987) Computed agement. Am J Ophthalmol 125(6):865–867
tomography of posterior uveal melanomas. Arch Ophthalmol Nishioka H, Haraoka J, Izawa H, Ikeda Y (2006) Magnetic reso-
105(11):1512–1516 nance imaging, clinical manifestations, and management of
Barkovich AJ (2005) Intracranial, orbital, and neck masses of Rathke’s cleft cyst. Clin Endocrinol (Oxf) 64(2):184–188
childhood. In: Barkovich AJ (ed) Pediatric neuroimaging, 4th Noth D, Gebauer M, Müller B et al (2001) Graves’ ophthalmopa-
edn. Lippincott Williams & Wilkins, Philadelphia, pp 573–603 thy: natural history and treatment outcomes. Swiss Med
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cleft cysts: a radiological, surgical and pathological review. neck inflammatory pseudotumor. AJR Am J Roentgenol
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histological features. J Clin Neurosci 13(4):438–442 cavernous hemangioma from schwannoma of the orbit: a
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growth hormone-producing and non-growth hormone-pro- Thorn-Kany M, Arrue P, Delisle MB, Lacroix F, Lagarrigue J,
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Harold Lee HB, Garrity JA, Cameron JD, Strianese D, Bonavolontà Tominaga JY, Higano S, Takahashi S (2003) Characteristics of
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gioma in children. Surv Ophthalmol 53(6):543–558 2(1):1–8
Ing E, Abuhaleeqa K (2007) Graves’ ophthalmopathy (thyroid- Turbin RE, Pokorny K (2004) Diagnosis and treatment of orbital
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trum of orbital pathologies that involve the lacrimal gland Yadav BS, Sharma SC (2009) Orbital lymphoma: role of radia-
and the lacrimal fossa. Korean J Radiol 8(4):336–342 tion. Indian J Ophthalmol 57(2):91–97
Ear, Nasal, and Paranasal Sinuses 3
Contents
Case 1 Antrochoanal Polyp . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 46
Case 2 Inverted Papilloma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 48
Case 3 Nasosinusal Polyposis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 50
Case 4 Sinus Mucocele . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 52
Case 5 Osteoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 54
Case 6 Rhinocerebral Mucormycosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 56
Case 7 Sinonasal Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 58
Case 8 Acoustic Neuroma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 60
Case 9 Cholesteatoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 62
Case 10 Jugulotympanic Glomus Tumor . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 64

Case 1
Antrochoanal Polyp
a b
Fig. 3.1
A 41-year-old female presented with right hemifacial tenderness, posterior rhinorrhea, and History
nasal obstruction, predominantly in the right side. On physical examination, a polypoid
mass in the right nasal fossa was found.
The antrochoanal polyp (ACP) is a solitary benign tumor that arises from the mucosa of Comments
the maxillary antrum. The polyp grows by extension from the antrum through its ostium
into the middle meatus and, thereafter, protruding to the posterior choana and nasopharynx
causing nasal obstruction. The occurrence of choanal polyps is equally frequent in both
males and females and is most common in teenagers and younger patients.
ACP was first described by Professor Killian, in 1906, giving specificity among polyposis;
it represents about 4–6% of all nasal polyps. Although its physiopathology remains unclear,
some authors have proposed a pathogenetic origin. According to these authors, development
of ACP could be due to an increased pressure level in the Highmoro antrum caused by
an inflammatory-anatomical alteration at ostio-meatal complex/middle meatus level, in a
patient with a preexisting silent antral cyst, subsequently forced to herniate outside, through
the accessory ostium.
The most common clinical symptom of ACP is unilateral nasal obstruction (especially
during the expiratory phase) that can be associated with mucopurulent rhinorrhea, bleed-
ing, snoring, foreign body sensation, halitosis, headache, postnasal drip, and loss of sense
of smell.
Nasal endoscopy and computed tomography (CT) are the main diagnostic techniques.
During nasal anterior rhinoscopy, ACP usually presents as a firm, smooth, bright, and white
mass occupying the nasal cavity. On CT scans, using both coronal and axial images, ACP
appears as a dumbbell-shaped, hypoattenuating mass that fills the maxillary sinus with an
extension into the nasal fossa and posterior choana. The walls can be distended or thinned,
but never eroded. An enlargement of the maxillary ostium can occasionally be observed.
Magnetic resonance imaging (MRI) should be performed only in cases of suspected malig-
nancy. On MRI, ACP appears hypointense on T1-weighted images and hyperintense on
T2-weighted images. The polyp do not show enhancement after gadolinium administration.
The two general differential diagnoses are as follows: (1) inverted papilloma, a tumor
that may contain calcifications and cause destruction of surrounding bone and (2) fungus
infection; in this condition, the maxillary sinus wall may appear thickened and the sinus
may contain hyperdense material or faint intraluminal calcifications.
Surgery is the treatment of choice for ACP, with endoscopic resection the most
recommended. Incomplete excision of ACP almost always leads to recurrence.
Un-enhanced CT scan acquired in the axial plane (a) with posterior reconstructions in Imaging Findings
coronal (b) and sagittal (c) planes demonstrate a large polypoid tumor in the right maxillary
antrum, with mild mass effect. The lesion protrudes through the ostium (arrow in Fig. 3.1b)
into the right nasal cavity and extends into the posterior choana.
History A 48-year-old male presented with a 3-year history of

Case 2 nasal obstruction and rhinorrhea that had become more
severe for the past month. Physical examination showed
Inverted Papilloma a large polypoid mass filling the right nasal cavity.
Comments Inverted papilloma is a rare type of tumor that constitutes

only 0.5–4% of all nasal tumors. It is a benign, locally
aggressive, epithelial neoplasm that arises from the nasal
a b
c d
Fig. 3.2
or paranasal sinus mucosa with a characteristic histological feature: The epithelium

proliferates into the underlying stroma, rather than proliferating outward from the surface,
hence the name “inverted.” The etiology of this lesion remains unclear, among proposed
causes are allergies, chronic sinusitis, airborne pollutants, and viral infection. Despite the
benign nature of the tumor, it tends to destroy bone, to recur after incomplete removal and
can be associated with malignancy (squamous cell carcinoma) in approximately 5% of
patients, especially in older patients. Squamous cell carcinoma may be present with inverted
papilloma at the initial diagnosis or it may occur metachronously after prior treatment.
Inverted papilloma has been reported in all age groups but presents a higher incidence in
the sixth and seventh decades of life. Males are affected four times more often than females.
The three main clinical characteristic attributes of the tumors are the tendency to recur,
their destructive capacity to surrounding structures, and their propensity to be associated
with malignancy. Clinical manifestations of inverted papilloma are nonspecific and depend
on the location and extent of the tumor. Unilateral nasal obstruction is the most common
presenting symptom. Other symptoms are epistaxis, rhirorrhea, epiphora, and facial pain.
Radiological diagnosis has traditionally been based on CT. On CT scan, inverted papil-
loma appears as a unilateral soft-tissue mass, with a lobulated surface configuration, located
in the nasal cavity, predominantly in the central portion of the middle meatus and involving
the ostiomeatal complex and maxillary sinus. Seventy-five percent of patients have evidence
of various degrees of bone destruction. These may include thinning, remodeling, erosion,
and (less commonly) sclerotic bony changes. The presence of bone destruction alone does
not indicate dedifferentiation into malignancy. CT scanning is more accurate than conven-
tional radiography for identifying the areas of bony erosion. MRI, especially T2-weighted
images, is perhaps a better tool in differentiating inverted nasal papilloma from other nasal
lesions, and has recently been advocated as the imaging modality of choice. MRI is superior
to CT scanning in distinguishing papillomas from inflammation and for providing better
delineation of the lesions in contrast to surrounding soft tissue. On MRI, inverted papilloma
presents a heterogeneous appearance. On T1-weighted images, they are slightly hyperin-
tense to muscle; however, on T2-weighted images, they have intermediate signal intensity.
After gadolinium administration, the tumor shows strong enhancement. MRI can more
accurately define the true extent of the lesion and can help in treatment planning.
Surgery is the primary therapy of inverted papilloma, however, this tumor is characterized
by a high recurrence rate after excision, which emphasizes the importance of accurate
tumor mapping and total tumor extirpation. There is likely no significant difference in the
risk of local recurrence after open compared with endoscopic surgery.
Axial and coronal CT scan images (Fig. 3.2a, b) reveal a large expansile polypoid mass filling the Imaging Findings
right nasal cavity. The lesion causes displacement and thinning of the medial maxillary sinus
wall and the nasal septum. The tumor also erodes the anterior portion of lamina papiracea and
extends into the orbit (arrow in Fig. 3.2a). Note the presence of dysmorphic calcifications within
the mass. On axial STIR (Fig. 3.2c) and gadolinium-enhanced T1-weighted (Fig. 3.2d) MR
images, the right nasal cavity mass and the maxillary sinus opacification are clearly depicted.
After contrast administration, the lesion shows intense enhancement (Fig. 3.2d).
Case 3
Nasosinusal Polyposis
a b
Fig. 3.3
A 34-year-old male presented with a long history of nasal obstruction. Physical examination History
revealed multiple nasal polyps involving both nasal cavities.
Nasal polyps are tumor-like, hyperplastic swellings of the nasal mucosa or paranasal sinuses, Comments
with variable histology. Nasosinusal polyps may be associated with different inflammatory
nasal diseases such as chronic rhino-sinusitis, cystic fibrosis, and Kartagener’s syndrome.
The pathogenesis of nasal polyposis is unclear. Polyp formation has been linked to a variety
of causes including allergy, chronic inflammation, autonomic nervous system dysfunction,
and genetic predisposition. Most theories consider polyps to be the ultimate manifestation
of chronic inflammation; therefore, conditions leading to chronic inflammation in the nasal
cavity can lead to nasal polyps. Benign multiple nasal polyposis is more common in patients
older than 40 years and is rare in children younger than 10 years. There is male gender
predominance with a male-to-female ratio of 2–4:1 in adults.
The clinical manifestation of nasal polyps depends on the size of the polyp. Small pol-
yps may not produce symptoms and may be identified only during routine examination.
When the polyps become larger, they can cause progressive nasal airway obstruction,
rhinorrhea, postnasal drainage, dull headaches, snoring, and anosmia. Massive polyposis
or a single large polyp can cause obstructive sleep symptoms and chronic mouth
breathing.
The diagnosis is made by clinical examination, endoscopy, and radiological examina-
tions. CT scan is a useful tool to diagnose the polyp or polyps, to define the extent of the
lesion in the nasal cavities, sinuses, and beyond, and to narrow the differential diagnosis.
The findings seen on CT scan, coronal view, in cases of nasosinusal polyposis include poly-
poid masses in the nasal cavity, partial or complete pansinus opacification, enlargement of
infundibula, bony attenuation of the ethmoid trabeculae and nasal septum, opacified eth-
moid sinuses with convex lateral walls, and air-fluid levels. The air-fluid levels may corre-
late with symptoms and signs of acute sinusitis in some patients.
The therapeutic options are medical and surgical treatment. Glucocorticoids play a
dominant role in conservative therapy, and topical application of steroids is the preferred
route. In cases of surgical treatment, recognition of sinonasal polyposis is important to the
endoscopic surgeon since it can be the most troubling sinonasal inflammatory disease to
manage due to its aggressive nature and tendency to recur despite appropriate treatment.
Surgical therapy should not be radical, but should focus on the lateral nasal wall rather
than on the healthy mucosa of the sinuses itself.
Coronal (Fig. 3.3a) and axial (Fig. 3.3b, c) un-enhanced CT scan images demonstrate near- Imaging Findings
complete pansinus and nasal cavity opacification consistent with nasosinusal polyposis. In
pneumatized areas, small polypoid lesions can be easily identified (arrows).
History An 81-year-old woman presented with a tumor located

Case 4 in superomedial portion of the right orbit that displaced
the ocular globe inferiorly. The lesion had been growing
Sinus Mucocele progressively for several years.
a b
Fig. 3.4
Mucoceles are chronic, slow-growing, benign, cystic lesions of the sinuses. They consist of a Comments
collection of mucoid fluid from obstructed sinus, which forms an expansile mass surrounded
by a cuboid epithelium. Even benign, they have a tendency to expand by eroding the
surrounding bony walls that displaces and destroys adjacent structures such as the orbit or
the anterior base of the skull by pressure and bony resorption. Mucoceles usually occur in
adults, with no gender predilection. Several predisposing factors have been identified for the
development of paranasal mucoceles, corresponding to conditions that cause a traumatic,
inflammatory, or tumoral distortion of the sinus drainage pathways. The occurrence of
mucoceles has usually been associated with previous nasosinusal surgery, facial trauma, and
chronic sinusitis with or without polyps. There are other less common predisposing factors
such as the presence of nasosinusal tumor lesions, cranial fibrous dysplasia, and cystic
fibrosis. Paranasal mucoceles predominantly affect the frontal sinus (60–65%), followed in
frequency by the ethmoidal (20–30%), maxillary (10%), and sphenoid (2–3%) sinuses.
Clinically, mucoceles remain asymptomatic until they become large enough to produce
symptoms secondary to their mass effect. Symptoms and signs of frontal mucoceles include
pain, swelling, exophtalmos, diplopia, and visual loss. An erosive mucocele may lead to men-
ingitis, meningoencephalitis, pneumocephalus, brain abscess, seizures, or cerebrospinal fluid
(CSF) fistulas. Mucoceles can turn, in approximately 50% of cases, into mucopioceles due to
Staphylococcus aureus or Albus colonization and, less frequently, to Streptococcus, Hemophilus
influenzae, and Escherichia coli. In these cases, there is an increase in volume, hence a faster
clinical evolution.
The diagnosis of mucocele is made on the basis of symptoms, imaging, and surgical explo-
ration and histological confirmation. The most informative radiologic technique is CT. On
CT, almost all mucoceles appear as airless sinuses with an expanded sinus cavity surrounded
by remodelled bone. Mucoceles are usually homogenous lesions, isoattenuating to brain, and
do not show enhancement after contrast administration, unless infected. On MRI, mucoceles
may show varied appearances on T1- and T2-weighted images that reflect changing protein
concentration, amount of free water, and viscosity. Gadolinium-enhanced MR imaging is
useful in differentiating mucoceles from sinonasal tumors. Mucoceles characteristically
reveal a thin peripheral linear enhancement with central low-signal intensity on T1-weighted
images and sinonasal tumors demonstrate diffuse enhancement. The intrasinus origin of the
process, the expanded sinus cavity, and the remodelled sinus walls suggest the diagnosis.
The recommended therapy for a mucocele consists of endoscopic surgical removal or
marsupialization.
Axial (Fig. 3.4a), coronal (Fig. 3.4b), and sagittal (Fig. 3.4c) un-enhanced CT scan images Imaging Findings
demonstrate an expansile mass arising from right frontal sinus that extends into the right
orbit and displaces extraocular muscles (arrows in Fig. 3.4b) and ocular globe. The tumor
has eroded the medial sinus wall and has extended into the contralateral frontal sinus. Note
the mass is homogeneous and water density.
Case 5
Osteoma
a b
Fig. 3.5
A 32-year-old female presented with a several-month history of progressive right History

exophthalmos and diplopia.
Osteomas are the most common tumors of the paranasal sinuses. They are benign, slowly Comments
growing, bone tumors of unknown etiology, although three theories have been postulated
regarding to their origin: traumatic, infectious, and developmental. The most common
locations are frontal sinus and ethmoidal cells although the maxillary and sphenoid sinuses
may also be involved. Osteomas are usually diagnosed between the second and third
decades of life and there is slight male gender predominance. Osteomas are commonly
sporadic isolated lesions, but can be multiple in Gardner’s syndrome, a hereditary disease
with autosomal dominant inheritance. This syndrome manifests with intestinal polyposis,
epidermal cysts, fibromatosis, and multiple osteomas in the mandible, the cranium, and the
long bones. Osteomas are histologically “bone forming” tumors, which are usually covered
by intact sinus mucosa. According to their structure, they can be divided into three types:
(1) compact osteoma, also called eburnated osteoma. In this type the bone is very dense
and lacks haversian canals. These osteomas develop from membranous elements. (2)
Osteoma spongiosum, also known as mature osteoma. It is composed of softer bone. This
type of osteoma arises from cartilagenous elements. These osteomas have little medullary
component containing fibrofatty tissue. (3) Mixed osteoma: This type contains elements of
both eburnated and mature types.
The majority of paranasal osteomas are asymptomatic and incidentally found on rou-
tine radiological examination. Some cases may cause symptoms as chronic frontal head-
ache, which is the most common presenting manifestation. Obstruction of the sinus ostium
may lead to infection or formation of a mucocele. When osteomas grow significantly and
go beyond the limits of the sinus, they can cause visual disturbances, facial dysmorphia and
even severe neurological problems if intracranial invasion happens.
Plain sinus radiographs are adequate for detecting osteomas of paranasal sinuses. CT
scans are more sensitive in demonstrating even small ones. Radiologically, osteomas appear
as well-circumscribed, sharply marginated, round and very dense lesions, usually less than
2 cm in size, attached to the sinus wall with a narrow pedicle or a broad base. MRI may be
helpful in cases of orbital or intracranial extension.
Clinical management of a small osteoma of the paranasal sinuses is still a matter of debate
and different approaches are recommended. Serial observation may be suitable for very small
lesions, however, resection should be considered if the osteoma shows significant growth, is
blocking the sinus and causing a mucocele, or is very large and threatening to produce an
acute sinus blockage. Osteomas, as benign lesions, do not tend to recur after excision.
Coronal (Fig. 3.5a) and axial (Fig. 3.5b, c) noncontrast CT scan images show a large calcium Imaging Findings
density mass arising from right ethmoidal cells with erosion of the lamina papiracea and
extension into the right orbit. The tumor displaces extraocular muscle (arrow in Fig. 3.5b)
and the ocular globe.
Case 6
Rhinocerebral Mucormycosis
a b
c d
Fig. 3.6
A 30-year-old male, with a recent history of bone marrow transplantation for treatment History
of acute myelomonocytic leukemia 2 months ago, presented with right periorbital
inflammation and pain.
Rhinocerebral mucormycosis is an invasive fungal opportunistic infection that usually Comments

affects patients with diabetes in poor control, however, any immunocompromised patient
may be infected. It is an acute and fulminant infection; indeed, it is the most lethal form of
fungal sinusitis with a reported mortality of 50–80%, caused by one of the members of the
mucoraceal family, including Absidia, Mucor, and Rhizopus. After inhalation into the nasal
cavity and paranasal sinuses, the fungi causes a necrotizing vasculitis of the nose and
sinuses, and rapidly progresses to the orbits, deep face, and cranial cavity. If recognized
early, involvement is limited to the nasal cavity and paranasal sinuses.
The most common presenting symptoms and signs are headache, fever, facial swelling,
sinusitis, and unilateral proptosis. Neurologic deficits may occur due to intracerebral
abscess formation and septic thrombosis of major intracranial vessels.
When a mucor infection is clinically suspected, CT and MRI findings can be diagnostic. The
most common imaging findings include opacification of sinuses with hyperdense material and
nodular mucosal thickening. On MRI, sinus contents present a variety of signal intensities, including
T2-WI hyperintensity or marked hypointensity on all pulse sequences, possibly secondary to the
presence of iron and manganese in the fungal elements. Soft-tissue infiltration of the deep face
and obliteration of the normal fat planes in the infratemporal fossa, pterygopalatine fossa, ptery-
gomaxillary fissure, and periantral fat are often present. Proptosis occurs because of enhancing
soft-tissue masses crowding the orbital apex and the cavernous sinuses. Lack of enhancement of
the superior ophthalmic vein or ophthalmic and internal carotid arteries may be seen and is
related to vasculitis and thrombosis. Intracranial findings include infarcts related to vascular
thrombosis, mycotic emboli, and frontal lobe abscesses. CT is better to assess for bone changes,
and MR imaging is superior in evaluating intracranial and intraorbital extension of the disease.
The mainstays of treatment are reversal of immunosuppression, systemic amphortericin
B, and surgical debridement. Although survival has improved dramatically during the last
years, deaths still occur if the infection is not recognized and not treated early in its course
or if the source of immunocompromise is not reversible.
Axial un-enhanced CT scan image of paranasal sinuses (Fig. 3.6a) demonstrates partial Imaging Findings
opacification with fluid levels in both maxillary sinuses, thickening of masticatory muscles
in the right infratemporal fossa (arrow in Fig. 3.6a), and obliteration of the normal fat
planes. CT scan also shows opacification of right ethmoidal cells and right orbital
involvement (Fig. 3.6b, c) with thickening of the extraocular muscles (arrow in Fig. 3.6b)
and inflammatory changes within the orbital fat (arrow in Fig. 3.6c). The findings are
consistent with mucormycosis. Despite the patient was treated with intravenous antifungal
therapy and underwent endoscopic surgery, he presented with left neurologic deficit 3 days
later. An unenhanced cerebral CT scan (Fig. 3.6d) was performed showing hypoattenuation
of the right caudate and lenticular nuclei consistent with ischemic infarct (arrow).
History A 32-year-old man presented with a 1-week history of

Case 7 frontoparietal headache. The patient also complained
about having visual loss for the past 2 days.
Sinonasal Carcinoma
a b
c d
Fig. 3.7
Malignant tumors of the sinonasal tract are rare and account for about 3% of head and Comments
neck cancers. However, they are important because their location is close to vital structures
and usually grow to considerable size before diagnosis as they remain paucisymptomatic
in their early stages. These tumors are most frequently found during the fifth to the seventh
decades of life and are more common in males than females (ratio 2:1). The majority of
sinonasal malignancies (60–70%) originate from the maxillary sinus and 20–30% occurs in
the nasal cavity itself. An estimated 10–15% occurs in the ethmoid air cells, with the
remaining minority of lesions found in the frontal and sphenoid sinuses. Over 80% of all
malignancies that arise in the nasal cavity and paranasal sinuses are squamous cell
carcinomas. The prognosis depends on the extent of the tumor and the site of origin.
Adenocarcinomas account for only 4–8% of all sinonasal cancers. They originate most
commonly at the ethmoids and nasal cavity and are locally aggressive.
The presenting symptoms are widely varied and may include a nasal mass or obstruc-
tion, pain, rhinorrhea, epistaxis, nasal discharge, swelling of the cheek, or cranial neuropa-
thies. As the majority of symptoms are similar to those of chronic sinusitis, the diagnosis
may be delayed by several months. Although the incidence of nodal metastasis from sinon-
asal malignancies is low, lymph node involvement implies a poor prognosis.
All patients with a suspicious nasal lesion should undergo a physical examination, endos-
copy, and radiological imaging studies. CT scan is excellent for depicting opacification, mass
effect, or bone destruction. It also allows the evaluation of the orbital apex, infratemporal
fossa, posterior ethmoid sinus, cribriform and pterygoid plates, and sphenoid sinus. MRI
can differentiate tumor and retaining secretions and demonstrates perineural spread and
other factors that determine resectability as orbital invasion, skull base invasion, intracra-
nial extension, and invasion of the masticator and parapharyngeal spaces by tumor. Both
modalities, CT scan and MRI, are complementary in evaluating and staging a sinonasal tract
mass and both should be performed prior to surgical intervention. On CT scan, sinus carci-
noma appears as a soft tissue mass in the sinus cavity usually associated with bony destruc-
tion. After contrast administration, the lesion shows mild and variable enhancement. On
MRI, tumors present intermediate signal intensity on T1-WI and high signal intensity on
T2-WI with enhancement following gadolinium injection. Small lesions are homogeneous,
whereas large lesions may contain internal areas of necrosis or hemorrhage.
Currently, therapy of sinus and nasal cavity malignancy is often multimodal with a
combination of radiation therapy, surgery, and chemotherapy.
Urgent non-enhanced CT scan (Fig. 3.7a) revealed a large mass filling the nasal cavity and Imaging Findings
extending into the anterior cranial fossa. MRI scan was performed the next day. Coronal
STIR images (Fig. 3.7b) demonstrated a mass arising from the sinonasal region with
intracranial extension. Sagittal (Fig. 3.7c) and coronal (Fig. 3.7d) T1-weighted MR images
obtained after intravenous injection of gadolinium clearly showed a large lobulated margin
tumor with strong enhancement. The lesion occupied the nasal cavity and extended into
the ethmoid cells, cribriform plates, sphenoid sinus, rhinopharynx, and anterior cranial
fossa. The gadolinium administration allowed the differentiation between tumor and
retaining secretions.
Case 8
Acoustic Neuroma
a b
c d
Fig. 3.8
A 42-year-old male presented with unilateral left sensorineural hypoacusia and tinnitus. History
Acoustic neuromas, also called vestibular schwannoma or cerebellopontine angle tumor, Comments
are intracranial, extra-axial, benign, slow-growing, capsulated tumors that arise from
vestibular or cochlear nerve sheaths, although the vast majority (95%) of acoustic neuromas
develop from the vestibular portion of the vestibulocochlear nerve. The tumor consists of
Schawnn cells in a collagen matrix. Acoustic schwannomas account for 8–10% of intracranial
tumors and 60–90% of cerebellopontine angle masses. Its incidence is slightly higher in
females and the majority affects individuals between 30 and 70 years, particularly between
fourth and sixth decades of life. The tumor may be found in younger patients with
neurofibromatosis 2 (NF2), an autosomal dominant disorder located on chromosome 22
that predisposes to the formation of bilateral acoustic neuromas. Patients with NF2 present
with bilateral vestibular schawnnomas, which are the hallmark of the disease, associated
with other Schwann cell tumors, multiple meningiomas, neurofibromas, and glial tumors.
Acoustic tumors produce symptoms by any of the four recognizable mechanisms: (1)
compression or distortion of the spinal fluid spaces, (2) displacement of the brain stem, (3)
compression of vessels producing ischemia or infarction, or (4) compression and/or atten-
uation of nerves. Unilateral hypoacusia is the most common symptom present at the time
of diagnosis and is generally the symptom that leads to diagnosis. Indeed, any unilateral
sensorineural hearing loss is caused by an acoustic neuroma until proven otherwise. Other
less common symptoms are tinnitus, vertigo, and headache.
The diagnostic technique of choice is gadolinium-enhanced MRI. Well-performed scan-
ning can demonstrate tumors as small as 1–2 mm in diameter. Gadolinium contrast is criti-
cal because nonenhanced MRI can miss small tumors. Based on the MRI scan, acoustic
neuromas can be divided into three types: (a) entirely intracanalicular, which means the
entire tumor is completely within the bony canal; (b) intracranial extension without brain
stem distortion, which means the intracranial portion of the tumor is small (1–2 cm), and
(c) intracranial extension with brain stem distortion, which means the intracranial portion
of the tumor is larger than 2 cm and pressing on the pons of the brain stem. On MRI, acous-
tic neuromas appear hypointense or isointense on T1-weighted images and hyperintense
on T2-weighted images. After gadolinium administration, the tumor shows intense and
homogeneous enhancement. Large lesions may have cystic degeneration. In addition to the
routine MR protocol, 3D high-resolution T2-weighted images help the diagnosis when the
tumors are intracanalicular because the sensitivity of this sequence is very high.
Surgical removal remains the treatment of choice for tumor eradication. Elderly patients
or patients with small tumors and preserved hearing can be managed conservative with
serial observation. Stereotactic radiotherapy is an alternative to microsurgery for selected
patients. This radiation therapy tries to prevent further tumor growth.
Axial and coronal T1-weighted MR images before (Fig. 3.8a, b) and after gadolinium Imaging Findings
administration (Fig. 3.8c, d) show a left intracanalicular tumor (arrows) that enhances with
contrast consistent with acoustic neuroma. Note that the lesion presents a small cystic/
necrotic component.
History A 21-year-old woman presented with a 2-year history of

Case 9 otorrhea. In the last month, the discharge became more
frequent and profuse. On otoscopic examination,
Cholesteatoma tympanic membrane perforation was found.
Comments Cholesteatomas are non-neoplastic keratinizing masses,

formed by aberrant epithelial rests of exfoliated keratin
within stratified epithelium that most commonly occurs
in the middle ear and mastoid region. Cholesteatoma
usually results from chronic otitis media, with squamous
metaplasia or extension of squamous epithelium inward
Fig. 3.9
to line and expanding cystic cavity that may involve the mastoid and erode surrounding
bone. Cholesteatomas can be divided into congenital and acquired types. Congenital
cholesteatomas occur due to abnormal migration of external canal ectoderm beyond the
tympanic ring. In these cases, there is no prior history of otorrhea, tympanic membrane
perforation, or previous otologic procedures. Congenital cholesteatoma appears as a pearly
white mass behind the tympanic membrane with a normal pars flaccida and tensa. The
most common sites of presentation on physical examination are the anterior-superior and
posterior-superior quadrants of the tympanic membrane. Congenital cholesteatomas most
commonly present with unilateral conductive hearing loss. Acquired cholesteatomas can
be subdivided into primary and secondary. Primary acquired cholesteatomas arise as a
result of tympanic membrane retraction. The classic primary cholesteatoma develops from
a progressively deeper medial retraction of the pars flaccida into the epitympanum.
Secondary acquired cholesteatomas occur as a direct consequence of injury to the tympanic
membrane. This injury can be a perforation secondary to acute otitis media or trauma, or
it may be due to surgical procedures in the drum.
Complications of growing cholesteatomas occur due to bony erosion and include intrac-
ranial abscesses, lateral sinus thrombosis, and meningitis. Bony erosion is a result of two
mechanisms: pressure effects that produce bony remodeling and enzymatic activity at the
margin of the cholesteatoma, which enhances osteoclastic activity with increase in bone
resorption. Bone erosion occurs later in congenital than acquired cholesteatomas.
CT is the study of choice for imaging of the temporal bone in cholesteatoma. On high-
resolution CT of the temporal bone, cholesteatoma appears as an avascular lobular middle
ear mass behind an intact tympanic membrane with bony erosion of the middle ear ossi-
cles, with the long process of incus and the stapes most commonly destroyed. As the mass
becomes larger, it erodes the middle ear wall, lateral semicircular canal, or tegmen tym-
pani. If the aditus ad antrum is occluded, the mastoid air cells opacify with retained secre-
tions. If the Eustachian tube becomes obstructed then middle ear effusions and otitis can
occur. MRI can be a complimentary technique when intracranial extension and bony
defects are observed or suspected. MRI is also indicated for cases of facial nerve involve-
ment and unexplained sensorineural hypoacusia. Cholesteatomas commonly show low
signal intensity on T1-weighted images and moderately high signal intensity on T2-weighted
images. The tumor typically does not enhance after gadolinium administration, except
rarely at the margins of the lesion and shows variable signal intensities on contrast-
enhanced studies. MRI is useful as well to detect intracranial complications including
abscesses, lateral sinus thrombosis, and meningitis.
Treatment for congenital cholesteatomas includes myringotomy for small lesions, and
mastoidectomy for larger lesions. Ossicular chain reconstruction may also be necessary.
Axial (Fig. 3.9a) and coronal (Fig. 3.9b, c) un-enhanced CT scan images reveal a soft-tissue Imaging Findings
attenuating right middle ear mass, which surrounds and erodes middle ear ossicles. Coronal
images clearly depict that the lesion extents into epytimpanum (asterisk in Fig. 3.9b) and
Prusack’s space (arrow in Fig. 3.9c) and erodes scutum (double arrow in Fig. 3.9c) and,
partially, the head of the malleus.
History A 67-year-old female presented with conductive hearing loss in the left ear. The otoscopic
Case 10 examination revealed a reddish pulsatile mass in the middle ear.
Jugulotympanic
Glomus Tumor Glomus tympanicum is the most common primary neoplasm of the middle ear and the
second most common tumor of the temporal bone. This tumor arises from nonchromaffin
paraganglia or glomus bodies located in the adventitia of the dome of the jugular bulb.
When the tumor arises from the middle ear is termed as glomus tympanicum and when
Comments arises from jugular fossa is called glomus jugulare tumor. Glomus tympanicum is a
benign, slow-growing tumor that may become locally aggressive and may spread along
a b
c d
Fig. 3.10
paths of least resistance.According to the area and degree of involvement, glomus tympanicum
is divided into four types: Type 1, glomus limited to the promontory; Type 2, tumor that fills
completely the middle ear; Type 3, tumor extending further into mastoid; Type 4, tumor
spreading into external auditory canal with or without intracranial extension.
Glomus tympanicum is more common in females than males (ratio 4:1) and usually
affects middle-aged patients with a peak incidence in the fifth decade. Clinically, the most
common presenting symptoms are conductive deafness and pulsatile tinnitus. Other symp-
toms may include aural hemorrhage or otorrhea, otalgia, and facial palsy. When the tumors
enlarge within the jugular foramen, neuropathies of IX, X, XI, and XII may occur.
Diagnosis is made by otoscopy and imaging studies. On otoscopy the tumor usually appears
as a reddish, pulsatile mass behind an intact tympanic membrane. Nevertheless, otoscopy can
be misleading sometimes. When the margins of the meso-tympanic mass are visible at 360°,
the lesion is classified as a glomus tympanicum. If the margins cannot be clearly identified
during otoscopy, the tumor must be assumed to be a glomus jugulare until proven otherwise.
CT and MRI scans are the imaging techniques of choice for evaluating the size and extent of
glomus tumors. On CT scan, glomus tympanicum appears as a soft tissue mass abutting the
promontory of the middle ear. The most important assessment when a jugulotympanic tumor
is suspected is the state of the jugular fossa. An intact jugular fossa and the demonstration of
air or bone between the tumor mass and the jugular bulb helps immediately identify the
lesion as a glomus tympanicum and almost exclude the existence of a glomus jugulare com-
pletely. In addition, CT scan clearly shows ossicle status or bony erosion of the tympanic cav-
ity, which is a hallmark of jugulotympanic glomus tumors. These tumors enhance intensely
after contrast material administration. Sometimes, it may not be possible on a contrast-
enhanced CT study to differentiate the normal opacified internal jugular vein from glomus
jugulare tumor. Besides, CT may not be totally reliable for assessing whether the tumor has
arisen from the jugular fossa or from the middle ear in two situations: (1) when the tumor
reaches the floor of the middle ear and (2) when the tumor produces obstruction of the
Eustachian tube with secondary fluid accumulation in the rest of the middle ear and mastoid.
This fluid is indistinguishable from the tumor on CT scans. In the above situations, gadolin-
ium-enhanced MRI may be useful. MRI also helps to assess the intracranial extension and the
relation of the glomus to the regional neurovascular anatomy. On gadolinium-enhanced MR
images, the tumor also enhances intensely. Angiography is no longer mandatory, but it should
be considered for preoperative evaluation of selected cases when embolization can aid the
surgeon by reducing blood loss during surgery in cases of large tumors.
Therapeutic options for glomus tumors of the temporal bone include surgical excision,
radiotherapy, combined therapy and, in selected cases, observation. Surgical excision offers
the only chance for total tumor eradication.
Axial CT scan (Fig. 3.10a) shows a soft tissue mass occupying the left tympanic cavity that Imaging Findings
erodes the promontory and extends into the jugular foramen (Fig. 3.10b) causing bony
erosion and enlargement of the jugular foramen. Note opacification of the left mastoid air
cells due to retaining secretions. Axial T1-weighted un-enhanced (Fig. 3.10c) and gadolinium-
enhanced (Fig. 3.10d) MR images demonstrate intense enhancement of the lesion (arrows).
Further Reading Noujaim SE, Pattekar MA (2000) Paraganglioma of the temporal

bone: role of magnetic resonance imaging versus computed
Aribandi M, McCoy VA, Bazan C 3rd (2007) Imaging features of tomography. Top Magn Reson Imaging 11:108–122
invasive and noninvasive fungal sinusitis: a review. Obeso S, Llorente JL, Rodrigo JP et al (2009) Paranasal sinuses
Radiographics 27(5):1283–1296 mucoceles. Our experience in 72 patients. Acta Otorrinolaringol
Berrylin J, Ferguson MD (2000) Fungal rhinosinusitis: a spec- Esp 60(5):332–339
trum of disease mucormycosis of the nose and paranasal Phelps PD, Cheesman AD (1990) Imaging jugulotympanic glo-
sinuses. Otolaryngol Clin North Am 33(2):349–365 mus tumours. Arch Otolaryngol Head Neck Surg 116(8):
Chan LL, Singh S, Jones D, Diaz EM Jr, Ginsberg LE (2000) 940–945
Imaging of mucormycosis skull base osteomyelitis. AJNR Am Phelps PD, Stansbie JM (1988) Glomus jugulare or tympanicum?
J Neuroradiol 21(5):828–831 The role of CT and MR imaging with gadolinium DTPA.
Dammann F, Pereira P, Laniado M et al (1999) Inverted papil- J Laryngol Otol 102(9):766–776
loma of the nasal cavity and the paranasal sinuses: using CT Pitts LH, Jackler RK (1998) Treatment of acoustic neuromas
for primary diagnosis and follow-up. AJR Am J Roentgenol [editorial; comment]. N Engl J Med 339(20):1471–1473
172(2):543–548 Raghavan P, Phillips CD (2007) Magnetic resonance imaging of
Drutman J, Harnsberger HR, Babbel RW et al (1994) Sinonasal sinonasal malignancies. Top Magn Reson Imaging 18(4):
polyposis: investigation by direct coronal CT. Neuroradiology 259–267
36(6):469–472 Rodríguez Prado N, Llorente Pendás JL, Del Campo Rodríguez A
Earwaker J (1993) Paranasal sinus osteomas: a review of 46 et al (2004) Paranasal sinus osteoma. Revision of 14 cases.
cases. Skeletal Radiol 22:417–423 Acta Otorrinolaringol Esp 55(5):225–230; in Spanish
Fatterpekar GM, Doshi AH, Dugar M, Delman BN, Naidich TP, Roland PS, Marple B (1998) Diagnosis and management of
Som PM (2006) Role of 3D CT in the evaluation of the tempo- acoustic neuromas. American Academy of Otolaryngology,
ral bone. Radiographics 26(Suppl 1):S117–S132; Review Alexandria
Frosini P, Picarella G, De Campora E (2009) Antrochoanal polyp: Rosenberg SI (2000) Natural history of acoustic neuromas.
analysis of 200 cases. Acta Otorhinolaryngol Ital 29(1):21–26 Laryngoscope 110(4):497–508
Jackson CG (2001) Glomus tympanicum and glomus jugulare Silverman CS, Mancuso AA (1998) Periantral soft-tissue infiltra-
tumours. Otolaryngol Clin North Am 34(5):941–970, vii; Review tion and its relevance to the early detection of invasive fungal
Karkos PD, Khoo LC, Leong SC et al (2009) Computed tomogra- sinusitis: CT and MR findings. AJNR Am J Neuroradiol
phy and/or magnetic resonance imaging for pre-operative 19:321–325
planning for inverted nasal papilloma: review of evidence. Terk MR, Underwood DJ, Zee C, Colletti PM (1992) MR imaging
J Laryngol Otol 123(7):705–709; Epub 2009 Feb 16 in rhinocerebral and intracranial mucormycosis with CT and
Kazahaya K, Potsic WP (2004) Congenital cholesteatoma. Curr pathological correlation. MRI 10:81–87
Opin Otolaryngol Head Neck Surg 12(5):398–403 Tiwari R, Hardillo JA, Mehta D et al (2000) Squamous cell carci-
Levie P, Orban D, Verheyden PJ, Monnoye JP, Daele J (2000) The noma of maxillary sinus. Head Neck 22(2):164–169
antrochoanal polyp. Acta Otorhinolaryngol Belg 54(2):109–113 Towbin R et al (1979) Antrochoanal polyps. AJR 132:27–31
Maldonado M, Martínez A, Alobid I, Mullol J (2004) The antro- Vashist S, Goulatia RK, Dayal Y, Bhargava S (1985) Radiological
choanal polyp. Rhinology 42(4):178–182 evaluation of mucocoele of the paranasal sinuses. Br J Radiol
McLean FM, Ginsberg LE, Stanton CA (1996) Perineural spread 58(694):959–963
of rhinocerebral mucormycosis. AJNR Am J Neuroradiol Weissman JL, Hirsch BE (2000) Imaging of tinnitus: a review.
17:114–116 Radiology 216:342–349
Mendenhall WM, Hinerman RW, Malyapa RS et al (2007) Woodruff WW, Vrabec DP (1994) Inverted papilloma of the
Inverted papilloma of the nasal cavity and paranasal sinuses. nasal vault and paranasal sinuses: spectrum of CT findings.
Am J Clin Oncol 30(5):560–563 AJR Am J Roentgenol 162:419–423
Moriyama H, Nakajima T, Honda Y (1992) Studies on muco- Yousem DM, Gad K, Tufano RP (2006) Resectability issues with
coeles of the ethmoid and sphenoid sinuses: a report of 47 head and neck cancer. AJNR Am J Neuroradiol 27(10):
cases. J Laryngol Otol 106(1):23–27 2024–2036
Orocervical Region 4
Contents
Case 1 Adenoid Cystic Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 68
Case 2 Branchial Cleft Cyst . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 70
Case 3 Carotid Body Paraganglioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 72
Case 4 Cervical Abscess . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 74
Case 5 Juvenile Nasopharyngeal Angiofibroma . . . . . . . . . . . . . . . . . . . . . . . 76
Case 6 Laryngeal Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 78
Case 7 Nasopharyngeal Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 80
Case 8 Pleomorphic Adenoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 82
Case 9 Warthin’s Tumor . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 84
Case 10 Sialolithiasis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 86

A 60-year-old man presented with a several-month history of progressive right-sided facial

Case 1 swelling that caused facial deformation.
Adenoid
Cystic Adenoid cystic carcinoma (ACC) is a malignant epithelial tumor developing in the salivary glands.
Carcinoma Although the tumor may be located in the parotid and submandibular glands, the minor salivary
glands are the most common place of origin of this carcinoma and ACC accounts for approximately
33% of all malignancies of the minor salivary glands. The tumor occurring in these glands has a
worse prognosis than when it occurs in the major glands. This carcinoma is more frequently found
Comments in patients between the fourth and seventh decades of life, predominantly in the fifth decade, and
shows a slight predominance in females (ratio female:male is 3:2). ACC has a marked tendency to
invade nerve tissue and this condition occurs in up to 60% of cases. Specifically, it involves the
nerve sheaths, with a typical pattern of skip metastasis, covering a wide locoregional area of the
nerves peripheral to the primary tumor. Cervical metastases are rare and occur in only 8–13% of
c d
Fig. 4.1
patients. Distant metastases may present in up to 50% of ACC patients during the course of
the disease, with the lungs and liver as the most common sites.
Clinically, the tumor usually presents as a mass that may be accompanied by a dull pain
and/or paralysis of a cranial nerve especially the facial nerve when the tumor occurs in the
parotid gland. Perineural invasion is an almost invariably microscopic finding and defi-
nitely seen in the head and neck area, more often with primary squamous cell carcinomas
of cutaneous origin, followed by ACC. On histologic examination, these neoplasms are clas-
sified according to their predominant histologic pattern into three types: tubular, cribri-
form, and solid. They may occur either separately or together in the same tumor. Cribriform
subtype is the most common and solid subtype is the most aggressive.
The diagnosis is made on the basis of histological analysis of a biopsy and imaging tech-
niques. On imaging studies, ACC may show both benign and malignant features. Tumors located
in parotid gland usually present as well-defined lesions with benign pattern, whereas tumors of
the minor salivary glands usually appear as infiltrative malignant lesions. Computed tomogra-
phy (CT) and magnetic resonance imaging (MRI) scans should be done preoperatively for all
minor salivary gland ACC in order to determine the tumor stage, to plan proper surgical inter-
vention, and as a reference for future follow-up. On T2-weighted images, ACC may show variable
signal intensity depending on the tumor subtype; lesions with low signal intensity correspond
to highly cellular tumors (solid subtype) and lesions with high signal intensity correspond to
less cellular tumors (cribriform or tubular subtype) with a better prognosis. CT and MR images
are not specific in the differentiation of ACCs from other types of tumors, therefore, a biopsy
must be done to ensure correct diagnosis. The optimal method for imaging perineural tumor
infiltration depends on the involved cranial nerve. Both high-resolution direct coronal CT and
magnetic resonance show clearly the perineural tumor below the skull base. MRI best depicted
skull base, cisternal, and brain stem perineural tumor infiltration. Some authors suggest that
T1-weighed MRI before and after administration of gadolinium is the method of choice in the
investigation of perineural invasion whereas others defend that visualization of the tumoral
infiltration around the cranial nerves is probably best evaluated by using nonenhanced and
gadolinium-enhanced fat-saturated T1-weighted MRI sequences. The appearance of perineural
tumor spread includes thickening and abnormal enhancement or both along the main branches
of the nerves, foraminal widening erosion, and obliteration or both of the perineural fat pads.
ACC is classically considered unresponsive to chemotherapy, maybe because of its slow
growth rate. When feasible, wide surgical resection followed by radiation therapy is the
only curative treatment option. Nevertheless, the long-term outcome for patients with ACC
is poor with a relatively high recurrence rate that may be due to incomplete primary
surgical resection and a perineural tumor extension. There is no effective therapy for
advanced, recurrent, or metastatic disease.
Axial (Fig. 4.1a) and coronal (Fig. 4.1b) CT scan images show a large lobulated tumor Imaging Findings
located in the right superior maxillary bone with extension into the homolateral maxillary
sinus. Note erosion of the superior maxillary bone and hard palate. Axial contrast-enhanced
fat-saturated T1-weighted image (Fig. 4.1c) and coronal contrast-enhanced T1-weighted
image (Fig. 4.1d) better demonstrate the lesion boundaries. The tumor extends into the
right nasal fossa and crosses the midline.
History A 24-year-old woman presented with a 2-month history

Case 2 of a left neck mass.
Branchial Cleft Cyst

Comments Branchial cleft cysts are congenital cysts, which arise on the
lateral part of the neck, formed by incomplete involution of
branchial cleft structures during embryonic development.
The second branchial cleft accounts for 95% of branchial
anomalies, and they are most frequently found along the
anterior border of the upper third of the sternocleidomastoid
muscle between the muscle and the overlying skin.
Branchial cleft cysts are the most common congenital
a c
Fig. 4.2
cause of a cervical mass. An estimated 2–3% of cases are bilateral. Branchial cleft cysts usually
present in the second to fourth decades of life with no gender predilection. A tendency exists for
cases to cluster in families. Branchial cleft cysts are lined by stratified squamous epithelium and
can contain keratin, hair follicles, sweat and sebaceous glands, and hyaline cartilage. The lumen
is usually filled with a thick yellow fluid containing a large amount of cholesterol crystals.
The majority of branchial cleft cysts are asymptomatic. When they become symptom-
atic, they commonly appear as a solitary, round, painless, compressible, fluctuant mass
located in the lateral aspect of the neck. A history of intermittent swelling and tenderness
of the lesion during upper respiratory tract infection may exist. They may also develop
abscesses due to the lymphoid tissue located beneath the epithelium. Spontaneous rupture
of an abscessed branchial cleft cyst may result in a purulent draining sinus to the skin or
the pharynx. Depending on the size and the anatomical extension of the lesion, local symp-
toms, such as dysphagia, dysphonia, dyspnea, and stridor, may occur.
The diagnosis is based on clinical history and imaging methods such as ultrasound, CT,
and MRI. On CT and MRI, contrast is needed to differentiate cyst from solid mass.
Ultrasonography (US) is useful to determine the cystic nature of the mass but it does not
adequately evaluate the extent and depth of neck lesions. On US, branchial cleft cyst appears
as an anechoic thin-walled cyst with posterior acoustic enhancement. The lesion may be
hypoechoic or variably echogenic if infected. CT scan findings are usually diagnostic for
branchial cleft cysts. Contrast-enhanced CT scan reveals a unilocular, smooth, and well-
circumscribed mass of fluid attenuation in a characteristic location, immediately anterior
to the upper third of the sternocleidomastoid muscle. The lesion does not associate strand-
ing or induration of surrounding structures, or significant wall enhancement, or enhancing
nodule. Rarely, if infected, the cyst may display minimal enhancement, but that is the excep-
tion to the rule. CT may aid preoperative planning and identify compromise of local struc-
tures. MRI allows for finer resolution during preoperative planning. On MRI, branchial cleft
cysts show high signal intensity on T2-weighted images. On T1-weighted images, the signal
intensity is usually low, but previous infection can produce proteinaceous debris that
increases the T1 signal intensity. Uninfected branchial cleft cysts should not enhance on
MRI and lesions should not associate stranding or induration of surrounding structures.
Infiltration of surrounding tissue may indicate another diagnosis as lymphangioma.
Differential diagnosis includes lymphangioma, lymphadenopathy, abscess, glandular
cysts, ranulas, dermoid cysts, laryngoceles, thyroglossal duct cysts, hemangiomas, and
paragangliomas.
Regarding treatment, surgical excision is definitive therapy for these lesions.
US (Fig. 4.2a) shows a well-defined cystic lesion containing heterogeneous material with Imaging Findings
no vascularization detected by Doppler ultrasound (not shown). Axial (Fig. 4.2b) and
coronal (Fig. 4.2c) contrast-enhanced CT scan images demonstrate a well-circumscribed
mass of fluid attenuation located immediately anterior to the upper third of the
sternocleidomastoid muscle (white arrow in Fig. 4.2b), posterior to left submaxillary gland
(black arrow in Fig. 4.2b), and lateral to cervical vessels (white arrow in Fig. 4.2c). The mass
is consistent with complicated second branchial cleft cyst.
History A 48-year-old man presented with a left cervical

Case 3 pulsatile mass with progressive growth for the last six
months.
Carotid Body Paraganglioma
a b
c d
Fig. 4.3
Paraganglioma arising from the carotid body are relatively rare tumors although they Comments
constitute the majority of head and neck paragangliomas (60–70%). Carotid paraganglioma
are more common in women with female-to-male ratio of 2.7:1 and can occur at any age,
with a peak incidence in the 45–50-year-old age group. Bilateral carotid body lesions occur
in approximately 10% of the cases. Most of these neoplasms are benign.
Clinically, carotid body paraganglioma usually presents as a slow-growing, nontender
neck mass in an otherwise asymptomatic patient. The lesion is located just anterior to the
sternocleidomastoid muscle at the level of the hyoid and is mobile in the lateral plane but its
mobility is limited in the craniocaudal direction (fontaine`s sign). Occasionally the tumor
may transmit the carotid pulse or demonstrate a bruit or thrill. Due to its location in close
contact to carotid vessels and X–XII cranial nerves, tumor enlargement may cause progres-
sive symptoms such as dysphagia, odynophagia, hoarseness, or other cranial nerve deficits.
Diagnosis of carotid body tumors is based on physical examination and imaging tech-
niques such as US, CT, MR, and angiography. Imaging studies depict the location and extent
of tumor involvement, help determine the surgical planning, and help predict operative
morbidity and mortality. US scanning is the primary diagnostic technique. The character-
istic appearance of a carotid body tumor on gray-scale US scans is a round-to-oval, well-
circumscribed, heterogeneously hypoechoic solid mass in the lateral neck with splaying of
external and internal carotid arteries. Contrast-enhanced CT and MRI detect these lesions
equally well. The typical CT appearance of a carotid body tumor is a well-defined, soft-
tissue mass within the carotid space of the infrahyoid neck. After contrast material
administration, the tumor presents homogeneous and strong enhancement due to its
hypervascularity. Widening of the common carotid bifurcation is very suggestive of a
carotid body tumor. On MRI, paragangliomas typically appear hypointense on T1-WI and
hyperintense on T2-WI. As with CT, the lesion exhibits a homogeneous and intense enhance-
ment following gadolinium administration. Multiple serpentine and punctate areas of sig-
nal void characterize the typical paraganglioma with all MR sequences; these areas are
variably distributed throughout the mass and are believed to represent flow voids in the
larger intratumoral vessels. The typical angiographic appearance of a paraganglioma is
that of a hypervascular mass with enlarged feeding arteries, intense tumor blush, and early
draining veins. Widening of the carotid bifurcation by the tumor is known as the “lyre sign,”
which is a classic pathognomonic angiographic finding.
Surgical removal is the treatment of choice. Preoperative embolization can be used to
shrink tumor vascularity and size, with a consequent decrease in intraoperative blood loss.
Axial contrast-enhanced CT scan (Fig. 4.3a) shows a hypervascularized tumor that splays Imaging Findings
the internal and external carotid arteries (arrows). Contrast-enhanced CT scan image
(Fig. 4.3b), obtained at a higher level than (Fig. 4.3a), demonstrates another lesion with
similar features located posteriorly to the first one (arrow). Sagittal contrast-enhanced CT
(Fig. 4.3c) and preoperative digital subtraction angiography (Fig. 4.3d) delineate the lesions
in a better way. Another lesion can be seen in a more superior location (arrow in Fig. 4.3c).
The findings are consistent with three concomitant paragangliomas, a carotid body
paraganglioma, a vagal paraganglioma, and a jugular paraganglioma.
History A 25-year-old woman presented with a 2-week history

Case 4 of a left laterocervical painful mass with no response to
antibiotic therapy. The patient developed rapid tumor
Cervical Abscess growth accompanied by trismus.
Comments Infection of the neck is a common clinical problem in

all age groups, especially children and young adults.
Despite the widespread availability of antibiotics and
a b
c d
Fig. 4.4
early surgical intervention, it continues to be a serious clinical condition with potentially

devastating complications. Complex neck anatomy can frequently obscure or delay
diagnoses, so timely and appropriate radiological interpretation is critical to patient care.
Infections of the neck may be of nasal, oral, otitic, or bony origin. Currently, tonsillitis
remains the most common etiology of deep neck space infections in children, whereas odon-
togenic origin is the most common etiology in adults. The causative organisms of neck infec-
tion including lymphadenitis are varied and can be subdivided into bacterial, fungal, parasitic,
and viral inflammatory disorders. Accumulation of pus, which constitutes an abscess, may be
contained within various fascial spaces but is more commonly found in the retropharyngeal,
danger, or parapharyngeal spaces. Complications of deep neck abscesses include arterial ero-
sion, venous thrombosis, caudal extension leading to mediastinitis, pharyngeal rupture with
subsequent aspiration and pulmonary abscess, and intracranial spread.
The clinical manifestations are often suggestive of the diagnosis. The symptoms and
signs of neck infections include fever, pain with limitation of neck motion, trismus,
anorexia, dysphagia, odynophagia, adenopathy, and a neck mass secondary to phlegmon or
an abscess associated with an elevated leukocyte count. In advanced neck infections with
laryngeal edema there may be dysphonia, hoarseness, stridor, and dyspnea.
Imaging studies, including CT and MR imaging, are frequently required to confirm the
diagnosis but more importantly to localize the infectious process and search for and delin-
eate an abscess cavity. US has also been used in the evaluation of superficial neck infec-
tions, especially to determine fluid accumulation. Enhanced CT scan is the gold standard
in the evaluation of neck infections. CT scan indicates the location, boundaries, and rela-
tion of infection to surrounding neurovascular structures. The typical appearance of an
abscess is a low-density lesion with rim enhancement, occasional air fluid levels, and locu-
lations. CT scanning of the chest may be helpful if extension into the mediastinum is sus-
pected. MRI is not the initial modality of choice and it is not commonly used for
inflammatory disease. However, when obtained, MRI scans can give excellent soft tissue
resolution to help localize the region of involvement. On MRI, abscesses appear hypoin-
tense on T1-weighted images and hyperintense on T2-weighted images. After gadolinium
administration, a central area of no enhancement, indicating pus collection, can readily be
demonstrated as well as the typical peripheral enhancement.
The treatment varies depending on whether an abscess has developed. Intravenous
antimicrobial therapy is the treatment of choice for phlegmon; however, if an abscess is
present surgical intervention is mandated. The type and approach has to be tailored
according to the location and size of the abscess.
Axial contrast-enhanced CT scan images (Fig. 4.4a, b) demonstrate an abscess located in Imaging Findings
the left submaxillary region. The abscess is surrounding the mandible and causes
compression and posterior displacement of the left submaxillary gland (arrow).
Inflammatory changes in the perilesional fat are also evident. On coronal CT image
(Fig. 4.4c), cortical erosion in the medial margin of the mandible can be seen (arrow). This
finding suggests the odontogenic origin of the abscess. On sagittal CT image (Fig. 4.4d),
reactive lymphadenopathies are better depicted (arrows).
Case 5
Juvenile Nasopharyngeal Angiofibroma
a b
c d
Fig. 4.5
A 14-year-old male presented with nasal stuffiness, continuous rhinorrhea and occasional History
epistaxis. On the endoscopic examination, a polypoid lesion was found arising from the
rhinopharynx and filling the left posterior nasal cavity.
Nasopharyngeal angiofibroma is a benign vascular tumor that occurs in the nasopharynx Comments
of prepubertal and adolescent males. The most frequent symptoms are unilateral nasal
obstruction and spontaneous epistaxis. The lesion typically arises in the sphenopalatine
foramen and it often acts in a malignant manner by eroding into the surrounding sinuses,
orbit, or cranial vault. This tumor is highly vascularized, with its main blood supply coming
from the internal maxillary artery, although the ascending pharyngeal or vidian arteries
may also feed the lesion.
Treatment is usually preoperative embolization to reduce blood loss followed by surgical
resection (preferably endoscopic). Radiation therapy is a treatment option reserved for
patients with incomplete resection, intracranial disease, or recurrent cases.
CT scan after contrast administration (Fig. 4.5a) shows an enhancing mass in the left Imaging Findings
posterior nasal cavity extending into the cavum (open arrow). The lesion extends laterally
through the pterygomaxillary fissure into the infratemporal fossa and erodes the posterior
wall of the maxillary sinus. Note the aggressiveness of the tumor, which produces erosion
of the greater wing of the sphenoid and left side of the clivus.
Axial (Fig. 4.5b) and coronal (Fig. 4.5c) gadolinium-enhanced fat-saturated T1-weighted
MR images demonstrates intense enhancement of the lesion. The borders are clearly
depicted. A nodule is seen protruding into the dura of the middle cranial fossa (open
arrow); no cerebral involvement is detected.
Digital subtraction angiography (Fig. 4.5d) was performed prior to surgery in order to
embolize the tumor. Angiography shows that the lesion is fed by branches of the internal
maxillary artery (open arrow).
A 72-year-old man referred to ENT department for a long history of hoarseness that had wors-
Case 6 ened over the previous weeks, associated with dyspnea of effort. On laryngoscopic examination, a
tumor involving left larynx with vocal cord paralysis and glottic stenosis were found.
Laryngeal
Carcinoma
Laryngeal carcinoma is the most common cancer of the upper aerodigestive tract. This tumor
accounts for 25% of head and neck cancer and 1% of all cancers. The incidence of laryngeal
tumors is closely correlated with two main risk factors—smoking and alcohol. The majority of
laryngeal tumors (85–95%) are squamous cell carcinomas that arise from the mucosal surface of
Comments the aerodigestive tract. Laryngeal cancer is most prevalent in the sixth and seventh decades of
life and has a male gender predilection (ratio 4:1). Laryngeal carcinomas are divided into three
a b
c d
Fig. 4.6
groups depending on tumor location: (1) supraglottic carcinoma (40%), (2) glottic
carcinoma (59%), and (3) subglottic carcinoma (1%). Supraglottic tumors are more
aggressive and tend to invade the preepiglottic space and metastasize to lymph nodes.
Glottic tumors grow slower and tend to metastasize late owing to a paucity of lymphatic
drainage. True subglottic tumors are rare, and most subglottic masses are extension from
glottic or supraglottic carcinomas.
Clinically, the most common and alarming symptom of this malignancy is hoarseness.
Patients presenting with hoarseness should undergo an indirect mirror examination and/
or flexible laryngoscope evaluation. Other symptoms include persistent pain/discomfort in
the throat, swallowing difficulty, dyspnea, hemoptysis, ear pain, aspiration, and neck mass.
The diagnosis is based on clinical examination. Malignant lesions can appear as friable,
fungating, ulcerative masses or be as subtle as changes in mucosal color. Biopsy is required
for diagnosis. Imaging plays a crucial role in pretherapeutic diagnosis and tumor staging,
which is critical due to the variety of therapeutic options available based on tumor exten-
sion. Cross-sectional imaging (CT or MRI) of the head and neck should be performed to
establish the extent of primary disease, the presence of bone or cartilage invasion, and the
presence of nodal metastases. Currently, multidetector CT is the first-line imaging investi-
gation for staging laryngeal carcinoma. The overall staging accuracy for multidetector CT,
with its rapid scanning time, high spatial resolution, and multiplanar reformatted images,
is about 93%. On CT, direct signs of laryngeal malignancy include: presence of a circum-
scribed tumor mass, infiltration of fatty tissue, muscle, or cartilage, asymmetric soft tissue
swelling, and abnormal pattern of contrast enhancement. Indirect signs include metastatic
cervical lymphadenopathy. Compared to CT, MRI has a similar ability to define the inter-
face between fat and tumor, but is superior for assessing muscle and cartilage invasion. MRI
is indicated if there are equivocal findings on multidetector CT, including possible cartilage
invasion. MRI examination should be performed before and after contrast enhancement.
On nonenhanced studies, tumors are of intermediate signal intensity and easily distin-
guished from adjacent fat. After gadolinium enhancement, fat suppression helps in differ-
entiating enhancing tumor from the normal preepiglottic and paraglottic fat.
The treatment plan for laryngeal carcinoma depends on several factors, including tumor
location, tumor stage, histology, and the patient’s age and other medical comorbidities. The
aim of therapy is to conserve the laryngeal function while achieving the best life expectancy
and quality of life. Treatment options include various types of surgery and radiation with
adjuvant chemotherapy.
Contrast-enhanced multidetector CT scan images (Fig. 4.6a–d) demonstrate a large tumor Imaging Findings
involving the entire left vocal cord (arrow in Fig. 4.6a), the anterior portion of the right vocal cord,
and the anterior commissure (arrows in Fig. 4.6b). The lesion invades through the thyroid cartilage
(Fig. 4.6c) and extends into the thyrohyoid muscles (arrow in Fig. 4.6d) causing airway stenosis.
History A 48-year-old man presented with a 3-month history of

Case 7 right nasal obstruction and otalgia. On physical
examination, occupation of the nasal fossa and left
Nasopharyngeal Carcinoma serous otitis media were found.
a b
c d
Fig. 4.7
Nasopharyngeal carcinoma (NPC) is a tumor arising from the epithelial cells that covers Comments
the surface and lines the nasopharynx. There are three histologic subtypes of NPC that
vary in their clinical behavior and prognostic significance. Type I (keratinizing squamous
cell carcinoma) is the least common, found in nonendemic areas, carries the least favorable
prognosis, and is similar to squamous cell carcinomas of oropharyngeal origin with a
relation to alcohol and tobacco abuse. This type is typically found in the older adult
population; Types II (nonkeratinizing squamous cell carcinoma) and III (undifferentiated
carcinoma, 60%) are more common, carry a more favorable prognosis, and are more
sensitive to radiotherapy. Men are twice as likely to develop NPC as women. The peak
incidence is usually in the fifth to sixth decades of life. NPC usually originates in the lateral
wall of the nasopharynx, which includes the fossa of Rosenmuller; indeed, this fossa is the
most common location. It can then extend within or out of the nasopharynx to the other
lateral wall and/or posterosuperiorly to the base of the skull or the palate, nasal cavity, or
oropharynx. This tumor typically spreads to cervical lymph nodes. Distant metastases may
occur in bone, lung, mediastinum, and, more rarely, the liver.
NPC presents most commonly as a unilateral painless neck mass in 50–70% of patients,
from enlarged cervical lymphadenopathy. The tumor may not be clinically apparent at the
time of presentation and the diagnosis of NPC is often made by lymph node biopsy.
Symptoms related to the primary tumor include nasal obstruction, epistaxis, hearing loss,
tinnitus, recurrent otitis media, cranial nerve dysfunction, sore throat, and headache.
Diagnostic methods include clinical examination of the size and location of cervical lymph
nodes, indirect nasopharyngoscopy to assess the primary tumor, and radiological cross-
sectional techniques (CT and MRI) for staging and evaluation. Endoscopic examination of the
nasopharynx with biopsy should be performed in patients suspected of having NPC, particu-
larly if risk factors are present. CT scan remains the most common imaging technique for tumor
mapping and nodal staging. Indeed, contrast-enhanced CT scan of the head and neck, with
both bone and soft tissue windows, is used to determine tumor extent, base of skull erosion, and
cervical lymphadenopathy. Nevertheless, MRI is superior to CT in demonstrating the extent of
soft tissue tumors, the perineural invasion, the bone marrow involvement, and the intracranial
extension. MRI is also recommended for use in staging tumor recurrences. On imaging tech-
niques, the earliest sign of NPC is asymmetry of the fossa of Rosenmuller with blunting of the
fossa. The tumor shows a tendency for infiltration through the pharyngobasilar fascia into the
parapharyngeal space and then disease spread can occur anteriorly, laterally, or superiorly.
The recommended treatment schedule consists of three courses of neoadjuvant
chemotherapy, irradiation, and adjuvant interferon (IFN)-beta therapy.
Axial contrast-enhanced CT scan images (Fig. 4.7a, b) show a mass in the right Imaging Findings
nasopharyngeal region (asterisk) extending into the parapharyngeal space and encasing
the homolateral internal carotid artery. On images obtained at a lower level than Fig. 4.7a,
several nonspecific lymphadenopaties can be seen (arrow in Fig. 4.7b). On coronal MR
images (Fig. 4.7c, d), the lesion margins are clearly depicted. Extension to the base of skull,
meningeal infiltration (white arrow), and cavernous sinus involvement (black arrow) are
better demonstrated on MRI.
A 50-year-old man presented with a 3-month history of a slow-growing 2-cm mass in the
Case 8 left preauricular region.
Pleomorphic
Adenoma Salivary gland neoplasms are relatively rare and represent less than 3% of all tumors in the
general population. Most of them are benign (70–80%) and found in the parotid glands (80–
90%). Approximately 88% of salivary gland neoplasms are of epithelial origin. The most
History
common benign neoplasms of major salivary glands are pleomorphic adenoma, also known
as benign mixed tumor, and Warthin tumor, also known as adenolymphoma or
Comments cystadenolymphoma. Pleomorphic adenomas usually manifest in the fourth and fifth
decades of life but may arise at any age. The average age at presentation is from 43 to 46 years.
They show a slight predominance in females (ratio female to male 2:1).
Clinically, the tumor commonly presents as a solitary, unilateral, slow growing, painless
mass located in the parotid gland.
a c
Fig. 4.8
Diagnosis is made by physical examination and imaging techniques such as US, CT, and
MRI. US is considered to be the first imaging method in assessment of the lymph nodes and
soft-tissue diseases of the head and neck, including the parotid gland. A complete US exami-
nation should determine whether the mass has an intraglandular or extraglandular location,
if it is a solid, cystic, or mixed mass, poor or highly vascularized, with well- or ill-defined
margin, associated or not with lymphadenopathy, and whether it is solitary or multicentric.
If deep tissue extension or malignancy is suspected or confirmed on cytology, an MRI or CT
is mandatory to evaluate tumor extent, local invasion, and perineural spread. On US, the typi-
cal appearance of the lesion is a hypoechoic, well-defined, lobulated nodule with posterior
acoustic enhancement. The feature of lobulated margin is being emphasized in differential
diagnosis and it appears in 55–91% of the cases. The tumor structure is usually mostly homo-
geneous, solid, sometimes with calcifications and the vascularization is poor or absent. On CT,
pleomorphic adenomas are typically smooth and well-defined tumors. The attenuation val-
ues of the mass are usually homogeneous and higher than that of the surrounding gland,
though lower attenuation masses that resemble cysts are occasionally seen. Tumor enhance-
ment after contrast material administration may be variable and can result in a missed diag-
nosis if delayed images are not acquired. Pleomorphic adenomas are poorly enhancing in the
early phase, and the amount of enhancement increases over time. Delayed images obtained at
5–10 min are often useful. When these tumors increase in size and become large, they may
develop a heterogeneous appearance with areas of necrosis, hemorrhage, cysts, and calcifica-
tion. Large tumors commonly have a lobulated margin, which strongly suggests the diagno-
sis. On MRI, the expected characteristic appearance of pleomorphic adenoma is usually of a
well-circumscribed, homogeneous mass with low signal intensity, lower than that of the sur-
rounding tissue, on T1-WI and high signal intensity on T2-WI with the fibrous capsule form-
ing a hypointense rim on T2-weighted and fat-suppressed T1-weighted MR images. After
gadolinium administration, the use of a fat suppressed technique is preferred to increase the
depiction of the lesion, which usually shows homogeneous enhancement. When adenoma
becomes large, the tumor may present areas of fibrosis, necrosis, and hemorrhage, with inho-
mogeneous signal intensity apparent in these cases.
Surgical resection is the treatment of choice. All pleomorphic adenomas should be
surgically removed because of the risk of malignancy of nontreated tumors after decades.
In most cases, the prognosis of patients with benign, well-circumscribed tumors tends to
be excellent following surgery.
Axial contrast-enhanced MDCT scan images (Fig. 4.8a, b) and coronal reconstruction Imaging Findings
image (Fig. 4.8c) show a well-defined, lobulated mass located in the deep lobe of the left
parotid gland consistent with pleomorphic adenoma. Note the retromandibular vein
anterior to the lesion (black arrow in Fig. 4.8b).
A 75-year-old man presented with a right laterocervical painless mass that had been slowly
Case 9 growing over a period of more than 20 years.
Warthin’s
Tumor Warthin’s tumor, also known as papillary cystadenoma or adenolymphoma, is the second most
common neoplasm of the parotid gland, representing approximately 2–10% of all parotid tumors,
History and it is located almost exclusively in this gland. Warthin tumor is more common in men than in
women (3:1), with peak incidence in the fifth and sixth decades of life. The relationship between
smoking and development of this tumor has been proved. The risk of malignant transformation
Comments
is extremely low (<1%).
c d
Fig. 4.9
Warthin tumor usually presents as a solitary, unilateral, slow growing, painless mass.
Sometimes, it may manifest as an inflammatory local process with pain. In about 10–60% of the
cases, tumors may occur bilaterally or multifocally either synchronously or metachronously.
Diagnosis of Warthin tumor may be supported by physical examination and imaging
techniques such as US, CT, MRI, and technetium 99m scintigraphy. At US, Warthin’s tumor
appears as a rounded or lobulated, hypoechoic mass with a heterogeneous echotexture in
about 50% of cases due to the cystic component. The proportion of the cystic component
tends to be higher in larger tumors and the presence of multiple anechoic cysts is a sensitive
marker of Warthin’s tumor. The borders are well-defined in the majority of the cases, and
ill-defined margins should be interpreted as a warning sign for malignancy. The acoustic
enhancement is less frequently encountered when compared with pleomorphic adenoma.
The lesion is usually hypervascularized but may also contain short vessel segments. On CT,
Warthin’s tumor usually manifests as a benign-looking smooth, well-marginated, mostly
solid and cystic mass. It presents homogenous soft-tissue density without calcifications. The
cyst wall is usually thin and fairly smooth. The presence of a mural nodule helps to distin-
guish Warthin’s tumors with large cystic components, septae or multiple adjacent cystic
lesions from first branchial cleft cysts, or lymphoepithelial cysts. After contrast administra-
tion, solid components show poor or weak enhancement and cystic contents of tumors show
slightly increased or decreased attenuation on delayed CT images due to the diffusion of
contrast material from the wall to the cystic spaces. On MRI, solid and cystic components
show low signal intensity on T1-weighted images, although cystic areas may appear hyper-
intense due to proteinaceous debris and/or hemorrhage. On T2-weighted images, solid com-
ponents show intermediate-to-high signal intensity and cystic foci appear hyperintense. On
STIR images the lesions become more conspicuous, especially the cystic components.
Warthin’s tumors show significant restriction of diffusion (high signal in DWI images and
low signal in ADC maps). The mean ADC values are very low (0.81–0.96 × 10–3 mm2/s), a
finding that is helpful to differentiate them (together with their morphologic features) either
from pleomorphic adenomas (2 × 10–3 mm2/s) or from malignant tumors (1.29–1.38 × 10–3
mm2/s). After gadolinium administration, solid components show minimal enhancement.
Regarding treatment, a limited partial parotidectomy is considered as an effective and
curative therapy. This tumor does not tend to recur.
US performed on the patient (Fig. 4.9a) demonstrated a nodule in the right parotid gland, Imaging Findings
which corresponded to the palpable abnormality, and two nodules in the left parotid gland. All
the three nodules had similar echographic features, were well-defined, hypoechoic lesions with
heterogeneous echotexture and hypervascularization. On contrast-enhanced CT scan
(Fig. 4.9b–d), the three parotid nodules are detected as well (arrows in Fig. 4.9d). The nodule in
the right parotid gland was well-circumscribed and involved both superficial and deep lobes.
The nodules of the left side were located in the superficial lobe, and one of them showed a small
cystic component. The findings are consistent with bilateral and multifocal Warthin tumor.
A 28-year-old man presented with a 48-h history of pain and swelling of the left submaxillary
Case 10 region. The patient also reported occasional discomfort in the left part of the floor of the
mouth related to food intake.
Sialolithiasis
History Sialolithiasis is the most common disease of salivary glands and is defined as the presence of
one or more calculi (composed of calcium and/or oxalate salts) in the salivary glands.
Sialolithiasis shows a male gender predilection (ratio 2:1). More than 80% of sialolithiasis
Comments occur in the submandibular gland or its duct, 10% in the parotid gland, and 2–7% in the
sublingual gland or minor salivary glands. The submandibular gland is affected much more
frequently than the parotid gland due to the different secretions that they produce. The
submandibular secretion is thicker, more mucinous, and has a greater salt content, whereas
the parotid gland has a more serous secretion. Besides, the submandibular main duct is narrow
a c
Fig. 4.10
and tortuous, and this can cause stagnation of salivary secretion, increasing the risk of
salivary stone formation. Submandibular calculi are usually unilateral and solitary (75%).
Bilateral sialolithiasis is rare.
Sialolithiasis typically causes pain and swelling of the involved salivary gland by
obstructing the food-related surge of salivary secretion. Calculi may cause stasis of saliva,
leading to bacterial ascent into the parenchyma of the gland, and therefore infection, pain,
and swelling of the gland. Some may be asymptomatic until the stone passes forward.
With regard to the diagnosis, there are several imaging modalities such as conventional
radiography, US, sialography, CT, and MR. On plain X-ray a submandibular stone is seen as
a round or oval radiopaque body projecting over the glandular image. Nevertheless, conven-
tional radiography has limitations. Nonenhanced CT scan is much more sensitive than X-ray
to depict calcium. CT can detect the majority of stones not visible on conventional radio-
graphs. US has proven value with regard to the diagnosis of sialolithiasis. US is easily per-
formed and widely available, and is a cheap and reliable test to assess a patient presenting
with suspected sialolithiasis. Submandibular calculi are seen as hyperechoic oval or rounded
formations casting an acoustic shadow below them. The submandibular duct may be dilated
if the calculus is obstructing it, and the gland may appear swollen. However, US also has
limitations. Sialoliths smaller than 2–3 mm may be overlooked because the acoustic shadow
may be absent. Digital sialography and digital subtraction sialography are the favored tech-
niques for help in the detection of sialolithiasis of the submandibular duct. Even nonra-
diopaque sialoliths may be detectable with this technique. Sialography shows the presence
of a stone as a filling defect within Wharton’s duct or one of its branches. Indirect signs
include dilatation in the ducts, stasis of contrast medium in the ductal system, incomplete
parenchymal opacification, and alterations in glandular structure. Recently, MR sialography
may overcome the limitations of current sialographic techniques (invasive method, bleed-
ing, traumatic perforation, or rupture of the submandibular duct and side effects from con-
trast material). On MR sialography images, saliva displays a very high signal intensity,
whereas the normal submandibular gland had intermediate signal intensity. Therefore, the
intraglandular salivary ductal system and the submandibular duct are clearly depicted as
hyperintense structures. Sialoliths appear as round or oval hypointense structures sur-
rounded by hyperintense saliva. The examination can be combined with conventional MR
studies for the assessment of inflammatory involvement of the gland and for the detection
of possible glandular lesions or other causes of the patient’s symptoms.
The goal of treatment is to remove the stone. Most often, the lithiasis can be flushed out
by increasing the flow of saliva with sour candy or citrus (which stimulate the flow of
saliva) combined with increased fluids and massage.
Axial (Fig. 4.10a, b) and coronal (Fig. 4.10c) contrast-enhanced CT scan images show a Imaging Findings
lithiasis in the distal portion of the left Wharton duct causing retrograde dilatation of the
duct (arrow in Fig. 4.10b). On coronal CT image (Fig. 4.10c), submaxillary glands asymmetry
can be seen. The left submandibular gland is enlarged due to the inflammatory process
(lithiasic submaxillitis).
Further Reading Mattei TA, Nogueira GF, Ramina R (2011) Juvenile nasopharyn-
geal angiofibroma with intracranial extension. Otolaryngol
Albsoul NM, Alsmady MM (2009) Carotid body paraganglioma Head Neck Surg May 13 [Epub ahead of print]
management and outcome. Eur J Sci Res 37(4):567–574; ISSN Murakami R, Baba Y, Nishimura R, Baba T, Matsumoto N,
1450-216X Yamashita Y, Ishikawa T, Takahashi M (1988) MR sialogra-
Ayadi K, Ayadi L, Daoud E et al (2010) Adenoid cystic carcinoma phy using half-Fourier acquisition single-shot turbo spin-
of the parotid with facial nerve invasion. Tunis Med echo (HASTE) sequences. AJNR Am J Neuroradiol 19(5):
88(1):46–48 959–961
Becker M, Marchal F, Becker CD, Dulguerov P, Georgakopoulos Neves F, Huwart L, Jourdan G, Reizine D, Herman P, Guichard JP
G, Lehmann W, Terrier F (2000) Sialolithiasis and salivary (2008) Head and neck paragangliomas: value of contrast-
ductal stenosis: diagnostic accuracy of MR sialography with enhanced 3D MR angiography. Am J Neuroradiol 29:883–889
a three-dimensional extended-phase conjugate-symmetry Poe LB, Petro GR, Matta I (1996) Percutaneous CT-guided aspi-
rapid spin-echo sequence. Radiology 217(2):347–358 ration of deep neck abscesses. AJNR 17:1359–1363
Bialek EJ, Jakubowski W, Zajkowski P et al (2006) US of the major Rabinov JD (2000) Imaging of salivary gland pathology. Radiol
salivary glands: anatomy and spatial relationships, pathologic Clin North Am 38(5):1047–1057
conditions, and pitfalls. Radiographics 26(3):745–763 Rao AB, Koeller KK, Adair CF (1999) From the archives of the
Brennan B (2006) Nasopharyngeal carcinoma. Orphanet J Rare AFIP paragangliomas of the head and neck: radiologic-
Dis 1:23 pathologic correlation. Radiographics 19:1605–1632
Castelijns JA, van den Brekel MW, Niekoop VA, Snow GB (1996) Rosa PA, Hirsch DL, Dierks EJ (2008) Congenital neck masses.
Imaging of the larynx. Neuroimaging Clin N Am 6(2):401–415 Oral Maxillofac Surg Clin North Am 20:339–352
Cummings C (2005) Malignant tumors of the larynx and hypo- Salerno S, Cannizzaro F, Lo Castro A et al (2002) Interventional
pharynx. Otolaryngology – head and neck surgery, 4th edn. treatment of sialoliths in main salivary glands. Radiol Med
Mosby, London http://www.imagingpathways.health.wa.gov. 103(4):378–383
au/includes/pdf/laryngeal_ca.pdf Shah GV (2004) MR imaging of salivary glands. Neuroimaging
Davidovic LB, Djukic VB, Vasic DM, Sindjelic RP, Duvnjak SN Clin N Am 14(4):777–808
(2005) Diagnosis and treatment of carotid body paragan- Simon S et al. Imaging in nasopharyngeal squamous cell carci-
glioma: 21 years of experience at a clinical center of Serbia. noma. E-medicine. Medscape reference
World J Surg Oncol 3:10 Som P (2004) Radiología de Cabeza y Cuello, vol dos, 4th edn.
Dumitriu D, Dudea SM, Jid CB, Baciut G (2010) Ultrasonographic Mosby, London, pp 2090–2096
and sonoelastographic features of pleomorphic adenomas of Som PM, Curtin HD (2003) Larynx. Head and neck imaging, 4th
the salivary glands. Med Ultrason 12(3):175–183 edn. Mosby-Year Book, St Louis, pp 1595–1699
Fodor D, Pop S, Maniu A, Cosgaria M (2010) Gray scale and Som P et al (2004) Radiología de Cabeza y Cuello. Mosby,
Doppler ultrasonography of the benign tumors of parotid London, pp 2039–2040
gland (pleomorphic adenoma and Warthin’s tumor). Pictorial Ted L. Juvenile nasopharyngeal angiofibroma. E-medicine.
essay. Med Ultrason 12(3):238–244 Medscape reference
Goh J, Lim K (2009) Imaging of nasopharyngeal carcinoma. Ann Tincani AJ et al (124) Management of salivary gland adenoid
Acad Med Singapore 38(9):809–816; Review cystic carcinoma: institutional experience of a case series.
Howlett DC, Kesse KW, Hughes DV, Sallomi DF (2002) The role Sao Paulo Med J [online] 1:26–30; ISSN 1516–3180
of imaging in the evaluation of parotid disease. Clin Radiol Weber AL, Siciliano A (2000) CT and MR imaging evaluation of
57(8):692–701 neck infections with clinical correlations. Radiol Clin North
Hudgings PA, Gillison M (2009) Second branchial cleft cyst: Am 38(5):941–968
NOT!! Am J Neuroradiol 30:1628–1629 Woo EK, Connor SE (2007) Computed tomography and mag-
Ikeda M, Motoori K, Hanazawa T, Nagai Y, Yamamoto S, Ueda T, netic resonance imaging appearances of cystic lesions in the
Funatsu H, Ito H (2004) Warthin tumor of the parotid gland: suprahyoid neck: a pictorial review. Dentomaxillofac Radiol
diagnostic value of MR imaging with histopathologic corre- 36(8):451–458
lation. AJNR Am J Neuroradiol 25(7):1256–1262 Yoshino N, Yamada I, Ohbayashi N, Honda E, Ida M, Kurabayashi
Kakimoto N, Gamoh S, Tamaki J et al (2009) CT and MR images T, Maruyama K, Sasaki T (2001) Salivary glands and lesions:
of pleomorphic adenoma in major and minor salivary glands. evaluation of apparent diffusion coefficients with split-echo
Eur J Radiol 69(3):464–472 diffusion-weighted MR imaging-initial results. Radiology
Lawani A (2004) Malignant laryngeal lesions. Current diagnosis 221(3):837–842
and treatment in otolaryngology – head and neck surgery. Yousem DM, Kraut MA, Chalian AA (2000) Major salivary gland
McGraw-Hill and Lange, New York imaging. Radiology 216:19–29
Spine 5
Contents
Case 1 Spondylodiscitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 90
Case 2 Spinal Metastatic Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 92
Case 3 Spinal Hemangioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 94
Case 4 Spinal Neurinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 96
Case 5 Spinal Meningioma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 98
Case 6 Spinal Epidural Hematoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 100
Case 7 Spinal Cord Astrocytoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 102
Case 8 Myxopapillary Ependymoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 104
Case 9 Transverse Myelitis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 106
Case 10 Spinal Cord Vascular Malformation . . . . . . . . . . . . . . . . . . . . . . . . . . 108

History A 24-year-old man, with history of intravenous drug use, complained of pain localized in
Case 1 thoracolumbar junction (T12-L1) for several months. The pain had become more severe
in the last weeks. The patient also had daily vespertine fever in the 2 previous months.
Spondylodiscitis
Comments Spondylodiscitis is an infection that involves the extradural components of the spine.
It accounts for 2–4% of all osteomyelitis. In adults, the marrow in the region of a
vertebral body endplate usually is affected first (spondylitis), and the infection rapidly
spreads to the adjacent disk (diskitis) and to the closest adjacent vertebral body.
Children have disks with significant vascularity, so the initial infection may occur in
the disk and then spread secondarily to the adjacent bone. The incidence of
spondylodiscitis is 2.4/100,000 inhabitants and it increases with age. Adults older than
50 years are most often affected and there is a male predominance in the ratio
male:female of 1.5–3:1. In pyogenic spinal infections, the most frequent bacteria
a b
c d
Fig. 5.1
Spine 91
involved is Staphylococcus aureus (in 60% of patients) followed by Enterobacter species. In

nonpyogenic spinal infections, Mycobacterium tuberculosis is the most common cause.
The three main routes of infection are hematogenous spread from distant septic foci,
direct inoculation from spinal surgery or penetrating trauma, and contiguous spread. The
lumbar spine is most often involved, followed by the thoracic spine and the cervical spine.
Although the clinical manifestations of patients with spondylodiscitis are variable, the
patient usually refers insidious back pain accompanied by other nonspecific symptoms
such as malaise, fever, or weight loss. Laboratory tests may show elevation of erythrocyte
sedimentation rate in 95% of the patients and elevation of C-reactive protein level.
Leukocytosis may be present. Laboratory test can be normal in tuberculosis infection and
in immunocompromised or older patients.
Plain film radiograph of the spine is normal in the early stages of infection, and abnor-
malities are rarely detected before 2–4 weeks. Currently, magnetic resonance imaging
(MRI) is considered the most sensitive diagnostic technique for evaluating the presence
and severity of spinal infection. The advantages of MRI include direct visualization of the
bone marrow and simultaneous evaluation of the neural structures. The appearance of
the infection on MRI depends on the extent of disease and the body response to it at the
time of imaging. MRI findings consists of a triad that includes: (1) decreased signal inten-
sity of the intervertebral disk and adjacent vertebral body marrow on T1 weighted images,
(2) contrast enhancement of the disk and infected bone marrow on gadolinium-enhanced
T1-WI, and (3) high signal intensity of the disk on T2-WI. Associated findings that may be
detected are decreased disk height, destruction of the low signal intensity cortical endplate,
and subligamentous, epidural, or paraspinous inflammatory phlegmon or abscess.
Early stages of spondylodiscitis may be difficult to differentiate from degenerative
abnormalities (especially Modic type 1), fractures in seronegative spondyloarthropathy,
and less frequently encountered diseases such as amyloidosis. That is the reason why
some authors suggest computed tomography (CT)-guided core biopsy of intervertebral
disks and adjacent endplates prior to therapy.
Treatment depends on etiology. In cases of inflammatory autoimmune spondylodiscitis,
antiinflammatory therapy is administered; in cases of infectious spondylodiscitis, the
therapy is mainly based on antibiotics. Surgery is warranted in only a few specific situations
(neurological signs, vertebral collapse, spinal instability, etc). When diagnosis or treatment
is delayed, the infection may progress, causing progressive destruction of the vertebres
involved with pathological fractures and paraspinal and epidural abscess formation.
Sagittal T2-weighted (Fig. 5.1a) and T1-weighted (Fig. 5.1b) MR images reveal a decreased Imaging Findings
T12-L1 disk height accompanied by flattening of the T12 vertebral body and adjacent endplate
destruction. The involvement of a posterior epidural inflammatory component located
posteriorly to both vertebres can be seen (arrows). Axial (Fig. 5.1c) and coronal (Fig. 5.1d)
T1-weighted images after gadolinium administration show a fluid collection within the disk
space with extension into adjacent paravertebral spaces. Abscesses in both psoas muscles can
be seen, more conspicuous in the right side (arrow). A sample of the paravertebral collection
was obtained and Mycobacterium tuberculosis was isolated in culture.
History A 76-year-old man, with known history of stage IV

Case 2 bronchogenic carcinoma with adrenal and hepatic
metastases, presented with progressive lower extremities
Spinal Metastatic Disease paresis and severe back-pain radiating to the right
lower limb.
a b
c d
Fig. 5.2
Spine 93
Metastases are the most common vertebral tumors in adults. Spinal metastases are frequent Comments
in patients with malignant neoplasms and the spine is the third most common site for
malignant cells to metastasize, following the lung and the liver. In adults, approximately
50% of spine metastases arise from breast, lung, or prostate cancer. Spinal metastases
usually affect adults aged 40–65 years and they are slightly more common in male than in
female.
The majority of metastatic lesions of the spine occur in the extradural space. All verte-
bral levels can be involved, although the lower thoracic and lumbar spine are the most
common locations. Typically, metastases are multiple and of variable size with cortical dis-
ruption (osteolytic lesions). Osteoblastic metastases are less frequent and are usually
caused by prostate carcinoma in elderly men and by breast cancer in women. Some metas-
tases have a mixed pattern, with areas of osteolysis and areas of sclerosis.
Clinically, most patients (approximately 90%) present with bone and/or back pain fol-
lowed by radicular pain and progressive neurologic deficits. Spinal metastasis may be the
initial presentation in 10% of patients with systemic cancer.
Several imaging methods may be used to diagnosis spinal metastases although MRI is
the technique of choice. Plain films have poor sensitivity in the early detection of osseous
destruction because radiologic findings become apparent only when bone destruction
reaches 30–50%. CT scan has high sensitivity and specificity for depicting both osteolytic
and osteoblastic bone lesions. CT also allows evaluation of paraspinal soft tissues and
paraspinal lymph nodes. On T1-WI, intravertebral lesions are of low intensity and may be
extremely low for sclerotic metastases. Such images can be useful for demonstrating spinal
cord compression. On T2-weighted imaging, the signal characteristics of intravertebral
lesions are variable, although an increase in signal intensity is most usually found.
Intravenous gadolinium administration can vastly improve metastatic foci visibility,
especially when they are extravertebral. However, tumor enhancement is variable, may be
marked, slight, or absent (in cases of sclerotic metastases). Furthermore, enhancement may
be random, initially peripheral with subsequent central spread, or homogeneous. In cases
of highly fatty bone marrow, fat suppression or STIR may be necessary. STIR sequence is
more sensitive than T1-W1 and T2-WI for detecting metastases but, on the other hand, it is
less sensitive for identifying extravertebral involvement. Diffusion-weighted images
distinguish metastasis from osteoporotic bone. Osteoporotic fractures are hypointense,
and metastases are hyperintense.
Sagittal T1-weighted (Fig. 5.2a) and T2-weighted (Fig. 5.2b) MR images demonstrate Imaging Findings
decreased signal intensity in L2 vertebral body associated with a soft-tissue mass that
extends into the spinal canal. More lesions are identified in other vertebral bodies (white
arrows); they appear hypointense on both T1 and T2 MR pulse sequences. A disk protrusion
at the L4-L5 level, with cranial displacement of disk material (black arrow in Fig. 5.2b), is
incidentally detected. Note the involvement of posterior elements and the presence of a
right paraspinal mass occupying the neural foramen (white arrow in Fig. 5.2c). On axial
images (Fig. 5.2d), the occupation of the right neural foramen (white arrow) and the
epidural metastatic component (black arrow) are clearly identified.
History A 14-year-old male presented with a 2-week history of progressive

Case 3 right lower extremity paresis and cutaneous extensor plantar reflex.
Spinal Hemangioma
Comments Vertebral hemangiomas are the most common benign tumor of
the spinal column, and occur most frequently in the lower thoracic
and upper lumbar spine. Hemangiomas are composed of thin-
walled vessels lined by flat, bland endothelial cells infiltrating the
medullary cavity between bone trabeculae. They typically affect
the vertebral body more than posterior elements. Spinal
hemangiomas are most often solitary although they can be multiple
a b
c d
Fig. 5.3
Spine 95
in up to one-third of the cases. The prevalence of hemangiomas seems to increase with age
and is greatest after middle age, with a peak incidence in the fifth decade. Hemangiomas
present a slight female predilection, in the female:male ratio of 3:2.
Clinically, bone hemangiomas are usually small and asymptomatic lesions discovered
incidentally on imaging examination for another condition. When symptoms are present,
they are largely nonspecific and depend on the location, size, and aggressiveness of the
tumor. Occasionally, vertebral hemangiomas may increase in size and compress the spinal
cord and nerve roots. Hemangiomas are slow growing, and malignant degeneration is vir-
tually unknown. Rarely, locally aggressive growth patterns are recognized; hemangiomas
with these patterns can mimic malignant lesions.
Plain radiography is useful for evaluation as the first-line imaging modality in most
cases. The radiographic hallmark of bone hemangiomas is a prominent trabecular pattern
and the characteristic radiographic appearance is of a sclerotic vertebra with coarse, thick-
ened vertical trabeculae giving a corduroy, accordion, or honeycomb appearance. This
appearance is due to resorption of horizontal trabeculae, caused by vascular channels and
consequent reinforcement of vertical trabeculae. Bulging of the posterior cortex or expan-
sion of the vertebral body is sometimes present. When plain radiographs do not suffice and
appearances remain equivocal, cross-sectional imaging, such as CT or MRI, is crucial for
further characterization of these lesions. On CT, hemangiomas appear as punctate sclerotic
foci representing thickened vertical trabeculae seen in cross-section and giving a “polka-
dot” appearance. Bulging of the posterior cortex and paravertebral soft-tissue extension
are readily assessed on CT scans, as is bone destruction with aggressive hemangiomas. On
MRI, most hemangiomas have a classic appearance due to the histopathologic features of
these tumors—high vascularity, interstitial edema, and interspersed fat. On T1-weighted
images, they are round lesions of high signal intensity caused by the large fat component of
typical hemangiomas. On T2-weighted images, they are also of high signal intensity (higher
than fat) because of the slow-flowing blood in the lesions. Thick, low-signal-intensity verti-
cal trabecular struts may be seen within hemangiomas. Fatty vertebral hemangiomas may
represent inactive forms of this lesion, whereas low signal intensity at MR imaging may
indicate a more active lesion with the potential to compress the spinal cord.
Treatment depends on the size and location of the hemangioma. Transarterial
embolization is an effective treatment for painful intraosseous hemangioma and is useful
in reducing intraoperative blood loss before decompressive surgery. Embolization may be
used in combination of excision and radiation therapy.
Sagittal T2-weighted (Fig. 5.3a) and T1-weighted (Fig. 5.3b) MR images reveal high signal Imaging Findings
intensity in the vertebral body of T9 consistent with spinal hemangioma. On T2-WI,
expansion of the vertebral body seems to be present. Axial T2-weighted image (Fig. 5.3c)
demonstrates the epidural component of the hemangioma that causes spinal cord
compression (arrows). Preoperative digital subtraction angiography (Fig. 5.3d) and
transarterial embolization were performed to reduce intraoperative complications. DSA
showed that hemangioma vascularization was supplied by an intercostal artery. Note the
venous drainage (arrow).
History A 56-year-old female, with a history of liver hydatid

Case 4 cyst surgically removed 10 years ago, presented with a
right paravertebral tumor incidentally discovered on
Spinal Neurinoma abdominal follow-up CT.
a b
c d
Fig. 5.4
Spine 97
Nerve sheath tumors, including schwannoma, neurofibroma, and ganglioneuroma, are Comments
intradural extramedullary masses. These tumors, in conjunction with meningiomas,
account for 80–90% of all intradural extramedullary neoplasms. Schwannomas, also known
as neurinomas or neurilemomas, are usually solitary, benign, lobulated, well-circumscribed,
truly encapsulated, round or oval tumors that may occur nearly anywhere in the body but
have a predilection for the head, the neck, and the major nerve trunks. Indeed, most
neurinomas arise from dorsal sensory roots. Depending on their origin, they can be
intradural, extradural, or combined intra- and extradural “dumbbell” tumors. These
neoplasms are commonly found in middle-aged adults with no gender predominance.
Neurinomas arise eccentrically from their parent nerve with the nerve displaced to the
periphery of the mass. Nerve fibers do not course through the lesion but are confined to the
capsule. Neurinomas often present hemorrhage, cystic degeneration, and xanthomatous
changes but malignant transformation is very rare.
Clinically, the most common presenting manifestations are pain and radiculopathy, fol-
lowed by paresthesias and limb weakness. Some intradural neurinomas can compress the
spinal cord causing myelopathic symptoms.
On CT scan, schwannomas usually present as well-circumscribed and homogeneous
masses, with attenuation slightly less than or equal to that of surrounding muscles with
variable homogeneous or heterogeneous enhancement. Calcifications and hemorrhage are
rare. The tumor often causes osseous changes with pedicle erosion and/or neural foramen
enlargement, which are clearly depicted on CT. On MR imaging, schwannomas appear
isointense to skeletal muscle on T1-weighted images and hyperintense with slightly hetero-
geneous signal intensity on T2-weighted images. Approximately 40% of spinal neurinomas
have a cystic component, which appears hyperintense on T2-WI, secondary to cystic,
necrotic, or hemorrhagic degeneration. After contrast material administration, schwan-
nomas commonly show intense enhancement. Unfortunately, the patterns of signal inten-
sity on MRI are neither specific for neural tumors, nor do they allow differentiation between
benign and malignant nerve sheath tumors. However, some imaging features may help to
differentiate between schwannomas and neurofibromas. A tumor eccentrically positioned
in relation to the parent nerve suggest schwannoma, while centrally located suggests a
neurofibroma. The presence of cystic degeneration areas in the mass due to vascular
thrombosis and subsequent necrosis are usually found in schwannomas and are uncom-
mon in neurofibroma.
The treatment of choice is surgical excision, which is generally curative with recurrences
being rare.
On axial contrast-enhanced abdominal CT scan (Fig. 5.4a), a right paravertebral soft-tissue Imaging Findings
mass causing neural foramen enlargement was incidentally found. Axial T1-weighted image
(Fig. 5.4b) and axial and coronal contrast-enhanced T1-weighted images (Fig. 5.4c, d)
demonstrate a large tumor, with a predominantly extraforaminal component that enhances
strongly after contrast material administration, consistent with nerve sheath tumor.
Case 5
Spinal Meningioma
a b
c d
Fig. 5.5
Spine 99
A 42-year-old female presented with a several-month history of progressive weakness of History

lower extremities. The neurological examination revealed paraparesis and hypoaesthesia
from the T6 dermatome.
Meningiomas account for approximately 25% of all spinal canal lesions. They are the Comments
second most frequent neoplasm in the intradural extramedullary location, after tumors of
the nerve sheath. Ninety percent of spinal meningiomas are intradural, whereas 5% are
extradural. The thoracic spine is the most common location (80%) followed by the cervical
spine (15%), lumbar spine (3%), and the foramen magnum (2%). Meningiomas are usually
benign and slow-growing tumors. They show a female gender predominance (with a
male:female ratio of 1:4) and the peak incidence is in the fifth and sixth decades of life.
Clinically, patients usually present with back or radicular pain, sensitive and motor defi-
cits, and sphincter dysfunction. These symptoms are secondary to spinal cord compression
because of mass effect. Typically, back or radicular pain precedes the weakness and sensory
changes; the sphincter disturbance is normally a late finding. Additionally, signs of myel-
opathy are present in most patients.
Gadolinium-enhanced MRI is the imaging modality of choice. MRI can determine the
intradural extramedullary location of meningiomas. If MRI is not available, CT scan and
CT myelography may demonstrate the lesion. On CT, meningiomas appear as solid, well-
defined masses, isoattenuating to skeletal muscle. Occasionally, the tumor may appear as a
hyperattenuating lesion due to calcification or dense tumor tissue. After intravenous con-
trast administration, meningiomas typically show intense and homogeneous enhance-
ment. On MRI, meningiomas are usually isointense to spinal cord on all pulse sequences.
Sometimes, they appear hypointense on T1-weighted images and hyperintense on
T2-weighted images. Following intravenous injection of gadolinium, homogeneous and
intense enhancement of the tumor is typically found. Most spinal meningiomas demon-
strate a broad-based dural attachment, and a dural “tail” sign may be seen in some cases.
Calcified meningiomas appear hypointense in all pulse sequences with only minimal con-
trast enhancement. The spinal cord is usually displaced and compressed by the tumor.
The treatment of choice is complete surgical removal of the mass. However, spinal
meningiomas may recur, especially when total resection is not achieved.
Sagittal T2-weighted (Fig. 5.5a) and T1-weighted (Fig. 5.5b) MR images demonstrate an Imaging Findings
intradural extramedullary mass in the thoracic spine, posterior to T1 to T3 vertebral bodies,
with a broad-based dural attachment. On sagittal and axial postgadolinium T1-weighted
images (Fig. 5.5c, d), the mass shows moderate enhancement. Spinal cord compression is
better depicted in the axial plane (Fig. 5.5d). The spinal canal is almost completely occupied
by the tumor. Note the posterolateral displacement and compression of the spinal cord
(arrow in Fig. 5.5d).
History A 80-year-old male presented with acute onset of

Case 6 paraplegia and back pain. The patient had no prior
history of anticoagulant therapy or trauma.
Spinal Epidural Hematoma
a b
c d
Fig. 5.6
Spine 101
Spinal epidural hematomas (SEH) are focal collections of blood in the epidural space of the Comments
spine. Their etiology is related to several conditions including acute trauma, surgery
complication, anticoagulation therapy, vascular malformation, neoplasms, infections, or
spontaneous occurrence. Spontaneous cases account for approximate 40% of all cases and
the most common locations are the cervicothoracic region or thoracolumbar region. They
are believed to occur from tearing of fragile epidural veins at the time of an acute disk
disruption. Spontaneous epidural hematomas are largest in the anteroposterior diameter
at the midvertebral body level (adjacent to the basivertebral plexus) and taper as they
extend to the adjacent disk level. SEH shows a slight male predominance, and the peak
incidence is seen in patients older than 50 years.
The usual clinical presentation of spontaneous SEH is acute onset of back pain and
rapid progression with development of spinal cord or cauda equine signs that progress to
radicular pain and paresis that may lead rapidly to total paralysis. The progression of
symptoms may take about 48–72 h and result from the compression of the spinal cord.
Currently, MRI is considered as the first choice diagnostic imaging technique for spon-
taneous SEH. MRI provides information about the nature and extent of the spontaneous
SEH, as well as the degree of cord compression. Prior to advent of MRI imaging, myelogra-
phy and CT were used to evaluate this condition, but nowadays, these methods are not used
routinely. Sagittal MR imaging typically shows biconvex hematomas in the epidural space
with well-defined borders tapering superiorly and inferiorly. The dura mater separates the
hematoma from the spinal cord on T1- and T2-weighted images. The appearance of the
hematoma on MRI depends on its age. In acute stage (within 24 h of onset), the spontane-
ous SEH is usually isointense on T1-weighted image. On T2-weighted image, there may be
homogeneous high signal intensity or heterogeneous areas of high and low signal intensity.
After 24 h there is usually high signal intensity on T1-weighted images whereas T2 presents
with analogous to cerebrospinal fluid signal intensity.
The differential diagnosis of spontaneous spinal epidural hematoma includes epidural
tumor or abscess, spondylitis, disk prolapse, acute tranverse myelopathy, and intraspinal
tumor. Spontaneous SEH are often quite small and may be difficult to distinguish from a
sequestered disk or extrusion, depending on their signal intensity at the time of imaging.
Early surgical intervention is the general treatment for spontaneous spinal epidural
hematomas. The procedure includes decompressive laminectomy and hematoma removal.
Conservative treatment has also been proposed, but it is employed only when neurological
deficits improved in the early phase or with the coexistence of coagulopathy.
Sagittal T1-weighted (Fig. 5.6a) and T2-weighted (Fig. 5.6b) MR images demonstrate a Imaging Findings
large posterior epidural collection (arrow) extending from lower thoracic spine to L3. The
collection appears hyperintense on T1-weighted images (Fig. 5.6a, c) and T2-weighted
images (Fig. 5.6b, d) and is consistent with epidural hematoma. Axial images show anterior
displacement and compression of the spinal cord (arrows) with intramedullary high signal
intensity on T2-WI (Fig. 5.6d) related to compressive myelopathy.
History A 42-year-old female presented with a several-day

Case 7 history of progressive right hemibody numbness
leading to paralysis and pyramidal tract signs.
Spinal Cord Astrocytoma
a b
c d
Fig. 5.7
Spine 103
Spinal cord astrocytomas are rare tumors that arise from cells within the spinal cord. Most Comments
of them are benign, low-grade tumors. This neoplasm accounts for 4–10% of all the central
nervous system tumors and may occur in both adults and children. Peak incidence is
between the third and fifth decades of life but they can be found at any age. There is a
slight male predominance (1:1 to 1.5:1). Grossly, intramedullary astrocytomas diffusely
expand the spinal cord. Intratumoral cyst formation is frequent, and associated syrinxes are
commonly observed. The tumor can arise anywhere in the spinal cord, from the
cervicomedullary junction to the filum terminale, but they are found most commonly in the
cervical cord, presumably because it contains more neural tissue than the thoracic or lumbar
segments. Holocord tumors are sometimes seen in children but are rarely found in adults.
Patients usually present with symptoms at or below the level of the spinal cord tumor.
The most common initial signs and symptoms are generalized back pain, which is very
difficult to distinguish clinically from back pain from musculoskeletal conditions, numb-
ness and paresthesias, extremities weakness, ataxia, dysfunction of the bowel or bladder,
mild spasticity, and gait difficulties. Intramedullary spinal cord astrocytomas may also
cause increased reflexes, clonus, mild spasticity, and a positive Babinski sign.
MRI is the diagnostic imaging technique of choice. Both pre- and postcontrast examina-
tions are required using multiplanar sequences. CT scan is of limited value in the assess-
ment of spinal cord tumors. CT scans may depict bony changes of the spine, which may
occur as late secondary findings in patients with spinal cord tumors, such as pedicular ero-
sion, widening of the spinal canal, and dorsal scalloping of the vertebral bodies. MRI typi-
cally shows enlargement of the spinal cord at the level of tumor. This finding is useful for
differentiating tumors from inflammatory lesions (which cause no or minimal increase in
cord size appearance). Most tumors are isointense or slightly hypointense compared with
the normal cord signal on T1-weighted images and hyperintense on T2-weighted sequences.
Following gadolinium administration, tumors generally exhibit some irregular or hetero-
geneous enhancement. MRI can also demonstrate the extent of tumor, the solid and cystic
components, spinal cord edema, reactive cysts, and syringeal cavities.
Differential diagnosis includes ependymoma, hemangioblastoma, and meningioma.
Surgical resection is the therapy of choice and should be considered where possible,
especially for patients with lower-grade astrocytomas. The value of radiation therapy
remains controversial but may be useful for highly aggressive tumors, and for inoperable,
residual, and recurrent tumors. The overall 5-year survival rate for patients with spinal
cord tumors (astrocytomas) is about 50%.
Sagittal (Fig. 5.7a) and axial (Fig. 5.7b) T2-weighted MR images demonstrate an Imaging Findings
intramedullary mass in the cervicomedullary junction that extends caudally to C4 level.
The tumor presents ill-defined margins. Note that it is difficult to differentiate tumor from
surrounding peritumoral edema (that extends into the cerebellar tonsils). Sagittal (Fig. 5.7c)
and axial (Fig. 5.7d) postgadolinium T1-weighted images reveal focal enhancement of the
lesion (arrows). The findings are consistent with spinal astrocytoma.
History A 17-year-old female presented with a long history of lower back pain
Case 8 that radiated to both the lower limbs. The patient also complained of
weakness of the lower extremities in the last two weeks.
Myxopapillary Ependymoma
Comments Ependymomas are the most common primary spinal cord tumors,
accounting for 40–60% of them, followed by astrocytoma. Myxopapillary
ependymoma is considered a variant of ependymoma, with distinct
biological and morphological features. This tumor arises from
a b
c d
Fig. 5.8
Spine 105
ependymal cells of filum terminale, hence it occurs almost exclusively in the region of the
cauda equina. Myxopapillary ependymoma is a benign and slow-growing neoplasm that
demonstrates a slight male gender predilection. It manifests earlier than other spinal
ependymomas, with a mean patient age at presentation of 36 years. Distant metastases can
occur, especially in extradural tumors, but they are rare.
Clinically, patients with these tumors typically present with lower back pain and radicu-
lopathy. These presenting symptoms are nonspecific and may be misdiagnosed as disco-
genic in origin. Other symptoms such as sensory and motor disturbances and bowel and
bladder dysfunction are much less common.
The diagnosis of myxopapillary ependymoma is often delayed, because the majority of
ependymomas of the cauda equina region are slow-growing lesions. There is an average
elapsed time of 2.3 years between symptom onset and diagnosis. On noncontrast CT scans,
myxopapillary ependymomas usually appear isoattenuating compared with the spinal
cord. Small tumors may cause only nonspecific spinal canal expansion, whereas larger
tumors may produce neural foraminal enlargement, scalloped vertebral bodies, and bone
destruction. Following intravenous administration of iodinated contrast material, the
lesion typically demonstrates marked homogeneous enhancement. Myxopapillary
ependymomas do not show specific imaging features on MRI. The tumors usually appear
hypointense to isointense relative to the normal spinal cord signal intensity on T1-weighted
images and hyperintense on proton density- and T2-weighted images. Occasionally, they
may be hyperintense compared to the spinal cord on un-enhanced T1-weighted images due
to the proteinaceous mucoid matrix. After gadolinium intravenous administration, myxo-
papillary tumors typically show intense enhancement, similar to the other ependymoma
subtypes. Enhancement is usually homogeneous but may be heterogeneous when hemor-
rhage or necrosis is present. MRI may also demonstrate enlargement of the spinal canal and
neural foramina, bone destruction, and invasion of the surrounding soft tissues.
The therapy of choice for lumbosacral ependymomas is complete surgical resection.
This can be curative treatment without the need for adjuvant therapy. Radiation therapy is
probably recommended in cases of incomplete resection or recurrence of intradural tumors
but is controversial in cases of total resection and extradural ependymomas. Chemotherapy
has not proved to be beneficial and is usually reserved for recurrent disease that is refractory
to resection and irradiation.
MRI shows a lobulated intradural mass at the L3 vertebral level with a predominantly Imaging Findings
necrotic-cystic component. The tumor appears hyperintense on T2-weighted image
(Fig. 5.8a) and hypointense on T1-weighted image (Fig. 5.8b) and fills entirely the thecal
sac. Note how the nerve roots drape around the mass. The tumor shows intense peripheral
enhancement following intravenous gadolinium administration (Fig. 5.8c, d). The findings
are consistent with myxopapillary ependymoma.
Case 9
Transverse Myelitis
a b
Fig. 5.9
Spine 107
A 28-year-old woman presented with numbness in the left lower extremity that began several History
days earlier. The patient also reported gait disturbance. Physical examination revealed
increased osteotendinous reflexes and hypoaesthesia from the T4 to T5 dermatomes.
Myelitis is a nonspecific term that refers to inflammatory disorder of the spinal cord that Comments
can be associated with several etiologies as infectious agents, postinfectious or postvaccinal
states, demyelinating diseases and systemic inflammatory diseases. Transverse myelitis
refers to a heterogeneous group of inflammatory diseases that are characterized by an
acutely developing, rapidly progressing lesion that affects both halves of the spinal cord
causing motor, sensory, and autonomic spinal cord dysfunction.
The precise etiology in most cases remains unknown but this syndrome has been asso-
ciated with various causes, as referred above. It most often occurs as an autoimmune phe-
nomenon after an infection or vaccination (accounting for 60% of the cases in children) or
as a result of a direct infection, an underlying systemic autoimmune disease, or an acquired
demyelinating disease such as multiple sclerosis or the spectrum of disorders related to
neuromyelitis optica.
This syndrome occurs in both adults and children with no gender predilection, and
typically begins with a rather rapid development of symptoms over the course of several
hours, days, or weeks. Symptoms may include lower back pain, weakness in the extremities,
sensory disturbance, spasms leading to gradual paralysis, and bowel or bladder
dysfunction.
Neuroimaging should be performed to rule out treatable conditions that can mimic
transverse myelitis such as hematoma, epidural abscess, acute disk herniation, or compres-
sion myelopathies. Imaging features of transverse myelitis are nonspecific and may resem-
ble other noninfectious diseases. In the acute phase, MRI studies may be normal in up to
50% of cases and nonspecific in the remainder. The most common imaging findings are
focal spinal cord enlargement on T1-weighted images and poorly defined high signal
intensity lesions within the spinal cord on T2-WI. Enhancement after gadolinium admin-
istration may occur in some cases.
Corticosteroids are the treatment of choice. Unfortunately, the prognosis for significant
recovery is poor in approximately 80% of the cases, and severe residual neurologic deficits
are common.
Sagittal (Fig. 5.9a) and axial (Fig. 5.9b) T2-weighted MR images of the cervicothoracic Imaging Findings
spine demonstrate focal high signal intensity at the left lateral side of the spinal cord with
mild mass effect, consistent with myelitis (arrow). After intravenous gadolinium
administration (Fig. 5.9c), the lesion shows incomplete and predominantly peripheral
enhancement.
History A 40-year-old woman presented with a 3-day history of paraparesis accompanied by bowel
Case 10 and bladder-sphincter dysfunction.
Spinal Cord
Vascular Spinal vascular malformations consist of an abnormal connection between the normal arterial
Malformation and venous channels. They are relatively rare, accounting for 3–11% of spinal space-occupying
lesions, and represent a heterogenous group of blood vessel disorders that affect the spinal cord
Comments parenchyma either directly or indirectly. Most common vascular spinal lesions are arteriovenous
malformations (AVM) and dural arteriovenous fistulae (AVF). Less common vascular lesions
are cavernous and venous angiomas, capillary telangiectasias, and aneurysms.
Spinal cord vascular malformations can be classified into four types, which are distinct
entities with unique gender and age predominance, location of spinal cord involvement,
angiographic features, pathophysiology, prognosis, and treatment. These four categories are
summarized below:
a b
c d
Fig. 5.10
Spine 109
Type 1: Dural arteriovenous fistula. It is the most common type of malformation. It

consists of a single arterial feeder that creates a fistula to the spinal venous circulation.
Symptoms in this type of malformation are related to venous congestion and hypertension,
resulting in hypoperfusion of the spinal cord. These lesions are most frequently seen in
men between the fifth and eighth decades of life.
Type 2: Intramedullary glomus AVMs. They consist of a tightly compacted group of
abnormal arterial and venous vessels (nidus) inside a short segment of the spinal cord. These
malformations usually present in younger patients with acute neurologic deterioration.
Type 3: Juvenile or combined AVMs. These malformations are extensive arteriovenous
abnormalities of the spinal cord parenchyma fed by multiple vessels, both intramedullary
and extramedullary. These lesions are typically seen in young adults and children.
Type 4: Intradural perimedullary AVFs. These malformations result from a direct com-
munication between a spinal artery and a spinal vein without an interposed vascular net-
work. They are usually found in patients between the third and sixth decades of life.
The clinical presentation of spinal cord vascular malformations is variable in the dif-
ferent types. The majority of patients present with progressive neurological deficits, pare-
sis, sensory changes, loss of bladder and bowel function, and impotence. The symptoms
are caused by hemorrhage, venous hypertension, compression, and ischemia of the spinal
cord.
CT scan, MRI, and myelography are not adequate to delineate the vascular anatomy and
classification of the vascular malformations of the spinal cord. CT scanning may show
dilated vessels in the thecal sac, but findings are usually unremarkable. Myelography is able
to demonstrate dilated vessels in the intradural space but it is an invasive procedure that is
not routinely performed. On MRI, dilated intradural vessels can be seen as flow voids.
Following contrast administration, the abnormal vessels show enhancement. Edema or
hemorrhage in the spinal cord parenchyma can be also detected with MRI. Advanced imag-
ing methods as MRI angiography and digital subtraction angiography (DSA) are crucial to
identify the lesion and also to precisely delineate the angioarchitecture of the malforma-
tion for appropriate therapy. Indeed, DSA is the modality of choice for assessing arterio-
venous malformations. This is a dynamic technique that allows visualization of the lesion
in real time, allowing evaluation of high-flow versus low-flow malformations. In addition,
the site of the fistula can be identified.
Accurate and timely diagnosis is crucial for the treatment outcome. Currently, the
therapy options include open surgical ligation or resection of the malformation,
endovascular embolization, spinal radiation, or a combination of these techniques.
Sagittal (Fig. 5.10a) and axial (Fig. 5.10b) T2-weighted images show multiple tubular flow Imaging Findings
voids related to dilated serpiginous perimedullary vessels (black arrows). Spinal cord
thickening associated with areas of intramedullary high signal intensity consistent with
edema can be seen, as well. After gadolinium administration (Fig. 5.10c), enhancement of the
abnormal perimedullary vessels is detected. 3D DSA image reconstruction (Fig. 5.10d) shows
vascular malformation. Note the presence of a small aneurysm (arrow). A hemangiomatous
vertebra in the lower dorsal spine is incidentally found (open arrow in Fig. 5.10a, c).
Further Reading King AT, Sharr MM, Gullan RW, Bartlett JR (1998) Spinal menin-
giomas: a 20-year review. Br J Neurosurg 12(6):521–526
Beaman FD et al (2004) Schwannoma: radiologic-pathologic Ledermann HP, Schweitzer ME et al (2003) MR imaging findings
correlation. Radiographics 24:1477–1481 in spinal infections: Rules or myths? Radiology 228(2):506–
Fukui MB, Swarnkar AS, Williams RL (1999) Acute spontaneous 514; Epub 2003 Jun 11
spinal epidural hematoma. AJNR Am J Neuroradiol 20(7): Ogden A et al. (2009) Intramedullary spinal cord tumors. eMedi-
1365–1372 cine. Medscape reference
Gerukis T, Voultsinou D, Anastasiadou K, Pilavaki M, Petridis A, Osborn AG (1994a) Diagnostic neuroradiology. Mosby, London,
Palladas P. (2009, Nov 24). Spontaneous spinal epidural pp 895–898
hematoma: MRI findings {online}. http://www.eurorad.org/ Osborn AG (1994b) Diagnostic neuroradiology. Mosby-Year
case.php?id=7005 Book, St. Louis
Gezen F, Kahraman S, Canakci Z, Beduk A (2000) Review of 36 Osborn A (1994c) Diagnostic neuroradiology. Mosby, London
cases of spinal cord meningioma. Spine 25(6):727–731 Osborn AG (1994d) Diagnostic neuroradiology. Mosby, London,
Guillevin R, Vallee JN, Lafitte F et al (2007) Spine metastasis imag- p 828
ing: review of the literature. J Neuroradiol 34(5):311–321 Perrin RG (1992) Metastatic tumors of the axial spine. Curr Opin
Harrop JS, Jennifer Malone RN, Przybylski G. Vascular malfor- Oncol 4(3):525–532
mations of the spinal cord. Available at http://www.emedi- Rao AVC, Maharaj P (2007) High cervical spinal cord vascular
cine.com malformation masquerading as Guillian-Barre syndrome: a
Holtas S, Heiling M, Lonntoft M (1996) Spontaneous spinal epi- case report. Internet J Radiol 5(2)
dural hematoma; findings at MR imaging and clinical corre- Rodallec M, Feydy A, Larousserie F et al (2008) Diagnostic imag-
lation. Radiology 199:409–413 ing of solitary tumors of the spine: what to do and say.
Hong SH, Choi JY, Lee JW, Kim NR (2009) MR imaging assess- Radiographics 28:1019–1041
ment of the spine: Infection or an imitation? Radiographics Sarma SK, Rahman MLA, Ahmed GS (2010, Apr 25) Spinal cord
29(2):599–612 astrocytoma {online}. http://www.eurorad.org/case.php?id=8389
Kaplan P, Helms C (2001) Musculoskeletal MRI. Saunders, Shors SM et al (2006) Myxopapillary ependymoma of the
Pennsylvania, p 316 sacrum. Radiographics 26:S111–S116
Kaplan C et al (2001a) Musculoskeletal MRI. Saunders, Pennsylvania, Stabler A, Reiser MF (2001) Imaging of spinal infection. Radiol
pp 301–302 Clin North Am 39(1):115–135
Kaplan C et al (2001b) Musculoskeletal MRI. Saunders, Pennsylvania, Tobias ME (2011) Spinal imaging in astrocytoma. eMedicine.
pp 313–314 Medscape reference
Kilickesmez O, Barut AY, Mutlu IN, Ubic H (2001, Dec 31). Vilanova JC et al (2004) Hemangioma from head to toe: MR
Cervical spinal meningioma {online}. http://www.eurorad. imaging with pathologic correlation. Radiographics 24(2):
org/case.php?id=1240. doi:10.1594/EURORAD/CASE.1240 367–385
Kim LJ, Spetzler RF (2006) Classification and surgical manage- Wippold FJ et al (1995) MR imaging of myxopapillary
ment of spinal arteriovenous lesions: arteriovenous fistulae ependymoma: findings and value to determine extent of tumor
and arteriovenous malformations. Neurosurgery 59(5 suppl and its relation to intraspinal structures. AJR 165:1263–1267
3):195–201
Neuropediatric 6
Luiz Celso Hygino da Cruz Jr., Raquel Ribeiro Batista, Taísa Davaus Gasparetto,
and Cláudio de Carvalho Rangel
Contents
Case 1 X-Linked Adrenoleukodystrophy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 112
Case 2 Alexander Disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 114
Case 3 Septo-Optic Dysplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 116
Case 4 Pilocytic Astrocytoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 118
Case 5 Lissencephaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 120
Case 6 Acute Disseminated Encephalomyelitis (ADEM) . . . . . . . . . . . . . . . . 122
Case 7 Chiari I Malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 124
Case 8 Congenital Cytomegalovirus Infection . . . . . . . . . . . . . . . . . . . . . . . . . 126
Case 9 Dandy-Walker Malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 128
Case 10 Neurofibromatosis Type 1 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 130

112 Luiz Celso Hygino da Cruz Jr., Raquel Ribeiro Batista, Taísa Davaus Gasparetto, and Cláudio de Carvalho Rangel
Case 1
X-Linked Adrenoleukodystrophy
Fig. 6.1
Neuropediatric 113
A 7-year-old boy presents with bilateral moderate hearing loss evolving with progressive History
loss of vision, aggressiveness, gait and coordination disturbances, and history of partial left
hemibody seizures.
X-linked recessive adrenoleukodystrophy (ALD) is the most frequent peroxisomal disorder, Comments
affecting the white matter of the central nervous system (CNS), adrenal cortex, and testes.
This genetic defect causes deficiency of an enzyme, acyl-CoA synthesase, which prevents
the breakdown of very long chain fatty acids that accumulate in tissue and plasma.
ALD has several clinical phenotypes. In childhood, the classical cerebral phenotype
usually onsets between 4 and 8 years, but signs of adrenal insufficiency may appear earlier.
Neurological manifestations are generally slowly progressive. The first manifestations are
behavioral disorder, poor concentration, gait disturbances, progressive loss of vision
leading to cortical blindness and hearing problems, or seizures.
There is no involvement of gray matter structures. In the early stages, symmetric white Imaging Findings
matter demyelination occurs typically in the peritrigonal regions (Fig. 6.1a, b). The
subcortical U-fibers are spared for quite a long time. The progression pattern of white
matter abnormalities is centrifugal, postero-anterior, and typically shows three different
zones (Schaumberg zones). The central or inner zone appears moderately hypointense at
T1-weighted magnetic resonance (MR) images and markedly hyperintense at T2-weighted
MR images, and corresponds to irreversible gliosis and scarring. Dystrophic calcifications
may be detected within this zone on computed tomography (CT). The intermediate zone is
only faintly hyperintense on T2-weighted images. Enhancement after intravenous contrast
injection (Fig. 6.1c, d) and relatively restricted water diffusion on diffusion-weighted
images represents active inflammation and blood–brain barrier breakdown. The peripheral
or outer zone represents the leading edge of active demyelination. Although axons are
usually preserved at this zone.
Case 2
Alexander Disease
Fig. 6.2
Neuropediatric 115
A 4-month-old boy presents with seizures and macrocephaly. Only a slight face asymmetry History
was noted at birth.
Alexander disease, or fibrinoid leukodystrophy, is a rare sporadic leukodystrophy, without Comments

clear inheritance pattern, characterized by massive deposition of Rosenthal fibers
(cytoplasmic inclusions found in astrocytes) in the subependymal, subpial, and perivascular
regions.
Some clinical subgroups are recognized, but the most frequent form is the infantile form
that is characterized by early onset of delayed psychomotor development, loss of acquired
developmental milestones, hypotonia, swallowing difficulties, spastic quadriparesis,
seizures, and rapidly leading to death. Macrocephaly is very characteristic. The average age
at onset is 6 months and the death occurs within 2–3 years.
Supratentorially, extensive white matter disease is seen, showing a centripetal and antero- Imaging Findings
posterior progression, with predilection for the frontal lobe and characteristic areas of
hyperintensity at T2-weighted and fluid-attenuated inversion recovery (FLAIR) MR images
(Fig. 6.2a–c). The subcortical white matter is affected early. The cortex appears to be normal,
but basal ganglia abnormalities are common. The cerebellum also shows abnormalities,
with involvement of the hemispheric white matter, dentate nuclei, and atrophy. Enhancement
is often seen near the tips of the frontal horns early in the disease course. In the late stages,
large cystic lesions are typically seen in the frontal and temporal regions.
MR spectroscopy evaluation of frontal white matter abnormalities showed markedly
decreased NAA and abnormal lactate (Fig. 6.2d).
Case 3
Septo-Optic Dysplasia
Fig. 6.3
Neuropediatric 117
A 6-month-old girl presents with absent of visual fixation. Ophthalmological examination History
revealed bilateral optic disk hypoplasia.
Septo-optic dysplasia (SOD) refers to a heterogeneous group of disorders that variably Comments
include the association of optic nerve hypoplasia (Fig. 6.3a, b) and absence or hypoplasia
of the septum pellucidum, and a constellation of lesions like pituitary-hypothalamic
dysfunction, olfactory aplasia, and brain abnormalities, such as schizencephaly, cortical
dysplasia, and agenesis of the corpus callosum.
The embryological basis of this association has been proposed to be an insult to the
brain during the late 7th or 8th week of gestation, when the optic nerve, germinal matrix, and
septum are being formed. The presence of diffuse calcifications is often the result of intra
utero infection, and cytomegalovirus infection should be remembered as a potential dif-
ferential diagnosis.
The clinical presentation is highly variable. Visual disturbances may range from
blindness to normal vision. Hypothalamic-pituitary dysfunction, mainly represented by
growth deficit, is seen in approximately two-thirds of the patients. Various degrees of
psychomotor retardation and thermoregulatory disturbances are also seen. About half of
patients have schizencephaly and usually present with seizures. Spastic motor deficits may
be related to other malformations of cortical development, such as polymicrogyria.
According of the spectrum of abnormalities, SOD can be categorized into: isolated SOD, Imaging Findings
presenting with endocrine dysfunction, complete absence of the septum pellucidum
(Fig. 6.3a, c) and diffuse white matter hypoplasia that results in ventriculomegaly
(Fig. 6.3c, d); and SOD-plus, in which schizencephaly or other cortical malformations are
associated, normal size ventricles, a remnant of the septum pellucidum and normal
appearing optic radiations; these patients usually present with seizures and/or spastic
motor deficit associated with neurodevelopmental delay.
Case 4
Pilocytic Astrocytoma
Fig. 6.4
Neuropediatric 119
8-year-old girl presents with sudden headache, vomiting, and gait disturbance. History
Pilocytic astrocytoma is the most common pediatric CNS glial neoplasm and the most Comments
common pediatric cerebellar tumor. The incidence of cerebellar astrocytomas peaks
between 5 and 15 years with a slight predominance in females. Most of the lesions occur in
or near the midline, usually arising from the cerebellum, the optic nerve and chiasm, or the
region of the hypothalamus-thalamus.
Clinical presentation of patients with a pilocytic astrocytoma varies with its site of
origin. Headache, vomiting, gait disturbance, blurred vision, diplopia, and neck pain are
common symptoms in patients with a cerebellar pilocytic astrocytoma.
Most cerebellar pilocytic astrocytomas have a well-demarcated appearance with a round Imaging Findings
or oval shape smaller than 4 cm in size, cyst-like features (Fig. 6.4a, b), smooth margins,
and occasional calcifications.
At MR imaging, the solid portion of the tumor is typically hypointense on T1-weighted
images and hyperintense on T2-weighted images. This MRI feature is consistent with the
low-grade nature of this lesion, showing low cellularity and nuclear-cytoplasmic ratio. The
cyst portion is markedly hypointense on T1-weighted images and on T2-weighted images
is usually hyperintense to cerebrospinal fluid (CSF). As expected for a tumor of low bio-
logic activity, the degree of surrounding vasogenic edema is diminished in comparison
with that seen in high-grade glial neoplasms (Fig. 6.4a). Almost all of pilocytic astrocy-
tomas enhance, typically intensely, on postcontrast images obtained after intravenous
administration of contrast material (Fig. 6.4b).
Diffusion-weighted images typically demonstrate a high apparent diffusion coefficient
values within the cystic portion, whereas the solid component of the lesion shows ADC
values similar to the cerebellum parenchyma. These findings could be used to distinguish
this entity from medulloblastoma, another common tumoral lesion in children, which has
high cellularity and correlates with low values of ADC (Fig. 6.4c, d).
Case 5
Lissencephaly
Fig. 6.5
Neuropediatric 121
A 4-month-old girl presents with seizures. Routine ultrasound examination at 20 weeks History
gestation showed hydrocephalus and microcephaly.
Lissencephaly, or smooth brain, is a severe malformation of the cerebral cortex that results Comments
from impaired neuronal migration between about 8 and 14 weeks of gestation. The affected
brain shows either an absence (agyria) or a paucity of gyri (pachygyria).
The most common clinical manifestations include severe psychomotor retardation,
developmental delay, generalized hypotonia with superimposed spasticity, feeding prob-
lems that often require gastrostomy, as well as seizures, and failure to thrive. The prognosis
depends on the degree of failure of cortical development. In severe cases, death occurs in
infancy or early childhood.
The lissencephalies are currently classified as classic (formerly know as type 1)
lissencephaly (formerly know as type 2), cobblestone lissencephaly, and lissencephaly not
otherwise classified.
Patients with lissencephaly are usually microcephalic (Fig. 6.5a), although they may be Imaging Findings
normocephalic. Neuroimaging studies in classic type demonstrate a smooth brain with
vertically oriented Sylvian fissures (Fig. 6.5b–d). In severe cases, the brain is completely
smooth with the Sylvian fissures being the only definable fissures. In milder cases, a few
shallow sulci are seen to surround broad flat gyri.
MR imaging can provide a complete and global depiction of cerebral sulci and gyri. T1-
and T2-weighted images are obtained to define brain anatomy. Additional diffusion-
weighted and T2*-weighted sequences may be obtained for detection of blood products or
ischemia. The shallow Sylvian fissures may result in a so-called “figure of 8” deformity
(Fig. 6.5d). Hetenotopias are commonly recognized within the white matter, especially near
the corners of the lateral ventricles. Bands of heterotopic gray matter can be present bilat-
erally (Fig. 6.5b, c) arrows.
Case 6
Acute Disseminated Encephalomyelitis (ADEM)
Fig. 6.6
Neuropediatric 123
A 5-year-old male patient presented with headache, nausea, vomiting, and seizures 5 days History
after vaccination.
Acute disseminated encephalomyelitis (ADEM) represents an anomalous immune Comments

response to viral infection or vaccination. Clinical manifestations of this condition usually
occur 4–7 days after the onset of a viral infection or vaccination characterized by seizures,
headache, fever, irritability, vomiting, and nuchal rigidity. The symptoms may resolve
spontaneously over a period of weeks. However, 10–30% of patients may have some
permanent neurological injures. The etiology of ADEM is thought to result from an
antigen–antibody reaction against a CNS antigen, leading to a microvascular inflammatory
pathological mechanism and consequently to areas of demyelination. This condition is
typically monophasic and self-limited, however, some patients may evolve to a multiphasic
disseminated encephalomyelitis that is thought to represent a variant of multiple sclerosis
(MS), suggesting that those entities represent two extremes of the same pathophysiological
process. If relapses occur with dissemination in space and time, a diagnosis of MS should
be considered. Generally, lesions resolve completely after steroid therapy. However, MRI
findings may persist on MR imaging for a long time, with partial resolution of the lesions
and a permanent damage of the brain parenchyma.
The imaging features of ADEM are nonspecific and the MR imaging just suggests the Imaging Findings
presence of demyelinating disease. Although the differential diagnosis between ADEM and
NS is difficult based on imaging findings, some MR imaging characteristics may presume
this diagnosis. MR imaging shows moderate to large areas of demyelination involving both
white and gray matter of one or both hemispheres, usually asymmetrically. Deep cerebral
nuclei, corpus calosum, brain stem, cerebral white matter, and spinal cord may be involved,
whereas the white matter is more frequently and severely injured. On MR imaging, the
T2-weighted images demonstrate the lesions more easily than T1-weighted images. These
areas usually have high T2 signal whereas on T1-weighted images the larger lesions may
present low signal while most lesions will not be easily seen due to a signal similar to the
brain parenchyma (Fig. 6.6a, b). The lesions are usually large, with poorly defined margins
and minimum or absent mass effect. Contrast enhancement is evident in larger lesions
with a peripheral predominant enhancement characteristic (Fig. 6.6c, d). Advanced MR
imaging techniques such as diffusion-weighted imaging (DWI) and MR spectroscopy
(MRS) may help in the differentiation from other disorders affecting the white and gray
matter. At DWI the lesions may demonstrates restricted diffusion in the periphery of the
lesion, indicating the elevated inflammatory response. Peak MR spectroscopy may show
low NAA levels, mild increased choline, and a lactate.
Case 7
Chiari I Malformation
Fig. 6.7
Neuropediatric 125
A 8-year old male patient presented with headache. History
The Chiari malformations are congenital abnormalities of the hindbrain, usually associated Comments
with hydrocephalus. The Chiari I malformation is defined as caudal cerebellar tonsillar
ectopia, which is dislocated below the foramen magnum into the cervical spinal canal. A
caudal displacement and flattening or kinking of the medulla can also be identified. A
reduction of CSF flow at the foramen magnum may occur as a consequence of the restricted
space due to the tonsillar dislocation. The etiology of Chiari I malformation is thought to
depend on several different processes. There is a strong association between the posterior
fossa size and the degree of cerebellar ectopia, with the tonsillar herniation occurring as a
result of mechanical factors. Well-known associated abnormalities include craniovertebral
malformations such as occipitalization of atlas, Klippel-Feil anomaly, and platybasia.
Syringomyelia may occur in 20–65% of patients probably due to a pathologic CSF dynamics.
Clinical symptoms associated with Chiari I malformation include the following: suboccipital
headaches, ocular symptoms, otoneurologic disturbances, hindbrain compression
symptoms, and spinal cord dysfunction.
MRI is the imaging modality of choice to identify and grade the cerebellar ectopia (3-5 mm Imaging Findings
below foramen magnum) best visualized at sagittal planes on both T1- (Fig. 6.7a) and
T2-weighted images. Usually, the degree of tonsillar ectopia and the presence and severity
of neurologic symptoms and signs are directly correlated. The extent of this ectopia is
measured on a midsagittal MR image from the tips of the cerebellar tonsils to a line drawn
from the basion to the opisthion. A tonsillar dislocation of less than 3 mm often has no
clinical significance; between 3 and 5 mm the clinical significance is variable; and when the
tonsils extend more than 5 mm below the foramen magnum clinical symptoms are more
likely to develop. In all patients with Chiari I malformation, the spine should be imaged to
look for concurrent syringohydromyelia, recognized as a cystic spinal cord cavity without
enhancement after endovenous gadolinium administration.
Case 8
Congenital Cytomegalovirus Infection
Fig. 6.8
Neuropediatric 127
A 2-month-old baby presented with seizures and microcephaly. History
Cytomegalovirus is the most common congenital infection that is transmitted to the fetus Comments
by the hematogeneous spread from maternal infection. About 10% of the infected fetus will
develop signs and symptoms related to the infection including hepatosplenomegaly,
microcephaly, and impaired hearing. Clinical manifestations comprise seizures, hearing
loss, psychomotor retardation, and hepatosplenomegaly. Optic atrophy, chorioretinitis, and
visual defect may also occur. The CNS manifestations are strongly associated with the
gestational phase when the infection happened. First trimester infections usually cause
germinal matrix necrosis, with secondary migrational abnormalities and malformations.
Second and third trimester infections are most commonly associated with periventricular
white matter lesions and delayed myelination. However, in some cases, the infectious time
may be longer leading to both migrational malformations and parenchyma lesions.
Brain lesions due to congenital cytomegalovirus infection are variable, depending on the Imaging Findings
degree of brain destructions and the timing of injury. MR imaging plays an important tool
in the characterization of possible abnormalities (Fig. 6.8a–d). Patients infected in the first
trimester will usually have lissencephaly, hypoplastic cerebellum, delayed myelination,
ventriculomegaly, and periventricular calcifications. Patients injured later will usually have
polymicrogyria, less ventricular dilatation, and less cerebellar hypoplasia. Finally, patients
infected near the end of gestation will probably have normal gyral patterns, mild ventricular
and sulcal prominence. Also, periventricular or subcortical white matter lesions with
periventricular calcifications and hemorrhage are frequent lesions that are best visualized
on T2* gradient or susceptibility weighted images (Fig. 6.8b). The intracranial calcifications
may be seen as T1 bright, T2 and T2* dark foci usually located in the periventricular regions
and less frequently involving the cerebral cortex, white matter, basal ganglia, cerebellum,
brain stem and spinal cord. Microcephaly is often identified due to cerebral atrophy.
Case 9
Dandy-Walker Malformation
Fig. 6.9
Neuropediatric 129
A 4-year-old girl presented with macrocephaly and headache. History
The classic Dandy-Walker malformation is characterized by a cystic dilatation of the fourth Comments
ventricule, hypoplastic or absence of the cerebellar vermis, and a large posterior fossa with
superior displacement of the dural sinuses and tentorium. Approximately 70–90 % of
patients will present with hydrocephalus. Associated malformations comprise corpus
calosum agenesis (25%), corpus calosum lipoma, malformation of cerebral gyri,
holoprosencephaly (25%), cerebellar heterotopia (25%), dysplasia of the cingulated gyrus
(25%), and gray matter heterotopia (5- 10%). The etiology of this condition is varied and a
familial occurrence has been reported. Some predisposing factors that may be involved in
the genesis of Dandy Walker malformation include gestational exposure to rubella,
cytomegalovirus, toxoplasmosis, warfarin, alcohol, and isotretinoin. Patients with Dandy-
Walker malformation usually present with developmental delay, enlarged head
circumference, as well as signs and symptoms of hydrocephalus. Specific symptoms and
prognosis usually depends on the severity of the hydrocephalus and associated
malformations.
Dandy-Walker malformation consists of an abnormality that involves the roof of the fourth Imaging Findings
ventricle and cerebellum. The cystic dilatation of the four ventricule fills the posterior fossa
and usually extends into the cisterna magna. The cystic lesion may also connect anteriorly
with the residual vermis, laterally with the cerebellar hemispheres, and inferiorly with the
medulla. The cerebellar hemispheres are hypoplastic, with rudimentary vermis foliation,
or absent and may be displaced anterolaterally. The brainstem is generally thin mainly due
to hypoplasia of the pons. The most common associated abnormalities described are:
hydrocephalus, callosal anomalies, cortical dysplasia, and heterotopia. 3D MR sequences
with multiplanar reconstructions may help to identify associated lesions (Fig. 6.9).
Case 10
Neurofibromatosis Type 1
Fig. 6.10
Neuropediatric 131
A 6-year-old boy presented with seizure, progressive decrease of visual acuity and café au History
lait macules.
Neurofibromatosis type 1 (NF1), also called von Recklinghausen disease, is one of the most Comments
common autosomal dominant neurocutaneous disorders (phakomatosis) of the CNS
caused by a mutation that involves the long arm of chromosome 17. Intracranial
manifestations of NF1 include optic pathway and cerebral gliomas, hydrocephalus,
schwannomas of cranial nerves, hamartomas, craniofacial plexiform neurofibromas, and
dysplastic white matter lesions (focal areas of signal intensity [FASI]). Also, multisystemic
involvement is common and patients usually present with associated lesions affecting the
spine, musculoskeletal system (short stature, scoliosis, pseudoarthrosis), the endocrine
system (precocious puberty, hypothalamic dysfunction), and the circulation (hypertension,
renal artery stenosis). Several pathological states may present in childhood, including
seizures and intellectual compromise, optic and acoustic symptoms, intracranial and spinal
tumors, as well as an increased incidence of malignancies. However, the clinical presentation
is variable and the course of the disease in each patient is unpredictable.
The diagnostic criteria of NF1 are met if two or more of the following signs are found:
Six or more café au lait macules larger than 5 mm in the greatest diameter in prepuber-
tal children and larger than 1.5 cm in postpubertal individuals;
Two or more neurofibromas of any type or 1 plexiform neurofibroma;
Multiple freckles in the auxiliary or inguinal region;
A distinctive osseous lesion, such as sphenoid dysplasia or thinning of long bone
cortex;
Optic glioma;
Two or more iris hamartomas (Lisch nodules);
A first-degree relative with NF1.
MRI is the ideal imaging modality for the evaluation of the CNS in NF1. The most common Imaging Findings
intracranial lesions found in patients with neurophibromatosis type I are the FASI, seen on
T2-weighted and FLAIR images (Fig. 6.10a, b) as hyperintense and poorly defined areas
without an evident enhancement and mass effect (Fig. 6.10c), involving the dentate nuclei
of cerebellum, globus pallidus, thalamus, brain stem, pons, midbrain, and hippocampus.
These lesions may increase in number and size until the end of childhood and then tend to
decrease or even, disappearing spontaneously in some cases. However, proliferative changes
and development of tumors from previously FASI have been described. Advanced MR
techniques, such as MR spectroscopy, may help in the differentiation of FASI, which present
normal NAA and choline, from gliomas that usually have lower NAA with elevated choline.
Optic nerve gliomas are usually isohyperintense to normal parenchyma on T2-weighted
imaging (Fig. 6.10d) arrow with a variable enhancement after endovenous gadolinium
administration. Plexiform neurofibromas lesions tend to grow from the nerve of origin
into the intracranial region with a local aggressive characteristic that may compress the
adjacent structures.
Further Reading Malm G, Engman ML (2007) Congenital cytomegalovirus infec-

tions. Semin Fetal Neonatal Med 12(3):154–159
Abdel Razek AA, Kandell AY, Elsorogy LG, Elmongy A, Basett AA Mejaski-Bosnjak V (2008) Congenital CMV infection: a com-
(2009) Disorders of cortical formation: MR imaging features. mon cause of childhood disability. Dev Med Child Neurol
AJNR Am J Neuroradiol 30(1):4–11 50(6):403
Aitken LA, Lindan CE, Sidney S, Gupta N, Barkovich AJ, Sorel M, Menor F, Marti-Bonmati L, Arana E, Poyatos C, Cortina H (1998)
Wu YW (2009) Chiari type I malformation in a pediatric Neurofibromatosis type 1 in children: MR imaging and fol-
population. Pediatr Neurol 40(6):449–454 low-up studies of central nervous system findings. Eur J
Barkovich AJ, Fram EK, Norman D (1989) Septo-optic dysplasia: Radiol 26(2):121–131
MR imaging. Radiology 171(1):189–192 Nelson MD Jr, Maher K, Gilles FH (2004) A different approach to
Cheon JE, Kim IO, Hwang YS, Kim KJ, Wang KC, Cho BK et al cysts of the posterior fossa. Pediatr Radiol 34(9):720–732
(2002) Leukodystrophy in children: a pictorial review of MR Noorbakhsh F, Johnson RT, Emery D, Power C (2008) Acute dis-
imaging features. Radiographics 22(3):461–476 seminated encephalomyelitis: clinical and pathogenesis fea-
Doneda C, Parazzini C, Righini A, Rustico M, Tassis B, Fabbri E, tures. Neurol Clin 26(3):759–780
Arrigoni F, Consonni D, Triulzi F (2010) Early cerebral lesions North KN (1998) Neurofibromatosis 1 in childhood. Semin
in cytomegalovirus infection: prenatal MR imaging. Radiology Pediatr Neurol 5(4):231–242
255(2):613–621 Poussaint TY, Rodriguez D (2006) Advanced neuroimaging of
Friedman JM (2002) Neurofibromatosis 1: clinical manifesta- pediatric brain tumors: MR diffusion, MR perfusion, and MR
tions and diagnostic criteria. J Child Neurol 17(8):548–554; spectroscopy. Neuroimaging Clin N Am 16(1):169–192, ix
discussion 571–572, 646–651 Rossi A (2008) Imaging of acute disseminated encephalomyeli-
Gasparetto EL, Warszawiak D, de Carvalho Neto A, Benites Filho tis. Neuroimaging Clin N Am 18(1):149–161
PR, Bruck I, Antoniuk S (2003) Septo-optic dysplasia plus: Rumboldt Z, Camacho DL, Lake D, Welsh CT, Castillo M (2006)
case report. Arq Neuropsiquiatr 61(3A):671–676 Apparent diffusion coefficients for differentiation of
Ghai S, Fong KW, Toi A, Chitayat D, Pantazi S, Blaser S (2006) cerebellar tumors in children. AJNR Am J Neuroradiol 27(6):
Prenatal US and MR imaging findings of lissencephaly: 1362–1369
review of fetal cerebral sulcal development. Radiographics Tonsgard JH (2006) Clinical manifestations and management of
26(2):389–405 neurofibromatosis type 1. Semin Pediatr Neurol 13(1):2–7
Khong PL, Goh WH, Wong VC, Fung CW, Ooi GC (2003) MR van der Knaap MS, Naidu S, Breiter SN, Blaser S, Stroink H,
imaging of spinal tumors in children with neurofibromatosis Springer S et al (2001) Alexander disease: diagnosis with MR
1. AJR Am J Roentgenol 180(2):413–417 imaging. AJNR Am J Neuroradiol 22(3):541–552
Kim JH, Kim HJ (2005) Childhood X-linked adrenoleukodystro- Vazquez E, Macaya A, Mayolas N, Arevalo S, Poca MA, Enriquez
phy: clinical-pathologic overview and MR imaging manifes- G (2008) Neonatal Alexander disease: MR imaging prenatal
tations at initial evaluation and follow-up. Radiographics diagnosis. AJNR Am J Neuroradiol 29(10):1973–1975
25(3):619–631 Weinberg JS, Freed DL, Sadock J, Handler M, Wisoff JH, Epstein
Koeller KK, Rushing EJ (2004) From the archives of the AFIP: FJ (1998) Headache and Chiari I malformation in the pediat-
pilocytic astrocytoma: radiologic-pathologic correlation. ric population. Pediatr Neurosurg 29(1):14–18
Radiographics 24(6):1693–1708 Zimmerman RA, Bilaniuk LT (2005) Magnetic resonance
Kollias SS, Ball WS Jr, Prenger EC (1993) Cystic malformations evaluation of fetal ventriculomegaly-associated congenital
of the posterior fossa: differential diagnosis clarified through malformations and lesions. Semin Fetal Neonatal Med 10(5):
embryologic analysis. Radiographics 13(6):1211–1231 429–443
Vascular Diseases 7
Contents
Case 1 Carotid Artery Dissection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 134
Case 2 Cerebral Venous Thrombosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 136
Case 3 Cavernous Angioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 138
Case 4 Cerebral Dural Arteriovenous Fistula . . . . . . . . . . . . . . . . . . . . . . . . . 140
Case 5 Subarachnoid Hemorrhage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 142
Case 6 Moyamoya Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 144
Case 7 Posterior Reversible Encephalopathy Syndrome . . . . . . . . . . . . . . . . 146
Case 8 Intraparenchymal Haematoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 148
Case 9 Vein of Galen Malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 150
Case 10 Cranial Nerve Neurovascular Compression . . . . . . . . . . . . . . . . . . . . 152

134 F. de Asís Bravo-Rodríguez and R. Diaz-Aguilera
History A 56-year-old man presented with acute onset of right

Case 1 hemiparesis with fluctuating course accompanied by
pain on the head and neck. The symptoms began after
Carotid Artery Dissection carrying heavy bags.
a b
c d
Fig. 7.1
Vascular Diseases 135
Carotid artery dissection represents an important cause of ischaemic stroke in young and Comments
middle-aged patients accounting for 10–25% of such cases. Although dissections affect all age
groups, there is a distinct peak in the fifth decade of life, with no gender-based differences.
Dissection of the internal carotid artery can occur intracranially or extracranially, with the
latter being more frequent due to its greater mobility and the potential for it to be injured.
Internal carotid artery dissection can be caused by major or minor trauma, or it can be
spontaneous, in which case, genetic, familial, and/or heritable disorders are likely aetiologies.
The typical presenting symptoms are pain on one side of the head, face, or neck accom-
panied by a partial Horner’s syndrome (oculosympathetic palsy) and followed hours or
days later by cerebral or retinal ischemia. This classic triad is found in less than one-third
of patients, but the presence of any two symptoms should strongly suggest the diagnosis.
When this entity is diagnosed early, prognosis is usually good. A high index of suspicion is
required to make this difficult diagnosis.
Computed tomograph angiography (CTA), magnetic resonance angiography (MRA),
and conventional angiography are the main diagnostic techniques. Currently, helical CTA
has an established role in the diagnosis of internal carotid artery dissection, and the
increased use and availability of high-resolution multidetector scanners has replaced
angiography and possibly MRA as the diagnostic modality of choice. The hallmark of
injury to the internal carotid artery using CT angiography is a change in the Caliber of the
vessel. Other findings indicating a dissection may include oval, irregular, or slit-like cross-
section of the vessel lumen. In comparison to conventional angiography, CTA has the added
benefit of imaging extravascular structures. MR angiography may be useful as well for the
diagnosis of internal carotid artery dissection. MRI scans with fat saturation can show
intramural blood, the pathological hallmark of dissection, and mural expansion, thus con-
firming the diagnosis of carotid artery dissection. This is visualised as a semilunar hyper-
intensity (the mural hematoma) partially surrounding a circular hypointense signal (the
residual lumen). Conventional angiography was the criterion standard for the diagnosis of
internal carotid artery dissection. The most common angiographic finding is termed the
“string sign,” which is a long, tapered, narrowing column of contrast material in the distal
segment of the internal carotid artery.
A general consensus does not exist for the management of internal carotid artery
dissection, but endovascular and medical options may depend on the type of injury,
anatomic location, mechanism of injury, co-existing injuries, and co-morbidities. Patients
are usually treated with anticoagulation therapy to prevent thromboembolic events. Anti-
platelet therapy has also been used alone especially when systemic anticoagulation is
contraindicated. Angioplasty and stent placement should be considered when ischaemic
symptoms persist despite adequate anti-coagulation.
Coronal FLAIR MR image (Fig. 7.1a) shows cortical lesions in the left internal carotid artery Imaging Findings
territory. Diffusion-weighted imaging (Fig. 7.1b) reveals diffusion restriction in the lesions
(arrows).Axial fat saturation T1-weighted image (Fig. 7.1c) shows a semilunar hyperintensity
in the left internal carotid artery related to mural haematoma (arrow). MR angiography
(Fig. 7.1d) demonstrates left internal carotid artery occlusion at its origin (arrow).
Case 2
Cerebral Venous Thrombosis
a b
c d
Fig. 7.2
A 24-year-old woman presented with headache and confusion followed by upper left limb History
paresthesia, incoordination, and motor dysphasia. Cerebral venous thrombosis was suspected
after an urgent CT scan. The diagnosis was confirmed by MRI and MR venography.
Cerebral venous thrombosis (CVT) represents 1% of all acute strokes. This entity consists Comments
of venous occlusion that may affect dural sinuses (venous dural thrombosis), cortical veins
(cortical thrombosis), the internal cerebral veins, Galen veins, or sinus rectus (deep cerebral
venous thrombosis). CVT is more common in women than men and has no age predilection.
Over 100 causative conditions have been described in CVT. In general, any condition that
produces hypercoagulability or decreased cerebral blood flow may predispose to CVT, for
instance, dehydratation, coagulopathies, pregnancy, oral contraceptives, infections, or
malignancies. However, no cause is identified in approximately 25% of the cases.
The most common presentation is progressive headache with nausea and vomiting.
Focal neurologic deficits can occur as well.
The venous occlusion produces oedema in the adjacent cerebral parenchyma. Venous
thrombosis may progress to infarction, characteristically cortical and haemorrhagic.
Prognosis is variable depending on extension of thrombosis and the patient’s clinical con-
dition at admission.
Anti-coagulation is the treatment of choice for CVT. Endovascular thrombolytic therapy
is reserved for cases with unfavourable evolution despite anti-coagulation.
Un-enhanced CT scan (Fig. 7.2a) may show a hyperattenuating sinus (open arrow) or a Imaging Findings
hyperdense cortical vein, known as the “cord sign.” Cortical infarctions appear as hypodense
cortical areas or hyperdense zones if haemorrhage is present. After contrast material
administration, the “empty delta sign,” which corresponds to the filling defect of the clot
inside the sinus, can be seen.
T1-weighted (Fig. 7.2b) and proton-density-weighted (Fig. 7.2c) MR images show a lack
of normal flow in the involved vessels and an increased signal within the thrombosed vein
on all pulse sequences (open arrows). Oedema or haemorrhage can be detected as well
(solid arrows). After gadolinium administration, the empty delta sign can be seen.
MR venography may be useful for determining the extension of thrombosis. MR venog-
raphy (Fig. 7.2d) demonstrates enlargement of the affected sinus and filling defects (open
arrows) secondary to the presence of thrombi.
Digital subtraction angiography (DSA) is not necessary in the majority of cases and is
used only when the diagnosis is not clear.
Case 3
Cavernous Angioma
a b
c d
Fig. 7.3
A 35-year-old woman, with a prior medical history of seizures and surgical resection of a History
cavernoma 10 years before, was being treated with carbamazepine and presented with
isolated seizures. Brain and spine MRI were performed for surveillance of multiple
cavernomatosis.
Cavernous malformations (cavernomas, cavernous angiomas, or haemangiomas,…) Comments

consist of well-circumscribed sinusoidal vascular channels containing blood in various
stages of evolution. They represent approximately 1% of intracranial vascular lesions and
15% of cerebrovascular malformations. With the advent of MRI, cavernomas have become
the most commonly identified vascular malformations in the brain. Cavernous angiomas
vary from several millimetres to several centimetres in diameter although they are usually
<3 cm in size. Multiple lesions are seen in approximately 10–30% of spontaneous cases. A
familial form of the disorder exists and is inherited as an autosomal dominant trait with
variable expression. Multiple lesions are more common in the familial form, occurring in
as many as 80% of patients. About 80–90% of lesions are supratentorial and only 5% of
cavernomas are seen in the spinal cord of adult patients. There is no gender predilection.
Cavernous angiomas are considered to be a congenital entity although de novo lesions
may occur in patients with previous radiation exposure or in familial forms cases. In early
studies of major autopsy reports, the calculated prevalence was 0.3–0.9%. Cavernomas can
occur at any age, but they are most likely to become clinically apparent in patients aged
20–40 years.
Common clinical symptoms are seizures secondary to cortical lesions, focal neurologic
deficits secondary to deep cerebral white matter and pons lesions, and headache as a result
of intralesional haemorrhage.
The treatment of choice is surgical removal. Resection is indicated even in cases affecting
the pons, due to the increased risk of subsequent and progressive neurologic disability in
cases of rehaemorrhage.
The sensitivity of CT for these lesions is 70–80%. Cavernous angiomas are typically Imaging Findings
hyperdense and present calcifications in 30% of cases. Lesions show a mottled pattern of
enhancement after contrast administration. When surrounding oedema is present, it is
indicative of a recent bleed.
MRI is the imaging modality of choice. Gradient-echo imaging, with its increased sensi-
tivity to susceptibility artefacts, is useful in the detection of smaller and concomitant
lesions, which may not be detected with SE and FSE sequences (Fig. 7.3a, open arrow).
Cavernous angiomas present as a typical “popcorn-like” lesion with large amounts of hae-
mosiderin surrounding the core of haemorrhage in different stages of evolution. The cen-
tral area appears hyperintense on T1-weighted images (Fig. 7.3b) and the haemosiderin
rim is hypointense on gradient-refocused and FSE T2-weighted images (Fig. 7.3c, d).
In general, cavernomas are considered angiographically occult because of the extremely
low flow of blood through these lesions.
History A 71-year-old man referred to the emergency room with sudden onset of severe headache,
Case 4 blurred vision, vomiting, and decreased level of consciousness.
Cerebral Dural
Arteriovenous Intracranial dural arteriovenous fistulae (DAVF) are uncommon lesions that consist of an
Fistula abnormal connection between dural arteries or pachymeningeal branches of cerebral
arteries and dural veins. DAVFs are thought to be acquired or developmental anomalies
responsible for about 10–15% of all intracranial vascular malformations.
Comments Classification of cerebral DAVFs attempts to explain the significance of the angiographic
anatomy (the pattern of venous drainage) and the clinical presentation and outcome.
DAVFs are classified into five types as follows:
Type I lesions consist of one or more fistulae between a meningeal artery and a dural
venous sinus or dural meningeal vein, with normal direction of blood flow. These lesions
a b
c d
Fig. 7.4
usually present with pulsatile tinnitus, bruit, or a cranial nerve deficit depending on
their location. Their overall clinical course is commonly benign.
Type II lesions consist of one or more fistulae draining into a dural sinus and pial suba-
rachnoid vein with reversed blood flow. These lesions commonly manifest with symp-
toms related to venous hypertension or haemorrhage.
Type III lesions are characterised by direct drainage into subarachnoid veins located at
or in the wall of dural sinuses. Type III lesions behave more aggressively than types I
and II and usually present with haemorrhage or other manifestations of venous
hypertension.
Type IV lesions include type III lesions with venous ectasia.
Type V lesions are type III lesions associated with drainage into spinal veins.
Dural arterio-venous fistula can result in a wide range of signs and symptoms, as referred
above; this variation makes the clinical diagnosis difficult. Symptoms of dural arterio-
venous fistula depend on the location and hence the drainage pattern. Drainage of a petrous
region DAVF to the transverse or sigmoid sinus commonly produces pulsatile tinnitus,
sometimes in association with an audible bruit. Cavernous sinus DAVFs may develop
orbital signs such as congestion, chemosis, and ophthalmoplegia. More aggressive behav-
iour may manifest as focal neurological deficits, a dementia-type of syndrome or cerebral
haemorrhage, including subarachnoid, subdural, or intraparenchymal bleeds.
CT, MRI, and angiography all have roles to play in the investigation of patients with a
possible DAVF. Because the clinical and imaging features can be nonspecific, the diagnosis
of a DAVF is often delayed or missed. Multi-detector CT angiography (MDCTA) can now
provide a high-resolution detail of vascular anatomy. T2-weighted MRI is more sensitive to
the white matter changes of venous congestion or infarction when compared to CT. It has
the drawback of being less sensitive to the changes of acute haemorrhage. If dilated cortical
veins are present they may be seen on conventional spin echo sequences and visualised
using MR angiographic techniques such as phase-contrast venography or contrast-
enhanced MR angiography. Benign disease, without cortical venous reflux, can be missed
using both CT and MRI. Intra-arterial catheter angiography therefore remains the gold
standard and should be performed in most cases when the diagnosis of dural arterio-
venous fistula is being considered. Conventional MRI is less successful than DSA in show-
ing the exact fistula site.
The goal of treatment is obliteration of cortical venous drainage and venous hypertension.
A number of therapy modalities have been postulated: transarterial or transvenous embolisation,
retrograde transvenous embolisation, ligature of the feeding arteries, or a combination.
Urgent un-enhanced CT scan (Fig. 7.4a) demonstrates an intraparenchymal haematoma in Imaging Findings
the right cerebellar hemisphere causing fourth ventricle obliteration. Note the presence of
blood in tentorium and pre-pontine cistern as well. A diagnostic DSA was performed next
(Fig. 7.4b, c) showing a dural Arterio-venous fistula supplied by middle meningeal artery
(black arrow) and occipital artery (yellow arrow) and draining into infratentorial cortical
veins. Note a small aneurysm (double arrow). Transmastoid and tentorial branches were
embolised and the fistula was obliterated (Fig. 7.4d).
Case 5
Subarachnoid Hemorrhage
b c
Fig. 7.5
A 47-year-old man was admitted to the emergency room with sudden occipital headache, History
vomiting, and progressive loss of consciousness. CT scan showed a subarachnoid
haemorrhage. The patient was transferred to intensive care unit and DSA carried out 12 h
after the onset of symptoms revealed an anterior communicating artery aneurysm. In the
same procedure, the aneurysm was completely occluded with GDC coils. A contrast-
enhanced MRA performed 6 months after embolisation for surveillance showed total
occlusion of the aneurysm.
Subarachnoid haemorrhage (SAH), which implies the presence of blood within the Comments
subarachnoid space, could be traumatic or non-traumatic. The common medical use of the
term SAH refers to the non-traumatic type, usually from rupture of an intracranial berry
aneurysm (in 85% of cases), which accounts for 5% of strokes. The incidence is 6–8 events
per 100,000 person years, being higher in women than in men. The mortality rate is as high
as 25–50%.
Clinically, the most common presenting symptom is an unusually severe headache that
starts suddenly. Other possible manifestations are vomiting, focal deficits, and/or loss of
consciousness.
If SAH is suspected, unenhanced CT scan is the first-line imaging modality because the
characteristically hyperdense appearance of extravasated blood in the basal cisterns and
sulci is virtually pathognomonic. In addition, the pattern of haemorrhage often suggests
the location of any underlying aneurysm (sentinel clot). CT scanning presents a high
sensitivity (93–100%) when it is performed within the first 24 h. Nevertheless, the method
of choice to detect an intracranial aneurysm is DSA, which allows a better assessment of
the architecture of the aneurysm in order to decide on the most appropriate therapeutic
procedure, endovascular embolisation (the current treatment of choice) or surgery. On the
other hand, as DSA is an invasive technique, other less invasive methods, such as CT
angiography or MR angiography, should be considered during follow-up.
Un-enhanced CT scan (Fig. 7.5a) shows subarachnoid haemorrhage with blood in Imaging Findings
perimesencephalic cisterns (open arrow), Sylvian fissures (arrows), and sulci. Notice the
sentinel clot in the anterior inter-hemispheric fissure (arrowhead).
DSA (Fig. 7.5b) reveals a 2-mm anterior communicating artery aneurysm (arrow), which
was coiled in the same procedure. DSA after coiling with GDC (Fig. 7.5c) shows complete
occlusion of the aneurysm (arrow).
Gadolinium-enhanced MR angiography, MIP reconstruction, was obtained 6 months
after treatment (Fig. 7.5d). No recurrence of the aneurysm is seen. Notice the magnetic
susceptibility artefact secondary to the coils in the aneurysm. Magnetic susceptibility arte-
facts can be a limitation in the MR angiography follow-up of intracranial aneurysms.
A 37-year-old man presented with a 1-week history of dysarthria and paresis of the left upper
Case 6 extremity.
Moyamoya
Disease The moyamoya syndrome is a progressive occlusive disease of the cerebral vasculature with
particular involvement of the circle of Willis and the arteries that feed it. This disease predisposes
affected patients to stroke due to progressive stenosis of the intracranial internal carotid arteries
and their proximal branches. Reduced blood flow in the major vessels of the anterior circulation
of the brain leads to compensatory development of collateral vasculature. Moyamoya (i.e.,
Japanese for “puff of smoke”) refers to the appearance on angiography of abnormal vascular
Comments collateral networks that develop adjacent to the stenotic vessels. The steno-occlusive areas are
usually bilateral, but unilateral involvement does not exclude the diagnosis. The disease may
occur by itself in a previously healthy individual, which is known as moyamoya disease, or may
occur in association with other conditions including immunological, infections, haematologic
a b
c d
Fig. 7.6
disorders, congenital syndromes, and vascular diseases, which is known as moyamoya

syndrome. The aetiology of moyamoya disease is unknown. Histopathologically, moyamoya
disease is characterised by intimal thickening in the walls of the terminal portions of the
internal carotid vessels bilaterally. The incidence of moyamoya disease is highest in Japan
but it also may affect American and European people. It presents a bimodal peak of
incidence, with symptoms occurring either in the first decade or in the third and fourth
decades of life. There is a slight female gender predilection (female-to-male ratio is 1.8:1).
Children and adults with moyamoya disease may have different clinical manifestations.
The symptoms and clinical course vary widely from asymptomatic to transient events to
severe neurologic deficits. The symptoms can be divided into two groups according to aeti-
ology: (1) those related to ischaemia (i.e., stroke, TIA, seizures) and (2) those related to the
compensatory mechanisms responding to the ischaemia (i.e., haemorrhage from collateral
vessels rupture, headache from dilated transdural collaterals). Adults experience haemor-
rhage more commonly whereas cerebral ischaemic events are more common in children.
Children may have hemiparesis, monoparesis, sensory impairment, involuntary movements,
headaches, dizziness, or seizures. Adults may have symptoms and signs similar to those in
children, but intraventricular, subarachnoid, or intracerebral haemorrhage of sudden onset
is more common. Typically, no remarkable findings appear in the laboratory data.
Diagnostic imaging in patients with suspected moyamoya disease usually requires sev-
eral studies. CT scan may be normal or may show small hyperattenuating areas suggestive
of haemorrhage or a stroke in the cortical watershed zones, deep white matter, basal ganglia,
or periventricular regions. MR imaging shows infarctions and bleeding secondary to cere-
brovascular hypoperfusion. In some cases multiple, small, round or tortuous low-intensity
areas arising in the basal ganglia are seen, which are thought to represent an abnormal net-
work of parenchymal collaterals, the so-called moyamoya vessels. The most suggestive fea-
ture of moyamoya disease on MRI is reduced flow voids in the ICA, MCA, and ACA coupled
with prominent flow voids in the basal ganglia and thalamus from moyamoya vessels.
Cerebral angiography is the criterion standard for diagnosis. The following findings sup-
port the diagnosis: (1) stenosis or occlusion of the distal intracranial ICA or the proximal
portion of the anterior or middle cerebral arteries; (2) abnormal vascular networks at the
base of the brain; and (3) bilaterality of the described findings (although some patients may
present with unilateral involvement and then progress). MR angiography can be performed.
Any of these findings on MRA may preclude the need for conventional angiography.
As the aetiology of the disease remains unknown, there is no treatment to reverse the
pathological process. Current therapies pretend to prevent strokes by improving blood
flow to the affected cerebral hemisphere by surgical revascularisation.
Axial T2-weighted images (Fig. 7.6a) show multiple ischemic lesions located in both Imaging Findings
cerebral hemispheres. On diffusion-weighted imaging (Fig. 7.6b), a recent ischaemic lesion
is detected in the right Sylvian territory. DSA (Fig. 7.6c, d) demonstrates stenosis of the
right M1 segment (white arrow) and the left MCA (not shown) and collateral vessels at the
base of the brain (yellow arrows in Fig. 7.6d). These findings are consistent with moyamoya
disease.
History A 50-year-old man, with a history of liver transplantation 5 days ago and treated
Case 7 with immunosuppressive drug Tacrolimus, developed recurrent seizures and altered
mentation.
Posterior Reversible
Encephalopathy
Syndrome Posterior reversible encephalopathy syndrome (PRES) or acute hypertensive
Comments encephalopathy is a neurologic disorder with characteristic CT and MR imaging
a b
c d
Fig. 7.7
findings. Initially recognised in association with eclampsia, cyclosporine after transplantation,

and in the setting of severe hypertension, PRES has become synonymous with a unique
pattern of brain vasogenic oedema seen in the setting of neurotoxicity. Currently, this
syndrome has been associated with different clinical entities, apart from eclampsia and
cyclosporine use, such as acute glomerulonephritis, systemic lupus erythematosus,
thrombotic thrombocytopenic purpura, and haemolytic-uremic syndrome, as well as drug
toxicity including immunosuppressive and chemotherapeutic agents (i.e., tacrolimus,
cisplatin) and erythropoietin, and infection, sepsis, and shock. There are two pathophysiologic
mechanisms proposed to explain this syndrome. One propounds cerebral vasospasm with
resulting ischaemia within the involved territories, whereas the other postulates a breakdown
of the blood–brain barrier with ensuing interstitial extravasation of fluid. Diffusion MR
imaging can be used to discriminate between these two possibilities, as the cytotoxic oedema
of cerebral ischaemia demonstrates decreased water mobility, whereas vasogenic oedema
due to cerebrovascular autoregulatory dysfunction results in increased water mobility.
Clinically, symptoms at toxicity are broad and include acute or subacute onset of hyper-
tension, headache, visual disturbance, paresis, haemianopsia, nausea, and altered mental
status. Generalised seizures are common and coma may develop.
Since PRES is often unsuspected by clinicians, radiologists may be the first to suggest the
diagnosis. This diagnosis has important therapeutic and prognostic implications, in fact,
when the syndrome is not recognised, it may progress to permanent neurologic deficit or
death, but if it is diagnosed and appropriately managed, PRES is potentially fully reversible.
That is the reason why radiologists should be aware of the spectrum of imaging findings in
PRES. Imaging plays an important role in early diagnosis. MRI is the most sensitive imag-
ing technique for recognising PRES. On CT/MR imaging, the brain typically demonstrates
focal regions of symmetric hemispheric oedema involving the cortical and subcortical
white matter. The parietal and occipital lobes are most commonly affected, followed by the
frontal lobes, the inferior temporal-occipital junction, and the cerebellum. Lesion conflu-
ence may develop as the extent of oedema increases. The oedema usually completely
reverses. Un-enhanced CT shows bilaterally symmetric patchy non-confluent hypodense
foci usually in the posterior parietal and occipital lobes. MR imaging demonstrates hyper-
intensity on T2-weighted and FLAIR images in the same distribution. On diffusion-
weighted imaging the abnormal areas appear hypointense or isointense. DSA may show
diffuse vasoconstriction, focal vasoconstriction, vasodilation, and even a string-of-beads
appearance, consistent with vasospasm or arteritis.
The treatment of PRES depends on the underlying cause. In cases of hypertension, blood
pressure control will accelerate the resolution of the cerebral abnormalities. If the likely
cause is medication, the withdrawal of the drug in question is needed.
Urgent un-enhanced CT scan (Fig. 7.7a) shows bilaterally symmetric hypodense foci in the Imaging Findings
frontal lobes involving the cortical and subcortical white matter. Axial FLAIR MR images
(Fig. 7.7b–d) better depict cortical and subcortical white matter involvement in the frontal
lobes. They also demonstrate bilateral involvement of the parietal and occipital lobes
(arrows).
Case 8
Intraparenchymal Haematoma
a b
c d
Fig. 7.8
A 45-year-old woman presented to the emergency room with sudden onset of left History
hemiparesis and left facial palsy.
Intraparenchymal haemorrhage (IPH) is defined as the spontaneous extravasation of Comments

blood into the brain parenchyma. IPH accounts for 10–15% of all cases of acute stroke and
is associated with high disability, morbidity, and mortality. The most common cause of IPH
in adults is arterial hypertension followed by amyloid angiopathy and coagulopathy; other
less frequent causes are drug abuse and vascular lesions such as arteriovenous malformations
(AVMs), ruptured intracranial aneurysms, and dural venous sinus thrombosis.
Hypertensive IPH is usually located in areas supplied by penetrating branches of the
middle cerebral and basilar arteries, therefore, the most common locations of IPH are the
external capsule and putamen, thalamus, and pons. Clot dissection into the ventricular
system occurs in about 50% of cases and implies a poor prognosis, especially when intra-
ventricular haemorrhage involves the fourth ventricle.
The clinical presentation is characterized by acute neurologic deterioration associated
with signs and symptoms of elevated intracranial pressure.
CT scan is the initial diagnostic technique of choice to evaluate a patient with suspected
IPH. CT scan easily depicts the size and location of the haematoma, extension into the
ventricular system, degree of surrounding oedema, and anatomical disruption. On CT,
parenchymal haematomas are typically hyperdense relative to brain. Recently, multidetec-
tor CT angiography has been proposed as an accurate imaging tool to demonstrate vascu-
lar aetiology of the IPH and should be performed in patients with increased likelihood to
present vascular anomalies as individuals younger than 40–50 years, absence of pre-existing
hypertension or impaired coagulation, presence of associated subarachnoid (SAH) or intra-
ventricular hemorrhage (IVH), and temporal or frontal lobe location.
MRI techniques, such as gradient-echo (GRE, T2*), are highly sensitive for the diagnosis
of IPH. MRI and CT are equivalent for the detection of acute IPH but MRI is significantly
more accurate than CT for the detection of chronic IPH. The appearance of IPH on MRI
depends on the age of the haematoma and on the imaging sequence or parameters.
Hyperacute haemorrhage (<24 h) appears slightly hypointense or isointense relative to
brain on T1-WI and slightly hyperintense to the brain on T2-WI. Acute haematoma
(1–2 days) appears iso- or hypointense on T1-WI. A thin hyperintense rim is sometimes
seen in the periphery. Subacute haemorrhage (after 2–7 days) appears marked hyperintense
on T1-WI and hypointense on T2-WI. Late subacute haematoma (>7 days) manifests as
high signal intensity on both T1-WI and T2-WI.
Urgent un-enhanced CT scan (Fig. 7.8a) showed a right basal ganglia haematoma causing Imaging Findings
mild compression of ipsilateral frontal horn of the lateral ventricle, with no subfalcine
herniation. MRI was performed 5 days later. On sagittal T1-weighted image (Fig. 7.8b), the
haematoma showed peripheral high signal intensity due to the presence of extracellular
metahaemoglobin. On axial T2-weighted images (Fig. 7.8c), the hematoma appeared
isohypointense. Axial FLAIR image (Fig. 7.8d) allowed the differentiation between haematoma
and oedema.
History A 20-day-old neonate, with bacterial meningitis due to

Case 9 Escherichia coli, underwent a transfontanelar ultrasound.
A large dilated vascular structure in the theoretical
Vein of Galen Malformation location of the vein of Galen was found.
a b
c d
Fig. 7.9
The vein of Galen malformations (VGM) are rare congenital malformations that consist of Comments
arterio-venous shunts between one or more arteries, usually subependymal arteries,
anterior and posterior choroidal, and anterior cerebral arteries, and a vein in the region of
the vein of Galen. As VGM are believed to develop early in embryogenesis, the dilated
midline draining vein is not the vein of Galen, but rather the medial prosencephalic vein.
There are two types of VGMs, choroidal and mural. The first one is the most common
type and is characterised by multiple arteriovenous connections in the anterior wall of the
prosencephalic vein supplied by numerous choroidal, pericallosal, and thalamoperforator
vessels. Choroidal type usually manifests during the neonatal period with congestive heart
failure. Mural type consists of fewer but larger calibre connections between the posterior
choroidal or collicular arteries and the medial prosencephalic vein, which is usually aneu-
rysmal because of outlet stenosis. This type usually presents in infancy with loss of devel-
opmental milestones, increasing head circumference and focal neurological deficits.
Diagnosis is mainly based on both pre- and post-natal cerebral ultrasound.Ultrasonography
typically shows a median tubular cystic lesion with internal high-velocity turbulent flow
demonstrated on colour Doppler. Ultrasonography may also show brain infarction or leu-
komalacia and hydrocephalus. Computed tomography reveals tubular vascular structures
within ambiens cistern, and torcular Herophili and transverse sinuses enlargement. After
contrast material administration, intense enhancement of these vascular structures is dem-
onstrated. On MRI, areas of signal void related to serpentine vascular structures, vein of
Galen, and straight sinus are seen. Un-enhanced MR angiography (2D and 3D TOF sequences)
and gadolinium-enhanced MR angiography allow the anatomic characterisation of the vas-
cular malformation with better assessment of arterial supply, dilation degree, and vascular
stenosis. Angiography could be performed on the same session of endovascular intervention
to define precise vascular anatomy.
The goal of treatment is to occlude the arterio-venous shunts and this is done
endovascularly either from an arterial or a venous approach.
Cerebral MRI study performed after birth (not shown) demonstrated aneurysmatic Imaging Findings
dilatation of the vein of Galen, and straight, sigmoid, and transverse cerebral venous
sinuses. As the newborn presented low weight and he was clinically stable with no heart or
neurological symptoms, no therapy but follow-up was decided.
MRI performed 4 months later (Fig. 7.9a) showed enlargement of the vascular malfor-
mation (arrow), cerebral venous sinuses, and ventricular system, as compared to previous
MRI study. No therapy was considered at this moment.
MRI performed 3 months later (Fig. 7.9b) revealed hydrocephalus with transependymal
resorption (arrow). DSA (Fig. 7.9c, d) was performed next for planning endovascular treat-
ment. On DSA, vein of Galen malformation was clearly seen. Note the arterial supply by
both posterior medial choroidal arteries (arrow in Fig. 7.9c) and the left pericallosal artery
(arrow in Fig. 7.9d).
Case 10
Cranial Nerve Neurovascular Compression
a b
c d
Fig. 7.10
A 37-year-old man, with uncontrolled hypertension, presented with a 1-year history of History
right eye blepharospasm worsened by stress and recurrent occipital headaches. On physical
examination, facial paresis and left eye blefarospasm were also found.
Facial hemispasm is a nervous system disorder characterised by irregular and involuntary Comments
contractions of facial muscles innervated by the facial nerve (cranial nerve VII). This
chronic syndrome usually manifests with blepharospasm, secondary to orbicularis oculi
muscle involvement, but other muscles may be involved as well. Hemifacial spasm typically
affects middle-aged patients with peak incidence of 40–60 years. There is a slight female
predominance. Symptoms are almost always unilateral, although bilateral involvement
may occur rarely in severe cases (0.6–3%). Facial nerve compression within the
cerebellopontine angle due to vascular structures has been postulated as a common cause
of facial hemispasm. Anterior inferior cerebellar artery is the most common vessel
involved.
MRI is the imaging diagnostic technique of choice, especially if an underlying compres-
sive lesion is suspected. Currently, high-field magnets can be used to produce images of
higher resolution, which are better than MR angiography and FSE T2-weighted image in
evaluating posterior fossa structures and cranial nerve vascular compressions. Botulinum
toxin injection and surgical decompression are different therapeutic options.
Proximal portion of the trigeminal nerve (fifth cranial nerve) and glossopharyngeal
nerve (ninth cranial nerve) can be compressed by vascular structures, as well. In these
cases, patients present with chronic neuralgia. Pain distribution in fifth cranial nerve
neuralgia depends on the site of the cranial nerve compression; if the nerve is compressed
in its superior aspect, the first branch will be affected, and if is compressed in its inferior
portion, the third branch will be affected. Surgical decompression is considered the
treatment of choice in these cases.
Axial T2-weighted MR image (Fig. 7.10a) shows an elongated vascular structure within Imaging Findings
right cerebellopontine angle (arrow). MR angiography, MIP reconstruction (Fig. 7.10b)
reveals an elongated and tortuous vertebrobasilar junction (arrow) with distal right
vertebral artery and basilar artery ectasia. 3D FIESTA high-resolution images (Fig. 7.10c, d)
demonstrate seventh and eighth right cranial nerves displacement and compression by a
tortuous right vertebral artery (arrow in Fig. 7.10c). Fig. 7.10d. Image obtained at a lower
level than Fig. 7.10c shows displacement and compression of ninth, tenth, and eleventh
cranial nerves by a marked elongated left vertebral artery (arrow in Fig. 7.10d).
Further Reading Ishimori T, Nakano S, Kagawa M, Yokoe K, Togami T, Asakura H,

Kusuhara T, Ohkawa M, Nagao S, Yamashita Y, Sugiura S
Adams W, Whitfield P (2007) Intracranial dural arteriovenous (2003) Virtual endoscopic images by 3D FASE cisternogra-
fistulae. Management topic. ACNR 7(3) phy for neurovascular compression. Magn Reson Med Sci
Alvarez H, Garcia Monaco R, Rodesch G, Sachet M, Krings T, 2(3):145–149
Lasjaunias P (2007) Vein of galen aneurysmal malformations. Kyo N et al (2004) Intracranial dural arteriovenous fistulas: eval-
Neuroimaging Clin N Am 17(2):189–206 uation with combined 3D Time-of-Flight MR angiography
Ashtekar JL et al. Brain, MRI appearance of hemorrhage. and MR Digital Subtraction Angiography. AJR 182:183–190
E-medicine. Medscape reference Lanzino G, Cloft H, Hemstreet MK et al (1997) Reversible poste-
Bartynski WS (2008) Posterior reversible encephalopathy syn- rior leukoencephalopathy following organ transplantation:
drome, part 1: fundamental imaging and clinical features. description of two cases. Clin Neurol Neurosurg 99:222–226
Am J Neuroradiol 29:1036–1042 Loumiotis I, Lanzino G (2011) Vein of galen aneurysmal malfor-
Bonneville F et al (2006) Intracranial aneurysms: an overview. mation. Arch Neurol 68(6):822–823
Neuroimaging Clin N Am 16(3):371–382 McKinney AM, Short J, Truwit CL et al (2007) Posterior revers-
Brisman JL et al (2006) Cerebral aneurysms. N Engl J Med ible encephalopathy syndrome: incidence of atypical regions
355(9):928–939 of involvement and imaging findings. AJR Am J Roentgenol
Cakirer S (2001, Aug 28). Vein of Galen malformation {online}. 189:904–912
http://www.eurorad.org/case.php?id=1032. doi:10.1594/ Michael Scott R, Smith ER (2009) Moyamoya disease and
EURORAD/CASE.1032 Moyamoya syndrome. N Engl J Med 360:1226–1237
Delgado Almandoz JE, Schaefer PW, Forero NP et al (2009) Ornia Rodríguez M, Pérez Carbajal AJ, Gavela Ramón RM,
Diagnostic accuracy and yield of multidetector CT angiogra- Castañón Hevia R (2007) Vein of Galen malformation in an
phy in the evaluation of spontaneous intraparenchymal adult: MRI diagnosis. Radiologia 49(5):347–350
cerebral hemorrhage. AJNR Am J Neuroradiol. Published Rahman EA, Triobe JD, Gebarski SS (2002) Hemifacial spasm
on-line caused by vertebral artery dolichoectasia. Am J Ophthalmol
Fewel ME, Thompson BG Jr, Hoff JT (2003) Spontaneous intrac- 133(6):854–856
erebral hemorrhage: a review. Neurosurg Focus 15(4):E1 Rincon F, Mayer SA (2008) Clinical review: critical care manage-
Fugate JE, Claassen DO, Cloft HJ et al (2010) Posterior reversible ment of spontaneous intracerebral hemorrhage. Crit Care
encephalopathy syndrome: associated clinical and radiologic 12(6):237
findings. Mayo Clin Proc 85(5):427–432 Tabrizi P, Spetzler RF, McDougall C et al (2003) Cerebral dural
Hiwatashi A, Matsushima T, Yoshiura T, Tanaka A, Noguchi T, arteriovenous fistula. Interv Neuroradiol 9(Suppl 2):101–102
Togao O, Yamashita K, Honda H (2008) MRI of glossopha- Tan EK, Chan LL (2004) Clinico-radiologic correlation in
ryngeal neuralgia caused by neurovascular compression. AJR unilateral and bilateral hemifacial spasm. J Neurol Sci 222
Am J Roentgenol 191(2):578–581 (1–2):59–64
Diffusion and Spectroscopy 8
Luiz Celso Hygino da Cruz Jr. and Rafael Ferracini Cabral
Contents
Case 1 Acute Ischemic Stroke . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 158
Case 2 Bipolar Affective Disorder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 160
Case 3 Canavan Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 162
Case 4 Epidermoid Tumor . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 164
Case 5 Epidural Empyema . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 166
Case 6 Hepatic Encephalopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 168
Case 7 HIV Encephalitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 170
Case 8 Leigh Disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 172
Case 9 Lymphoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 174
Case 10 Pyogenic Brain Abscess . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 176

156 Luiz Celso Hygino da Cruz Jr. and Rafael Ferracini Cabral
Diffusion-weighted (DW) magnetic resonance (MR) imaging provides potentially unique

information on the viability of brain tissue. It provides image contrast that is dependent on
the molecular motion of water, which may be substantially altered by disease.
The primary application of DW MR imaging has been in brain imaging, mainly because
of its exquisite sensitivity to ischemic stroke, a common condition that is very common
nowadays.
Some clinical applications:
Ischemic stroke: Within minutes after the onset of ischemia, a profound restriction in
water diffusion occurs in the affected brain tissue. One likely important contributor is
cytotoxic edema. Ischemia causes disruption of energy metabolism, leading to failure of
the Na1/K1 adenosine triphosphatase pump and other ionic pumps. This leads to loss of
ionic gradients and a net translocation of water from the extracellular to the intracel-
lular compartment, where water mobility is relatively more restricted.
Extraaxial masses—arachnoid cyst versus epidermoid tumor: Epidermoid tumors are
solid masses, however, which demonstrate ADCs similar to those of gray matter and
lower than those of cerebrospinal fluid (CSF). With the combination of T2 and diffusion
effects, epidermoid tumors are markedly hyperintense compared with CSF and brain
tissue on diffusion MR images. Conversely, arachnoid cysts are fluid-filled, demonstrate
very high ADCs, and appear similar to CSF on both T2 and DW MR images.
Intraaxial masses: In the preoperative assessment, DWI may play an important role in
the characterization of brain neoplasms. Lesions with restricted components are
mostly liked to represent high grade tumors or lymphoma. Metastasis may also have
low ADC values. Although, these findings should be of great importance in this evalu-
ation, DWI is not able to differentiate low from high grade gliomas, as these tumors are
heterogeneous and areas of high and low ADCs value may coexist. This may in turn
lead to a underestimation of the real degree of the neoplasm.
Proton MR spectroscopy provides information regarding tissue biochemistry and meta-

bolic changes in vivo. The parameters that characterize each peak include its resonance
frequency, height, and width at half-height. The resonance frequency position of each peak
on the plot is dependent on the chemical environment of that nucleus and is usually ex-
pressed as parts per million from the main MR frequency of the system used (i.e., chemical
shift). The height (maximum peak intensity) or the area under the peak may be calculated
and yield relative measurements of the concentration of protons.
Metabolite Symbol ppm Importance

N-acetyl aspartate NAA 2.01 Marker of tumor viability and density
Creatine Cr 3.03 Involved in ATP production, a marker of energy reserves
Choline Cho 3.23 Marker for membrane breakage and cell proliferation
Myoinositol mI 3.56 Marker of gliosis and cell osmolarity
Lipids Lip 0.9–1.3 Marker of demyelination and necrosis
Lactate Lac 1.32 Marker of anaerobic processes
MR spectroscopy has been used with some success in psychiatric illnesses limited to
understanding some metabolic changes and to assessing the effects of lithium in the treat-
ment of bipolar affective disorder.
Case 1
Acute Ischemic Stroke
a b
c d
Fig. 8.1
A 67-year-old man presented with acute left hemiplegia. He referred irregular treatment History
for hypertension and diabetes mellitus for 20 years.
Stroke is the third leading cause of death in the United States after myocardial infarction Comments
and cancer. Approximately 80% of strokes are due to ischemia or cerebral infarction.
Atherosclerotic infarction can result from thrombosis directly at the site of the athero-
sclerotic plaque or from emboli produced at the plaque that lodge downstream, causing an
artery to artery embolism. The clinical manifestations of focal ischemic stroke result from
interference with blood circulation to the brain and the precise signs and symptoms depend
on the region deprived of flow.
Proximal occlusions of the middle cerebral artery may affect both superior and inferior
branches as well as perforating branches to the internal capsule, optic radiations, and basal
ganglia. The result includes contralateral hemiplegia, hemianesthesia, dense homonymous
hemianopsia, and global aphasia with dominant or anosognosia with nondominant hemi-
sphere involvement.
Several risk factors besides age and gender have been described that predispose to
stroke: hypertension, diabetes mellitus, a history of smoking, underlying cardiac disease,
and elevated levels of cholesterol and lipids.
In a few instances, intra-arterial or intravenous thrombolysis has been instituted, but in
most cases this is not feasible because of the narrow therapeutic window. Thrombolytic
treatment has a risk of hemorrhagic complications, which is why it has become important
to establish the potential benefit of thrombolysis for the individual patient. The treatment
of complications of stroke has been given greater import, and management of patients is
often done in dedicated stroke units.
Computed tomography (CT) scan shows hyperdensity of the affected vessel, loss of gray- Imaging Findings
white matter distinction in first 3 h, parenchymal hypodensity, gyral swelling, and sulcal
effacement.
The conventional MR imaging findings in evolving cerebral infarction are well character-
ized and follow a stereotypical temporal evolution similar in many ways to that seen on CT.
There is an early cortical swelling showing hyperintensity in affected distribution on fluid-
attenuation-invertion-recovery-weighted (FLAIR) and T2-weighted images (Fig. 8.1a, arrow).
MR angiography usually shows the occluded right middle cerebral artery (Fig. 8.1b, arrow).
Diffusion-weighted imaging (DWI) has revolutionized the MR evaluation of early or
hyperacute infarction due to its high sensitivity to otherwise occult infarction, and it has
already become a routine imaging sequence in stroke patients. The ischemic event results
in restricted diffusion of the affected tissue, which can be seen as early as 30 min after ictus,
as a hyperintense signal from cytotoxic edema (Fig. 8.1c, d). DWI improves hyperacute
stroke detection to 95% and usually correlates to final infarct size, however rarely some
diffusion abnormalities are reversible. The high signal can persist up to 60 days postictus,
but after 10 days, T2 effect may predominate over low ADC because of predominance of
vasogenic edema (called “T2 shine-through”).
Case 2
Bipolar Affective Disorder
a b
Fig. 8.2
A 34-year-old woman presented with a severe bipolar affective disorder. History
Bipolar affective disorder (BPAD) is a severe, chronic, and incapacitating illness that may Comments
begin during childhood. Its course may be nonepisodic and often manifests as a mixed
state with both manic and depressive symptoms, which may cycle rapidly.
Studies of adults in whom affective illnesses develop after brain injuries have found
associated abnormalities in the frontal or temporal lobes. Mania may be related to right
frontotemporal or left parietooccipital lesions and depression to left frontotemporal and
right parietooccipital lesions.
Neuroimaging techniques have been used to show structural and neurometabolic altera- Imaging Findings
tions in adults with BPAD. These alterations include cortical atrophy, sulcal widening,
cerebellar vermis atrophy, and an enlarged third ventricle. T2-weighted MR imaging has
shown nonspecific cortical atrophy in the periventricular white matter hyperintensities.
In adults with BPAD, there is enlargement of the lateral and third ventricles, decreased
cerebellar size, deep white matter T2 hyperintensities, and temporal lobe asymmetry.
Proton MR spectroscopy provides information regarding tissue biochemistry and meta-
bolic changes in vivo. MR spectroscopy has been used with some success in psychiatric
illnesses limited to understanding some metabolic changes and to assessing the effects of
lithium in the treatment of BPAD. The presence of increased levels of glutamine/glutamate
(Fig. 8.2a, b, arrow), which are excitatory amino acids, suggests a possible dysregulation in
neurotransmitter activity in the frontal and temporal lobes.
Case 3
Canavan Disease
a b
c
Fig. 8.3
A 15-month-old male presented with delayed psychomotor development and increased History
head circumference.
Canavan disease is an autosomal recessive disorder of amino acid metabolism and it occurs Comments
due to deficiency of N-acetyl aspartic (NAA) acylase with excessive accumulation of NAA.
Clinical signs and symptoms become manifest within the first few months of life, marked
by hypotonia, head lag, increased head circumference, seizures, and failure to achieve
motor milestones. Canavan disease is a rapidly progressive illness with a mean survival
time of 3 years, although protracted cases do occur. Diagnosis can be made by quantitative
study of acetylaspartic acid in urine and aspartoacylase level in cultured fibroblasts.
Salient histologic features include vacuolization of both gray and white matter and
proliferation of Alzheimer type II astrocytes. The distribution of spongiotic changes is
most prominent in the deeper cortex and subcortical white matter, with relative sparing of
the deeper white matter and internal capsule. As the disease progresses, a more diffuse
pattern of demyelination will develop. During the first 2 years of life, the ventricles are
usually narrowed and gradually increase in size as a result of loss of tissue.
The signal abnormality in Canavan disease has a centripetal distribution beginning in the Imaging Findings
subcortical white matter of the cerebrum and cerebellum, with early U-fiber involvement.
The subcortical white matter may appear swollen with broadening of the gyri. Typically,
there are diffuse symmetric hypointense signal on T1-weighted images throughout cerebral
white matter (WM) (Fig. 8.3a, b) and increased signal intensity on the T2-weighted images
throughout WM with relative sparing of the internal and external capsules and corpus
callosum (Fig. 8.3c). Lesions do not enhance.
The central white matter becomes involved with disease progression. High signal inten-
sity is always seen within the globus pallidus, with frequent involvement of the thalamus
and relative sparing of the putamen and caudate nucleus. Cerebral and cerebellar atrophy
is a late finding.
The underlying pathology of excessive accumulation of NAA in the brain is readily dem-
onstrated by proton MR spectroscopy with a characteristic increase in the NAA peak
(Fig. 8.3d, arrow).
History A 27-year-old woman presented with progressive dizziness and left sensorineural
Case 4 hearing loss for 9 months.
Epidermoid Tumor
Comments Epidermoid tumor is the third most frequent tumor of the cerebellopontine angle
(CPA). It arises from normal epithelial cells included during neural tube closure. Its
growth is due to accumulation of keratin and cholesterol produced by desquamation
of the squamous epithelium lining the mass. These slow-growing tumors encase
a b
c d
Fig. 8.4
and surround nerves and arteries in the cisterns rather than displacing them. Rarely, the
tumor ruptures and produces a chemical granulomatous meningitis.
The epidermoid tumor accounts for approximately 0.5–1.5% of all brain tumors.
Although congenital in nature, they usually present clinically in the second to fourth
decades. That lesion may be extradural or intradural in location and is predominantly
located in the basal cisterns and are lateral in position. The CPA is the most frequent site of
occurrence, followed by the parasellar region.
The symptoms depend on location and growth pattern of tumor and the principal
symptom is dizziness, however sensorineural hearing loss, trigeminal neuralgia (tic dou-
loureux), facial neuralgia (hemifacial spasm), and headache may be present.
Large epidermoid tumor causing symptoms are treated by surgical removal, which is
generally quite successful in resolving symptoms. Because the tumor lining can be very
adherent to the brain structures, a complete tumor removal may not be possible in many
patients. If only a subtotal removal is accomplished, follow-up MR imaging is necessary for
many years to monitor for recurrence. Chemotherapy and radiotherapy are generally not
used for treating epidermoid tumor.
On computed tomographic (CT) scans, epidermoid tumor appears hypoattenuating, almost Imaging Findings
isoattenuating to CSF, and have characteristic irregular, lobulated margins. As opposed to
arachnoid cyst, which is the main differential diagnosis, epidermoid tumor produces no
reaction of the adjacent bone structures.
At MR imaging, epidermoid tumor usually exhibits poor contrast from surrounding
CSF and resembles other cystic masses. However, epidermoid tumor commonly presents
slightly higher signal intensity than CSF on T1- and T2-weighted images (Fig. 8.4a, arrow),
often with heterogeneous and marbled features. Sometimes, when the signal intensity is
very similar to that of CSF, diffusion-weighted imaging (DWI) has been shown to be a use-
ful method to differentiate epidermoid tumor from arachnoid cysts, by revealing the solid
nature of epidermoid tumor as opposed to the fluid properties of arachnoid cysts (Fig. 8.4d,
arrow) according to their apparent diffusion coefficients (ADC).
With FLAIR imaging, an inversion time is chosen such that the signal from CSF is nulled.
Because epidermoid tumor does not contain free water, they are of increased signal inten-
sity on FLAIR imaging, whereas arachnoid cysts containing CSF are of very low signal
intensity (Fig. 8.4c, arrow).
Because DWI basically is a diffusing proton-attenuated T2-weighted sequence, diffu-
sion-weighted (DW) contrast depends not only on the ADC but also on the T2 values.
Epidermoid tumor shows a mean DW signal intensity higher than that of brain tissue
(Fig. 8.4b, arrow), however, the ADC of epidermoid tumor is higher than that of the brain.
Because of the remarkable T2 prolongation, the DW hyperintensity of epidermoid tumor
should be attributed to the T2 shine-through effect, meaning that the T2 properties domi-
nated the contributions to the DW signal intensity and even overwhelmed the effect of
signal attenuation resulting from the increase in ADC.
Recently, DWI has also been reported to allow confirmation of the presence of residual
postoperative tumor.
History A 52-year-old man presented with a history of surgery

Case 5 for removal of meningioma 8 years ago, actually with
fever.
Epidural Empyema
a b
c d
Fig. 8.5
Purulent collections in the subdural and/or epidural space are uncommon, occurring one-fourth Comments
to one-half as often as intracerebral abscesses. In 65–90% of cases, the empyema is secondary to
otorhinologic infection, which has spread by direct invasion of the extraaxial space and/or by
retrograde thrombophlebitis via bridging emissary veins. The remaining cases of empyema are
related to previous head trauma (penetrating injury, infected subdural hematoma) or to
neurosurgical procedure and, less often, as a complication of bacteremia or meningitis. Cortical
venous thrombosis or brain abscess develops in approximately one fourth of cases and purulent
meningitis is a less common accompaniment.
Symptoms initially reflect those of chronic otitis or sinusitis, as well as lateralized head-
ache (a common feature) and fever. Vomiting, meningeal signs, and focal neurologic abnor-
malities (hemiparesis or seizures) usually follow. If the disease remains untreated, obtundation
progresses, and the septic mass and swelling of the underlying brain soon lead to venous
thrombosis or death from herniation. The major differential diagnosis is meningitis.
The result of lumbar puncture, obtained because of the suspicion of meningitis, reveals
an elevated intracranial pressure accompanied by an increased protein content and a poly-
morphonuclear pleocytosis with usually a normal glucose concentration in the CSF.
Treatment requires both prompt surgical drainage of the empyema cavity and high-dose
intravenous antibiotics directed toward organisms found at the time of craniotomy. The
bacteriologic characteristics of epidural empyemas are similar to those of sinusitis and cerebral
abscess. Anticonvulsants should be administered prophylactically, as seizures are common.
Contrast-enhanced CT shows a biconvex low-density collection between dura and Imaging Findings
calvarium, contained by cranial sutures with strong peripheral rim enhancement.
MR imaging is the study of choice in the evaluation of patients with suspected empy-
ema. The increased sensitivity and specificity of MR are due to direct multiplanar imaging,
increased contrast resolution, and the absence of artifact from bone. It is possible to dif-
ferentiate subdural empyema from epidural empyema when the latter is continuous across
the midline and/or when there is a hypointense margin on both T1- and T2-weighted
images, representing medially displaced dura, which is seen at the interface between an
extraaxial collection and the brain.
On T1-weighted images, epidural empyemas appear hyperintense to CSF but hypointense
relative to brain parenchyma (Fig. 8.5a, arrow) and on FLAIR, hyperintense to CSF (Fig. 8.5b,
arrow). On T2-weighted images, the collections are isointense to hyperintense relative to CSF.
These signal characteristics are typical of proteinaceous fluid with T1 and T2 values interme-
diate between gray/white matter and CSF. There may be abnormal signal in the underlying
brain as a result of ischemia or cerebritis. Prominent enhancement at the margin of an empy-
ema is due to formation of a membrane of granulomatous tissue on the leptomeninges and
inflammation in the subjacent cerebral cortex. On diffusion-weighted images, an empyema
may demonstrate high signal intensity with a corresponding reduction in the apparent diffu-
sion coefficient (Fig. 8.5c, d, arrows), directly related to the cellularity and viscosity of the pus.
Because the source of the infection may be otorhinologic, MR is quite sensitive in detecting
inflammatory changes in the paranasal sinuses and mastoid air cells, usually as areas of increased
signal intensity on T2-weighted images. Enhancement is usually prominent and peripheral.
Case 6
Hepatic Encephalopathy
a b
c d
Fig. 8.6
A 47-year-old man presents with cirrhosis lasting 2 years. At the moment, change in mood History
and behavior is seen.
Hepatic encephalopathy is a neuropsychiatric disorder caused by hepatic insufficiency Comments

characterized by changes in personality, cognition, motor function, or level of consciousness.
It is usually reversible and portends a poor prognosis, with a 1-year survival of 40%. The
precise pathogenesis of hepatic encephalopathy is unknown. Nitrogenous gut-derived
substances accumulate and produce adverse effects on brain function.
Hepatic encephalopathy may cause mild cognitive abnormalities recognizable only by
psychometric testing, or it can present as recurrent or chronic cognitive or motor disor-
ders. Altered behavior or level of consciousness can range from subtle personality changes
to lethargy to coma. Subclinical encephalopathy occurs in 50–80% of patients with cirrho-
sis, with the most common symptoms being insomnia, reversal of the day–night sleep
cycle, and subtle deficits in concentration and hand–eye coordination.
Medical treatment is based on efforts to control the generation of putative neuroactive
toxins. Nonabsorbable disaccharides, such as lactulose, are used as laxatives to decrease
ammoniagenesis and to reduce ammonia absorption from the gastrointestinal tract.
Antibiotics, which may be alternatives or adjuncts to lactulose, work by altering colonic flora.
A variety of different findings are seen on MR, such as bilateral hyperintense dentate nuclei Imaging Findings
on T2-weighted images, symmetric lesions of the white matter, pallidal hypointensity on
MR with calcification on CT and hyperintensity on T1-weighted images in the globus
pallidus, putamen (Fig. 8.6a, b, arrows), subthalamic region, surrounding the red nucleus,
quadrigeminal plate, and anterior pituitary.
Proton MR spectroscopy of patients with chronic hepatic encephalopathy shows ele-
vated glutamine with reductions in choline metabolites and specially myoinositol, which
may be undetectable (Fig. 8.6c, d).
Case 7
HIV Encephalitis
a
b
c d
Fig. 8.7
A 40-year-old man HIV-infected with difficulties with memory and concentration followed History
by apparent apathy and social withdrawal.
The human immunodeficiency virus (HIV) is a human retrovirus that may cause neurologic Comments
disease. The neurologic manifestations of HIV infection include encephalopathy, myelopathy,
and peripheral neuropathy. In the brain, replicating HIV is most frequently associated with
multinucleated giant cells and macrophages. These cells appear to be the chief targets of
infection by HIV and are related to the progressive encephalopathy seen in acquired
immunodeficiency syndrome (AIDS). Polymorphic microglia is frequently infected.
The most common neurologic complication seen in AIDS patients is subacute encepha-
litis. Clinical presentation includes a progressive dementia associated with motor and/or
behavioral dysfunction. Early difficulties with memory and concentration are often fol-
lowed by apparent apathy and social withdrawal and may be mistaken for symptoms of
depression. Headache is also a frequent complaint, and seizures are seen in approximately
10% of cases.
Clinicopathologic correlation in patients with AIDS-dementia complex suggests the
primary pathologic substrate is a subacute encephalitis with multinucleated giant cells
rather than microglial nodules. Electron microscopy reveals retrovirus particles within
these multinucleated giant cells. Diffuse atrophy is usually present. Initially, lesions are
present in the white matter and extend to the basal ganglia and cortex with disease
progression.
CT is often negative or reveals atrophy only. Cortical atrophy is the most frequent MR Imaging Findings
finding and is usually the only early alteration. Both cortical and central atrophy progress
on serial MR examination. T2-weighted and FLAIR images reveal hyperintense lesions
without mass effect in the periventricular white matter and centrum semiovale that
correspond to foci of demyelination and vacuolation (Fig. 8.7a, arrows). Lesions do not
enhance. The extent of disease roughly parallels the clinical neurologic deterioration.
The lesions of HIV demyelination tend to be somewhat more symmetric and central
(periventricular white matter) than those of progressive multifocal leukoencephalopathy
(PML), which are typically multifocal and asymmetric areas of T1-weighted and
T2-weighted images in the periventricular and subcortical white matter (Fig. 8.7c).
Although both entities appear hyperintense on T2-weighted images, the lesions of HIV
demyelination are usually inapparent on T1-weighted images, whereas those of PML are
often hypointense and well demarcated on T1-weighted images. Clinical correlation, how-
ever, is extremely helpful because HIV encephalitis often manifests as a global encephal-
opathy, whereas PML is usually associated with a focal neurologic deficit.
Proton MR spectroscopy of HIV encephalitis in subcortical region shows reduction of
NAA (neuronal loss) and elevated choline in white matter (Fig. 8.7b). Therefore, proton MR
spectroscopy may helps in differentiation with PML because in this case it demonstrates a
reduction in NAA, presence of lactate, and increased amounts of choline and lipids
(Fig. 8.7d).
Case 8
Leigh Disease
a b
c d
Fig. 8.8
A 4-year-old female presented with important developmental delay, hypotonia, seizures, History
and increased lactate in cerebrospinal fluid.
Leigh disease is often referred to as subacute necrotizing encephalomyopathy. In the Comments

majority of cases, this disease is caused by enzyme deficiencies (respiratory chain complex
I, III, and IV, or pyruvate dehydrogenase), followed by mitochondrial DNA mutations.
The disease typically appears during early infancy, average age of 6 months, and pres-
ents with failure to thrive and progressive neurological deterioration, including develop-
mental delay or loss of milestones, hypotonia, weakness, ataxia, dystonia, and seizures.
Laboratory tests reveal lactic acidosis and increased pyruvate concentrations both in blood
and CSF.
The pathologic hallmarks include focal, bilateral, and symmetric spongiform lesions in
the thalamus, pons (tegmentum), inferior olives, and the posterior columns of the spinal
cord.
It is currently treated with thiamin (vitamin B1), but even with prompt treatment, infants
rarely live longer than 2 or 3 years after the onset of the symptoms. In cases of older people,
the disease takes longer, but is still almost always fatal.
The characteristic MR findings in Leigh disease are bilateral symmetric areas of high signal Imaging Findings
intensity on T2-weighted images in the basal ganglia (putamen, globus pallidus, caudate
nucleus) (Fig. 8.8a, arrows), thalamus, brainstem (Fig. 8.8b, arrow), cerebellar white matter,
cerebellar cortex, cerebral white matter, and the gray matter in the spinal cord. Delayed and
hypomyelination in Leigh disease are common findings on MR imaging.
As an uncommon imaging feature of Leigh disease, signal enhancement after intrave-
nous gadolinium injection has also been described within some of the affected brain areas,
including the periventricular white matter, periaqueductal and hypothalamic structures,
and mammillary bodies.
Diffusion-weighted images may show restricted diffusion during acute metabolic attacks
within the lesions in brainstem, basal ganglia, and dentate nuclei.
Proton MR spectroscopy with voxel placement in the basal ganglia or lateral ventricles
typically demonstrates an abnormal lactate peak (Fig. 8.8c, d, arrows) with a decrease in the
NAA/Cr. The lactate peak can be easily demonstrated using a higher TE, as it presents as an
inverted double peak. On successful therapy, the cerebral lactate peak may disappear, but in
cases of metabolic deterioration it may reappear.
History A 67-year-old woman presented with left hemiparesis

Case 9 for 2 weeks.
Lymphoma
a b
c d Co Lip.
Naa
Cr
Fig. 8.9
Lymphomas are solid tumors of the immune system. Non-Hodgkin’s lymphomas account Comments
for about 4% of new cancers in the United States. For most cases of non-Hodgkin’s
lymphoma, the etiology is unknown, although genetic, environmental, and infectious
agents have been implicated. In some cases, lymphoma may be related to Epstein–Barr
virus. The risk of non-Hodgkin’s lymphoma is also increased more than 100-fold in patients
with the human immunodeficiency virus (HIV).
The clinical signs and symptoms of central nervous system (CNS) lymphoma are usu-
ally nonspecific but may be related to the affected area. Personality changes (especially
with thalamic or corpus callosal lesions), cerebellar signs, headaches, seizures, and motor
dysfunction are the most common symptoms and signs at presentation.
The gross pathologic manifestations of CNS lymphoma may be intraaxial nodules and
diffuse meningeal or periventricular involvement. At histologic analysis, CNS lymphoma
exhibits sheets of monotonous, closely packed blue cells, has a high degree of cellularity,
and infiltrates far beyond the borders of the grossly observed mass.
Radiotherapy and chemotherapy are frequently used for localized disease.
The classic imaging findings of parenchymal lymphoma include masses that involve the Imaging Findings
deep gray matter structures, periventricular regions, and corpus callosum.
On CT scans, lesions typically have high attenuation and may show enhancement after
administration of contrast material. However, negative findings from a CT scan do not
exclude the diagnosis of CNS lymphoma. Lymphomatous masses do not calcify, and hem-
orrhage is distinctly uncommon on imaging studies.
On T1-weighted images, the lesions are typically slightly hypointense to isointense rela-
tive to gray matter, produce relatively little mass effect for their size and show dense con-
trast enhancement (Fig. 8.9a). The lesions are known to be more commonly hypointense to
isointense relative to gray matter on T2-weighted images (Fig. 8.9b). This appearance likely
reflects the increased nuclear-cytoplasmic ratio in these densely packed and highly cellular
tumors. T2 hypointensity, when seen, helps differentiate CNS lymphoma from gliomas
(with the exception of high-grade gliomas) and demyelinating disease, which are more
commonly hyperintense on T2-weighted images.
Toxoplasmosis can have very similar imaging appearance of lymphoma. Because toxo-
plasmosis is fairly common in acquired immunodeficiency syndrome (AIDS) patients,
those who present with typical clinical and radiographic findings are placed on appropri-
ate medical therapy, and follow-up scans are obtained in 2 weeks. Findings of irregular,
sinuous, or even gyral-like contrast enhancement suggest CNS lymphoma rather than tox-
oplasmosis, which typically has smooth, peripheral ring enhancement.
Diffusion-weighted imaging typically shows a restricted diffusion with low ADC map,
due to hypercellularity (Fig. 8.9c).
At proton MR spectroscopy, lymphoma demonstrates elevated peaks of choline (Fig. 8.9 d),
and MR perfusion typically shows an elevated relative cerebral blood volume in lymphoma
but not in toxoplasmosis. Toxoplasmosis demonstrates elevated peaks of lipid and lactate,
findings that, unfortunately, can also be seen in lymphoma if the voxel is placed over the
necrotic, rather than cellular portion, of the lesion.
History A 23-year-old man presented with a recent onset of

Case 10 headache and speech disorder.
Pyogenic Brain Abscess
a b
c d
Fig. 8.10
Brain abscess is an uncommon disorder accounting for only 2% of intracranial mass Comments
lesions. CNS abscesses are circumscribed, enlarging, focal infections that produce symptoms
and findings similar to those of other space-occupying lesions, such as brain tumors. Brain
abscesses, however, often progress more rapidly than tumors and more frequently affect
meningeal structures.
Infections resulting in brain abscess originate in or extend from extracerebral locations. The
most frequent predisposing factors are hematogenous spread, direct extension from paramen-
ingeal sites (otitis, cranial osteomyelitis, sinusitis), recent or remote head trauma or neurosurgi-
cal procedures, and infections associated with cyanotic congenital heart disease. Extension of
infection from otitis and mastoiditis involves contiguous brain regions of the temporal lobe and
cerebellum, whereas abscesses resulting from sinusitis affect the frontal and temporal lobes.
The pathogenic organisms vary considerably, depending on the clinical circumstances.
The most commonly isolated pathogens are aerobic and microaerobic streptococci and
gram-negative anaerobes such as Bacteroides and Prevotella spp. Less commonly, gram-
negative aerobes and Staphylococcus spp. are isolated.
Signs of infection may be minimal or absent. Almost half of affected patients maintain a
normal body temperature, and fewer than one third show a peripheral white cell count
greater than 11,000/mL.
A headache of recent onset is the most common symptom, representing distortion or
irritation of pain-sensitive structures within the cranial vault, especially those of the great
venous sinuses and the dura mater about the base of the brain. Seizures may occur with
abscesses that involve the cortical gray matter.
Pyogenic brain abscesses are treated with antibiotics combined with surgical aspiration
or excision. Prophylactic anticonvulsants may reduce the risk of seizure.
CT images reveal a low-density lesion with a ring enhancement. Imaging Findings

MR imaging in a typical abscess with central liquefactive necrosis shows a center of the cavity
slightly hyperintense to cerebrospinal fluid, whereas the surrounding edematous brain is slightly
hypointense to normal brain parenchyma on T1-weighted images (Fig. 8.10a). On T2-weighted
images the signal intensities are quite variable depending on the protein composition and vis-
cosity of the material in the central cavity (Fig. 8.10b). The rim is isointense to slightly hyperin-
tense to white matter on T1-weighted images and is hypointense on T2-weighted images.
The ring enhancement of an abscess capsule on postgadolinium MR images parallels
the enhancement seen on postcontrast CT images. The ring is usually smooth and thin
walled (approximately 5 mm in thickness) and is often thinner along the medial margin,
possibly due to variation in perfusion of gray and white matter. Daughter abscesses appear
as adjacent smaller enhancing rings, often along the medial margin of the parent abscess,
which helps in differentiation from other necrotic lesions, especially in necrotic or cystic
degeneration of tumors (including gliomas).
Edema surrounding an abscess may be greater in volume than the abscess itself and
causes much of the associated mass effect.
On diffusion-weighted echo planar images, an abscess demonstrates restricted diffusion
directly related to the cellularity and viscosity of the pus contained within an abscess cavity
(Fig. 8.10c, d).
Further Reading Koeller KK, Smirniotopoulos JG, Jones RV (1997) Primary central
nervous system lymphoma: radiologic-pathologic correla-
Bonneville F, Sarrazin J, Marsot-Dupuch K, Iffenecker C, tion. Radiographics 17:1497–1526
Cordoliani Y, Doyon D, Bonneville J (2001) Unusual lesions Lovblad KO, Laubach HJ, Baird AE, Curtin F, Schlaug G, Edelman
of the cerebellopontine angle: a segmental approach. Radio- RR, Warach S (1998) Clinical experience with diffusion-
graphics 21:419–438 weighted MR in patients with acute stroke. AJNR Am J
Castillo M, Kwock L, Courvoisie H, Hooper SR (2000) Proton MR Neuroradiol 19:1061–1066
spectroscopy in children with bipolar affective disorder: pre- Smith AB, Smirniotopoulos JG, Rushing EJ (2008) Associated
liminary observations. AJNR Am J Neuroradiol 21:832–838 with human immunodeficiency virus infection: radiologic-
Chen S, Ikawa F, Kurisu K, Arita K, Takaba J, Kanou Y (2001) pathologic correlation. Radiographics 28:2033–2058
Quantitative MR evaluation of intracranial epidermoid Srinivasan A, Goyal M, Al Azri F, Lum C (2006) State-of-the-art
tumors by fast fluid-attenuated inversion recovery imaging imaging of acute stroke. Radiographics 26(Suppl 1):S75–S95
and echo-planar diffusion-weighted imaging. AJNR Am J Tortori-Donati P (2005) Pediatric neuroradiology: brain, head
Neuroradiol 22:1089–1096 and neck and spine. Springer-Verlag. ISBN-13: 3540410775
Ho VB, Fitz CR, Chuang SH, Geyer CA (1993) Bilateral basal ganglia
lesions: pediatric differential considerations. Radiographics
13:269–292
DTI and Bold MR Imaging 9
Luiz Celso Hygino da Cruz Jr., Isabela Garcia Vieira, and Tatiana Chinem Takayassu
Contents
Case 1 Semilobar Holoprosencephaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 182
Case 2 Low-Grade Glioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 184
Case 3 Gliomatosis Cerebri . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 186
Case 4 Alzheimer Disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 188
Case 5 Arteriovenous Malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 190
Case 6 Amyotrophic Lateral Sclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 192
Case 7 Diffuse Axonal Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 194
Case 8 Multiple Sclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 196
Case 9 Focal Cortical Dysplasia and Seizure . . . . . . . . . . . . . . . . . . . . . . . . . . 198
Case 10 High-Grade Tumor . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 200

Diffusion tensor imaging (DTI) is a new technique that allows the mapping of mean water Introduction
diffusion in fibrous tissues. It depends on a property, known as diffusion anisotropy, which
is based in the fact that water molecules diffuse more easily in the direction aligned with
internal structure and with difficulty as it moves perpendicular to the preferred direction.
Diffusion
The acquired data enable the reconstruction of 3D images of white matter tracts
Tensor
(tractography) and the measurement of fractional anisotropy, which reflects the anisotropy Imaging
of the analyzed structure.
Some clinical applications of DTI are in the localization of white matter lesions such as
trauma and demyelinating diseases, such as multiple sclerosis; the relation of tumor to the
white matter tracts (infiltration, deflection) in presurgical evaluation; and the assessment
of white matter in development, pathology, and degeneration diseases.
Blood oxygen level–dependent functional (BOLD) MR imaging is a noninvasive technique Blood Oxygen
that offers an unprecedented opportunity to explore the neuronal basis of human cognition, Level-dependent
perception, and behavior. In BOLD imaging, as its name implies, neuronal activation is
inferred from small, local MR signal changes (on the order of 3% at 1.5 T field strength)
proportional to hemodynamically induced alterations in net deoxyhemoglobin
concentration caused by task-related increases in neuronal metabolism.
Clinical applications of BOLD imaging have focused primarily on the evaluation of
functional cortex plasticity in patients with stroke, congenital malformations, and trauma;
on the preoperative localization of the motor, sensory, and language centers of the brain in
anticipation of tumor or vascular malformation resection, and in the functional evaluation
of focal cortical dysplasia in epilepsy.
182 Luiz Celso Hygino da Cruz Jr., Isabela Garcia Vieira, and Tatiana Chinem Takayassu
History A 20-day-old white male neonate presented with

Case 1 dehydration and was found out to have diabetes
insipidus and hypoglycemia. There were microcephaly,
Semilobar Holoprosencephaly hypotelorism, and microphthalmia. This infant had
been delivered at 37 weeks by elective cesarean delivery
after an uncomplicated full-term pregnancy.
Fig. 9.1
Holoprosencephaly (HPE) denotes an incomplete or absent division of the embryonic Comments

forebrain (prosencephalon) into distinct lateral cerebral hemispheres. It is roughly
categorized into three types (from most severe to least severe): (1) alobar holoprosencephaly,
or complete absence of midline forebrain division resulting in a monoventricle and fused
cerebral hemispheres; (2) semilobar holoprosencephaly, or incomplete forebrain division
resulting in partial separation of the cerebral hemispheres, typically posteriorly; and (3)
lobar holoprosencephaly, or complete ventricular separation with focal areas of incomplete
cortical division or anterior falcine hypoplasia. These lesions are often diagnosed on
prenatal sonography or MRI, allowing adequate prenatal management.
The age of onset is at 3–4 weeks of gestation, being present in 1:16,000 in live births and
1:250 in conceptuses.
The cause of HPE is currently unknown. Often, no specific cause can be identified.
Suggested risk factors include maternal diabetes, infections during pregnancy, and various
drugs taken during pregnancy.
Semilobar HPE is intermediate in severity, with partial formation of the interhemi-
spheric fissure, particularly posteriorly. A somewhat H-shaped monoventricle with par-
tially developed occipital and temporal horns is common. A rudimentary falx cerebri and
incompletely formed interhemispheric fissure are often seen, with partial or complete
fusion of basal ganglia.
In some cases of semilobar HPE, craniofacial abnormalities are associated, including
hypotelorism, and median and lateral cleft lip.
Also not uncommon are seizures, hypotonia and/or hypertonia, extrapiramidal symp-
toms, such as dystonia and/or chorea; hypothalamic and brainstem dysfunction leading to
autonomic dysfunction and swallowing difficulties; pituitary dysfunction, which can man-
ifest as partial or complete panhypopituitarism with resultant endocrine deficiencies.
Isolated semilobar HPE have empiric survival rates of about 50% to age 12 months.
Brain MR imaging is the diagnostic imaging modality of choice. On MR imaging, semilobar Imaging Findings
HPE is characterized by partial ventricular differentiation but with a single ventricular
cavity, a partial interhemispheric fissure and falx (posterior-ventral axis), partial or
incomplete formation of the corpus callosum, and variable degree of thalamic fusion. The
olfactory bulbs are often absent.
Axial T1-weighted (Fig. 9.1a) and T2-weighted (Fig. 9.1b) MR imaging demonstrate
primitive occipital horns (large white arrows) and partial, rudimentary interhemispheric
fissure (small red arrows). The basal ganglia and anterior white matter are fused (Fig. 9.1b,
black arrows).
On the fractional anisotropy (FA) color-coded derived from diffusion tensor MR imag-
ing (Fig. 9.1c), a thick bilobed subfrontal structure is shown; the red color indicates a pref-
erential transverse orientation. This bilobed structure is composed of the fused caudate
nuclei and thalami, and a transversely oriented white matter bundle (red arrow).
On the axial image derived from tractrography (Fig. 9.1d), there are large symmetric
fiber bundles having the expected course of the frontooccipital fasciculus (Fig. 9.1c, d, green
arrows); the fasciculus was connected across the midline in the subfrontal region. A band
of transversely oriented white matter is seen over the frontal convexity (red arrow).
Case 2
Low-Grade Glioma
Fig. 9.2
A 9-year-old girl presented with nausea and poor motor coordination for 2 months. History
Low-grade glioma located at the pons has been described as a locally invasive tumor. It Comments
has been traditionally considered to have a dismal prognosis, and to be malignant by
location and not by histology.
Approximately three-fourths of patients are younger than 20 years, with a peak age
around 7 years. The clinical presentation includes headaches, nausea, vomiting, gait distur-
bances, ataxia, visual deficits, seizures, hemiparesis, and cranial nerve dysfunction. It typi-
cally appears as mass that expands the pons and flattens the fourth ventricle, as the pontine
tegmentum becomes convex posteriorly. It may show hemorrhage at the onset or during
the follow-up studies, possibly reflecting anaplastic transformation. Hemorrhage heralds a
dismal prognosis, and is due to rupture of newly formed vessels within malignant portions
of the mass.
Focal radiotherapy has been the treatment of choice. Typically, biopsy and/or surgery
are not required for diagnosis or treatment of diffuse intrinsic pontine gliomas and cannot
be recommended routinely; diagnosis can be made by MRI alone.
CT may detect diffuse pontine glioma as hypodense or isodense mass that expands the Imaging Findings
brain stem, but MRI provides a far better appraisal. On MRI, most pontine gliomas are
diffuse and tend to invade the midbrain, medulla, and cerebellar peduncles. Diffuse tumors
may infiltrate the whole brain stem and extend upward to the thalami. They may impinge
on or engulf the basilar artery when projected anterolaterally.
MRI generally depicts an aspecific increase of both T1 and T2 relaxation times, as well
as high signal in proton density-weighted and FLAIR images. Both CT density and MRI
signal intensity frequently are heterogeneous and nonenhancing.In the case herein pre-
sented, MR imaging shows an expanded lesion at the pons with hyperintense signal on
axial T2-weighted image (Fig. 9.2a white arrow) and hypointense signal on axial T1-weighted
image (Fig. 9.2b, white arrow). On axial T1-weighted postgadolinium image (Fig. 9.2c), this
lesion (white arrow) does not demonstrate significant enhancement.
Axial diffusion tensor color-coded maps of the pons at the level of middle cerebellar
peduncles (Fig. 9.2d) demonstrate a destruction and reduction of fractional anisotropy in
the main fiber tracts, including the corticospinal tracts (blue arrow) and the transverse
pontine fibers (red arrow).
Case 3
Gliomatosis Cerebri
Fig. 9.3
A 39-year-old woman presented with a short history of visual disturbance and an episode History
of generalized seizure.
Gliomatosis cerebri is a rare diffusely infiltrating glial tumor that involves at least two lobes Comments
of the brain and are frequently bilateral. Patients usually present between the third and
fifth decades of life, but cases have been described in patients of all ages.
Clinical findings are nonspecific and are characteristically mild in comparison with
imaging findings. The most common symptoms are alterations in mental status and per-
sonality, followed by headaches and seizures. Focal neurologic deficits appear late in the
course of the disease.
According to autopsy studies, gliomatosis cerebri may affect (in decreasing order of fre-
quency) the cerebral hemispheres, midbrain, pons, thalamus, basal ganglia, cerebellum,
medulla oblongata, and more rarely, the hypothalamus, optic nerve and chiasm, and spinal
cord. The corpus callosum may also be involved in up to 50% of cases. In the cerebral hemi-
spheres, the white matter is always invaded typically, whereas the cortex is involved in only
19% of cases.
The prognosis is generally poor, with survival rates of 50% at 1 year.
Few treatment options exist. Steroid therapy may help as initial treatment; however, the
lesions are too widespread for surgery, and radiation therapy and chemotherapy are of
questionable benefit, due their poor response.
CT may demonstrate normal findings or may show diffuse low attenuation in affected Imaging Findings
areas, and minimal or no enhancement after intravenous contrast administration.
The MR examination reveals extensive bilateral poorly defined areas of hyperintensity
on T2-weighted images (Fig. 9.3a) and iso- to hypointense on T1-weighted images in
affected regions with little mass effect. Areas that display loss of gray-white matter differ-
entiation correspond to areas of neoplastic infiltration.
On T1-weighted postgadolinium (Fig. 9.3b), it usually shows no or minimal enhance-
ment. If areas of significant enhancement are seen, they can represent both higher-grade
tumor and dense tumor infiltration.
Single-voxel short-echo MR spectroscopy may show elevated levels of creatine (Cr) (at
3.0 ppm) and myoinositol (mI) (at 3.55 ppm), a reduced level of NAA (at 2.02 ppm), and a
mildly elevated level of choline (Cho) (at 3.2 ppm).
Gliomatosis cerebri cells invade myelinated tracts in a parallel orientation (infiltration
pattern). Therefore, diffusion tensor color-coded maps (Fig. 9.3c) and tractography
(Fig. 9.3d) are useful to demonstrate the preservation of underlying histoarchitecture, with
normal directionality of nerve fibers, although extensive and diffuse lesion that infiltrates
areas of the brain.
Case 4
Alzheimer Disease
Fig. 9.4
A 80-year-old man presented with language impairment and progressive memory loss for History
recent events.
Alzheimer disease (AD), the most common cause of dementia among the elderly, is an Comments
acquired cognitive and behavioral impairment of sufficient severity that markedly interferes
with social and occupational functioning.
The prevalence of AD increases with age. It is estimated that more than 14% of individu-
als older than 65 years have AD, and the prevalence increases to at least 40% in individuals
older than 80 years.
The anatomic pathology of AD includes neurofibrillary tangles and senile plaques at the
microscopic level and cerebrocortical atrophy, which predominantly involves the associa-
tion regions and particularly the medial aspect of the temporal lobe.
It primarily affects the gray matter, first involving the entorhinal cortex and then other
portions of the limbic system, such as the hippocampi, temporal lobes and posterior cingu-
late gyri. Although Alzheimer’s disease pathology mainly affects cortical grey matter, pre-
vious pathological and MRI studies showed that also the brain white matter of patients is
damaged. This fact is probably secondary to wallerian degeneration of fiber tracts due to
neuronal loss in cortical associative areas.
The clinical manifestations are insidiously progressive memory loss, initially for recent
events; language impairment; cognitive decline, such as visuospatial dysfunction, apraxia,
agnosia, and executive dysfunction.
The clinical diagnosis of AD is based on identifying elements within the medical history
and clinical examination that are suggestive of the disease together with exclusion of other
causes of dementia by means of brain imaging and laboratory tests.
No current treatments can either cure or permanently arrest AD. However, AD-specific
drug therapies are available.
Brain MR imaging or CT scans are not diagnostic of AD. However, these imaging modalities Imaging Findings
can show diffuse cortical and/or cerebral atrophy, or more commonly, parietal and temporal
atrophy, better demonstrated on coronal T2-weighted image (Fig. 9.4a) and sagittal
T1-weighted image (Fig. 9.4b).
Coronal T2-weighted image can also demonstrates bilateral hippocampal and entorhi-
nal cortical atrophy (Fig. 9.4a, white arrow). Nonspecific areas of high signal intensity on
white matter may be seen in patients with AD (double white arrows).
Fractional anisotropy (FA) maps derived from diffusion tensor MR imaging (Fig. 9.4c, d)
show reduction of FA values on right and left hippocampi (Fig. 9.4c, ROI 1 and 2, respectively)
and also on right and left posterior cingulate gyri (Fig. 9.4d, ROI 1 and 2, respectively).
Single-voxel proton spectroscopy MR in posterior cingulate gyrus or medial temporal
lobes usually shows decreased N-acetyl aspartate level (neuronal loss) and elevated myo-
inositol level (gliosis).
A pattern of bilateral temporoparietal hypometabolism on PET has been shown to have
high specificity for AD and may be useful in confirming its diagnosis.
Case 5
Arteriovenous Malformation
Fig. 9.5
A 15-year-old boy presented with left hemiparesthesia and generalized headache. Previous History
brain MR imaging showed a lesion consistent with arteriovenous malformation.
AVMs are congenital lesions composed of a complex tangle of arteries and veins connected Comments
by one or more fistulae. The vascular conglomerate is called the nidus. The nidus has no
capillary bed, and the feeding arteries drain directly to the draining veins.
Despite the presumed congenital origin of AVMs, the clinical presentation most com-
monly occurs in young adults.
AVMs tend to be clinically silent until the presenting event occurs. AVMs produce neu-
rological dysfunction through hemorrhage, seizure, or more rarely, a progressive neuro-
logical deficit. A history of headaches is present in as many as half of patients with cerebral
AVM.
The diagnosis usually is made at the time of the first seizure or hemorrhage.
Treatment planning for AVMs depends on risk of subsequent hemorrhage, through
invasive treatment of AVMs that may include endovascular embolization, surgical resection,
and focal beam radiation, alone or in any combination. An older individual or a patient
with no high-risk features may be best treated with anticonvulsants for seizure control and
appropriate analgesia for headaches.
Axial FLAIR MR image (Fig. 9.5a) shows a lesion composed of tangle of vessels (arrow) Imaging Findings
with flow voids, located in the right precentral gyrus and central sulcus, involving the
anatomically expected area of hand representation.
On T1-weighted postgadolinium image (Fig. 9.5b), besides flow voids, shows small foci
of enhancement anteriorly (arrow) in this arteriovenous malformation.
Axial blood oxygen level–dependent (BOLD) functional MR images show cortical acti-
vation at the rolandic areas contralaterally to the hand movement (Fig. 9.5c, d). Compared
with the left hemisphere primary motor area (M1) representation, which is in the expected
anatomic location, activation in the right hemisphere is displaced posteriorly. Note addi-
tional activation in bilateral supplementary motor areas (Fig. 9.5d, arrow).
In some studies, there are evidences suggest that brain AVMs lead to reorganization
within the somatotopic representation in M1 and to occasional abnormal expansion into
nonprimary motor areas.
Case 6
Amyotrophic Lateral Sclerosis
t. córtico-espinhal
t. temp.-par-
occi-pontino
t. fronto-pontino
Fig. 9.6
A 40-year-old man presented with progressive dysphonia, dysarthria, and motor weakness History
of a 3-year duration. He noticed atrophy and fasciculation involving muscles of extremities
and trunk. He has no significant alteration on eye movement, sphincter control, and
cognitive and sensitivity functions.
Amyotrophic lateral sclerosis is a progressive and fatal neurodegenerative disease, Comments

characterized by selective degeneration of motor neurons of brain stem and spinal cord
(lower) and neurons of motor cortex (upper).
The disease name refers to the “sclerosis” of the nerve fibers originate from upper motor
neurons, which are located in the lateral horn of the spinal cord that forms the cortico-
spinal tract (CST) side, leading to muscle atrophy. The lack of innervations leads to muscle
weakness, atrophy, and fasciculation. These motor neurons are responsible for the volun-
tary muscle movements, and their injury causes motor paralysis. Bladder and bowel
sphincters, muscles responsible for eye movement, sensitivity, and cognitive functions are
generally spared.
There is loss of skills in walking, speech, and swallowing, and, as the disease progresses,
the patient eventually died of respiratory failure when the muscles associated with breath-
ing are affected. The disease does not affect their intellectual abilities. With speech muscles
commitment, there are also communication difficulties.
There is a male predilection with onset usually between fourth to seventh decades of life.
The exact cause is not well known and the majority of cases are sporadic (90%). There is no
cure or effective treatment. Palliative care is very important for improving the quality of life
for patients. Regardless life expectancy, 20% of patients survive more than 5 years.
Serial CT examinations may show progressive atrophy, initiating in frontal and temporal Imaging Findings
lobes that that extend to pre- and postcentral gyrus, corpus callosum, and tegmentum.
The most specific finding on unenhanced brain MR imaging are bilateral and symmet-
ric areas of hyperintensity along CST extending from corona radiata through the posterior
aspect of the internal capsule and the cerebral peduncles (Fig. 9.6a, b) to the anterolateral
column of the spinal cord on T2-weighted images.
DTI can assess CST lesion before pyramidal symptoms become apparent. There is a
significant reduction on FA values (Fig. 9.6c) in middle portion of the cerebral peduncle as
well as in the posterior limb of internal capsule, which probably indicates axonal degenera-
tion. There is also an increase in ADC values that may reflect increases in the extracellular
water volume secondary to axonal loss. FA values can be affected in early disease process
whereas the ADC values alteration can indicate more chronic changes with neurons loss.
Case 7
Diffuse Axonal Injury
Fig. 9.7
A 21-year-old man lost consciousness in a car accident 6 months ago. He persists in a History
vegetative state.
Diffuse axonal injury (DAI) is a frequent result of traumatic acceleration-deceleration Comments

injury, most commonly caused by high-speed motor vehicle accident. It is also related to
the shaken baby syndrome, in which throwing or violent shaking of a baby causes brain
injury.
DAI rarely causes death, but it is a frequent cause of persistent vegetative state in patients
(90%), leading to a high medical cost.
This type of impact causes rotation forces that lead to injury in areas of great density
difference, such as the gray-white matter interface. The visible spots are the tip of the ice-
berg, since 80% of lesions are microscopic, not bleeding, and most are not detectable.
There are no racial or sex predilection, and it can occur at any age. There is no efficient
treatment, the prognostic is no good, and is related with the numbers and size of lesions.
In the acute setting, CT can be normal most of the time (50–80%). However, diffuse and Imaging Findings
bilateral hyperdense small areas of bleeding (1–15 mm) in characteristic locals, such as the
gray-white matter junction, corpus callosum, and dorsolateral rostral brainstem; or even
small areas of low density of edema can be demonstrated. It is not common to find skull
fractures. Subarachnoid and subdural hemorrhage may not be present.
In Fig. 9.7a, CT scan shows a fluid-fluid level in the posterior aspect of the left lateral ven-
tricle, corresponding to blood. No other abnormal findings are seen in brain parenchyma.
Although CT is not so sensitive as MRI is, it is more available and practical in emergency
head injury.
Typical MRI images findings are multiple hypointenses foci on T2* GRE sequences in
characteristic distribution, secondary to hemorrhage, demonstrated in Fig. 9.7b in the cor-
pus callosum.
Conventional MRI on acute setting has poor correlation with prognostic as discussed
earlier, since 80% of lesions are not detectable on MR images. DTI is an extension of the dif-
fusion, which allows the quantification of white matter architecture. The extent of injuries is
much greater than is seen in conventional images, but DTI is more sensitive to demonstrate
these lesions by reducing the fractional anisotropy (FA), which characterize cytoarchitec-
ture disorder (Fig. 9.7c). Tractography may show the fiber destruction (Fig. 9.7d).
Spectroscopy can show decreasing of NAA levels, which is related to the patient’s prog-
nosis in acute setting. There are also elevated Cho levels, which represent destruction of
myelin and cell membrane.
History A Caucasian 37-year-old woman presented with acute

Case 8 onset of difficulty in speaking and moving her left arm
and leg, besides double vision.
Multiple Sclerosis
Fig. 9.8
Multiple sclerosis (MS) is a perivenular demyelinating disease, probably autoimmune- Comments

mediated, which is the most common disabling central nervous system disease of young
adults. It is more common in Caucasian, young (30–40 years old) women (M:F = 1:2), and
mostly occurs at temperate zones. One important characteristic of this disease is its distribution
in time and space. Imaging findings can achieve this alteration, in association to clinic and
laboratorial examinations. The most common is relapsing-remitting form of the disease, with
latter shifting to chronic-progressive form. Immunomodulators and/or immunosuppressants
is the treatment of choice in almost all cases of MS. Depending on evaluation of the disease,
patients can progress with motor deficits, severe disability, and cognitive impairment.
CT images are not so sensitive as MRI are to detect demyelinating lesions. In early stages, Imaging Findings
CT can be normal. CT can demonstrate iso- or hypodense lesions. When the lesions are
acute, mild to moderate enhancement can be observed.
MRI is the most important imaging examination to demonstrate MS lesions and the
classic findings are multiple bilateral and asymmetric ovoid T2 hyperintensity (5–10 mm)
lesions, usually perpendicular at the callososeptal region (Fig. 9.8a) with extension to
periventricular, perivenular (Dawson’s fingers), and infratentorial (10%) location. It is
important to remember that hyperintensity on T2-WI is not specific, and it is necessary to
evaluate other imaging aspects and associate these data to clinical and laboratory results
for the correct diagnosis. Ten to 20% of chronic lesions are hypointense on T1-WI, the so-
called black holes. Images with gadolinium demonstrate transient enhancement during
acute setting of demyelination (Fig. 9.8b) that disappears within 6 months (90%). The
enhancement pattern can be nodular, ring, or incomplete (horseshoe-shaped) enhance-
ment. The Barkhof MRI criteria are used to establish the diagnosis of clinically isolated
syndromes that can suggest MS. These criteria are defined by spatial and time progress,
and MR images are very helpful to access this information. For example, MRI may demon-
strate chronic lesions (black holes), enhancement plaques (acute lesions) at the same exam-
ination, and determining different time-related lesions, even though follow-up examinations
are indicated to demonstrate the time progress. Serial examinations are also very impor-
tant to evaluate treatment response.
Although MRI is the most sensitive technique available for the detection of lesion, the
images findings on conventional examination do not always correspond to the clinical man-
ifestation. Conventional MRI lacks of specificity and is not able to detect subtle abnormali-
ties in the normal-appearing white matter (NAWM). Advanced MRI, such as magnetization
transfer (MT), DWI, and DTI may, however, help overcome these limitations. In the acute
setting, the center of the lesion is a demyelinated area with inflammatory tissue, seen at
advanced MRI with high ADC values and reduction of FA, as compared to the rim, NAWM,
and chronic plaques (Fig. 9.8c). The surrounding rim is probably an acute demyelination
region with an important inflammatory process, which may show enhancement and restrict
diffusion. In chronic lesions, one can see reduced cellularity associated with increase in
extracellular water, which appears as intermediate ADC elevation and reduced FA when
compared with NAWM. The typical location of spinal cord lesion is on posterior region. and
also can have low FA values (Fig. 9.8d, e).
Case 9
Focal Cortical Dysplasia and Seizure
Fig. 9.9
A 32-year-old male patient presented with tonic-clonic seizures since childhood. Lately, the History
epileptic episodes are medically refractory. He is a candidate for epilepsy surgery treatment
and is on presurgical evaluation.
Focal cortical dysplasia is a congenital abnormality where neurons failed to migrate. This Comments
abnormal area sends an incorrect signal to the cortex, being a common cause of intractable
epilepsy. It can be treated with anticonvulsant medication. However, surgery has become a
viable treatment option. It consists of removing or disconnecting the affected region, by
hemispherectomy, or by resecting only the epileptogenic zone.
CT rarely demonstrated cortical dysplasia as a cause of seizure. High-field MRI with high- Imaging Findings
spatial resolution 3D volumetric images is more sensible for depicting subtle cortical
dysplasia.
The standard MR clinical protocol includes T1-WI high-spatial resolution 3D volumet-
ric images of the entire brain that allows postprocessing and reformatting images into mul-
tiple planes; axial FLAIR and coronal T2WI focusing the hippocampus. The MRI findings
of cortical dysplasia are undefined of gray-white matter junction, cortical thickening on at
least three or more contiguous slices, and focal thinning with volume loss of the underlying
white matter (Fig. 9.9a). On FLAIR and T2-WI, it can be seen as a hyperintensive area that
extends in direction to the ventricles wall (Fig. 9.9a).
Functional neuroimaging techniques, such as functional MRI (fMRI) are not widely
available, but can be very helpful as a presurgical tool for mapping the eloquent cortex and
for indicating its relation to the epileptogenic zone. Functional MRI topography eloquents
regions of the brain, such as auditive (Fig. 9.9b), motor/sensitive (Fig. 9.9c), and language
(Fig. 9.9d) zones. These sequences are relevant in the surgical setting because it is helpful
to determine whether the lesion is located close to functionally important areas.
d
Case 10
High-Grade Tumor
a b
Fig. 9.10
A 73-year-old man presented with left hemiparesis and seizures. MR images show a large History
right parietal mass. Biopsy specimen confirmed glioblastoma mutiforme.
Glioblastoma multiform (GBM) is the most aggressive and most common primary brain Comments
tumor. This tumor is slightly more common in white males, with the peak incidence at
45–70 years. Rapidly enlarging, neovascularization, and necrosis characterize it. It has a
poor prognosis, despite new and more advanced types of treatment, including surgery,
radiotherapy, and/or associated to chemotherapy.
The symptoms are nonspecific, since they depend on the location of the tumor and may
be characterized by headache, seizure, nausea, vomiting, hemiparesis, progressive neuro-
logical, personality, and memory deficit.
The differential diagnosis is cerebral mestastasis, primary CNS lymphoma, cerebral
abscess, anaplasic astrocytoma, tumefactive demyelination, and cerebral toxoplasmosis.
The most common image finding of GBM is a poorly marginated and infiltrating Imaging Findings
supratentorial mass that presents thick and irregular surrounding enhancement (Fig. 9.10a)
with a central area of necrosis. Supratentorial white matter is the most common location
(frontal, temporal, or parietal lobes), but it can occur in brainstem and cerebellum. GBM
can cross white matter tracts (corpus callosum and anterior/posterior commissures) and
involve the contralateral hemisphere.
On CT, GBM can show as iso- to slightly hyperdense mass (high cellularity) with
central hypodense area of necrosis. Hemorrhage occasionally is seen, but calcification is
uncommon.
On conventional MRI, GBM is hypo- to isointense on T1-WI and hyperintense in T2-WI.
Flow voids can occasionally be seen. There is an adjacent area hypointense on T1-WI and
hyperintense on T2-WI that can represent vasogenic edema and/or tumor infiltration
(Fig. 9.10a). The viable tumor frequently extends beyond the visible tumor margins. On
magnetic susceptibility images, one can see artifact related to blood products.
Diffusion-weighted images can show areas of restricted diffusivity in the solid portions
of the lesion due tumor hypercellularity. Areas of necrosis show increased diffusivity.
MR spectroscopy demonstrates a decreased NAA and myo-inositol levels and elevated
Cho and lactate/lipid peak (Fig. 9.10c). In dynamic susceptibility contrast MR imaging, a
high rCBV is demonstrated (Fig. 9.10b), which can be helpful in distinguishing high-grade
tumor from low-grade tumor and radionecrosis. Diffusion tension imaging (DTI) may
help presurgical planning as this technique can establish the relationship between the
lesion and the main fiber tracts adjacent to it. In the present example, a displacement and
invasion caused by high-grade tumor is seen, better demonstrated on the FA color-coded
map, as a reduction on the FA value of the corticospinal tract, which is dislocated contral-
aterally (Fig. 9.10d).
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Miscellaneous 10
Contents
Case 1 Arachnoid Cyst . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 206
Case 2 Colloid Cyst . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 208
Case 3 Cerebral Toxoplasmosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 210
Case 4 Herpes Simplex Encephalitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 212
Case 5 Meningitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 214
Case 6 Neuro-Behçet’s Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 216
Case 7 Progressive Multifocal Leukoencephalopathy . . . . . . . . . . . . . . . . . . . 218
Case 8 Intracranial Hypotension Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . 220
Case 9 Acute Head Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 222
Case 10 Ranula . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 224

History A 17-year-old man presented with predominantly

Case 1 occipital headache.
Arachnoid Cyst
a b
c d
Fig. 10.1
Miscellaneous 207
Arachnoid cysts, also called leptomeningeal cysts, are congenital, benign, space-occupying Comments
cystic lesions that occur in the cerebrospinal axis in relation to the arachnoid membrane.
They usually contain clear fluid that resembles cerebrospinal fluid (CSF) although they do
not communicate with the ventricular system. Most cysts are unilateral, smoothly rounded,
and adhere loosely to the dura. Their size ranges from small incidentally found cysts to
large space-occupying lesions. Arachnoid cysts represent approximately 1% of all
nontraumatic intracranial masses. The most common location is the floor of the middle
cranial fossa, particularly anteriorly and parasagittally in the interhemispheric fissure.
Cysts in the middle cranial fossa are more frequent in males than in females (ratio 3:1) and
they occur predominantly on the left side. Other locations of arachnoid cysts include the
suprasellar/chiasmatic site (cysts in this location may cause endocrinopathy); the
cerebellopontine angle (11%); the quadrigeminal plate cistern (10%); in relationship with
the vermis (9%); and in the prepontine/interpeduncular cistern (3%).
The majority of patients with arachnoid cysts are asymptomatic and the lesions are
incidentally discovered on imaging studies. In symptomatic patients, clinical features
include headache, seizures, calvarial bulging, intracranial hypertension, craniomegaly,
developmental delay, visual loss, and precocious puberty. Occasionally, arachnoid cysts are
complicated by intracystic or subdural hemorrhage.
Magnetic resonance imaging (MRI) is the diagnostic method of choice because of its abil-
ity to demonstrate the exact location, extent, and relationship of the arachnoid cyst to adjacent
brain or spinal cord. On computed tomography (CT), arachnoid cysts appear as well-defined,
noncalcified, extraaxial mass, with attenuation similar to CSF, that does not enhance after con-
trast administration. On a bone window, remodeling of the adjacent skull may be evident. On
MR images, arachnoid cysts appear as well-circumscribed nonenhancing masses that are
isointense to CSF on all pulse sequences, including diffusion-weighted imaging.
The main differential diagnosis of arachnoid cyst is epidermoid tumor. The two masses
may have similar characteristics on T1-weighted and T2-weighted images, and neither
shows enhancement with gadolinium. However, arachnoid cysts follow CSF signals on all
sequences—in particular, on the fluid attenuation inversion recovery (FLAIR) sequence—
in contradistinction to epidermoid cysts. Diffusion-weighted images allow easier differen-
tiation of the two lesions because arachnoid cysts, as a result of the CSF contents, show low
signal intensity on DWI, and epidermoid cysts tend to have a high signal on DWI. Other
lesions that have to be included on the differential diagnosis are cystic tumors, infarcts,
open-lip schizencephaly, and loculated chronic subdural hygroma.
Small or asymptomatic arachnoid cysts do not require intervention. In cases of large
lesions that produce mass effect or complicated cysts, surgery is warranted.
Sagittal and axial T1-weighted (Fig. 10.1a, b), axial proton density-weighted (Fig. 10.1c), Imaging Findings
and axial T2-weighted (Fig. 10.1d) MR images demonstrate a large cystic lesion located in
the posterior fossa, posterior to the cerebellum, which show signal intensity similar to CSF
on all sequences. The lesion causes anterior displacement and compression of the cerebellar
hemispheres and vermis. Note the remodeling of the adjacent skull (arrow). These findings
are consistent with arachnoid cyst.
History A 27-year-old man presented with a 2-year history of

Case 2 recurrent effort syncope lasting for seconds. The patient
had experienced at least six syncopal episodes often
Colloid Cyst associated with vomiting. He also complained of cervical
and occipital pain worsened by Valsalva maneuver.
a b
c d
Fig. 10.2
Miscellaneous 209
Colloid cysts are benign, congenital epithelium-lined cysts that almost always arise in the Comments
anterior third ventricle, near the foramina of Monro. Colloid cysts account for approximately
1% of all intracranial tumors and are the most common tumor in the third ventricle. The
cysts are smooth and well-defined and range in size from a few millimeters to 3–4 cm. They
are lined by simple or pseudostratified epithelial cells and are usually filled with a thick,
viscid gelatinous material although other contents may be present as well, including blood
products, macrophages, cholesterol crystals, and numerous metallic ions, such as copper,
iron, magnesium, aluminum, and phosphorus. The composition of each cyst dictates its
imaging characteristics, especially with MRI. As colloid cyst is a benign lesion, malignant
degeneration does not occur.
Colloid cysts are usually found in adults and show no gender predominance. The lesion
may not produce any symptom and it is detected incidentally on imaging studies per-
formed for other reasons. Sometimes the cyst becomes symptomatic due to the obstruc-
tion of cerebrospinal fluid flow in the foramen of Monro, resulting in hydrocephalus. The
most common presenting manifestation is chronic headache. Headaches are characterized
as brief, lasting seconds to minutes, and are initiated, exacerbated, or relieved by a change
in position. Rarely, patient presents as an acute emergency with sudden neurological dete-
rioration secondary to acute hydrocephalus which may lead to sudden death.
Both CT and MR imaging may be used in the diagnosis of colloid cysts. The most com-
mon imaging finding is that of a rounded or oval mass in the anterior aspect of the third
ventricle. On CT scans, most colloid cysts are slightly hyperdense compared to the brain,
but may occasionally be hypodense or isodense to it. After contrast material administra-
tion, a thin rim of enhancement may be present and is thought to represent the cyst cap-
sule. On MR imaging, colloid cysts have a variable appearance. MR imaging may occasionally
show intracystic fluid levels or central and peripheral components in the lesion. Some col-
loid cysts are homogeneous. About 50% of colloid cysts are hyperintense on T1-weighted
images, and the rest are either isointense or hypointense compared to brain. On T2-weighted
images, most colloid cysts are hypointense to brain. Isointense cysts may be difficult to
identify on MR images and may be more easily seen on CT scans. Calcifications are rare in
colloid cysts.
Patients with few or no symptoms and small colloid cysts and large ventricles may be
managed conservatively with serial MRIs. The remaining patients with colloid cysts should
be treated because the reported risk of sudden death. The definitive therapy is surgical
removal, which is curative. Different neurosurgical techniques have been proposed, i.e.,
simple shunting of both lateral ventricles, open surgical removal techniques, and
percutaneous aspiration procedures.
MRI shows a rounded mass located in the anterior aspect of the third ventricle, near the Imaging Findings
foramina of Monro. The tumor appears hypointense on T2-weighted images (Fig. 10.2a),
hyperintense on FLAIR images (Fig. 10.2b) and iso-hyperintense on T1-weighted images
(Fig. 10.2c, d). The mass causes obstructive hydrocephalus without transependymal
resorption of CSF. These findings are consistent with colloid cyst.
History A 21-year-old woman referred to the emergency department for progressive decreased
Case 3 level of consciousness associated with motor deficit of the right upper and lower
extremities.
Cerebral
Toxoplasmosis
Comments Toxoplasmosis is a zoonotic infection in humans caused by Toxoplasma gondii, a ubiquitous
protozoan that is an obligate intracellular parasite. Cats are the primary hosts, while humans
serve as intermediate hosts. Infection with T. gondii is common among humans, however,
most infections are asymptomatic or associated with self-limited symptoms (e.g., fever,
malaise, lymphadenopathy). Disease typically only becomes apparent in congenitally
a b
c d
Fig. 10.3
Miscellaneous 211
acquired infection and in immunocompromised patients, such as those with acquired

immunodeficiency syndrome (AIDS) and malignancies. Indeed, toxoplasmosis encephalitis is
an opportunistic infection most commonly seen in AIDS patients.
AIDS-related central nervous system (CNS) toxoplasmosis may present with neurologic
deficits in 50–89% of patients, seizures in 15–25% of patients, altered mental status, and, rarely,
signs of raised intracranial pressure. Transplacental infection may occur if the mother acquires
the parasite acutely or if a latent infection is reactivated during immunosuppression. If the
mother was infected prior to pregnancy, there is virtually no risk of fetal infection, as long as she
remains immunocompetent; however, if the infection is acquired during the pregnancy, there is
risk of infection to the fetus. Infection during the first or second trimesters appears to be most
severe. The clinical features of congenitally acquired T. gondii infection include chorioretinitis,
blindness, seizures, microcephaly, anemia, and encephalitis, but the classic triad of congenital
toxoplasmosis is chorioretinitis, hydrocephalus, and intracranial bilateral calcifications.
Cerebral toxoplasmosis lesions are most commonly located in the cerebral hemispheric
white matter and subcortical gray matter, such as thalamus and basal ganglia. CT character-
istically shows single or multiple nodular lesions, with the presence of a moderate amount
of peripheral edema. The lesions appear as relatively isodense, rounded masses although
they can appear hyperdense due to hemorrhagic necrosis. Contrast-enhanced CT reveals
homogeneous nodular or ring-like enhancement. Enhancement may be mild or absent in
individuals with severely diminished cellular immunity. MRI is considered superior to CT
in the detection of brain toxoplasmosis. On MR imaging, lesions are multiple, commonly
located in the deep central nuclei, posterior fossa or lobar at the gray-white matter junction,
with prominent associated mass effect and edema. On T1-weighted images, the lesions are
hypointense to brain tissue. On T2-weighted MRIs, the foci of infection are usually hyperin-
tense, but they can occasionally be isointense to hypointense. Active lesions often are sur-
rounded by edema. After gadolinium administration, focal nodular or ring enhancement
occurs in approximately 70% of patients. Rarely, the lesions are hemorrhagic, and MRI find-
ings then depend on the hemorrhagic stage of evolution. The characteristic sign of CNS
toxoplasmosis is the asymmetric target sign (30% of patients), which represents a ring-
enhancing abscess containing similarly enhancing, eccentric nodules in the abscess cavity.
Currently, recommended drugs in the treatment of toxoplasmosis include a combination
of sulfamerazine, sulfamethazine, and sulfapyrazine. These agents are active against the
protozoan and are synergistic when used in combination.
Axial FLAIR MR image (Fig. 10.3a) shows a large mass located in left basal ganglia, Imaging Findings
surrounded by extensive edema, which produces contralateral displacement of midline
intracranial structures. The lesion seems to present a satellite nodule in left thalamus
(arrow). After gadolinium administration (Fig. 10.3b), an ill-defined predominantly
peripheral enhancement can be seen. On diffusion-weighted MR image (Fig. 10.3c), there
is no diffusion restriction. Perfusion-weighted MRI (Fig. 10.3d) demonstrates a decrease in
cerebral blood volume within the lesion. Findings are consistent with an inflammatory-
infectious process with no diffusion restriction. The patient’s serological analysis for
human immunodeficiency virus (HIV) was positive. She was treated with specific therapy
for toxoplasmosis with favorable response.
Case 4
Herpes Simplex Encephalitis
a b
c d
Fig. 10.4
Miscellaneous 213
A 71-year-old man presented with a 3-day history of temporal-spatial disorientation and History
inability to recognize objects and to express himself. The patient has had a fever for the last
5 days, although on admission he was afebrile.
Herpes simplex encephalitis (HSE) remains one of the most devastating infections of the Comments
CNS, with high morbidity and mortality despite available antiviral therapy. About half of
treated patients die or are left with severe impairment. HSE is divided into two different
entities. First, neonatal HSE, usually caused by herpes virus type 2 (genital herpesvirus),
which is acquired at the time of delivery, with diffuse brain involvement; and second, HSE
occurring in older infants, children and adults, caused by herpes virus type 1 (oral
herpesvirus), with mainly temporal and frontal lobes involvement. HSE represents a
primary herpes simplex virus (HSV) infection in about one-third of the cases, usually
patients younger than 20 years. The remaining cases occur in patients with serologic
evidence of preexisting HSV infection and are due to reactivation of a latent peripheral
infection in the olfactory bulb or trigeminal ganglion, or reactivation of a latent infection
in the brain itself. Brain infection is thought to occur by means of direct neuronal
transmission of the virus from a peripheral site to the brain via the trigeminal or olfactory
nerve. Factors that precipitate HSE are unknown. The mortality rate is high, 50–70%, with
significant long-term morbidity in surviving patients.
Clinically, patients present with altered mental status usually associated with fever,
headache, seizures, psychiatric symptoms, and vomiting. As the presenting symptoms are
similar to other neurological disorders such as tumors, non-HSV encephalitis, and brain
abscess, the definitive diagnosis should be made by the identification of HSV in the cere-
brospinal fluid (CSF) by means of a polymerase chain reaction (PCR) or by the identifica-
tion of HSV on brain biopsy.
In appropriate clinical settings, imaging findings can be highly suggestive of HSE. We
should bear in mind that CT is less sensitive than MRI, with approximately one-third of
patients having unremarkable CT findings at the time of HSE presentation. As the disease
progresses, CT may show low-density lesions in the temporal lobes with mild mass effect. On
contrast-enhanced CT, ill-defined patchy or gyriform enhancement can be seen. MRI is more
sensitive than CT in depicting early changes of HSE. These early findings include gyral edema
on T1-WI and high signal intensity in the temporal lobe or cingulated gyrus on T2-WI. No
evidence of enhancement is detected following gadolinium administration in the early stages.
After 1 to 2 weeks, abnormalities tend to spread with involvement of the contralateral tempo-
ral lobe, insula, and cingulated gyri and contrast enhancement become apparent.
Goals of therapy are to shorten the clinical course of the disease, to prevent complications,
and to prevent recurrences. The treatment of choice for HSE is the antiviral agent acyclovir.
Axial T1-weighted MR image (Fig. 10.4a) shows low signal intensity in both insular areas Imaging Findings
(arrows). Axial T2-weighted MR image (Fig. 10.4b), axial proton-density-weighted image
(Fig. 10.4c), and coronal FLAIR image (Fig. 10.4d) better depicted bilateral involvement of the
temporal lobes, more extensive within the left side. These findings are consistent with HSE.
A 23-year-old man presented to the emergency department with dizziness, right brachio-facial
Case 5 paresis, and altered sensorium. The patient underwent an urgent nonenhanced CT scan (in another
hospital), which was unremarkable. The CSF analysis was consistent with lymphocytic meningitis.
Meningitis
History Meningitis is the most common form of CNS infection. It consists of an inflammation of the
leptomeninges and underlying subarachnoid CSF. Infectious meningitis can be divided into
Comments three categories: acute (mostly bacterial infections), lymphocytic (usually viral), and chronic
(tuberculosis). Acute pyogenic meningitis is usually caused by bacteria. The most common agents
involved depend on age group. Neonatal meningitis is caused by group B streptococcus; in children
a b
c d
Fig. 10.5
Miscellaneous 215
under 7 years of age, Hemophilus influenza is the most frequent infective bacteria. Neisseria
meningitidis is found in children and young adults and Streptococcus pneumonia is the most
common cause of meningitis in adults. Nonepidemic meningitis occurs more frequently in
neonates, infants, and children, whereas epidemic meningitis can occur at any age. In neonates
there is a male gender predilection (ratio male-to-female, 3:1) but in adults gender
predominance does not exist.
Classic symptoms of meningitis include headache, nuchal rigidity (generally not pres-
ent in children <1 year or in patients with altered mental status), fever and chills, photo-
phobia, vomiting not preceded by nausea, prodromal upper respiratory infection symptoms,
seizures, focal neurologic symptoms, and altered sensorium (confusion may be sole pre-
senting complaint, especially in elderly).
The diagnosis is based on clinical manifestations, physical examination, and laboratory
test. Neuroimaging studies are used to rule out the complications of meningitis. Urgent
noncontrast CT scan is usually normal in children with acute bacterial meningitis.
Nevertheless, CT is crucial to identify contraindications of a lumbar puncture and compli-
cations that require prompt neurosurgical intervention such as symptomatic hydrocepha-
lus, subdural empyema, and cerebral abscess. After contrast administration, less than half
of patients with clinically documented meningitis have abnormally enhancing pattern.
MRI is superior to CT scanning in the evaluation of patients with suspected meningitis.
Gadolinium-enhanced MRI is the most sensitive modality for the diagnosis of bacterial
meningitis because it helps to detect the presence and extent of inflammatory changes in
the meninges as well as complications. Nonenhanced MRI of patients with uncomplicated
acute bacterial meningitis may be unremarkable or may demonstrate obliterated cisterns
and the distention of the subarachnoid space with widening of the interhemispheric fis-
sure, which is reported to be an early finding in severe meningitis. T2-weighted images are
sensitive to abnormal tissue water distribution and, thus, may show cortical hyperintensi-
ties that are reversible and believed to represent edema. Gadolinium-enhanced MRI stud-
ies can demonstrate abnormal leptomeningeal enhancement that more closely approximates
the extent of inflammatory cell infiltration. Dural enhancement may occur.
Treatment for meningitis depends on the organism causing the infection, patient’s age,
extent of the infection, and the presence of other medical conditions or complications of
meningitis. Early treatment is crucial to improve prognosis.
A cerebral MRI study was performed 24 h after admission. On axial FLAIR (Fig. 10.5a) and Imaging Findings
coronal T2-weighted (Fig. 10.5b) MR images, high signal intensity in the left Sylvian cortex
was demonstrated. Following gadolinium intravenous injection (Fig. 10.5c, d), a predominantly
leptomeningeal enhancement was found in this area (arrows). The patient’s chest CT scan
demonstrated pleural effusion and multiple small centrilobular nodules consistent with
tuberculosis. Therefore, intracranial findings were consistent with tuberculous meningitis.
Case 6
Neuro-Behçet’s Disease
a b
c d
Fig. 10.6
Miscellaneous 217
A 28-year-old man presented to emergency department with severe headache, blurred History
vision, vomiting, and mild weakness of left upper and lower extremities.
Behçet’s disease (BD) is a vasculitis of unknown cause that presents with multisystemic, Comments
recurrent, inflammatory disorder affecting the eyes, skin and mucosa, joints, vascular system
(mainly the veins), lungs, gastrointestinal tract and nervous system. Its neurologic symptoms,
neuro-Behçet’s disease (NBD), are relatively rare, between 5% and 30%. Both the central and
peripheral nervous systems can be involved although the involvement of the peripheral
nervous system is rare. CNS manifestations can be divided into two groups: (1) parenchymal
involvement, which includes brain stem involvement, hemispheric manifestations, spinal
cord lesions, and meningoencephalitic presentations; (2) nonparenchymal or vascular
involvement, including dural sinus thrombosis, arterial occlusion, and/or aneurysms.
The parenchymal form of NBD is the most common presentation of NBD (80% of cases) and
is characterized by focal or multifocal involvement of the brain parenchyma. This form usually
presents with an attack of hemiparesis, cognitive changes, sphincteric disturbances, and possi-
ble fever in men in their third decade of life. The most common clinical manifestations are
pyramidal tract and brain stem signs. An acute attack may be followed by a relapsing or pro-
gressive course. In the nonparenchymal group, CNS dysfunction is due to involvement of major
vessels (vascular NBD) or rarely aseptic meningitis. Vascular NBD usually presents with acute
neurological attacks. Nonparenchymal NBD has a better prognosis than parenchymal NBD.
MRI is the most sensitive imaging technique for assessing patients with NBD; it shows
focal or multifocal CNS abnormalities in the clinically affected areas. Lesions in parenchy-
mal NBD are usually located in the brain stem, thalami, and basal ganglia. The parenchy-
mal lesions of NBD are hyperintense on T2-weighted images. High-signal intensity lesions
represent demyelination, gliosis or transient inflammation, and secondary edema. Most
lesions are somewhat visible on T1-weighted images, but can be very subtle. Hypointense
lesions on T1-weighted images may be seen in chronic lesions. FLAIR sequences increase
MRI sensitivity for NBD abnormalities, especially juxta-cortical and periventricular lesions.
Some lesions may demonstrate contrast enhancement during the acute or the subacute
phase that resolves in remissions. The most common MRI findings in vascular NBD are
occlusion of the cerebral venous sinuses with or without venous infarcts.
Corticosteroids and adjuvant immunosuppressive therapy are used for parenchymal
manifestations, and corticosteroids and anticoagulants are used for treatment of dural
sinus thrombosis.
Urgent nonenhanced CT scan (Fig. 10.6a) showed a mass in the right basal ganglia Imaging Findings
surrounded by extensive edema with significant mass effect. It compressed the third
ventricle causing obstructive hydrocephalus. An MRI study was performed 24 h later.
Coronal T2-weighted images (Fig. 10.6b) demonstrated extensive edema. After gadolinium
administration (Fig. 10.6c, d), a marked enhancing mass located in the right thalamus and
cerebral peduncle was clearly identified. The patient underwent corticosteroids therapy.
Control MRI was performed (not shown) and shrinkage of the mass was seen. The patient
also presented oral ulcers which helped to establish the diagnosis of BD.
History A 55-year-old female presented with a 1-month history

Case 7 of disorientation and behavioral disturbances. Her past
medical history was remarkable for Sjogren’s syndrome
Progressive Multifocal occasionally treated with corticosteroids.
Leukoencephalopathy
a b
c d
Fig. 10.7
Miscellaneous 219
Progressive multifocal leukoencephalopathy (PML) is a disease of immunocompromised Comments

patients caused by central nervous system infection with JC virus, a ubiquitous human
papovavirus that is typically acquired during childhood and remains latent in the kidneys
and possibly other sites (e.g., mononuclear cells, CNS). The reactivated virus has a tropism
for oligodendrocytes and causes destruction of these cells with demyelination of deep and
superficial white matter regions. The areas involved are typically bilateral but asymmetric.
Cortical gray matter is relatively spared. This infection affects between 1% and 4% of adult
AIDS patients.
Progressive focal neurologic deficit is the clinical hallmark. Weakness and disturbance
of speech are the most common symptoms. Other manifestations include cognitive abnor-
malities, headache, gait disorders, visual impairment, and sensory loss. Most common
physical sign is limb weakness, which occurs in more than 50% of patients. Cognitive dis-
turbances and gait disorders affect 25–33%, and diplopia affects 9%. Optic-nerve disease
does not occur with PML, and spinal-cord involvement is rare.
Radiologic imaging strongly supports the diagnosis of PML in the appropriate clinical
context. CT is often the first neuroimaging technique performed and it usually demonstrates
single or multiple hypodense lesions in the subcortical white matter, with no surrounding
edema; but CT is significantly less sensitive than MRI in the detection of white matter involve-
ment. CT depicts the lesions at an advanced stage. MRI is the technique of choice because it
presents far greater sensitivity than other studies in detecting the lesions of PML and in defin-
ing their extent of involvement. On T2-weighted images, lesions appear hyperintense and
typically involve the periventricular and subcortical white matter. Lesions are more conspicu-
ously detected on FLAIR images, appearing hyperintense against a background of suppressed
CSF signal intensity. On T1-weighted images, lesions are hypointense and well demarcated,
though they may be isointense in the initial phase of the disease. The lesions may occur any-
where, but are most often seen in the parieto-occipital and frontal lobes. After contrast mate-
rial administration, the lesions typically do not enhance and do not have mass effect. Lesions
are initially multiple and discrete, but they eventually may coalesce into large lesions. On dif-
fusion-weighted images, lesions can show restricted diffusion, though this is uncommon.
Definitive diagnosis requires a brain biopsy or detection of JC virus DNA in CSF of
patients with radiographic and clinical findings consistent with PML. There is no specific
therapy for JC virus infection or PML, but the main approach to treatment currently
involves antiretroviral therapy to reverse the immunosuppression interfering with normal
host response to the virus. The median survival of patients with PML as a complication of
AIDS is 6 months.
Urgent nonenhanced CT scan (Fig. 10.7a) revealed low-density lesions in the subcortical Imaging Findings
white matter of both frontal lobes and right temporal lobe. MRI study was performed next.
Axial T1-weighted (Fig. 10.7b), axial T2-weighted (Fig. 10.7c) and sagittal T1-weighted
(Fig. 10.7d) MR images showed bilateral and asymmetric white matter lesions, with no mass
effect, that involved the subcortical U fibers resulting in the typical “scalloped” appearance
(arrows). The imaging findings were consistent with PML. The patient’s serology for HIV
was positive and JC virus DNA was detected by PCR in her cerebrospinal fluid.
Case 8
Intracranial Hypotension Syndrome
a b
c d
Fig. 10.8
Miscellaneous 221
A 37-year-old man presented with a 2-week history of severe holocranial headache with no History
response to common analgesic therapy. The cephalgia was relieved only by assuming a
supine position.
Intracranial hypotension (IH) is a clinical and pathologic entity in which imaging plays a Comments
crucial role. The causes that lead to IH is a CSF leak due to a dural trauma (e.g., diagnostic
lumbar puncture, craniospinal surgery, skull base trauma) or due to the presence of Tarlov’s
cyst and/or arachnoid diverticula. When no cause of CSF leak is found, the condition is
considered as spontaneous intracranial hypotension, which is the most frequent etiology.
The most common clinical presentation is positional, or orthostatic, headaches; patients
develop severe cephalgia on standing, which is relieved only by recumbency. Other symp-
toms that may coexist are nausea, neck stiffness, photophobia, diplopia, facial numbness,
dysguesia, vertigo, and tinnitus.
The imaging technique of choice for the diagnosis of spontaneous IH is MRI of the
brain. CSF volume depletion produces the typical imaging features of IH: diffuse dural
thickening and enhancement, expansion of the epidural venous plexus, downward dis-
placement of the brain with caudal tonsillar herniation, subdural fluid collections, and dis-
tention of the durovenous sinuses. The diffuse smooth thickening of the dura and its
intense contrast enhancement are considered to be the result of venous dilation that fol-
lows the CSF volume decrease produced by the leakage. According to the Monro–Kellie
doctrine, the intracranial volume of CSF is inversely related to the brain blood volume.
Thus, in spontaneous IH a decrease of the CSF volume leads to an increase of the dural
blood volume and venous engorgement. Furthermore, when venous engorgement becomes
chronic, fibrocollagenous proliferation contributes to the dural thickening as well. The MR
signs indicating descent of the brain are the decrease in the size of the ventricles and the
basal cisterns, decrease of the anteroposterior diameter of the prepontine cistern and the
anterior subarachnoid space, and the downward displacement of the cerebellar tonsils.
Subdural fluid collections are seen in 10% of cases and are typically bilateral, thin, and
often without any mass effect.
The majority of patients respond to conservative management with bed rest, hydration,
and oral or intravenous administration of caffeine. Lumbar epidural blood patches may be
tried as treatment in case of the failure of conservative therapy. If the patient fails to respond
to this treatment, then imaging is required to localize the leak for a more direct/closer
blood patch. The method of choice to localize leaks has been the CT myelography technique,
with spinal MRI and radionucleotide cisternography (RC) as guides for targeting the CT
myelography to appropriate levels. The CT myelography procedure has a sensitivity of 67%
compared to 50% and 55% of spinal MRI and RC, respectively.
Axial T1-weighted (Fig. 10.8a) and FLAIR (Fig. 10.8b) MR images demonstrate diffuse Imaging Findings
dural thickening (arrows) associated with a small ventricular system. After gadolinium
administration (Fig. 10.8c, d), diffuse and intense dural enhancement is clearly evident.
These findings are consistent with intracranial hypotension syndrome.
Case 9
Acute Head Trauma
a b
c d
Fig. 10.9
Miscellaneous 223
A 30-year-old man was admitted to the emergency room with head trauma after a motor History
vehicle accident. The patient was alert and oriented but slightly agitated. On physical
examination, bilateral otorrhea and probable left Horner’s syndrome were found. An urgent
un-enhanced CT of brain and skull base was performed. Diffusion-weighted MR imaging
and extracranial MR angiography were carried out next.
Approximately 15% of head trauma patients die and another 15% present chronic Comments
impairment.
Primary and secondary injuries occur in head trauma. The primary injury occurs at the
time of impact, either by a direct injury to the brain parenchyma or by an indirect injury
to the long white matter tracts caused by acceleration-deceleration forces. Specific types of
primary injury include scalp injury, skull fracture, cerebral contusion, subarachnoid hem-
orrhage, epidural hematoma, subdural hematoma, penetrating injuries, and diffuse axonal
injury.
The secondary injury consists of systemic and intracranial events that occur in response
to the primary injury and further contribute to neuronal damage and cell death. Secondary
injuries include brain herniation, cerebral edema, ischemia, cerebral death, extracranial
and intracranial vessel dissection, and carotid-cavernous fistula.
CT scanning is the imaging modality of choice in cases of severe head trauma. CT is
widely available and has excellent sensitivity for the detection of hemorrhage and osseous
involvement. Moreover, only a few minutes are required to perform the entire examination.
MRI is carried out when diffuse axonal injury is suspected because this entity may not
be detected on CT.
CT and MR angiography may be useful to study intracranial or extracranial vessels in
cases of suspected dissection. Digital subtraction angiography is performed in doubtful
cases or for therapeutic purposes.
Urgent un-enhanced CT scan (Fig. 10.9a) shows posttraumatic subarachnoid hemorrhage, Imaging Findings
with blood within the Sylvian fissures (arrow), and air bubbles consistent with
pneumoencephalus in the ventricular system and subarachnoid space (solid arrows).
CT scan of the skull base (Fig. 10.9b, c) demonstrates several fractures involving the
petrous temporal bone (open arrows), paranasal sinuses (solid arrow), and left carotid
channel (arrowhead in Fig. 10.9c).
MR angiography (Fig. 10.9d) shows irregularity and enlargement of the cavernous seg-
ment of left internal carotid artery consistent with posttraumatic pseudoaneurysm
(arrow).
Case 10
Ranula
a b
c d
Fig. 10.10
Miscellaneous 225
A 34-year-old female presented with a painful swelling in the right submandibular region. History
Ultrasound scan showed a cystic lesion. The submaxillary gland was not clearly identified.
Ranula is a mucous retention cyst of the floor of the mouth. The term derives from the Latin Comments
word rana (frog) and describes the bluish translucent swelling in the floor of the mouth that
resembles the underbelly of a frog. Ranulas can be divided into two types: simple ranulas and
plunging ranulas. Simple ranulas are confined to the sublingual space and represent
postinflammatory retention cysts of the sublingual glands or the minor salivary glands with
an epithelial lining. Plunging ranulas are simple ranulas that become large and rupture out the
back of the sublingual space and extend into one or more adjacent spaces such as submandibular
or parapharyngeal space. Plunging ranulas are pseudocysts because they lack the epithelial
lining. Ranulas are rare. The majority of them are unilateral and they do not demonstrate side
preference. They show slight female gender predominance and present most commonly in the
second and third decades of life (median age at presentation is 30 years).
Clinically, simple ranulas manifest as a painless swelling along the floor of mouth. If they
become larger they may interfere with speech, mastication, and swallowing because of the
supero-medial displacement of the tongue. In contrast, plunging ranulas present as a grad-
ually enlarging and painless mass in the neck with or without swelling in the mouth.
On ultrasound, ranulas appear as a thin-walled unilocular cystic mass. On CT scan they
are seen as a well-circumscribed non-enhancing fluid-attenuation mass. If the cyst has
been infected the contents may demonstrate increased attenuation, and occasionally may
show thin rim enhancement. MR imaging plays an important role in providing precise
information regarding the margins and extent of the lesion thereby assisting the operating
surgeons in better planning the surgical approach. On MRI, ranulas appear as well-defined,
homogeneous masses with low signal intensity on T1-weighted images and markedly high
signal intensity on T2-WI. After gadolinium administration, the thin wall of the cyst
enhances, even if it is uninfected.
The differential diagnosis includes dermoid, epidermoid-cyst, cystic hygroma, cervical
abscess, thyroglossal duct cyst, and second brachial cleft cyst. These entities present differ-
ent imaging findings on CT and MRI from a plunging ranula. Dermoid cyst demonstrates
intralesional fat. Cystic hygroma characteristically presents early in life (before 2 years of
age). Cervical abscess is associated with extensive fat stranding. Thyroglossal duct cyst is
usually midline, while the second brachial cleft cyst is classically located along anterior
border of sternocleidomastoid muscle.
Complete surgical excision of the ipsilateral sublingual gland and pseudocyst is
considered the therapy of choice. The removal of the lesion is performed via an oral route
in cases of simple ranulas and via a cervical approach in cases of plunging ranulas.
Axial (Fig. 10.10a, b), sagittal (Fig. 10.10c), and coronal (Fig. 10.10d) contrast-enhanced CT Imaging Findings
scan of the neck showed a cystic lesion adjacent to the right submaxillary gland causing
hyoglossus and mylohyoid muscle compression. These findings are consistent with a
plunging ranula. Some perilesional reactive lymphadenopathies (arrow in Fig. 10.10a) can
be seen as well.
Further Reading Macdonald AJ, Salzman KL, Harnsberger HR (2003) Giant ran-
ula of the neck: differentiation from cystic hygroma. AJNR
Afshin Borhani H (2005) Neuro-Behcet disease: a review. Am J Neuroradiol 24:757–761
Neurologist 11(2):80–89 Mandel L, Miremadi R (1997) CT scanning of the plunging ran-
Akman-Demir G, Serdaroglu P, Tasçi B, Neuro-Behçet Study ula. Case report. N Y State Dent J 63:38–42
Group (1999) Clinical patterns of neurological involvement Mann K, Jackson MA (2008) Meningitis. Pediatr Rev 29(12):417–
in Behçet’s disease: evaluation of 200 patients. Brain 429; quiz 430
122(11):2171–2182 Mathiesen T, Grane P, Lindgren L, Lindquist C (1997) Third ven-
Al-Araji A, Kidd DP (2009) Neuro-Behçet’s disease: epidemiol- tricle colloid cysts: a consecutive 12-year series. J Neurosurg
ogy, clinical characteristics, and management. Lancet Neurol 86(1):5–12
8(2):192–204 Mirsattari S, McGinn G, Halliday W (2004) Neuro-Behcet dis-
Armao D, Castillo M, Chen H, Kwock L (2000) Colloid cyst of the ease with predominant involvement of the brainstem.
third ventricle: imaging-pathologic correlation. AJNR Am J Neurology 63:382–384
Neuroradiol 21:1470–1477 Morton RP, Ahmad Z, Jain P (2010) Plunging ranula: Congenital
Arora A, Gupta R, Mahajan H (2010) A case of plunging ranula. or acquired? Otolaryngol Head Neck Surg 142:104–107
http://www.eurorad.org/case.php?id=8474. doi:10.1594/ Ohta K, Obara K, Sakauchi M et al (2001) Lesion extension
EURORAD/CASE.8474. Accessed July 7 2010 detected by diffusion-weighted magnetic resonance imaging
Balakrishnan J, Becker PS, Kumar AJ (1990) Acquired immuno- in progressive multifocal leukoencephalopathy. J Neurol
deficiency syndrome: correlation of radiologic and patho- 248(9):809–811
logic findings in the brain. Radiographics 10(2):201–215 Osborn A (1994a) Diagnostic neuroradiology. Mosby, London,
Benito-León J, Reina MA, Alvarez-Linera J (2001) Intracranial pp 639–642
hypotension syndrome. Neurologia 16(9):418–426 Osborn AG (1994b) Diagnostic neuroradiology. Mosby, London,
Bilginer B, Onal MB, Oguz KK, Akalan N (2009) Arachnoid cyst p 700
associated with subdural hematoma: report of three cases Pope T (2003) Aunt Minnie’s atlas imaging-specific diagnosis,
and review of the literature. Childs Nerv Syst 25(1):119–124 2nd edn. Lippincott Williams and Wilkins, Philadelphia
Brant WE, Helms CA (1999) Fundamentals of diagnostic radiol- Pritz T. Herpes simplex encephalitis in Emergency Medicine
ogy, 2nd edn. Lippincott Williams and Wilkins, Philadelphia Workup. E-medicine. Medscape reference
Chang KH, Han MH (1998) MRI of CNS parasitic diseases. Ramsey RG, Gean AD (1997) Neuroimaging of AIDS. I. Central
J Magn Reson Imaging 8(2):297–307 nervous system toxoplasmosis. Neuroimaging Clin N Am
Chang L et al (1995) Radiologic-pathologic correlation cere- 7(2):171–186
bral toxoplasmosis and lymphoma in AIDS. AJNR Am Spelle L, Boulin A, Tainturier C et al (2001) Neuroimaging features
J Neuroradiol 16:1653–1663 of spontaneous intracranial hypotension. Neuroradiology
Demaerel P et al (1992) MRI of herpes simplex encephalitis. 43:622–627
Neuroradiology 34(6):490–493 Thurnher MM, Post MJ, Rieger A et al (2001) Initial and follow-up
El Khoury C, Brugiéres P, Decq P et al (2000) Colloid cysts of the MR imaging findings in AIDS-related progressive multifocal
third ventricle: Are MR imaging patterns predictive of diffi- leukoencephalopathy treated with highly active antiretroviral
culty with percutaneous treatment? AJNR Am J Neuroradiol therapy. AJNR Am J Neuroradiol 22(5):977–984
21:489–492 van de Beek D, de Gans J, Tunkel AR, Wijdicks EF (2006)
Harnsberger et al (2002) Head and neck. Top 100 diagnosis. Community-acquired bacterial meningitis in adults. N Engl J
Pocket radiologist. Amirsys: 214–216 Med 354(1):44–53
Koelfen W et al (1996) MRI of encephalitis in children: compari- Van Tassel P, Cure JK (1995) Nonneoplastic intracranial cysts
son of CT and MRI in the acute stage with long-term follow- and cystic lesions. Semin Ultrasound CT MR 16(3):186–211
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Kurabayashi T, Ida M, Yasumoto M et al (2000) MRI of ranulas.
Neuroradiology 42:917–922

Learning Neuroimaging - 100 Essential Cases

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Learning Neuroimaging - 100 Essential Cases

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Learning Imaging

ISBN: 978-3-642-22998-5 e-ISBN: 978-3-642-22999-2

Springer Heidelberg Dordrecht London New York

Library of Congress Control Number: 2011940861

Printed on acid-free paper

Springer is part of Springer Science+Business Media (www.springer.com)

To my family, especially to Juan,

To my parents, Luiz Celso and Leonice

To Simone, incomparable woman,

Francisco de Asís Bravo-Rodríguez

Luiz Celso Hygino da Cruz jr.

2 Orbit and Sellar Region

3 Ear, Nasal, and Paranasal Sinuses

8 Diﬀusion and Spectroscopy

9 DTI and Bold MR Imaging

Antonio Cano Sanchez Isabela Garcia Vieira

Fernando Delgado Acosta Manuel J. Ramos Gómez

Rafael Ferracini Cabral Raquel Ribeiro Batista

Elisa Roldan Romero

Case 2 Anaplastic Astrocytoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4

Case 5 Dysembryoplastic Neuroepithelial Tumor (DNET). . . . . . . . . . . . . . . 10

Case 6 Intracranial Ependymoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12

Case 9 Pleomorphic Xanthoastrocytoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 18

F. de Asís Bravo-Rodríguez et al. (eds.), Learning Neuroimaging, Learning Imaging,

History A 56-year-old man was referred to a neurologist for

Dysembryoplastic Neuroepithelial Tumor (DNET)

1. The complex form is characterized by the association of a specific glioneuronal element

Neuroimaging studies usually show a predominantly cortical and well-deﬁned lesion

History A 27-year-old man presented with a 2-week history of

History A 37-year-old woman, with a several-month history of

History A 22-year-old woman presented with 1-month history

Medulloblastoma is an infratentorial primitive neuroectodermal tumor (PNET) that occurs Comments

History A 43-year-old woman presented with three limited episodes of

Further Reading Karremann M, Pietsch T, Janssen G, Kramm CM, Wolff JE (2009)

Case 2 Grave’s Ophthalmopathy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26

Case 3 Inflammatory Pseudotumor of the Orbit . . . . . . . . . . . . . . . . . . . . . . . 28

Case 4 Orbital Hemangioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30

Case 5 Orbital Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32

Case 6 Optic Nerve Sheath Meningioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34

Case 8 Hypothalamic Hamartoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38

Case 9 Pituitary Adenoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40

Case 10 Rathke’s Cleft Cyst. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42

F. de Asís Bravo-Rodríguez et al. (eds.), Learning Neuroimaging, Learning Imaging,

History A 57-year-old man presented with bilateral exophthalmos

Grave’s ophthalmopathy (GO) is a thyroid-associated orbitopathy that represents a part of Comments

History A 28-year-old woman presented with a mass located in

History A 40-year-old female presented with progressive visual

Craniopharyngioma is a benign, slow-growing, extra-axial, epithelial, calciﬁed cystic tumor Comments

Rathke’s Cleft Cyst

Further Reading Lee EJ et al (2005) MR Imaging of orbital inﬂammatory pseudotu-

Case 2 Inverted Papilloma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 48

Case 3 Nasosinusal Polyposis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 50

Case 4 Sinus Mucocele . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 52

Case 6 Rhinocerebral Mucormycosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 56

Case 7 Sinonasal Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 58

Case 8 Acoustic Neuroma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 60

Case 10 Jugulotympanic Glomus Tumor . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 64

F. de Asís Bravo-Rodríguez et al. (eds.), Learning Neuroimaging, Learning Imaging,

History A 48-year-old male presented with a 3-year history of

Comments Inverted papilloma is a rare type of tumor that constitutes

or paranasal sinus mucosa with a characteristic histological feature: The epithelium

History An 81-year-old woman presented with a tumor located

A 32-year-old female presented with a several-month history of progressive right History