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Neuroradiology Cases 1-25. Case directory. Case 1. Craniopharyngioma. Case findings: T2 and T1 weighted (pre-Gd): demonstrate a heterogeneous primarily cystic appearing mass in the suprasellar cistern Focal regions of decreased signal intensity, which may be secondary to calcifications
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Craniopharyngioma • Case findings: • T2 and T1 weighted (pre-Gd): demonstrate a heterogeneous primarily cystic appearing mass in the suprasellar cistern • Focal regions of decreased signal intensity, which may be secondary to calcifications • Post-Gd T1 weighted axial, sagittal, coronal image: extensive, primarily peripheral enhancement with intermixed regions of increased and decreased signal • Central region of signal void consistent with calcification • Cystic regions within high SI on T1 high protein contrast • Obstructive hydrocephalus
Craniopharyngioma • Arise from neuroepithelial rests within Rathke's cleft • Bimodal age distribution: MC pediatric age group, 2nd peak in 5th decade • Present with headache from obstructive hydrocephalus • Extremely heterogenous composed of intermixed cystic and solid components • Intrasellar with suprasellar extension • Calcifications (MC in pediatrics group) • DDX: • Germ cell tumor • Optic glioma • Epidermoid • Meningioma • Pituitary (macro)adenoma
Case directory Parasellar Masses • DDX “SATCHMO”: • Sphenoid sinus tumor, sarcoidois • Aneurysm, pituitary (macro)adenoma • Teratoma • Craniopharyngioma • Hypothalamic glioma, Langerhans cell histiocytoma (EG) • Meningioma, metastasis • Optic glioma • LC lesions: • Germinoma • Epidermoid • Hamartoma (hypothalamic, tuber cinereum) • Chordoma • Arachnoid cyst • Rathke’s cleft cyst
Diastematomyelia • Splitting of notochord during early development • Complete, longitudinal division of the spinal cord (MC upper LS) • May be bony or fibrous band between the two hemicords • Each hemicord gives off nerve roots from its lateral aspect and each has a separate anterior spinal artery • Associated with: • Myelomeningocele • Neurogenic and dermoid cysts • Teratoma • Lipoma • DDX: • Diplomyelia: true duplication of the spinal cord, rare
Case directory Diastematomyelia
Case directory Septo-optic dysplasia(de Morsier Syndrome) • Case findings: • Absence of septum pellucidum • Coronal T1: hypoplasia of the optic chiasm • Spectrum of holoprosencephaly: • Alobar holoprosencephaly • Semilobar holoprosencephaly • Lobar holoprosencephaly • Septo-optic dysplasia (most minor form)
Ependymoma • CT: • Obstructive hydrocephalus • Obliteration of the 4th ventricle • Internal calcifications • MRI: • Presence of flow voids • Mass extends laterally through the right foramen of Luschka • Heterogeneous enhancement • Marked mass effect on medulla and complete compromise of foramen magnum
Ependymoma • MC occur in 4th ventricle (arise from ependymal cells) • MC present in children < 5 years old • Slow growing, lobulated tumor that fills the ventricle and can extend out the exiting foramina (“plastic” lesion) • Calcifications (50%) • May demonstrate a large cystic component • Inhomogeneous enhancement • 3rd MC pediatric brain tumor (1st JPA, 2nd medulloblastoma)
Case directory Ependymoma
Agenesis of the corpus callosum • Findings: • High-riding 3rd ventricle • Frontal horns C-shaped on coronal view • Colpocephaly: dilated occipital horns • Parallel orientation of lateral ventricles: median bundles of Probst do not cross hemispheres • Associated with: • Chiari II malformation • Cephaloceles • Dandy-Walker malformation • CNS lipoma
Case directory Agenesis of the corpus callosum Frontal horns C-shaped
Case directory Vein of Galen malformation • Case findings: • Prominent dilatation of Vein of Galen, straight sinus, confluence of sinuses, transverse sinuses, and sigmoid sinus • Turbulent flow within the malformed vessels produces a mixed signal intensity • MRA: prominent Vein of Galen with multiple arteries leading to the malformation • Type 1 (this case): • Due to single or multiple arteries (usually choroidal or quadrigeminal arteries) flowing directly into the vein of Galen, resulting in a shunt that leads to high-flow CHF • Type 2: • Due to a parenchymal AVM in the thalamus or midbrain, which can present as developmental delay with ocular symptoms
Case directory Pituitary microadenoma • Clinical presentation: hormonal changes
Case 8 Axial T2
Coronal T1 post-Gd Coronal T1
Case directory Rathke's cleft cyst • Case findings: • Axial T2: homogeneously increased SI within the sella turcica • Coronal T1: decreased SI at the anterior aspect of the sella • Coronal T1 post-Gd: lesion does NOT enhance lesion does not appear to be a part of the adenohypophysis or neurohypophysis • Non-neoplastic, derived from embryonic remnants of Rathke's pouch • Variable T1: due to variable protein content and/or hemorrhage
Case 9 Axial T1 post-Gd
Pineoblastoma (PNET) • Contains peripheral calcifications • In contrast,germinoma (central) or teratoma (dispersed) calcifications • Dense enhancement, subarachnoid spread • MC in children and young adults • Clinical: • Parinaud’s syndrome: inability to gaze upward due to compression of the tectal plate • Hydrocephalus due to compression of the cerebral aqueduct
Pineal region mass • MC germ cell tumor (50%): • MC germinoma • LC teratoma, embryonal cell carcinoma, choriocarcinoma, endodermal sinus (yolk sac) tumor • Pineal cell tumor (25%): • Pineoblastoma (child) • Pineocytoma (adult) • LC meningioma, metastases, arachnoid cyst, epidermoid, dermoid
Case directory Intracranial germinoma • Pineal germinoma: • Hydrocephalus due to obstruction of the Sylvain aqueduct • Parinaud’s syndrome: failure of upward gaze from compression of the superior colliculi • Suprasellar germinoma: • Diabetes insipidus due to involvement of pituitary infundibulum • Visual disturbances from invasion of the optic chiasm • Precocious puberty if a ß-HCG secreting tumor • Hydrocephalus
Trigonocephaly • Case findings: • Abnormal “wedge shaped” or “triangular” appearance to the frontal bone of the skull • Premature closure of the metopic suture
Craniosynostosis • Scaphocephaly (dolichocephaly): premature closure of the sagittal suture long, narrow skull that looks "boat shaped" in appearance • Brachycephaly: premature close of the coronal suture harlequin eyes (orbital enlargement due to coronal sutural synostosis) • Plagiocephaly: isolated unilateral closure of a coronal or lamboid suture produces an asymmetrically shaped skull • Oxycephaly (turricephaly): vertical development of the skull from premature closure of coronal and sagittal sutures • Kleeblattschadel (cloverleaf skull): premature synostosis of multiple sutures (coronal, lambdoid, sagittal) • Associated with: thanatophoric dwarfism, Pfeiffer’s syndrome
Case directory Plagiocephaly with harlequin eye • Unilateral coronal suture synostosis • Results in an elevated sphenoid wing and distorted orbital shape
Case directory Cavernoma (cavernous angioma, cavernous malformation) • Case findings: • Axial T1 and T2 precontrast and post contrast axial and sagittal T1 images • Precontrast T1: mass in pons which is heterogeneously hyperintense • T2: areas of hypointensity within the mass mixed with hyperintensity • No enhancement • Vascular malformation consisting of endothelial lined sinusoidal spaces without normal intervening brain • Angiographic occult lesion • Popcorn-like lesions that have mixed signal intensity due to hemorrhage in different stages of evolution
Case directory Chiari II malformation • Case findings: • Herniation of cerebellar tonsils • Small posterior fossa • 4th ventricle displaced caudally and elongated • Tectal beaking • Hydrocephalus • Other findings (not shown): • Varying degrees of cerebellar dysplasia • Cervical medullary junction may be kinked • Interdigitation of gyri due to a hypoplastic falx or fenestrated falx • Agenesis of the corpus callosum • Associated with: myelomeningocele (MC in the lumbar spine)
Case directory Bilateral closed-lip schizencephaly • GM-lined clefts that extend through the entire hemisphere from the ependymal lining of the lateral ventricles to the pial covering of the cortex • Types: closed and open lip • Associated with absent cavum septum pellucidum
Case directory Juvenile pilocytic astrocytoma (JPA) • Case findings: • Low T1 and high T2 cystic mass in the right cerebellar hemisphere, with a moderate amount of mass effect on the 4th ventricle and cerebellum • Homogeneous enhancement of a mural nodule in the superior aspect of the mass • MC infratentorial neoplasm in pediatric age group • DDX: • Child: medulloblastoma, ependymoma • Adult: hemangioblastoma (flow voids due to hypervascularity)
Herpes encephalitis • Case findings: • Region of edema and abnormal signal in right insula and temporal lobe • No abnormal enhancement • MC affects mesotemporal lobe • Extends into the insular cortex with sparing of the putamen • DDX: • Infarct • Infiltrating glioma (low-grade)