Radiology & Imaging collections: 12/01/2015

Susac's Syndrome

32-year-old woman with confusion, vision distortion, scotoma and hearing loss.

Susac's syndrome is a self-limited syndrome, likely autoimmune, characterized by microangiopathy.

Fabry's Disease

47-year-old man with history of seizure, peripheral neuropathy, skin lesion, visual changes, cardiac murmur and proteinuria.

Fabry's disease is a multisystem X-linked disorder of glycosphingolipid metabolism with deficient activity of α-Galactosidase A, leading to progressive accumulation of glycosphingolipid in vascular endothelium, perithelium, smooth muscle cells, brain, peripheral nerves, heart, skin and kidneys.

Ventriculus Terminalis

Ventriculus terminalis (VT), also known as the "fifth ventricle", is an ependymal lined CSF-filled cavity within the conus medullaris.

Hypertrophic Olivary Degeneration

38-year-old man with headache and dizziness.

Hypertrophic olivary degeneration is a unique type of transsynaptic degeneration resulting in hypertrophy of degenerated inferior olivary neurons rather than atrophy.
Most commonly occurs following focal lesions to the brainstem, such as hemorrhage, which in this case was related to a cavernous hemangioma.

Band Heterotopia

25-year-old woman with seizures.

Heterotopia is the name given to focal or diffuse collections of ectopic neurons in the cerebral hemispheres, a neuronal migrational anomaly. It can be broadly classified into the subependymal, subcortical and band types of heterotopias.
In general, band heterotopia is seen largely in women. Men with a mutation of the related gene (called XLIS or DCX) usually die in utero or have a much more severe brain anomaly.
On MRI, it shows the characteristic 3-layers (double cortex), with a thin layer of white matter between the cortex and the heterotopic band. The cortex may be relatively normal or pachygyric.

"White" Dermoid

32-year-old man with headaches for 3 months. Intracranial dermoids are rare congenital lesions.

Dermoids contain skin adnexal structures (hair follicles, sebaceous and sweat glands).

Parry-Romberg Syndrome

5-year-old with facial asymmetry.

Parry-Romberg syndrome, also known as progressive facial hemiatrophy, is a rare sporadic disease characterized by unilateral wasting of facial skin and subcutaneous tissue with variable involvement of muscle, cartilage and bone.

Wernicke's Encephalopathy

Wernicke encephalopathy (WE) is an uncommon neurologic disorder characterized by changes in consciousness, ocular dysfunction and ataxia.
WE results from deficiency of vitamin B1 (thiamine) in patients with history of ethanol abuse, and in non-alcoholic patients the most frequent cause is intestinal malabsorption.

Reversible Cerebral Vasoconstriction Syndrome

Middle-aged women with thunderclap headache.
Causes of this syndrome: postpartum, exposure to vasoactive substances, catecholamine-secreting tumors, exposure to immunosuppressants or blood products, and miscellaneous.

Remote Cerebellar Hemorrhage

Remote cerebellar hemorrhage is a usually self-limiting complication of spinal and supratentorial surgery.
The streaky bleeding pattern in the sulci of one or both upper cerebellar hemispheres, also referred to as the "zebra-sign," sometimes extends into the cerebellar parenchyma

Pott's Puffy Tumor

3-year-old boy with progressive frontal scalp swelling, headache and seizures.

Pott's puffy tumor is defined as scalp swelling due to subperiosteal abscess resulting from osteomyelitis of the frontal bone secondary to bacterial sinusitis.