Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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April 9, 2015
Susac Syndrome
- Background: Susac syndrome, or retinocochleocerebral vasculopathy, is a microangiopathy of unknown cause affecting the arterioles of the brain, retina, and cochlea.
- Clinical Information: Typically, it affects young, healthy women between 18 and 40 years of age. No familial cases have been reported. Pathognomonic but not always a complete triad of encephalopathy, branch retinal artery occlusions, and hearing loss.
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- “Wheel spokes” or “snowballs” lesions of the corpus callosum.
- Involvement of the deep gray nuclei is more variable but, when associated, highly specific.
- Confounding supratentorial small white matter T2 hypersignals are often present.
- Recently, the "string of pearls" in the posterior limb of the internal capsule has been decribed in DWI Also, ill-defined leptomeningeal enhancement in the cerebellum can occadionally be seen.
- DDx:
- Multiple sclerosis
- Acute disseminated encephalomyelitis
- Primary or secondary CNS angeitis
- Treatment Options: Immunomodulation and/or anticoagulation or antiaggregation. Because of the rarity of the disease and its tendency to improve spontaneously, the efficacy of the different treatments is uncertain.
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