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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October 18, 2010
Susac's Syndrome
- Susac's syndrome is a self-limited syndrome, likely autoimmune, characterized by microangiopathy.
- Most often, women in the 20-40 year age group are affected. Patients present with a clinical triad of encephalopathy, hearing loss and vision distortion.
- If one of the features from the clinical triad is missing, it can be difficult to diagnose this entity. MRI then plays a critical role to establish the diagnosis.
- Key Diagnostic Features: Small infarctions involving the corpus callosum. These infarctions are typically located centrally within the corpus callosum. Involvement of callososeptal interface is less common. There is no volume loss of the corpus callosum. Additional imaging features include foci of bright signal intensity lesions on FLAIR and T2WI involving the periventricular white matter, deep gray matter, and posterior fossa. Leptomeningeal enhancement is often seen.
- Fluoroscein angiogram shows features consistent with microangiopathy.
- DDx includes Multiple sclerosis and ADEM.
- Rx involves use of immunosuppressive and antithrombotic agents.