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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November 15, 2010
CADASIL
- Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary small vessel vasculopathy.
- Patients present with mood disturbances, migraine with aura, depression, strokes, and progressive subcortical dementia.
- Key Diagnostic Features: Confluent, symmetric foci of signal abnormality involving the periventricular white matter, best appreciated on FLAIR are noted. Temporal lobe involvement is highly suggestive of this disease entity. Typically, there is relative sparing of the corpus callosum and infratentorial brain.
- DDx: Binswanger's disease, encephalitis