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Multiple Lacunar State - Case 3 (CADASIL)

A 46 year-old man presented with an acute right hemiparesis. His father had died of a stroke in his late forties. The patient's younger brother had a history of two ischemic infarcts.

Show the Acute Infarctions     Show All the Infarctions

Multiple Lacunar State (CADASIL): (Top) Diffusion-weighted axial MRIs; (Bottom) T2-weighted axial MRIs. Note the acute infarcts in the deep white matter in the left hemisphere on the diffusion-weighted images. On the Flair images, more widespread chronic ischemic changes are seen bilaterally.

This patient's strokes were due to a rare condition, Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). CADASIL is a familial non-atherosclerotic, non-amyloid arteriopathy which presents with recurrent subcortical ischemic strokes, beginning in middle age. Common symptoms include recurrent migraine headaches, stroke-like episodes, personality and mood changes, cognitive and behavioral disturbances, and dementia. The disease is caused by mutations in the NOTCH 3 gene on chromosome 19q.

Revised 11/30/06
Copyrighted 2006. David C Preston