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Past Issue:
Volume 19, Number 4 • October 2006
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Worsening neurologic deficits in a pilot

Nathaniel R. Jewell, MD, Gary L. Tunell, MD, John P. Bouffard, MD, and Michael J. Opatowsky, MD

A 58-year-old Caucasian man who worked as a pilot presented with symptoms of left-sided facial weakness, dysarthria, and incoordination of the left upper extremity. The patient reported a history of previous transient vision loss 9 years earlier, from which he recovered. He had no vascular risk factors based on his medical history, but his mother and many of his maternal relatives had suffered from strokelike symptoms. His mother and brother both died in their 50s. Laboratory studies during admission did not reveal a hypercoagulable state. Magnetic resonance (MR) imaging of the brain was performed (Figures 1-5).

What are the differential diagnostic considerations? What is the most likely diagnosis? What tests can confirm the diagnosis?

DIAGNOSIS: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).