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Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a rare autosomal dominant inherited disorder caused by a mutation on the notch3 gene. The disease usually begins during the third decade with migraines, stroke events, and is possibly associated with psychiatric disturbances, and early subcortical dementia.1 Phenotypic variants have been reported in families with dementia, psychiatric disorders, or seizures as the predominant symptoms. We report here the unusual presentation of two relatives with confusional episodes and coma.
Patients and methods.
Patient 1. The proband was a 50-year-old woman. Since age 15, she had occasional migraine headaches. The aura started with hemianopsia, followed after 15 minutes by right facial paresthesia, then occasionally followed by aphasia. The aura completely subsided within 40 minutes before the onset of the throbbing headache. At age 50, she was hospitalized for a confusional episode. She was agitated and disorientated with visual hallucinations, behavioral and memory disturbances, and …
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