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Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a rare, progressive, familial vasculopathy associated with mutations in the NOTCH3 gene, a transmembrane protein expressed in vascular smooth muscle cells.1 Although clinical symptoms are primarily neurologic, abnormalities are found in the systemic and cerebral vasculature.2 Migraines in people with CADASIL may begin as early as age 10 years,3 but TIAs and strokes are rare before age 20 years.3,4⇓ We describe a girl aged 14 years with migraines, hemiplegia, hypertension, and NOTCH3 mutation but unremarkable cerebral MRI and no family history of CADASIL.
Case report.
A right-handed girl aged 14 years came to our clinic with a 3-year history of severe headaches, three episodes of right hemiparesis (at ages 11, 12, and 14 years), persistent hypertension, and a 4.5-kg weight loss. Each episode began with sleepiness, weakness, and headache with photophobia, progressing to tingling of the right face and right …
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