A. Malandrini et al., UNUSUAL CLINICAL-FEATURES AND EARLY BRAIN MRI LESIONS IN A FAMILY WITH CEREBRAL AUTOSOMAL-DOMINANT ARTERIOPATHY, Neurology, 48(5), 1997, pp. 1200-1203
Cerebral autosomal dominant arteriopathy with subcortical infarcts and
leukoencephalopathy (CADASIL) is a recently described inherited disor
der. The pathologic gene maps on chromosome 19. The clinical spectrum
of the disease consists of recurrent strokes, migraine, transient isch
emic attacks, mood changes, and dementia. We report a genetically asse
ssed CADASIL family with atypical clinical presentations of epileptic
seizures, In two asymptomatic family members there were early brain ab
normalities on MRI. Our report expands the clinical spectrum of CADASI
L and suggests that it is possibly an undiagnosed disorder.