Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy
(CADASIL) is an adult-onset, autosomal dominantly inherited
small-vessel disease of the brain caused by NOTCH3 mutations. It is characterized
by migraine with aura, mood disorder, recurrent subcortical infarctions, and
dementia. We report a patient presenting sudden amnesia due to acute multiple
cerebral infarctions as the first manifestation of CADASIL. Acute multiple subcortical
infarctions were rarely reported in CADASIL. Acute multifocal infarction
in subcortical area of CADASIL might be the severe disease presentation of
cerebral small vessel disease. So, it should be differentiated with cardiogenic or
artery-to-artery embolic infarctions that usually involved both cortical and subcortical
areas.