title: [CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy)].
abstract: Recently identified, CADASIL is a diffuse disease of small arteries, predominating in the brain. It starts during mid-adulthood and is characterized by recurrent ischemic events (transient or permanent), attacks of migraine with aura, severe mood disorders, subcortical dementia and, at MRI, a white spread leukoencephalopathy. There is so far no specific treatment and death occurs after a mean of twenty years. CADASIL is an autosomal dominant condition and the gene Notch 3 is located on chromosome 19, in the same region as another neurological disorder, familial hemiplegic migraine.
authors.affiliation: Service de Neurologie, Hôpital Saint-Antoine, Paris.
authors.forename: H
authors.initials: H
authors.lastname: Chabriat
authors.forename: A
authors.initials: A
authors.lastname: Joutel
authors.forename: K
authors.initials: K
authors.lastname: Vahedi
authors.forename: M T
authors.initials: MT
authors.lastname: Iba-Zizen
authors.forename: E
authors.initials: E
authors.lastname: Tournier-Lasserve
authors.forename: M G
authors.initials: MG
authors.lastname: Bousser
mesh_terms: D002545:Brain Ischemia / Q000235:genetics / Q000473:pathology; D002539:Cerebral Arterial Diseases / Q000235:genetics* / Q000473:pathology; D015161:Dementia, Multi-Infarct / Q000235:genetics* / Q000473:pathology; D005799:Genes, Dominant; D006801:Humans; D007968:Leukoencephalopathy, Progressive Multifocal / Q000235:genetics* / Q000473:pathology; D008279:Magnetic Resonance Imaging