[CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy)].
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.
Chabriat
H
H
Service de Neurologie, Hôpital Saint-Antoine, Paris.
Joutel
A
A
Vahedi
K
K
Iba-Zizen
M T
MT
Tournier-Lasserve
E
E
Bousser
M G
MG
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