[CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy)]

  • Chabriat H
  • Joutel A
  • Vahedi K
 et al. 
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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.

Author-supplied keywords

  • *Genes, Dominant
  • Brain Ischemia/genetics/pathology
  • Cerebral Arterial Diseases/*genetics/pathology
  • Dementia, Multi-Infarct/*genetics/pathology
  • English Abstract
  • Humans
  • Leukoencephalopathy, Progressive Multifocal/*genet
  • Magnetic Resonance Imaging

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  • H Chabriat

  • A Joutel

  • K Vahedi

  • M T Iba-Zizen

  • E Tournier-Lasserve

  • M G Bousser

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