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Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy without anterior temporal pole involvement: a case report.

Abstract
The location of white matter lesions, especially in the anterior temporal poles (ATP), is helpful in the diagnosis of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). We report a 49-year-old man with CADASIL who developed migraine with atypical aura, silent lacunar infarcts, and leukoencephalopathy without involvement of the ATP. The prevalence of migraine with aura in subjects with CADASIL is several times greater than that in the general population. Particularly in patients with CADASIL, the aura is often atypical (hemiplegic, basilar, or prolonged). A diagnosis of CADASIL should be considered in patients with lacunar infarcts, leukoencephalopathy, and migraine with atypical aura, even in the absence of white matter lesion in the ATPs.
AuthorsJunpei Kobayashi, Shoichiro Sato, Kosuke Okumura, Fumio Miyashita, Akihiko Ueda, Yukio Ando, Kazunori Toyoda
JournalJournal of stroke and cerebrovascular diseases : the official journal of National Stroke Association (J Stroke Cerebrovasc Dis) Vol. 23 Issue 3 Pg. e241-2 (Mar 2014) ISSN: 1532-8511 [Electronic] United States
PMID24295602 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
CopyrightCopyright © 2014 National Stroke Association. Published by Elsevier Inc. All rights reserved.
Chemical References
  • NOTCH3 protein, human
  • Receptor, Notch3
  • Receptors, Notch
Topics
  • Brain (pathology)
  • CADASIL (complications, diagnosis, genetics, pathology)
  • DNA Mutational Analysis
  • Genetic Predisposition to Disease
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Migraine with Aura (complications)
  • Phenotype
  • Point Mutation
  • Receptor, Notch3
  • Receptors, Notch (genetics)

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