Abstract:
:Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a rare hereditary disease characterized by recurrent transient ischemic attacks, strokes, and vascular dementia. Various mutations in the Notch3 gene cause the disease, whereas the mechanism of how they cause the disorder remains unknown. We recently identified two Japanese CADASIL families with an R141C mutation. The mean age of onset was 44.6, and the main symptoms were recurrent strokes and progressive motor disturbances in extremities, as well as pseudobulbar palsy. Besides those in white matter and basal ganglia, ischemic lesions in temporal edge and corpus callosum were present on magnetic resonance images, which seemed to be characteristic of CADASIL. Moreover, in our cases, nocturnal arterial blood pressure fall was significantly lower in patients compared with control subjects, suggesting that it might be partly associated with ischemic lesions in deep white matter in CADASIL. We also compared Japanese and Caucasian CADASIL cases and found that dementia and pseudobulbar palsy were observed more frequently in Japanese patients, although typical migraine was rather rare. In the present study, we describe the clinical features of CADASIL, hoping to help reveal the mechanism of chronic ischemic brain diseases, including leukoaraiosis or Binswanger's disease.
journal_name
Ann N Y Acad Scijournal_title
Annals of the New York Academy of Sciencesauthors
Abe K,Murakami T,Matsubara E,Manabe Y,Nagano I,Shoji Mdoi
10.1111/j.1749-6632.2002.tb04825.xsubject
Has Abstractpub_date
2002-11-01 00:00:00pages
266-72eissn
0077-8923issn
1749-6632journal_volume
977pub_type
杂志文章abstract::In a placebo-controlled double-blinded study, the effect of 8 weeks of grape juice was compared to the effect of isocaloric placebo juice. Volunteers with a systolic blood pressure > 130 mmHg and diastolic blood pressure > 90 mmHg were recruited. A total of 40 healthy subjects were randomized to receive isocaloric jui...
journal_title:Annals of the New York Academy of Sciences
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abstract::An inactive form of erythrocyte CA I was found in patients with primary aldosteronism. The levels of CA I in the patients were within the normal range: however, the specific esterase activity of CA I from three patients was in the range of 0.12-0.26 u/mg CA I (normal control: 0.66 +/- 0.1 u/mg CA I). In one subject, w...
journal_title:Annals of the New York Academy of Sciences
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journal_title:Annals of the New York Academy of Sciences
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journal_title:Annals of the New York Academy of Sciences
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journal_title:Annals of the New York Academy of Sciences
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journal_title:Annals of the New York Academy of Sciences
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journal_title:Annals of the New York Academy of Sciences
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journal_title:Annals of the New York Academy of Sciences
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journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
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journal_title:Annals of the New York Academy of Sciences
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journal_title:Annals of the New York Academy of Sciences
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journal_title:Annals of the New York Academy of Sciences
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journal_title:Annals of the New York Academy of Sciences
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journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
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