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Sixty-eight patients(90%)had hyperintensities on T2-WI located in the white matter,more frequent in PV(96%)and WM(85%)than in the superficial white matter(25%).Hyperintensities also occurred in BG(60%)and brainstem (45%).Forty-seven patients(62%)presented with hypointensities on T1-WI.In one-third of the affected individuals,white matter hyperintensities occurred in the absence of small deep infarcts on T1-WI.The frequency and severity scores calculated for PV,WM,BG,or IT hyperintensities increase dramatically with age.These scores were higher in symptomatic compared with asymptomatic gene carriers.Dementia,Rankin score>1,or both occurred only in the presence of diffuse white matter signal abnormalities.Our results suggest that different subcortical areas have different vulnerabilities to ischemia in CADASIL.The age effect we observed may show an accumulation of lesions with aging during the course of the disease.A prospective study is needed to investigate if the rating of MRI lesions is of prognostic value in CADASIL. |
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basal ganglia,lesion of
brainstem,lesion of
cerebral autosomal dominate arteriopathy with subcortical infarction and leukoencephalopathy
leukoencephalopathy
MRI
MRI,abnormal
skin,biopsy
white matter disease
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