AUTOSOMAL DOMINANT LEUKOENCEPHALOPATHY AND SUBCORTICAL ISCHEMIC STROKE - A CLINICOPATHOLOGICAL STUDY

被引:283
作者
BAUDRIMONT, M
DUBAS, F
JOUTEL, A
TOURNIERLASSERVE, E
BOUSSER, MG
机构
[1] HOP ST ANTOINE,SERV NEUROL,184 RUE FAUBOURG ST ANTOINE,F-75571 PARIS 12,FRANCE
[2] CHU ANGERS,INSERM,U29,SERV NEUROL A,F-49036 ANGERS,FRANCE
[3] HOP ST ANTOINE,SERV ANAT PATHOL,F-75571 PARIS 12,FRANCE
[4] FAC MED NECKER ENFANTS MALAD,INSERM,CJF 9001,PATHOL IMMUN LAB,PARIS,FRANCE
关键词
GENETICS; LACUNAR INFARCTION; LEUKOENCEPHALOPATHY;
D O I
10.1161/01.STR.24.1.122
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Background and Purpose: We recently described an autosomal dominant syndrome characterized mainly by recurrent strokes and neuroimaging evidence of leukoencephalopathy. We now report the pathological findings in one of the affected subjects. Case Description: A 40-year-old woman experienced her first grand mal seizure in 1971. From 1983 on she suffered recurrent strokes, seizures, and psychiatric disturbances with depressions, manic episodes, and dementia. In 1988, after her fourth stroke, she became tetraplegic with a severe pseudobulbar palsy, and she died in 1990. Pathological examination disclosed a recent capsulolenticular hematoma, multiple small deep infarcts, a diffuse myelin loss and pallor of the hemispheric white matter, and a widespread vasculopathy of the small arteries penetrating the white matter. The arterial wall was markedly thickened with an extensive nonamyloid eosinophilic deposit in the media and reduplication of the internal elastic lamella. Conclusions: The underlying lesion of this hereditary disorder is located in the small arteries and is of unknown etiology. It differs from arteriosclerotic and amyloid angiopathies but is similar to that described in some cases of hereditary multi-infarct dementia.
引用
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页码:122 / 125
页数:4
相关论文
共 17 条
[1]  
BERTHIER E, 1992, REV NEUROL-FRANCE, V148, P146
[2]  
Binswanger O., 1894, KLIN WOCHENSCHR, V31, P1180
[3]  
Binswanger O., 1894, KLIN WOCHENSCHR, V31, P1137
[4]  
Binswanger O, 1894, KLIN WOCHENSCHR, V31, P1103
[5]  
Davous P, 1991, REV NEUROL-FRANCE, V5, P376
[6]   SCLEROSING VASCULOPATHY OF THE CENTRAL-NERVOUS-SYSTEM IN NONELDERLY DEMENTED PATIENTS [J].
ESTES, ML ;
CHIMOWITZ, MI ;
AWAD, IA ;
MCMAHON, JT ;
FURLAN, AJ ;
RATLIFF, NB .
ARCHIVES OF NEUROLOGY, 1991, 48 (06) :631-636
[7]  
GAMBLE CN, 1986, AM J PATHOL, V122, P410
[8]   HEREDITARY CEREBRAL-HEMORRHAGE WITH AMYLOIDOSIS-DUTCH TYPE - MAGNETIC-RESONANCE-IMAGING FINDINGS IN 7 CASES [J].
HAAN, J ;
ROOS, RAC ;
ALGRA, PR ;
LANSER, JBK ;
BOTS, GTAM ;
VEGTERVANDERVLIS, M .
BRAIN, 1990, 113 :1251-1267
[9]  
MAEDA S, 1976, Folia Psychiatrica et Neurologica Japonica, V30, P165
[10]   A FAMILIAL DISORDER WITH SUBCORTICAL ISCHEMIC STROKES, DEMENTIA, AND LEUKOENCEPHALOPATHY [J].
MAS, JL ;
DILOUYA, A ;
DERECONDO, J .
NEUROLOGY, 1992, 42 (05) :1015-1019