ALEXANDERS DISEASE - CLUES TO DIAGNOSIS

被引：61

作者：

PRIDMORE, CL

BARAITSER, M

HARDING, B

BOYD, SG

KENDALL, B

BRETT, EM

机构：

[1] HOSP SICK CHILDREN,DEPT CLIN GENET,GREAT ORMOND ST,LONDON WC1N 3JH,ENGLAND

[2] HOSP SICK CHILDREN,DEPT PAEDIAT NEUROL,LONDON WC1N 3JH,ENGLAND

[3] HOSP SICK CHILDREN,DEPT HISTOPATHOL,LONDON WC1N 3JH,ENGLAND

[4] HOSP SICK CHILDREN,DEPT CLIN NEUROPHYSIOL,LONDON WC1N 3JH,ENGLAND

[5] HOSP SICK CHILDREN,DEPT RADIOL,LONDON WC1N 3JH,ENGLAND

来源：

JOURNAL OF CHILD NEUROLOGY | 1993年 / 8卷 / 02期

关键词：

D O I：

10.1177/088307389300800205

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

The clinical, radiologic, neurophysiologic, and pathologic findings in 10 children with histologically proven Alexander's disease are described, and the presence of two broad clinical subgroups is confirmed. Macrocephaly, regression, and seizures are found in the infantile form, whereas bulbar signs predominate the so-called juvenile form. None of the clinical or neurophysiologic findings is pathognomonic, and radiologic features suggesting the diagnosis are not present in all cases. However, the combination of abnormalities may suggest the diagnosis and justify histologic confirmation.

引用

页码：134 / 144

页数：11

共 34 条

[1] PROGRESSIVE FIBRINOID DEGENERATION OF FIBRILLARY ASTROCYTES ASSOCIATED WITH MENTAL RETARDATION IN A HYDROCEPHALIC INFANT [J].