ACUTE BASAL GANGLIA INFARCTION IN PROPIONIC ACIDEMIA

被引：48

作者：

HAAS, RH

MARSDEN, DL

CAPISTRANOESTRADA, S

HAMILTON, R

GRAFE, MR

WONG, W

NYHAN, WL

机构：

[1] UNIV CALIF SAN DIEGO,DEPT NEUROSCI,LA JOLLA,CA 92093

[2] UNIV CALIF SAN DIEGO,DEPT PEDIAT,LA JOLLA,CA 92093

[3] UNIV CALIF SAN DIEGO,DEPT PATHOL,LA JOLLA,CA 92093

来源：

JOURNAL OF CHILD NEUROLOGY | 1995年 / 10卷 / 01期

关键词：

D O I：

10.1177/088307389501000104

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

An 8-year-old girl with propionic acidemia had acute and rapidly fatal symmetric necrosis of the caudate, globus pallidus, and putamen. Clinical presentation was with acute aphasia, generalized hypotonia, and muscle weakness. There was no evidence of metabolic decompensation, and analysis of the organic acids of the urine indicated good metabolic control. Organic acids in the cerebrospinal fluid were unremarkable. These observations indicate that the pathophysiology of ''metabolic stroke'' is more complicated than previously thought.

引用

页码：18 / 22

页数：5

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