ABNORMAL DENDRITIC DEVELOPMENT IN MAPLE SYRUP URINE DISEASE

被引：39

作者：

KAMEI, A ^{[1
]}

TAKASHIMA, S ^{[1
]}

CHAN, F ^{[1
]}

BECKER, LE ^{[1
]}

机构：

[1] HOSP SICK CHILDREN,DEPT PATHOL,TORONTO M5G 1X8,ONTARIO,CANADA

来源：

PEDIATRIC NEUROLOGY | 1992年 / 8卷 / 02期

关键词：

D O I：

10.1016/0887-8994(92)90038-Z

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

The neuropathology of a 6-year-old boy with maple syrup urine disease revealed spongy appearance of the white matter and marked edema of the brainstem. Golgi studies demonstrated aberrant orientation of neurons together with abnormalities of dendrites and dendritic spines. Similar changes were observed in a patient with dihydropteridine reductase deficiency. Disorders of amino acid metabolism may be associated with alterations in the terminal stages of neuronal migration and maturation.

引用

页码：145 / 147

页数：3

共 13 条

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