SEVERE GENERALIZED DYSTONIA ASSOCIATED WITH A MOSAIC PATTERN OF STRIATAL GLIOSIS

被引：36

作者：

GIBB, WRG

KILFORD, L

MARSDEN, CD

机构：

[1] Parkinson's Disease Society Brain Bank, Institute of Neurology and the National Hospital for Neurology and Neurosurgery, London

来源：

MOVEMENT DISORDERS | 1992年 / 7卷 / 03期

关键词：

CALBINDIN; DYSTONIA; HUNTINGTONS DISEASE; HYPOXIA; NEUROACANTHOCYTOSIS; STRIATUM;

D O I：

10.1002/mds.870070305

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

A mosaic pattern of striatal pathology is described in a male who developed severe generalised dystonia from the age of 10 years, and died at the age of 18 years. There was no family history of dystonia, and extensive investigations during his life failed to identify a cause for the dystonia. The caudate nucleus and putamen showed a network of cell loss and gliosis surrounding islands of preserved striatum. Dorsal parts showed confluent gliosis, and ventral parts were spared. The pattern suggested a correlation with patch-matrix organisation, but there was no correlation with the distribution of calbindin immunoreactive cells, which are present in the matrix of the classical striosome-matrix organisation. The pathological findings were unlike those in status marmoratus, perinatal hypoxia-ischaemia, Huntington's disease, and neuroacanthocytosis, but similar to those reported in a 44-year-old man with predominantly cranial dystonia.

引用

页码：217 / 223

页数：7

共 26 条

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