Movement Disorders (revue)

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Severe generalised dystonia associated with a mosaic pattern of striatal gliosis.

Identifieur interne : 004D35 ( PubMed/Corpus ); précédent : 004D34; suivant : 004D36

Severe generalised dystonia associated with a mosaic pattern of striatal gliosis.

Auteurs : W R Gibb ; L. Kilford ; C D Marsden

Source :

RBID : pubmed:1620138

English descriptors

Abstract

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.

DOI: 10.1002/mds.870070305
PubMed: 1620138

Links to Exploration step

pubmed:1620138

Le document en format XML

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