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Extrapyramidal features in patients with motor neuron disease and dementia; a clinicopathological correlative study.

Abstract：

:Motor neuron disease (MND) may be complicated by frontotemporal dementia and/or an extrapyramidal movement disorder. Several studies have identified the pathological substrate for dementia in MND as being ubiquitin-immunoreactive inclusions and dystrophic neurites in the extramotor neocortex and hippocampus. Although degenerative changes have previously been noted in the basal ganglia and substantia nigra in MND, detailed pathological studies with clinical correlation are lacking. We examined postmortem material from eight patients with a history of MND and dementia, four of whom also had prominent extrapyramidal features. All cases showed the expected degenerative changes in the pyramidal motor system and ubiquitin-positive inclusions in the extramotor cortex. In addition, the cases with a history of extrapyramidal features had striking pathology in the basal ganglia and substantia nigra; neuronal loss and gliosis ranged from moderate to severe and immunohistochemistry demonstrated numerous neuronal inclusions and dystrophic neurites, which were reactive for ubiquitin, but not tau or alpha-synuclein. Similar pathology was either absent or much milder in cases without extrapyramidal features. This study illustrates the utility of ubiquitin immunohistochemistry in demonstrating the range of pathology in MND and provides a neuropathological correlate for the extrapyramidal features which may occur in MND with dementia.

journal_name

Acta Neuropathol

journal_title

Acta neuropathologica

authors

Mackenzie IR,Feldman H

doi

10.1007/s00401-003-0814-y

subject

Has Abstract

pub_date

2004-04-01 00:00:00

pages

336-40

issue

4

eissn

0001-6322

issn

1432-0533

journal_volume

107

pub_type

杂志文章

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