Premium
Non‐Alzheimer‐type pattern of brain cholineacetyltransferase reduction in dominantly inherited olivopontocerebellar atrophy
Author(s) -
Kish Stephen J.,
Robitaille Yves,
ElAwar Munir,
Deck H. N.,
Simmons Jerry,
Schut Lawrence,
Chang LiJan,
DiStefano Linda,
Freedman Morris
Publication year - 1989
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410260309
Subject(s) - olivopontocerebellar atrophy , cholinergic , cerebral cortex , neuroscience , globus pallidus , hippocampus , nucleus basalis , alzheimer's disease , dementia , choline acetyltransferase , amygdala , atrophy , lesion , psychology , degenerative disease , cholinergic neuron , medicine , pathology , basal ganglia , central nervous system , central nervous system disease , disease
We recently reported reduced activity of the cholinergic marker enzyme cholineacetyltransferase (ChAT) in several brain regions of patients with dominantly inherited olivopontocerebellar atrophy (OPCA). To document the regional extent of these changes we performed a comprehensive examination of the behavior of ChAT throughout both cerebral cortical and subcortical brain areas in 5 patients from one large OPCA pedigree. As compared with the controls, mean ChAT activities in OPCA were redced by 39 to 72 in all (n = 27) cerebral cortical areas examined and in several thalamic subdivisions, caudate head, globus pallidus, red nucles, and medical olfacotry area. In contradistinction to findings in Alzheimer's disease (AD), mean ChAT levels in OPCA amygdala and hippocampal subdivisions were either normal or only mildly reduced. The lack of severe disabling dementia in our OPCA patients compared with AD patients having a similar cortical cholinergic reduction could be explained by an absence of either a marked cholinergic loss in amygdala or hippocampus or significant loss of noncholinergic cerebral cortical and limbic neurons as occurs in AD brain. We suggest that this and other OPCA pedigrees having a cortical cholinergic reduction represent a unique model for the study of behavioral consequences of a more selective cerebral cortical cholinergic lesion rather than a limbic cholinergic lesion.