Cognitive deficits in olivopontocerebellar atrophy: Implications for the cholinergic hypothesis of Alzheimer's dementia

✍ Scribed by Dr. Stephen J. Kish; Munir El-Awar; Lawrence Schut; Larry Leach; Marchlene Oscar-Berman; Morris Freedman

• Publisher: John Wiley and Sons
• Year: 1988
• Tongue: English
• Weight: 689 KB
• Volume: 24
• Category: Article
• ISSN: 0364-5134
• DOI: 10.1002/ana.410240205

✦ Synopsis

A cerebral cortical cholinergic reduction in dominantly inherited olivopontocerebellar atrophy (OPCA) was recently described. Although the magnitude of the cholinergic reduction was similar to that observed in Alzheimer's disease (AD), none of the OPCA patients was reported to have been demented. We now describe a comprehensive neuropsychological assessment of 11 patients from cine of the OPCA pedigrees which we examined biochemically. Detailed neuropsychological testing disclosed previously unrecognized deficits in verbal and nonverbal intelligence, memory, and frontal system function which were positively correlated with the severity of cerebellar ataxia. However, our OPCA patients appeared to be at most only mildly disabled by their cognitive impairment and scored within or close to the normal range on a simple mental statiis screening examination. This, as well as an absence of any aphasia, apraxia, or agnosia, contrasts with the profile and severity observed in advanced AD dementia, characterized by a similar cortical cholinergic deficit. This finding also suggests that cholinergic reduction may explain only part of the pathophysiology underlying the dementia of AD.