Abstract

Multiple system atrophy (MSA) is a neurodegenerative disorder involving basal ganglia and frontal-subcortical circuits. Dementia represents an exclusion criterion for the diagnosis of multiple system atrophy (MSA), but there have been reports of fronto-executive dysfunction in patients with MSA of the striatonigral type (MSA-P). To study the cognitive profile of MSA, 20 patients with MSA of the cerebellar type (MSA-C) were subjected to an extensive neuropsychological test battery comprising tests for attention, verbal and visuospatial memory, as well as executive function. We assessed 20 patients affected by probable MSA-C according to the criteria of Cilman et al (Mean age:59.8±7.2; mean disease duration: 8.5±2.3; M/F=13/7) using a neuropsychological battery which included general cognitive assessment(MMSE), executive and attentive functions test (Alternating square & triangle, Luria loop, COWAT), verbal long and short term memory (SVLT, Digit span) and visual-spatial short term memory (RCFT test). We also evaluated depression and neuropsychiatric symptoms using GDS. Mean MMSE was 26.8±2.1 All patients had pathological score in Alternating square & triangle, Luria loop, COWAT. All patients had borderline scores in visual-spatial short-term memory test. All patients had depression as showed by GDS. MSA-C patients were shown to have impaired verbal memory and executive function There was evidence for impaired verbal memory and verbal fluency. Regarding the similar cognitive syndrome of MSA-P, the otherwise subclinical problems in MSA-C result from subcortical rather than from cerebellar dysfunction. Depressive thoughts occur more frequently than PD. One possible explanation for the cognitive deficits in MSA-C is to assume that parts of the cerebral cortex undergo degeneration in parallel to the pontocerebellar system.

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