Background: Multiple system atrophy (MSA) type C is a rare, sporadic, progressive, neurodegenerative disease. Oligodendrocyte cytoplasmic inclusions of fibrillized alpha-synuclein represent the hallmark of the disease. Dementia is considered an exclusion criteria, but MMSE is abnormal in 26% of the patients. Executive dysfunction is the most common presentation, but memory or visual spatial functions may also be impaired.
Objective: To assess multiple, domain-specific cognitive functions in patients with the cerebellar type of MSA (MSA-C) and to compare them with normal controls and Parkinson's disease (PD) patients.
Patients and methods: We included patients with probable MSA-C, PD and normal controls, matched by age, sex and scholarity. We performed the following tests: The Montreal Cognitive assessment (global assessment); Naming Nouns and Pointing (language); Raven Colored Progressive Matrices (fluid intelligence); Symbol Digit Modalities Test, Trail Making Test, Phonetic and Semantic Fluencies (executive functions); Digit Span, Modified 10/36 Special Recall Test, Rey Auditory Verbal Learning Test (memory); Segment Length Discrimination, Mental Rotation (Visuospatial functions).
Results: We enrolled 20 MSA-C, 20 PD patients, and 20 normal controls (age, sex and scholarity matched). The most frequent finding in MSA-C patients was an impairment of the executive functions, followed by attention and memory and visuospatial impairment. The impairment of the executive functions was more severe in MSA-C than in PD patients.
Conclusions: Global cognitive impairment is uncommon in MSA-C but executive functions are frequently impaired.
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© 2015 Published by Elsevier Inc.
