Abstract
Introduction: Cognitive decline is not a characteristic feature of multiple system atrophy (MSA), but recent evidence suggests cognitive impairment as an integral part of the disease. We aim to describe the cognitive profile and its progression in a cohort of patients with MSA.Methods: We retrospectively selected patients referred to our department with a clinical diagnosis of MSA who were evaluated at least once a year during the course of the disease and underwent a comprehensive neuropsychological evaluation.Results: At the first evaluation (T0), 37 out of 60 patients (62%) were cognitively impaired, mainly (76%) in attention and executive functioning. Thirteen patients were impaired in one cognitive domain and 24 in more than one cognitive domain. Six out of the 24 had dementia. Twenty patients underwent a follow-up evaluation (T1) after a mean of 16.6 ± 9.3 months from the first evaluation (T0). Eight out of 20 patients were cognitively normal at both T0 and T1. Seven out of 12 patients presented with stable cognitive impairment at T1, while cognitive decline progressed in five patients. Patients with progression in cognitive decline performed significantly worse at T0 than cognitively stable patients. Education was significantly different between patients with and without cognitive impairment. No other differences in demographic and clinical variables and autonomic or sleep disturbances were found. Patients with dementia were older at disease onset and at T0 and had lower education and disease duration at T0 compared to those in other groups.Conclusions: In patients with MSA, we observed three different cognitive profiles: normal cognition, stable selective attention-executive deficits, and progressive cognitive deficits evolving to dementia. The detection of cognitive impairment in patients with suspected MSA suggests the need for comprehensive and longitudinal neuropsychological evaluation.
Highlights
Cognitive decline is not a characteristic feature of multiple system atrophy (MSA), but recent evidence suggests cognitive impairment as an integral part of the disease
Comparing the results on each task, we found that patients with cognitive impairment (CI) had significantly worse performance compared to those without CI mainly on tests of attention and executive function (Barrage total score, p < 0.0002, and Simple Verbal Analogies Test (SVAT), p < 0.001), followed by performance on tests of memory (RAVLT IR, p < 0.0001; Rey Auditory Verbal Learning Test (RAVLT) DR, p < 0.002; and immediate visual memory (IVM), p < 0.001) and semantic fluency (p < 0.001)
Our findings indicate that the cognitive profile of patients with Multiple system atrophy (MSA) can be characterized by normal cognitive functioning even in the sixth year of the disease or by a selective and stable impairment of attention and executive functions that could be related to fronto-striatal-cerebellar dysfunction
Summary
Cognitive decline is not a characteristic feature of multiple system atrophy (MSA), but recent evidence suggests cognitive impairment as an integral part of the disease. Multiple system atrophy (MSA) is a sporadic neurodegenerative disease characterized by autonomic failure associated with a combination of cerebellar and/or parkinsonian signs. It is subclassified into a cerebellar (MSA-C) or parkinsonian (MSA-P) variant, depending on the predominant clinical phenotype [1]. An evidence-based review of the “Neuropsychology Task Force of the Movement Disorders Society MSA” (MODIMSA) Study Group suggests cognitive impairment (CI) as an integral feature of the disease [5]. The frequency of CI varies largely among studies (between 33 and 83%)
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