Cognition in multiple system atrophy: a single‐center cohort study

Abstract

Objective Cognitive impairment in multiple system atrophy (MSA) is common, but remain poorly characterized. We evaluated cognitive and behavioral features in MSA patients and assessed between‐group differences for MSA subtypes and the effect of orthostatic hypotension (OH) on cognition. Methods This retrospective study included 54 patients with clinical diagnosis of possible and probable MSA referred to the Department of Neurology at Medical University of Innsbruck between 2000 and 2018. Neurological work‐up included comprehensive neuropsychological testing including Consortium to Establish a Registry for Alzheimer's Disease (CERAD‐plus) test battery, Frontal Assessment Battery (FAB), digit span test (DST), clock drawing task (CLOX1), and Hospital Anxiety and Depression Scale (HADS‐D). Results The mean MMSE score was 27.6 points. Overall, slight to moderate cognitive impairment was noted in up to 40% of patients, with predominant impairment of executive function and verbal memory. Patients with the cerebellar variant performed significantly worse than patients with the parkinsonian type (P < 0.05) in a screening of executive functions (FAB) and in phonemic verbal fluency. Depression and anxiety scores were elevated in 28% and 22% of MSA patients, respectively. Cognitive profile, depression, and anxiety levels were comparable between patients with and without OH. Interpretation Cognitive deficits are relatively frequent in MSA and primarily affect executive functions and verbal memory. Future comparative studies including Parkinson dementia, Lewy body disease, and MSA cases with and without OH are required to elucidate disease‐specific cognitive profiles in these synucleinopathies and to examine the influence of cardiovascular autonomic dysfunction on cognitive function in MSA.

Health-related quality of life in multiple system atrophy using EQ-5D-5L: a large cross-sectional study in China

“…Recent studies found that a varying degree of cognitive impairment was common in MSA assessed by comprehensive neuropsychological testing. [33,34] Our current study found that cognitive impairment was the determinant of health utility value in MSA-P but not in MSA-C. [35,36] Kawai reported that patients with MSA-P had wider and severer cognitive impairment than patients with MSA-C. [35] Patients with MSA-P were reported to have lower MoCA scores than patients with MSA-C in a Chinese study. [37] Additionally, we found that frontal lobe dysfunction was the determinant of EQ-VAS score in MSA-P but not in MSA-C, which was similar to our previous nding that frontal lobe dysfunction was the determinant of HRQoL assessed by PDQ-39 in MSA.…”

Neuropathology of multiple system atrophy: Kurt Jellinger`s legacy

“…Overt dementia is considered an exclusion criterion for the diagnosis of MSA, nevertheless cognitive impairment may occur, especially in the advanced disease stages (Eschlböck et al 2020 ). Regarding cognitively impaired MSA patients, concomitant amyloid-plaques, tau and LB co-pathology have been reported (Jellinger 2020 ).…”

Early autonomic and cognitive dysfunction in PD, DLB and MSA: blurring the boundaries between α-synucleinopathies

“…However, the presence of cognitive decline among MSA patients who undergo neuropsychological assessment has been also reported at disease onset and, in some patients, the cognitive impairment has preceded motor impairment [108]. Studies on cognitive function in MSA not only disclosed a broad spectrum of deficits in cognition and behavior describing frequently a profile that can be similar to that seen in PD and DLB, with predominant deficits in processing speed, verbal memory, and executive function [109,110], but visuospatial and constructional dysfunction have also been reported [111]. Similar to PD, MMSE does not seem sufficient to assess cognition in these patients [112] and MoCA seems to be more sensitive to detect the mild cognitive decline in MSA [113].…”