Annals of Clinical and Translational Neurology (Feb 2020)

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

  • Sabine Eschlböck,
  • Margarete Delazer,
  • Florian Krismer,
  • Thomas Bodner,
  • Alessandra Fanciulli,
  • Beatrice Heim,
  • Antonio Heras Garvin,
  • Christine Kaindlstorfer,
  • Elfriede Karner,
  • Katherina Mair,
  • Christoph Rabensteiner,
  • Cecilia Raccagni,
  • Klaus Seppi,
  • Werner Poewe,
  • Gregor K. Wenning

DOI
https://doi.org/10.1002/acn3.50987
Journal volume & issue
Vol. 7, no. 2
pp. 219 – 228

Abstract

Read online

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.