Clinical and imaging characteristics of dementia in multiple system atrophy

Han-Joon Kim1, Beom S Jeon, Young Eun Kim

  • 1Department of Neurology and Movement Disorder Center, Parkinson Study Group, and Neuroscience Research Institute, College of Medicine, Seoul National University, Seoul, Republic of Korea.

Abstract

Insights

Dementia affects 15% of multiple system atrophy (MSA) patients. Structural brain changes, not amyloid plaques, are linked to cognitive decline in MSA dementia, suggesting a complex pathology.

Area of Science:

  • Neuroscience
  • Neurology
  • Medical Imaging

Background:

  • Dementia is a known complication in multiple system atrophy (MSA), affecting 5-26% of patients.
  • The underlying structural and pathological causes of dementia in MSA remain poorly understood.

Purpose of the Study:

  • To investigate the neuroanatomical and pathological correlates of dementia in patients with multiple system atrophy (MSA).
  • To compare brain structure and amyloid burden between MSA patients with and without dementia, and healthy controls.

Main Methods:

  • 152 probable MSA patients were assessed; 9 (15%) had dementia (MSA-D).
  • Included 6 MSA-D patients, 9 MSA without dementia, and 10 controls.
  • Utilized clinical evaluations (UMSARS), neuropsychological tests, 3T-MRI, and Pittsburgh Compound B (PIB) PET imaging.
  • Assessed cortical thickness using surface-based morphometry.

Main Results:

  • MSA-D patients showed more severe motor disability than MSA without dementia.
  • Cognitive deficits in MSA-D included attention, visuospatial, and language impairments.
  • Cortical thinning was observed in MSA-D in regions like precuneus/cuneus and parahippocampal cortex.
  • Amyloid (PIB) binding was similar across groups, suggesting a limited role in MSA dementia.

Conclusions:

  • Dementia prevalence in probable MSA is approximately 15%.
  • Amyloid pathology appears to play a minor role in MSA dementia.
  • Observed cortical thinning in MSA-D overlaps with regions affected in Alzheimer's and Parkinson's disease dementia.
  • The neuropathogenesis of dementia in MSA is likely multifactorial and heterogeneous.

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