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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.
Background:
Recent reports show that dementia occurs in 5-26% of multiple system atrophy (MSA) patients. However, the structural or pathological correlates of dementia in MSA are unclear yet.
Methods:
Of 152 patients with MSA, 59 fulfilled the criteria of probable MSA and 9 (15%) had dementia. Six of those patients and 9 without dementia, in addition to 10 controls, were included. All subjects underwent clinical evaluation including UMSARS, neuropsychological examinations, 3T-MRI, and Pittsburgh Compound B (PIB) PET imaging. The cortical thickness was assessed using surface-based morphometry.
Results:
Age and disease duration were similar between MSA with dementia and without dementia, while motor disability was more severe in MSA with dementia. In neuropsychological tests, attention, visuospatial function, and language function were impaired in MSA with dementia. Mean PIB binding was similar among the three groups. Cortical thickness was reduced in precuneus/cuneus, uncus, and posterior cingulate in MSA with dementia compared to the controls, and in parahippocampal and lingual cortices compared to MSA without dementia.
Conclusions:
Dementia was found in 15% of the probable MSA patients, which was similar to those reported in previous studies. It appears that amyloid pathology has limited role in dementia in MSA, although some patients had increased cortical amyloid burden. Cortical thinning in MSA-D was observed in areas where cortical thinning was reported in Alzheimer disease or Parkinson disease dementia, but its pathological relevance is unclear. The neuropathological processes leading to the development of dementia in MSA appears to be multifactorial and heterogenous.
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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