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Clinical features of autopsy-confirmed multiple system atrophy in the Mayo Clinic Florida brain bank.
Clinical features of autopsy-confirmed multiple system atrophy in the Mayo Clinic Florida brain bank.
- Source :
-
Parkinsonism & related disorders [Parkinsonism Relat Disord] 2021 Aug; Vol. 89, pp. 155-161. Date of Electronic Publication: 2021 Jul 10. - Publication Year :
- 2021
-
Abstract
- Background: Multiple system atrophy (MSA) presents with various combinations of autonomic dysfunction, parkinsonism, and cerebellar ataxia. Although clinical diagnostic criteria have been widely used, the sensitivity and specificity are suboptimal. This study aims to provide evidence supporting the revision of the current diagnostic criteria for MSA.<br />Methods: Medical records of 171 patients with autopsy-confirmed MSA in the Mayo Clinic brain bank were reviewed with regard to their clinical features and diagnoses. Pathologic features, including concomitant pathologies (i.e., Alzheimer-related and Lewy-related pathologies), were also assessed.<br />Results: The cohort included 133 MSA-parkinsonian type, 36 MSA-cerebellar type, and 2 unclassified MSA patients who did not show significant motor symptoms. Twenty-three patients (13%) were not clinically diagnosed with MSA, but instead with progressive supranuclear palsy, Parkinson's disease (PD), PD with dementia (PDD), or dementia with Lewy bodies (DLB). Three patients with PDD and DLB also had concomitant Lewy body pathology. Six patients had late-onset MSA, with an age of onset greater than 75 years. Erectile dysfunction was frequent in male patients (60/63; 95%) in all age ranges. REM sleep behavior disorder (RBD) was present in 82 patients (48%) and was the initial symptom in 13 patients. Cognitive impairment was present in 60 patients (35%), but was an initial symptom in only two patients.<br />Conclusions: Our findings support the conclusion that late-onset presentation should not exclude MSA. The findings of this large autopsy-based cohort provides valuable insights for improving clinical criteria for MSA.<br /> (Copyright © 2021. Published by Elsevier Ltd.)
- Subjects :
- Aged
Autopsy
Biological Specimen Banks
Brain pathology
Cognitive Dysfunction diagnosis
Cognitive Dysfunction etiology
Diagnosis, Differential
Female
Florida
Humans
Male
Multiple System Atrophy etiology
Parkinson Disease diagnosis
Parkinson Disease etiology
Parkinsonian Disorders diagnosis
Parkinsonian Disorders etiology
REM Sleep Behavior Disorder diagnosis
REM Sleep Behavior Disorder etiology
Retrospective Studies
Sensitivity and Specificity
Supranuclear Palsy, Progressive diagnosis
Supranuclear Palsy, Progressive etiology
Symptom Assessment methods
Multiple System Atrophy diagnosis
Multiple System Atrophy pathology
Symptom Assessment statistics & numerical data
Subjects
Details
- Language :
- English
- ISSN :
- 1873-5126
- Volume :
- 89
- Database :
- MEDLINE
- Journal :
- Parkinsonism & related disorders
- Publication Type :
- Academic Journal
- Accession number :
- 34303202
- Full Text :
- https://doi.org/10.1016/j.parkreldis.2021.07.007