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. 2015 Aug 4;85(5):404-12.
doi: 10.1212/WNL.0000000000001807. Epub 2015 Jul 2.

When DLB, PD, and PSP masquerade as MSA: an autopsy study of 134 patients

Shunsuke Koga  1 , Naoya Aoki  1 , Ryan J Uitti  1 , Jay A van Gerpen  1 , William P Cheshire  1 , Keith A Josephs  1 , Zbigniew K Wszolek  1 , J William Langston  1 , Dennis W Dickson  2
Affiliations

When DLB, PD, and PSP masquerade as MSA: an autopsy study of 134 patients

Shunsuke Koga et al. Neurology. .

Abstract

Objective: To determine ways to improve diagnostic accuracy of multiple system atrophy (MSA), we assessed the diagnostic process in patients who came to autopsy with antemortem diagnosis of MSA by comparing clinical and pathologic features between those who proved to have MSA and those who did not. We focus on likely explanations for misdiagnosis.

Methods: This is a retrospective review of 134 consecutive patients with an antemortem clinical diagnosis of MSA who came to autopsy with neuropathologic evaluation of the brain. Of the 134 patients, 125 had adequate medical records for review. Clinical and pathologic features were compared between patients with autopsy-confirmed MSA and those with other pathologic diagnoses, including dementia with Lewy bodies (DLB), Parkinson disease (PD), and progressive supranuclear palsy (PSP).

Results: Of the 134 patients with clinically diagnosed MSA, 83 (62%) had the correct diagnosis at autopsy. Pathologically confirmed DLB was the most common misdiagnosis, followed by PSP and PD. Despite meeting pathologic criteria for intermediate to high likelihood of DLB, several patients with DLB did not have dementia and none had significant Alzheimer-type pathology. Autonomic failure was the leading cause of misdiagnosis in DLB and PD, and cerebellar ataxia was the leading cause of misdiagnosis in PSP.

Conclusions: The diagnostic accuracy for MSA was suboptimal in this autopsy study. Pathologically confirmed DLB, PD, and PSP were the most common diseases to masquerade as MSA. This has significant implications not only for patient care, but also for research studies in MSA cases that do not have pathologic confirmation.

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Figures

Figure
Figure. Flow chart of study design
DLB = dementia with Lewy bodies; MSA = multiple system atrophy; PD = Parkinson disease; PSP = progressive supranuclear palsy; OPCA = predominantly olivopontocerebellar involvement type of multiple system atrophy; SND = predominantly striatonigral involvement type of multiple system atrophy; SND/OPCA = equally severe striatonigral and olivopontocerebellar involvement type of multiple system atrophy.
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