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When DLB, PD, and PSP masquerade as MSA: an autopsy study of 134 patients.
Neurology. 2015 Aug 04; 85(5):404-12.Neur

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.

Authors+Show Affiliations

From the Departments of Neuroscience (S.K., N.A., D.W.D.) and Neurology (R.J.U., J.A.v.G., W.P.C., Z.K.W.), Mayo Clinic, Jacksonville, FL; the Department of Neurology (Behavioural Neurology) (K.A.J.), Mayo Clinic, Rochester, MN; and The Parkinson's Institute and Clinical Center (J.W.L.), Sunnyvale, CA.From the Departments of Neuroscience (S.K., N.A., D.W.D.) and Neurology (R.J.U., J.A.v.G., W.P.C., Z.K.W.), Mayo Clinic, Jacksonville, FL; the Department of Neurology (Behavioural Neurology) (K.A.J.), Mayo Clinic, Rochester, MN; and The Parkinson's Institute and Clinical Center (J.W.L.), Sunnyvale, CA.From the Departments of Neuroscience (S.K., N.A., D.W.D.) and Neurology (R.J.U., J.A.v.G., W.P.C., Z.K.W.), Mayo Clinic, Jacksonville, FL; the Department of Neurology (Behavioural Neurology) (K.A.J.), Mayo Clinic, Rochester, MN; and The Parkinson's Institute and Clinical Center (J.W.L.), Sunnyvale, CA.From the Departments of Neuroscience (S.K., N.A., D.W.D.) and Neurology (R.J.U., J.A.v.G., W.P.C., Z.K.W.), Mayo Clinic, Jacksonville, FL; the Department of Neurology (Behavioural Neurology) (K.A.J.), Mayo Clinic, Rochester, MN; and The Parkinson's Institute and Clinical Center (J.W.L.), Sunnyvale, CA.From the Departments of Neuroscience (S.K., N.A., D.W.D.) and Neurology (R.J.U., J.A.v.G., W.P.C., Z.K.W.), Mayo Clinic, Jacksonville, FL; the Department of Neurology (Behavioural Neurology) (K.A.J.), Mayo Clinic, Rochester, MN; and The Parkinson's Institute and Clinical Center (J.W.L.), Sunnyvale, CA.From the Departments of Neuroscience (S.K., N.A., D.W.D.) and Neurology (R.J.U., J.A.v.G., W.P.C., Z.K.W.), Mayo Clinic, Jacksonville, FL; the Department of Neurology (Behavioural Neurology) (K.A.J.), Mayo Clinic, Rochester, MN; and The Parkinson's Institute and Clinical Center (J.W.L.), Sunnyvale, CA.From the Departments of Neuroscience (S.K., N.A., D.W.D.) and Neurology (R.J.U., J.A.v.G., W.P.C., Z.K.W.), Mayo Clinic, Jacksonville, FL; the Department of Neurology (Behavioural Neurology) (K.A.J.), Mayo Clinic, Rochester, MN; and The Parkinson's Institute and Clinical Center (J.W.L.), Sunnyvale, CA.From the Departments of Neuroscience (S.K., N.A., D.W.D.) and Neurology (R.J.U., J.A.v.G., W.P.C., Z.K.W.), Mayo Clinic, Jacksonville, FL; the Department of Neurology (Behavioural Neurology) (K.A.J.), Mayo Clinic, Rochester, MN; and The Parkinson's Institute and Clinical Center (J.W.L.), Sunnyvale, CA.From the Departments of Neuroscience (S.K., N.A., D.W.D.) and Neurology (R.J.U., J.A.v.G., W.P.C., Z.K.W.), Mayo Clinic, Jacksonville, FL; the Department of Neurology (Behavioural Neurology) (K.A.J.), Mayo Clinic, Rochester, MN; and The Parkinson's Institute and Clinical Center (J.W.L.), Sunnyvale, CA. dickson.dennis@mayo.edu.

Pub Type(s)

Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

26138942

Citation

Koga, Shunsuke, et al. "When DLB, PD, and PSP Masquerade as MSA: an Autopsy Study of 134 Patients." Neurology, vol. 85, no. 5, 2015, pp. 404-12.
Koga S, Aoki N, Uitti RJ, et al. When DLB, PD, and PSP masquerade as MSA: an autopsy study of 134 patients. Neurology. 2015;85(5):404-12.
Koga, S., Aoki, N., Uitti, R. J., van Gerpen, J. A., Cheshire, W. P., Josephs, K. A., Wszolek, Z. K., Langston, J. W., & Dickson, D. W. (2015). When DLB, PD, and PSP masquerade as MSA: an autopsy study of 134 patients. Neurology, 85(5), 404-12. https://doi.org/10.1212/WNL.0000000000001807
Koga S, et al. When DLB, PD, and PSP Masquerade as MSA: an Autopsy Study of 134 Patients. Neurology. 2015 Aug 4;85(5):404-12. PubMed PMID: 26138942.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - When DLB, PD, and PSP masquerade as MSA: an autopsy study of 134 patients. AU - Koga,Shunsuke, AU - Aoki,Naoya, AU - Uitti,Ryan J, AU - van Gerpen,Jay A, AU - Cheshire,William P, AU - Josephs,Keith A, AU - Wszolek,Zbigniew K, AU - Langston,J William, AU - Dickson,Dennis W, Y1 - 2015/07/02/ PY - 2014/11/13/received PY - 2015/03/10/accepted PY - 2015/7/4/entrez PY - 2015/7/4/pubmed PY - 2015/10/27/medline SP - 404 EP - 12 JF - Neurology JO - Neurology VL - 85 IS - 5 N2 - 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. SN - 1526-632X UR - https://www.unboundmedicine.com/medline/citation/26138942/When_DLB_PD_and_PSP_masquerade_as_MSA:_an_autopsy_study_of_134_patients_ L2 - http://www.neurology.org/cgi/pmidlookup?view=long&pmid=26138942 DB - PRIME DP - Unbound Medicine ER -