Objective: This study aimed to validate the clinical utility of the newly proposed criteria for multiple system atrophy (MSA) by the International Parkinson and Movement Disorder Society (MDS).

Background: The second consensus statement has been widely used to diagnose MSA; however, it has been pointed out that the diagnostic accuracy is suboptimal. The new MDS criteria developed 4 categories: neuropathologically established MSA, clinically established MSA, clinically probable MSA, and possible prodromal MSA. Clinically established MSA was designed to have maximum specificity with acceptable sensitivity and clinically probable MSA was then aimed to balance sensitivity and specificity. The sensitivity and specificity of the new diagnostic criteria have not been fully examined.

Method: We identified consecutive patients with a clinical or pathological diagnosis of MSA from the Mayo Clinic Brain Bank between 1998 and 2021. We restricted the cohort to 352 patients with medical charts by neurologists or movement disorder specialists. Clinical signs and symptoms were extracted by systematic medical chart review. The MDS criteria and the second consensus criteria were applied to the cohort. The sensitivity, specificity, and area under the curve (AUC) of receiver operating characteristic curves were compared between these criteria. Comparison was conducted between clinical subtypes and among clinically challenging cases (those with different clinical diagnoses or those with suspected but undiagnosed MSA before death).

Results: The sensitivity and specificity of clinically established MSA and clinically probable MSA by the MDS criteria were 16% and 99% and 64% and 74%, respectively. The sensitivity and specificity of probable MSA and possible MSA by the second consensus criteria were 72% and 52% and 93% and 21%, respectively. Clinically established MSA exhibited the highest specificity and clinically probable MSA demonstrated the greatest AUC. The diagnostic performance did not differ between clinical subtypes. In clinically challenging cases, clinically established MSA kept high specificity and clinically probable MSA demonstrated the highest AUC.

Conclusion: In this retrospective autopsy cohort study, the MDS criteria showed high specificity with clinically established MSA and moderate sensitivity and specificity with clinically probable MSA.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/the-diagnostic-utility-of-the-new-mds-criteria-for-multiple-system-atrophy-a-retrospective-autopsy-cohort-study/