Objective: We investigated the utility of the Movement Disorder Society Criteria (MDS criteria) including sensitivity and specificity.

Background: Multiple system atrophy (MSA) is an adult-onset progressive neurodegenerative disease characterized by autonomic dysfunction, parkinsonism, and ataxia. In 2022, Wenning et al. proposed MDS criteria for the diagnosis of MSA, which criteria are expected to provide useful alternatives to the second consensus statement.

Method: We compared these criteria with those of patients who had been registered in the Hokkaido Rare-disease Consortium for multiple system atrophy (HoRC-MSA). The control group comprised patients with MSA-mimicking disorders (Parkinson’s disease, dementia with Lewy bodies, progressive supranuclear palsy, corticobasal syndrome).

Results: Of the 252 patients enrolled in the HoRC-MSA study, 241 were evaluated for these diagnostic criteria from November 2014 to July 2022. The MDS criteria were examined in 68 patients enrolled in the HoRC-MSA group and 35 patients in the control group. At the initial visit, the sensitivity for the categories of “clinically establish,” “clinically probable,” and “possible prodromal” was 66.2% [95% confidence interval (CI): 61.8–66.2%], 83.8% (CI: 78.3–86.6%), 89.7% (CI: 84.2–92.9%), and the specificity was 100% (CI: 91.4–100%), 94.3% (CI: 84.1–98.4%), 91.4% (CI: 81.2–96.7%), respectively. At the last visit, the sensitivity was 76.5% (CI: 72.1–76.5), 88.2% (CI: 82.5–91.9%), and 92.6% (CI: 87.1–96.1), and the specificity was 100% (CI: 91.6–100%), 91.4% (CI: 81–96.8), and 91.4% (CI: 81.6–96.6%), respectively. These criteria do not significantly change the diagnostic accuracy in the early and advanced stages of the disease compared to the second consensus statement.

About association between the clinical features used in the MDS criteria and the MSA/MSA-mimicking disorders, autonomic features, including unexplained urinary urge incontinence, neurogenic orthostatic hypotension, core cerebellar features, positivity for MRI markers, rapid progression and severe speech impairment within three years of motor onset, unexplained Babinski sign, stridor, and erectile dysfunction, were more common in patients with MSA.

Conclusion: The present study established the diagnostic usefulness of the MDS criteria. MDS criteria has been shown to be useful at any stage of the disease and may be helpful for patient selection in clinical trials that focus on disease-modifying therapies.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/investigation-of-usefulness-of-new-msa-diagnostic-criteria/