Objective: We evaluated the extent to which diagnoses of MSA align with the MDS criteria within a specialist neurology trust in the UK. We aimed to illuminate potential discrepancies in diagnosis, and make suggestions for improved clinical assessments.

Background: MSA is a progressive neurodegenerative disease that clinically presents with autonomic failure, parkinsonism & cerebellar syndrome in various combinations. The assessment of MSA has classically been clinical & the novel MDS diagnostic criteria has been developed to improve accuracy, especially in early disease stages.

Method: Retrospective analysis of 64 patients with an established diagnosis of MSA was performed, by undertaking case note review.  Patients were identified based on ICD-10 codes for MSA-P and MSA-C.

Results: 6% were assessed for post void residuals, and all had unexplained voiding difficulty. 75% had unexplained urinary urge incontinence. 55% had orthostatic hypotension within 10 minutes. 55% showed features of Parkinsonism of which 41% had poor L-Dopa response. 49% had limb ataxia, 55% had gait ataxia, 45% had cerebellar dysarthria & 38% had oculomotor features.[figure1]

Within 3 years of motor onset, 63% had rapid progression, 74% had moderate to severe disability, 3% had craniocervical dystonia, 58% had severe speech impairment, 48% had severe dysphagia, 8% had unexplained Babinski sign and 33% had jerky myoclonic tremor. 36% patients had postural deformities.[figure2]

9% had stridor, 3% had inspiratory sighs, 8% had cold discoloured hands/feet, 20% had erectile dysfunction & 3% had pathological laughing/crying.[figure3]

55% fulfilled the exclusion criteria.[figure4]

14% had a polysomnography proven REM sleep disorder. 72% had subtle Parkinsonism & 52% had subtle cerebellar signs.[figure5]

44% fulfilled the criteria for clinically established MSA, 48% for clinically probable MSA and 52% for possible prodromal MSA.

Conclusion: More than half the patients diagnosed with MSA did not fulfil the MDS criteria. Certain assessments outlined in the criteria have been seldom used. There are multiple mimics, some of which may require genetic testing to identify the cause. The exclusion criteria are extensive & do not account for the natural progression of Parkinson’s disease. The criteria are more suited for assessment of MSA at the early stages as the criteria has components which accounts for various presentations.

Core clinical features in diagnosis of MSA

Supporting clinical features in MSA

Supporting non-motor clinical features in MSA

Features within exclusion criteria

Possible prodromal MSA clinical features

References: The Movement Disorder Society Criteria for Diagnosis of Multiple System Atrophy
1 Poewe W, Stankovic I, Halliday G, et al. Multiple system atrophy. Nature Reviews Disease Primers 2022 8:1 2022; 8: 1–21.
2 Goh YY, Saunders E, Pavey S, et al. Multiple system atrophy. Pract Neurol 2023; 23: 208–21.
3 Miki Y, Foti SC, Asi YT, et al. Improving diagnostic accuracy of multiple system atrophy: a clinicopathological study. academic.oup.comY Miki, SC Foti, YT Asi, E Tsushima, N Quinn, H Ling, JL HoltonBrain, 2019•academic.oup.com https://academic.oup.com/brain/article-abstract/142/9/2813/5530238 (accessed March 11, 2025).
4 Koga S, Aoki N, Uitti RJ, et al. When DLB, PD, and PSP masquerade as MSA. Neurology 2015; 85: 404–12.
5 Wenning GK, Stankovic I, Vignatelli L, et al. The Movement Disorder Society Criteria for the Diagnosis of Multiple System Atrophy. Movement Disorders 2022; 37: 1131–48.
6 The Neuro Network Programme. ACNR, Advances in Clinical Neuroscience and Rehabilitation 2017. https://acnr.co.uk/articles/the-neuro-network-programme/ (accessed March 11, 2025).

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MDS Abstracts - https://www.mdsabstracts.org/abstract/retrospective-analysis-of-msa-diagnosis-in-a-neuroscience-hospital/