Objective: We aimed to assess the disease severity, non-motor symptoms and cardiac autonomic dysfunction in MSA phenotypes.

Background: Multiple system atrophy (MSA) is an adult-onset, sporadic, neurodegenerative disorder with parkinsonian (MSA-P) and cerebellar (MSA-C) phenotypes. There is limited data related to disease severity, non-motor symptom burden and autonomic dysfunction in Indians affected with probable MSA-P and MSA-C.

Method: This is a prospective cohort study. The study population comprised of 60 probable MSA patients (MSA-P=18; MSA-C=42). Clinical parameters, Unified MSA rating scale (UMSARS), Pittsburgh sleep quality index (PSQI), REM behavior disorder screening questionnaire (RBDSQ) and tilt table test were the study tools.

Results: Mean age of onset of MSA was 51±7 years for MSA-P and 52±6 years for MSA-C subgroups. Median disease duration was 2 years in both subgroups. Mean UMSARS I (historical review) was 17.7±5.2 in MSA-P and 18.2±5.5 in MSA-C patients. Mean UMSARS II (motor examination) was 23.1±7.6 in MSA-P and 22.3±6.2 in MSA-C patients. Among the non-motor symptoms, 88.9% MSA-P and 95.2% MSA-C patients had bladder disturbances; 92.9% MSA-P and 87.5% MSA-C males had erectile dysfunction; 77.8% MSA-P and 64.3% MSA-C patients had constipation; 88.9% MSA-P and 95.2% MSA-C patients had REM behavior disorder (RBDSQ score >5), 100% MSA-P and MSA-C patients had poor sleep quality (PSQI score>5); 50% MSA-P and 38.1% MSA-C patients reported postural symptoms. Tilt table test showed orthostatic hypotension in 88.9% MSA-P and 66.7% MSA-C patients. Interestingly, only 37.5% MSA-P and 42.9% MSA-C patients were symptomatic during the test. 50% MSA-P and 35.7% MSA-C patients had both supine hypertension and orthostatic hypotension. Median drop in BP was 39/23 mmHg in MSA-P and 41/25 mmHg in MSA-C patients. There was no statistically significant difference in any of the above parameters between MSA-P and MSA-C patients.

Conclusion: MSA-P and MSA-C patients exhibit a distinct motor phenotype. However, both the groups were similar in terms of disease severity, non-motor symptoms and cardiac autonomic dysfunction. A greater percentage of patients with both MSA phenotypes had disabling non-motor symptoms. Compensatory supine hypertension and cerebral auto-regulation might be responsible for majority of MSA patients being asymptomatic, despite having orthostatic hypotension.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/motor-and-non-motor-symptom-burden-in-parkinsonian-and-cerebellar-phenotypes-of-probable-msa/