Movement disorders clinical practice · 2025
Rapid Progression and Shorter Survival in Indians Affected with Probable MSA: A Prospective Cohort Study.
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Rukmani Malligurki Raghurama, Yadav Ravi, Bhaskarapillai Binukumar, Pal Pramod Kumar, Sathyaprabha Talakad Narasappa
Аннотация
BACKGROUND: Multiple system atrophy (MSA) is a fatal alpha-synucleinopathy characterized by variable combinations of parkinsonism and autonomic and cerebellar dysfunctions.
OBJECTIVE: We aimed to prospectively study the natural history of probable MSA, including cardiovascular autonomic dysfunction (CAD) and serum alpha-synuclein.
METHODS: Sixty probable MSA patients (MSA-P = 19; MSA-C = 41) and 30 age-and-gender-matched healthy controls were recruited. Disease severity (UPDRS III, UMSARS I, II, IV), CAD, and serum alpha-synuclein (ELISA) were assessed at baseline, 6-month (6 M-FU), and 12-month (12 M-FU) follow-ups. Survival probabilities (Kaplan-Meir analysis) and prognostic factors (Cox proportional hazards analysis) were assessed at the study's end period.
RESULTS: Total serum alpha-synuclein was higher in MSA patients than controls (P < 0.001). There was severe CAD in MSA patients at baseline (P < 0.001), which further deteriorated at 12 M-FU (P < 0.01). A greater proportion of MSA-P patients had a dopaminergic benefit than MSA-C patients (P < 0.001). CAD had a positive correlation with serum alpha-synuclein and disease severity. UPDRS III and UMSARS II scores were higher at follow-ups (P < 0.001). Disease progression was more rapid in MSA-C patients than MSA-P patients (P < 0.001). The median time to one-person support for walking and activities of daily living (ADL), wheelchair support, bedbound state, and death were 27, 48, 66, and 70 months, respectively. The probabilities of needing one-person support and wheelchair support were lower in MSA-P patients than MSA-C (P = 0.005; P = 0.03), whereas the probabilities of bedbound state and death were similar.
CONCLUSIONS: Probable MSA patients had a rapid progression, autonomic failure, and a shorter survival of 5.8 years in this Asian-Indian cohort compared to the other MSA cohorts worldwide (9-10 years). MSA-P patients had a better prognosis than MSA-C. Elevated serum alpha-synuclein and its positive correlation with CAD provide evidence for its role as a potential biomarker.
OBJECTIVE: We aimed to prospectively study the natural history of probable MSA, including cardiovascular autonomic dysfunction (CAD) and serum alpha-synuclein.
METHODS: Sixty probable MSA patients (MSA-P = 19; MSA-C = 41) and 30 age-and-gender-matched healthy controls were recruited. Disease severity (UPDRS III, UMSARS I, II, IV), CAD, and serum alpha-synuclein (ELISA) were assessed at baseline, 6-month (6 M-FU), and 12-month (12 M-FU) follow-ups. Survival probabilities (Kaplan-Meir analysis) and prognostic factors (Cox proportional hazards analysis) were assessed at the study's end period.
RESULTS: Total serum alpha-synuclein was higher in MSA patients than controls (P < 0.001). There was severe CAD in MSA patients at baseline (P < 0.001), which further deteriorated at 12 M-FU (P < 0.01). A greater proportion of MSA-P patients had a dopaminergic benefit than MSA-C patients (P < 0.001). CAD had a positive correlation with serum alpha-synuclein and disease severity. UPDRS III and UMSARS II scores were higher at follow-ups (P < 0.001). Disease progression was more rapid in MSA-C patients than MSA-P patients (P < 0.001). The median time to one-person support for walking and activities of daily living (ADL), wheelchair support, bedbound state, and death were 27, 48, 66, and 70 months, respectively. The probabilities of needing one-person support and wheelchair support were lower in MSA-P patients than MSA-C (P = 0.005; P = 0.03), whereas the probabilities of bedbound state and death were similar.
CONCLUSIONS: Probable MSA patients had a rapid progression, autonomic failure, and a shorter survival of 5.8 years in this Asian-Indian cohort compared to the other MSA cohorts worldwide (9-10 years). MSA-P patients had a better prognosis than MSA-C. Elevated serum alpha-synuclein and its positive correlation with CAD provide evidence for its role as a potential biomarker.