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Mari Tada, MD; Osamu Onodera, MD, PhD; Masayoshi Tada, MD; Tetsutaro Ozawa, MD, PhD; Yue-Shan Piao, MD, PhD; Akiyoshi Kakita, MD, PhD; Hitoshi Takahashi, MD, PhD; Masatoyo Nishizawa, MD, PhD

Arch Neurol. 2007;64(2):256-260.

Background  Multiple system atrophy (MSA) is diverse in clinical phenotype, disease progression, and prognosis. Sudden death is a leading cause of death in patients with MSA.

Objective  To determine what clinical factors affect the progression and survival prognosis of those with MSA.

Design  A retrospective review of the medical records of 49 consecutive Japanese patients with pathologically confirmed MSA (29 men and 20 women; mean ± SD age at onset, 59.8 ± 6.5 years). Cox proportional hazards models were used to compare the risks of being in a wheelchair-bound state, being in a bedridden state, and having a shorter survival.

Results  Thirty-one patients were diagnosed as having cerebellar type MSA, and 18 were diagnosed as having parkinsonian type MSA. Twenty-nine patients with cerebellar type MSA and 17 patients with parkinsonian type MSA had autonomic dysfunction. The median times from disease onset to being in a wheelchair-bound state, being in a bedridden state, death, and the development of autonomic dysfunction were 3.5, 5.0, 7.0, and 2.5 years, respectively. Patients with an early development of autonomic dysfunction (within 2.5 years from the onset of MSA) had significantly higher risks of being in a wheelchair-bound state (multivariate-adjusted hazard ratio [HR], 4.32; 95% confidence interval [CI], 2.04-9.15), being in a bedridden state (HR, 3.87; 95% CI, 1.77-8.48), having a shorter survival (HR, 3.40; 95% CI, 1.61-7.15), and sudden death (HR, 7.22; 95% CI, 1.49-35.07).

Conclusion  The early development of autonomic dysfunction is an independent predictive factor for rapid disease progression and shorter survival in patients with MSA.

Author Affiliations: Departments of Neurology (Drs Mari Tada, Masayoshi Tada, Ozawa, and Nishizawa) and Pathology (Drs Piao and Takahashi), and Departments of Molecular Neuroscience (Dr Onodera) and Pathology Neuroscience (Dr Kakita), Resource Branch for Brain Disease Research, Brain Research Institute, Niigata University, Niigata, Japan.

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