Medline ® Abstracts for References 3,4
of 'Multiple system atrophy: Prognosis and treatment'
Progression and prognosis in multiple system atrophy: an analysis of 230 Japanese patients.
Watanabe H, Saito Y, Terao S, Ando T, Kachi T, Mukai E, Aiba I, Abe Y, Tamakoshi A, Doyu M, Hirayama M, Sobue G
Brain. 2002;125(Pt 5):1070.
We investigated the disease progression and survival in 230 Japanese patients with multiple system atrophy (MSA; 131 men, 99 women; 208 probable MSA, 22 definite; mean age at onset, 55.4 years). Cerebellar dysfunction (multiple system atrophy-cerebellar; MSA-C) predominated in 155 patients, and parkinsonism (multiple system atrophy-parkinsonian; MSA-P) in 75. The median time from initial symptom to combined motor and autonomic dysfunction was 2 years (range 1-10). Median intervals from onset to aid-requiring walking, confinement to a wheelchair, a bedridden state and death were 3, 5, 8 and 9 years, respectively. Patients manifesting combined motor and autonomic involvement within 3 years of onset had a significantly increased risk of not only developing advanced disease stage but also shorter survival (P<0.01). MSA-P patients had more rapid functional deterioration than MSA-C patients (aid-requiring walking, P = 0.03; confinement to a wheelchair, P<0.01; bedridden state, P<0.01), but showed similar survival. Onset in older individuals showed increased risk of confinement to a wheelchair (P<0.05), bedridden state (P = 0.03) and death (P<0.01). Patients initially complaining of motor symptoms had accelerated risk of aid-requiring walking (P<0.01) and confinement to a wheelchair (P<0.01) compared with those initially complainingof autonomic symptoms, while the time until confinement to a bedridden state and survival were no worse. Gender was not associated with differences in worsening of function or survival. On MRI, a hyperintense rim at the lateral edge of the dorsolateral putamen was seen in 34.5% of cases, and a 'hot cross bun' sign in the pontine basis (PB) in 63.3%. These putaminal and pontine abnormalities became more prominent as MSA-P and MSA-C features advanced. The atrophy of the cerebellar vermis and PB showed a significant correlation particularly with the interval following the appearance of cerebellar symptoms in MSA-C (r = 0.71, P<0.01, r = 0.76 and P<0.01, respectively), but the relationship between atrophy and functional status was highly variable among the individuals, suggesting that other factors influenced the functional deterioration. Atrophy of the corpus callosum was seen in a subpopulation of MSA, suggesting hemispheric involvement in a subgroup of MSA patients. The present study suggested that many factors are involved in the progression of MSA but, most importantly, the interval from initial symptom to combined motor and autonomic dysfunction can predict functional deterioration and survival in MSA.
Department of Neurology, Nagoya University Graduate School of Medicine, Japan.
Survival in multiple system atrophy.
Schrag A, Wenning GK, Quinn N, Ben-Shlomo Y
Mov Disord. 2008;23(2):294.
We here report survival in patients with multiple system atrophy (MSA) in a large, prospectively studied group of patients with MSA. Eighty-five of 100 patients were known to have died. Three patients were rediagnosed as having PD. Twenty-four patients came to autopsy, which showed MSA in 22 and idiopathic Parkinson's disease in 2. The median survival time was 8.6 and 7.3 years for men and women, respectively (hazard ratio for women was 1.49, 95% CI 0.97-2.31, P = 0.07). Except for rediagnosis as PD, no predictive factors for better survival could be identified. These data confirm the relatively poor prognosis of MSA of less than 9 years on average.
Department of Clinical Neurosciences, Royal Free&University College Medical School, London, United Kingdom.