Multiple system atrophy: Prognostic indicators of survival

Abstract

Background Neurologic and autonomic presentation in multiple system atrophy (MSA) may predict early mortality. Quantification of early autonomic failure as mortality predictor is lacking. Methods Early neurologic and autonomic clinical features were retrospectively reviewed in 49 MSA cases (median age at onset, 56.1 years; 16 women) confirmed by autopsy at Mayo Clinic. When available, the 10-point composite autonomic severity score derived from the autonomic reflex screen provided quantification of the degree of autonomic failure and thermoregulatory sweat test quantitated body surface anhidrosis. Results Symptoms at onset were autonomic in 50%, parkinsonian in 30%, and cerebellar in 20% of cases. Survival (median [95% confidence interval]) was 8.6 [6.7–10.2] years. Survival was shorter in patients with early laboratory evidence of generalized (composite autonomic severity score ≥ 6) autonomic failure (7.0 [3.9–9.8] vs. 9.8 [4.6–13.8] years; P=0.036), and early requirement of bladder catheterization (7.3 [3.1–10.2] vs. 13.7 [8.5–14.9] years; P=0.003) compared to those without these clinical features. On Cox proportional analysis, prognostic indicators of shorter survival were older age at onset (hazard ratio [95% confidence interval], 1.04 [1.01–1.08]; P=0.03), early requirement of bladder catheterization (7.9 [1.88–38.63]; P=0.004) and early generalized (composite autonomic severity score ≥ 6) autonomic failure (2.8 [1.01–9.26]; P=0.047). Gender, phenotype, and early development of gait instability, aid-requiring ambulation, orthostatic symptoms, neurogenic bladder or significant anhidrosis (thermoregulatory sweat test ≥ 40%) were not indicators of shorter survival. Conclusions Our data suggests that early development of severe generalized autonomic failure more than triples the risk of shorter survival in patients with MSA.

Epidemiology of Multiple System Atrophy in Hokkaido, the Northernmost Island of Japan

“…Our study showed poor survival prognosis for patients with higher ages at disease onset, and this result was consistent with that of previous studies [15,16]. Our study…”

18F-FDG PET Is an Early Predictor of Overall Survival in Suspected Atypical Parkinsonism

“…Thereby we demonstrate that PET is a prognostic marker on its own. However, the good agreement between the disease-specific survival data of the present PET study and earlier postmortem studies also provides indirect support to the notion that metabolic patterns are valuable means for differential diagnosis (2)(3)(4)(5)7,13).…”

“…APSs are characterized by a rapid progression to disability and death. According to clinical and clinicopathologic studies, MSA, PSP, and CBD share a comparably short survival time of about 7-8 y from symptom onset or less than 3-4 y from clinical diagnosis (1)(2)(3)(4)(5)(6)(7)(8). Survival in PD is distinctly better: although some population-based studies did not find higher mortality (9), others convincingly demonstrated an increased age-adjusted mortality in PD (10,11).…”

“…Survival in PD is distinctly better: although some population-based studies did not find higher mortality (9), others convincingly demonstrated an increased age-adjusted mortality in PD (10,11). As in APS (1,2,5,8), higher age at onset is also associated with a higher PD mortality (9,10), but median survival time was still 10.3 y in a recent population-based cohort with a high average age of 70 y at diagnosis (9). The cumulative incidence of PD with dementia (PDD) increases with age and disease duration up to 80%-90% (12), being associated with a strong increase in mortality (9)(10)(11).…”

Clinical Features in a Danish Population-Based Cohort of Probable Multiple System Atrophy Patients

“…The symptoms include an array of additional clinical features, including pyramidal, respiratory, and behavioral symptoms [2] . MSA patients have a poor prognosis, with a reported survival of 6-9 years from disease onset [3][4][5] .…”