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  • Title: [Progression rate of MUNE at diagnosis: a prognostic factor of survival in patients with amyotrophic lateral sclerosis].
    Author: Liu XX, Fan DS, Zhang J, Zheng JY, Zhang S.
    Journal: Beijing Da Xue Xue Bao Yi Xue Ban; 2008 Dec 18; 40(6):629-32. PubMed ID: 19088836.
    Abstract:
    OBJECTIVE: To identify the correlation between the progression rate of motor unit number estimate (MUNE) at diagnosis and survival. METHODS: We included 129 patents with amyotrophic lateral sclerosis (ALS) enrolled in our hospital from January 2002 to December 2005. We recorded clinical features, ALS functional rating scale (ALSFRS), forced vital capacity (FVC) and electrophysiological data at diagnosis. The patients were monitored every 3 months from visit to death or tracheotomy. RESULTS: Mean age at onset was (52.19+/-11.00) years. The median survival time from symptom onset was 45.71 months (95% CI=35 to 51). In univariate analysis of Kaplan-Meier method, outcome was significantly related to progression rate of MUNE (P<0.05). In the Cox multivariate model, progression rate of MUNE was a significant prognostic factor of survival (P<0.01). Using 2.9/month as cutoff point, the value beyond this point at diagnosis tended to have a shorter survival (P<0.05). A 1-unit decrease in the progression rate of MUNE was associated with a 34.2% decrease risk of death. CONCLUSION: Progression rate of MUNE at diagnosis can provide the information of disease progression at one particular point, which may help to stratify patients and affect survival. It is the strongest prognostic factor of survival in patients with ALS compared with age, onset site and time from onset to diagnosis.
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