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- W2726514908 abstract "OBJECTIVE: To investigate the presence of naturally-occurring prognostic subgroups within the ALS population and to identify the phenotypic markers for any identified subgroups. BACKGROUND: ALS is a neurodegenerative disorder characterized by a highly variable rate of motor progression. Identification of reliable prognostic predictors would facilitate appropriate timing of medical interventions and effective patient stratification in clinical trials. DESIGN/METHODS: A population-based, case-control longitudinal study design was used.The motor and cognitive aspects of the disease were prospectively documented in a large population-based sample of ALS patients. Neuropsychological performance of ALS patients was compared to age, sex, and education matched healthy controls. Cluster analysis was applied to the motor data (survival time and prospectively estimated rates of motor progression) to identify prognostic subgroups. This was followed by an investigation of the characteristics and predictors of these subgroups. RESULTS: Cluster analysis of 88 patients with no evidence of C9orf72 repeat expansion suggested the presence of three prognostic subgroups, with the following median survival times: (1) 15 month (95%CI 11.6-18.4); (2) 20 months (95%CI 17.4-22.6); and (3) 60 months (95%CI 27.5-92.5, p CONCLUSIONS: Interrogation of a data-set generated using a deeply phenotyped ALS patient cohort suggests the presence of three prognostic subgroups within the C9orf72 negative population. Clinical and neuropsychological data gathered at the first evaluation of ALS patients may reliably predict poor prognosis. Supported by: Health Seventh Framework Programme, the Health Research Board and Research Motor Neuron. Disclosure: Dr. Elamin has nothing to disclose. Dr. Bede has nothing to disclose. Dr. Byrne has nothing to disclose. Dr. Pender has nothing to disclose. Dr. Hardiman has received personal compensation for activities with Ono Pharmaceutical, Sanofi-Aventis Pharmaceuticals, Inc., Biogen Idec, Merck Serono, and Schering. Dr. Hardiman has received personal compensation in an editorial capacity for the ALS Journal." @default.
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- W2726514908 date "2013-02-12" @default.
- W2726514908 modified "2023-09-24" @default.
- W2726514908 title "Naturally-Occurring Prognostic Subgroups within the ALS Population: A Data-Led Approach (P07.078)" @default.
- W2726514908 hasPublicationYear "2013" @default.
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