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Background: No prospective analyses of clinical characteristics and prognostic factors on amyotrophic lateral sclerosis(ALS) have been performed in Korea.Methods: The prognosis of 227 sporadic ALS patient with El-Escorial-compatible criteria were evaluated using the serial Korean Amyotrophic Lateral Sclerosis Functional Rating Scale-revised (K-ALSFRS-R), and the progression rate of K-ALSFRS-R (ΔFS=48-ALSFRS-R at the time of diagnosis). Their clinical characteristics and survival times were also analyzed. The endpoint was defined as either time to death or the tracheostomy state. The effects of individual prognostic factors on disease progression were assessed with the Kaplan-Meier life-table method and multivariate Cox proportional regression analysis. The declining ALSFRS-R curve, epidemiological data, and neuropsychological tests were evaluated.Results: Among 331 motor neuron disease (MND) patients, 227 were finally diagnosed as sporadic ALS. The mean age at onset in 227 sporadic ALS patients was 50.7 years (range 19-76 years). Analysis of sequential ALSFRS-R data revealed that a mean ΔFS of 0.73/month and a high ΔFS score were related with early death or a tracheostomy state. Neuropsychological testing revealed nonmotor symptoms, and especially cognitive impairment, had developed in half of our patients.Conclusions: Our data suggest that ΔFS is valuable parameter for evaluating prognosis and clinical course of ALS patients, and clinical characteristics obtained from this study would be informative data for understanding epidemiology of ALS in Korea.J Korean Neurol Assoc 29(1):16-24, 2011??? ????????? ????????????????????? ????????????????????????????????? ????????? ????????? ???????????? ??????(??????????????????: A091049)