Abstract: Objective: To study the clinical features of amyotrophic lateral sclerosis(ALS),and provide clinical data for the further research in pathogenesis and the rapy. Methods: We retrospectively reviewed the records of 105cases of ALS in our hospital during recent twenty years. Clinical characteristics、symptom、sign and data of laboratory examination on the patients were statistical analyzed. Results: The mean age at oneset was 42. 7years,earlier than the west country. The incidence of ALS increased with age until sixty years old. Sex ratio (male/female) was 2:1. Patient with initial bulbar symptoms tended to be predominant in female and more older than those with initial symptom in upper or lower extremities. ALS develops from the initial location to the near motor neuron vertically or horizontally. Conclusions: ALS is an age-related、male predominant disease. The development of the ALS follows some patterns.