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We examined the characteristic clinical features of one family of familial amyotrophic lateral sclerosis (FALS) with a His46Arg mutation in the enzyme Cu/Zn superoxide dismutase-1 (SOD1). The disease duration for this family was 18.1+/-13.2 (mean+/-S.D.) years, with the age at onset being 39.7+/-10.5 years old (mean+/-S.D.). The initial sign was distal weakness of the unilateral lower limb, extending...
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