title: Young-onset sporadic amyotrophic lateral sclerosis: a distinct nosological entity?
abstract: There are few reports describing young-onset amyotrophic lateral sclerosis (ALS). Age at onset is a prognostic factor in ALS, and thus it is relevant to investigate the clinical features of very young ALS patients. We describe three young-onset ALS cases and review the literature. SOD1 mutations were not identified. Our cases and 24 others from the literature indicate that young-onset ALS is characterized by slowly progressive symmetrical weakness; nevertheless, progression is variable. Young-onset ALS seems to be a distinct clinical syndrome but its aetiological background is largely unknown.
authors.affiliation: Department of Neurology, Hospital de Santa Maria, Lisbon, Portugal.
authors.forename: Liliana Olim
authors.initials: LO
authors.lastname: Gouveia
authors.forename: Mamede
authors.initials: M
authors.lastname: de Carvalho
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D017668:Age of Onset; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis* / Q000453:epidemiology* / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male