Familial amyotrophic lateral sclerosis. Report of a family with predominant upper limb pareses and late onset

Hestnes, A.; Mellgren, S.I.

Acta Neurologica Scandinavica 61(3): 192-199


ISSN/ISBN: 0001-6314
PMID: 7395463
DOI: 10.1111/j.1600-0404.1980.tb01482.x
Accession: 028323973

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Familial occurrence of neuromuscular disease similar to sporadic amyotrophic lateral sclerosis has been reported from several countries. A Norwegian family with muscular wasting in men and women of three generations is described. The propositus and his father's sister were examined, as well as a second cousin of the propositus. The disease was characterized by late onset, predominantly upper limb peripheral pareses, and "pyramidal" signs in the lower extremities. Although peripheral neuromuscular affection in the lower limbs tended to be subclinical, chonchotome biopsies from the tibialis anterior muscle showed neurogenic atrophy in all three cases.