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Familial amyotrophic lateral sclerosis
Author(s) -
Eestnes Arnulf,
Mellgren Svein Ivar
Publication year - 1980
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/j.1600-0404.1980.tb01482.x
Subject(s) - amyotrophic lateral sclerosis , medicine , subclinical infection , wasting , amyotrophy , cousin , progressive muscular atrophy , atrophy , norwegian , anatomy , pediatrics , disease , pathology , archaeology , history , linguistics , philosophy
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.