유년기 근위축성측삭경화증 2예

Abstract

Juvenile amyotrophic lateral sclerosis (ALS) is a rarely occurring chronic motor neuron disease, characterized by combined upper and lower motor neuron symptoms /signs with the onset before 25 years old. It is known that the inheritance is either autosomal dominant, autosomal recessive or sporadic. We report 2 cases of juvenile ALS without family history who showed severe weakness in distal limbs with definite upper motor neuron signs.