Familial amyotrophic lateral sclerosis: features of multisystem degeneration.

摘要: Two sibling cases of familial amyotrophic lateral sclerosis (ALS) revealed degerneration usually associated with other systemic degenerative disorders. The changes in the 41-year-old sister were compatible those reported ALS affecting upper and lower motor neurons, posterior columns, spinocerebellar tracts. 45-year-old more wide-spread involving not only neuron systems, but also proprioceptive, general somatic afferent systems. Intracytoplasmic hyaline inclusions observed oculomotor nuclei. Clinical manifestations urinary disturbance imparirment seldom seen sporadic interpreted to be due unusual distribution morbid process. These pathologic findings suggest that may a multisystemic disorder, frequently tracts, occasionally systems as well.