RESUMEN

A family in which 9 persons in three generations suffered from "pure" Strümpell's disease is described. The disease made is debut in early childhood and was characterized by slightly marked intrafamilial polymorphism. An electromyographic study was made. It has been proved that the pattern described is a "pure" disease variety inherited by the autosomal dominant type, with an early development of the clinical manifestations and slow progress of motor disturbances (up to 25 years). The nosology of the given disease pattern is under discussion.

Asunto(s)

Neuropatía Hereditaria Motora y Sensorial/genética , Paraplejía Espástica Hereditaria/genética , Adolescente , Adulto , Factores de Edad , Anciano , Brazo/inervación , Niño , Preescolar , Electromiografía , Humanos , Lactante , Pierna/inervación , Persona de Mediana Edad , Músculos/inervación , Músculos/fisiopatología , Linaje , Reflejo de Estiramiento/fisiología , Paraplejía Espástica Hereditaria/diagnóstico , Paraplejía Espástica Hereditaria/fisiopatología , Factores de Tiempo