Progressive proximal spinal and bulbar muscular atrophy of late onset

Publisher Website
Google Scholar
Abstract
Clinical, genetic and pathological findings in 2 families affected with an unusual anterior horn cell disease are described. The pathological changes in the spinal cord and skeletal muscle are similar to those of the other degenerative diseases involving the lower motor neuron. However, this disease may be distinguished by the following clinical characteristics: clinical onset in middle life, sex-linked recessive inheritance, proximal spinal and bulbar muscular weakness and atrophy, generalized fasciculations, especially prominent about the lips and chin, absence of pyramidal tract, sensory or cerebellar involvement and slowly progressive course with normal longevity.

This publication has 4 references indexed in Scilit: