Progressive Muscular Atrophy Definition
Progressive Muscular Atrophy: A group of disorders marked by progressive degeneration of motor neurons in the spinal cord resulting in weakness and muscular atrophy, usually without evidence of injury to the corticospinal tracts. Diseases in this category include Werdnig-Hoffmann disease and later onset SPINAL MUSCULAR ATROPHIES OF CHILDHOOD, most of which are hereditary. (Adams et al., Principles of Neurology, 6th ed, p1089)
Synonym: x-linked bulbo-spinal atrophy, spinal muscular atrophy, myelopathic muscular atrophy, adult spinal muscular atrophy, kennedy syndrome, distal spinal muscular atrophy, bulbospinal neuronopathy, scapuloperoneal form spinal muscular atrophy, scapuloperoneal form of spinal muscular atrophy, progressive proximal myelopathic muscular atrophy, progressive myelopathic muscular atrophy, oculopharyngeal spinal muscular atrophy.