Canadian Medical Guide > Diseases > Nervous System Diseases > Central Nervous System Diseases > Spinal Cord Diseases > Muscular Atrophy, Spinal Terms and Definitions




Muscular Atrophy, Spinal

Medical Definition: 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)
Guide Notes: MUSCULAR ATROPHY, SPINAL, INFANTILE see SPINAL MUSCULAR ATROPHIES OF CHILDHOOD is also available
Also Called: Bulbospinal Neuronopathy,Distal Spinal Muscular Atrophy,Myelopathic Muscular Atrophy,Progressive Muscular Atrophy,Oculopharyngeal Spinal Muscular Atrophy,Progressive Proximal Myelopathic Muscular Atrophy,Scapuloperoneal Form of Spinal Muscular Atrophy
Previously Indexed: Muscular Atrophy (1966-1987),Spinal Cord Diseases (1966-1987)
Muscular Atrophy, Spinal Categories.
Canadian Medical Guide Definition Spinal Muscular Atrophies of Childhood - A group of recessively inherited diseases that feature progressive muscular atrophy and hypotonia. They are classified as type I (Werdnig-Hoffman disease), type II (intermediate form), and type III (Kugelberg-Welander disease). Type I is fatal in infancy, type II has a late infantile onset and is associated with survival into the second or third decade. Type III has its onset in childhood, and is slowly progressive. (J Med Genet 1996 Apr:33(4):281-3)

Muscular Atrophy, Spinal Medical Definitions and Terms

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