Spinal 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)

Also Known As:

Progressive Muscular Atrophy; Adult Spinal Muscular Atrophy; Distal Spinal Muscular Atrophy; Spinal Muscular Atrophy, Distal; Muscular Atrophy, Spinal; Adult-Onset Spinal Muscular Atrophy; Amyotrophy, Neurogenic Scapuloperoneal, New England Type; Hereditary Motor Neuronopathy; Muscular Atrophy, Adult Spinal; Myelopathic Muscular Atrophy; Myelopathic Muscular Atrophy, Progressive; Progressive Myelopathic Muscular Atrophy; Progressive Proximal Myelopathic Muscular Atrophy; Proximal Myelopathic Muscular Atrophy, Progressive; Scapuloperoneal Spinal Muscular Atrophy; Spinal Amyotrophy; Spinal Muscular Atrophy, Oculopharyngeal; Spinal Muscular Atrophy, Scapuloperoneal; Spinal Muscular Atrophy, Scapuloperoneal Form; Adult Onset Spinal Muscular Atrophy; Amyotrophies, Spinal; Amyotrophy, Spinal; Atrophies, Progressive Muscular; Atrophy, Myelopathic Muscular; Atrophy, Progressive Muscular; Atrophy, Spinal Muscular; Bulbospinal Neuronopathies; Hereditary Motor Neuronopathies; Motor Neuronopathies, Hereditary; Motor Neuronopathy, Hereditary; Muscular Atrophies, Progressive; Muscular Atrophy, Myelopathic; Muscular Atrophy, Progressive; Neuronopathies, Bulbospinal; Neuronopathies, Hereditary Motor; Neuronopathy, Bulbospinal; Neuronopathy, Hereditary Motor; Progressive Muscular Atrophies; Spinal Amyotrophies; Bulbospinal Neuronopathy; Oculopharyngeal Spinal Muscular Atrophy; Scapuloperoneal Form of Spinal Muscular Atrophy

Nervous System Diseases: 14178
- Neurodegenerative Diseases: 42472
  - Motor Neuron Disease: 1823
    - Spinal Muscular Atrophy: 2240
- Central Nervous System Diseases: 4213
  - Spinal Cord Diseases: 4314
    - Spinal Muscular Atrophy: 2240
- Neuromuscular Diseases: 2005
  - Motor Neuron Disease: 1823
    - Spinal Muscular Atrophy: 2240

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Drug/Important Bio-Agent (IBA)