En grupp sjukdomstillstånd som kännetecknas av fortskridande nedbrytning av motornervceller i ryggmärgen, vilket leder till svaghet och muskelförtvining, oftast utan tecken på skada på de kortikospinala områdena (ryggmärgens ytterskikt). Till sjukdomar av denna art hör bl a Werdnig-Hoffmanns sjukdom och sent debuterande spinala muskelatrofier i barndomen. De flesta är ärftliga.
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)
Bulbospinal neuropati — Distal spinal muskelatrofi — Ärftlig motorisk neuropati — Progressiv muskelatrofi
Atrophy, Spinal Muscular — Spinal Amyotrophy — Amyotrophies, Spinal — Amyotrophy, Spinal — Spinal Amyotrophies — Spinal Muscular Atrophy — Distal Spinal Muscular Atrophy — Spinal Muscular Atrophy, Distal — Hereditary Motor Neuronopathy — Hereditary Motor Neuronopathies — Motor Neuronopathies, Hereditary — Motor Neuronopathy, Hereditary — Neuronopathies, Hereditary Motor — Neuronopathy, Hereditary Motor — Scapuloperoneal Form of Spinal Muscular Atrophy — Spinal Muscular Atrophy, Scapuloperoneal Form — Spinal Muscular Atrophy, Scapuloperoneal — Amyotrophy, Neurogenic Scapuloperoneal, New England Type — Scapuloperoneal Spinal Muscular Atrophy — Oculopharyngeal Spinal Muscular Atrophy — Spinal Muscular Atrophy, Oculopharyngeal — Progressive Muscular Atrophy — Atrophies, Progressive Muscular — Atrophy, Progressive Muscular — Muscular Atrophies, Progressive — Muscular Atrophy, Progressive — Progressive Muscular Atrophies — Progressive Myelopathic Muscular Atrophy — Myelopathic Muscular Atrophy, Progressive — Progressive Proximal Myelopathic Muscular Atrophy — Proximal Myelopathic Muscular Atrophy, Progressive — Bulbospinal Neuronopathy — Bulbospinal Neuronopathies — Neuronopathies, Bulbospinal — Neuronopathy, Bulbospinal — Myelopathic Muscular Atrophy — Atrophy, Myelopathic Muscular — Muscular Atrophy, Myelopathic — Adult-Onset Spinal Muscular Atrophy — Adult Onset Spinal Muscular Atrophy — Muscular Atrophy, Adult Spinal — Adult Spinal Muscular Atrophy