Motor Neuron Disease


The most common childhood forms of spinal muscular atrophy (SMA) are early-onset severe type 1 SMA (Werdnig-Hoffmann disease), chronic intermediate type 2 SMA, and chronic mild type 3 SMA (Kugelberg-Welander disease). Werdnig-Hoffmann disease (WHD) is an autosomal recessive, early-infantile form of spinal and bulbar muscular atrophy, sparing only the extraocular muscles. In late-infantile forms of SMA, the onset is between 3 and 24 months of age. The juvenile form of SMA (Kugelberg-Welander syndrome) has its onset between 2 and 10 years, or adolescence.


Spinal Muscular Atrophy Motor Neuron Disease Anterior Horn Anterior Horn Cell Spinal Nerve Root 
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23 Motor Neuron Disease

  1. 1.
    Weller RO, Cumming WJK, Mahan M, Ellison DW (2002) Diseases of muscle. In: Graham DI, Lantos PL (eds) Greenfield’s neuropathology, 7th edn, Vol 2. Arnold, London, pp 708–711.Google Scholar

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