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Motor Neuron Disease

Abstract

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.

Keywords

Spinal Muscular Atrophy Motor Neuron Disease Anterior Horn Anterior Horn Cell Spinal Nerve Root 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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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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© Springer 2007

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