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Werdnig-Hoffman disease

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Noun1.Werdnig-Hoffman disease - autosomal recessive disease in which the degeneration of spinal nerve cells and brain nerve cells leads to atrophy of skeletal muscles and flaccid paralysis; death usually occurs in early childhood
autosomal recessive defect, autosomal recessive disease - a disease caused by the presence of two recessive mutant genes on an autosome


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SMA type I, also called Werdnig-Hoffman disease, is the most severe form, which strikes infants between birth and six months old.
Types of SMA The most severe form of SMA is type I, or Werdnig-Hoffman disease.
Werdnig-Hoffman disease is a neuromuscular disorder caused by loss of anterior horn cells and motor nuclei of cranial nerves V through VIII.
 
 
 
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