Werdnig-Hoffmann disease


Werd·nig–Hoff·mann disease

noun \ˈvert-nik-ˈhof-ˌmän-\

Definition of WERDNIG-HOFFMANN DISEASE

: atrophy of muscles that is caused by degeneration of the ventral horn cells of the spinal cord, is inherited as an autosomal recessive trait, becomes symptomatic during early infancy, is characterized by hypotonia and flaccid paralysis, and is often fatal during childhood—called also Werdnig-Hoffmann syndrome; compare kugelberg-welander disease

Biographical Note for WERDNIG-HOFFMANN DISEASE

Werd·nig \ˈvert-ni\ Guido (1844–1919), Austrian neurologist, and Hoffmann, Johann (1857–1919), German neurologist. Independently of each other, Werdnig and Hoffmann published descriptions of Werdnig-Hoffmann disease in 1891. Hoffmann is also remembered for his 1897 descriptions of peroneal muscular atrophy and myopathy associated with hypothyroidism.

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