Medical Dictionary
Main Entry:
Werd·nig–Hoff·mann disease Pronunciation:
'vert-nik-'hof-"män- Function:
noun : atrophy of muscles that is caused bydegeneration 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-WELANDERDISEASE Werdánig /'vert-nik,/ Guido (1844–1919), Austrian neurologist, and
Hoffmann,
Johann (1857–1919), Germanneurologist. 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 muscularatrophy and myopathy associated with hypothyroidism.
