Werdnig-Hoffmann disease

Werdnig-Hoffmann disease

[verd′nig hôf′mun]

Etymology: Guido Werdnig, Austrian neurologist, 1844-1919; Johann Hoffmann, German neurologist, 1857-1919

a genetic disorder beginning in infancy or young childhood, characterized by progressive atrophy of the skeletal muscle resulting from degeneration of the cells in the anterior horn of the spinal cord and the motor nuclei in the brainstem. Onset occurs within the first year of life, with the condition usually apparent at birth. Symptoms include congenital hypotonia; absence of stretch reflexes; flaccid paralysis, especially of the trunk and limbs; lack of sucking ability; fasciculations of the tongue and sometimes of other muscles; and often, dysphagia. Treatment is symptomatic, and death generally occurs in early childhood, often from respiratory complications. The condition is transmitted as an autosomal-recessive trait and occurs more frequently in siblings than in successive generations. Also called familial spinal muscular atrophy, Hoffmann's atrophy, infantile spinal muscular atrophy, progressive spinal muscular atrophy of infants, Werdnig-Hoffmann paralysis. See also floppy infant syndrome.

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Werdnig-Hoffmann disease

a human disease which has a possible model in hereditary spinal muscular atrophy of Brittany spaniels.

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Werdnig-Hoffmann disease

Spinal muscle atrophy, see there