| Werdnig, Guido | <person> Austrian neurologist, 1862-1919. See: Werdnig-Hoffmann disease, Werdnig-Hoffmann muscular atrophy. (05 Mar 2000) |
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| werdnig-hoffmann disease | A recessively inherited acute infantile form of motor neuropathy with proximal muscle wasting particularly of the upper extremities. The chronic childhood form may be an arrested werdnig-hoffmann disorder. (12 Dec 1998) |
| Werdnig-Hoffmann muscular atrophy | Transmitted as autosomal recessive on chromosome 5q. Progressive dysfunction of the anterior horn cells in the spinal cord and brainstem cranial nerves with profound weakness and bulbar dysfunction occurring in the first two years of life. Three groups, based on age of clinical onset, are recognised. Synonym: familial spinal muscular atrophy, Hoffmann's muscular atrophy, infantile muscular atrophy, infantile progressive spinal muscular atrophy, progressive infantile spinal muscular atrophy, Werdnig-Hoffmann disease, Werdnig-Hoffmann muscular atrophy. (05 Mar 2000) |