KMID : 0381219810130030087
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Journal of RIMSK 1981 Volume.13 No. 3 p.87 ~ p.91
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A Case of Werdnig-Hoffmann Disease
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Abstract
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Werdnig-Hoffmann disease is a hereditary disorder causing progressive degeneration of lower motor neurons.
A case of Werdnig-Hoffmann disease in a 18 month male is presented. He was found to hypotonia at 1 month of age. At the time of admission he showed generalized muscular weakness, paradoxical respiration and dyspnea.
Muscle biopsy and electromyographic findings were compatible with Werdnig-Hoffmann disease.
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