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KMID : 0378019810240090079
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New Medical Journal
1981 Volume.24 No. 9 p.79 ~ p.84
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A Case of Werdnig-Hoffmann Disease
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Abstract
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A 21 day old male patient with Werdnig-Hoffmann disease was presented with autopsy finding.
He had histroy of deaths of 2 siblings. At birth, he had lethagy, weak crying, cyanosis of extremities, meconium staining skin, nail & umbilical cord, frog-like position, absence .of Moro reflex and paradoxical respiration. He survived until 12 days of life.
Electromyography showed patterns compatible with anterior horn cell disease.
Autopsy showed characteristic loss of large anterior horn cell of spinal cord & neuronophagia, gliosis of motor nerve cell. Muscle biopsy showed small round fibers with grouped atrophy.
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KEYWORD
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