| A Case of Aeute Werdnig-Hoffmann Disease. |
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Y M Song1, K S Lee1, J J Shin1, S C Kang1, J K Ji2 |
1Department of Pediatrics, Seoul Red Cross Hospital, Korea.
2Department of Pathology, College of Medicine, Seoul National University, Korea. |
| Acute Werdnig-Hoffmann 병 1 례 |
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송영명1, 이경숙1, 신미자1, 강석철1, 지제근2 |
1서울 적십자병원 소아과
2서울대학교 의과대학 병리학교실 |
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| Abstract |
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Acute Werdnig-Hoffmann disease is genetically determined progressive degenerative disease of anterior spinal neuron, characterized by fasciculations of the tongue usually occuring along with flaccid paralysis of extremities with intact mentality. We experienced a 5 months old female infant, whose sister died of pneumonia at the age of 2 months, had same symptoms and signs : revealing hypotonic state with flaccid extremities only responding to painful stimuli, and paradox-ical respiration. She was alert in mental state and showing typical tongue fasciculation even in quiet state. Muscle biopsy finding was compatible with acute werdnig-Hoffmann disease and serum enzymes were normal value. |
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