Journal of the Korean Pediatric Society 1985;28(8):823-828.
Published online August 31, 1985.
Acute Werdnig-Hoffmann Diseases in Siblings.
Y D Lee, Y W Lee, J H Lee, Y H Chung
Department of Pediatrics, Chungnam National University, College of Medicine
형제에서 발생된 급성 Werdnig-Hoffmaim씨 병
이영두, 이양원, 이재호, 정용헌
충남대학교 의과대학 소아과학교실
Acute Werdnig-Hoffmann diseases being the most common cause of floppy infant, in two male siblings were presented. The patients appeared characteristic clinical findings suggestive of spinal atrophy at 4 weeks and 2 months and all died about 8 months old. Previous expired another elder-brother was also related to this disease. Diagnosis was estabilished by clinical & pathologic findings. This is the autosomal dominant trait case of acute Werdnig-Hoffmann disease.
Key Words: Werdnig Hoffmann disease, Muscle biopsy

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