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A Case of Werdnig-Hoffmann Disease.

Journal of the Korean Pediatric Society 1987;30(10):1170-1175.
Published online October 31, 1987.
A Case of Werdnig-Hoffmann Disease.
C H Koo1, G J Lee1, H G Jung1, U J Jung1, B Huh2, M H Huh2
1Department of Pediatrics, Kosin Medical College, Pusan, Korea
2Department of Pathology, Kosin Medical College, Pusan, Korea
Werdning-Hoffmann병 (Kugelberg-Welander형) 의 1 례
구철회1, 이기지1, 정현기1, 정윤주1, 허 방2, 허만하2
1고신의대부속 복음병원 소아과
2고신의대부속 복음병원 병리학
Abstract
A case of Werdnig-Hoffmann disease (Kugelberg-Welander type), rare neuromuscular disease in childhood, was presented in a 11 years old Korean boy. The patient was well until 3yrs of age, when he was found to have muscle weakness of both upper and lower extremity. At the time of admission he showed emaciated, and flaccid apperance with tongue fasciculation. Diagnosis was established by clinical teature, eletromyography, and muscle biopsy. We report this case with brief review of some related literatures.
Key Words: Werdnig- Hoffmann disease, Kugelberg-Welander disease.


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