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 |
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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.
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Key Words:
Werdnig- Hoffmann disease, Kugelberg-Welander disease.
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