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A Case of Werdnig Hoffmann Disease.

Journal of the Korean Pediatric Society 1985;28(7):726-730.
Published online July 31, 1985.
A Case of Werdnig Hoffmann Disease.
Woo Yeong Chung1, Cheol Lee1, Tai Gyu Hwang1, In Soon Park1, Soon Young Lee1, Jong Eun Joo2
1Department of Pediatrics, Inje Medical College, Paik Hospital, Pusan, Korea
2Department of Pathology, Inje Medical College, Paik Hospital, Pusan, Korea
Werdnig Hoffmann 병 1례
정우영1, 이 철1, 황태규1, 박인순1, 이순용1, 주정은2
1인제의과대학 부산백병원 소아과학교실
2인제의과대학 부산백병원 병리학교실
We experienced a case of Werdnig Hoffmann disease in a 1 year 5 month old female child with the complaint of progressive muscular weakness on both lower extremities. Her development had been almost normal since birth and she could stand alone briefly at 12 months of age, but progressive muscular weakness had been apparent since then. Diagnosis was based on clinical pictures, EMG and muscle biopsy. A review of literatures was also attempted briefly.
Key Words: Werdnig Hoffmann disease, Progressive muscular weakness

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