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A Case of Kugelberg-Welander Syndrome

Journal of the Korean Pediatric Society 1992;35(9):1263-1266.
Published online September 15, 1992.
A Case of Kugelberg-Welander Syndrome
Sun Young Lee1, Myung Ik Lee1, Soon Wha Kim1, Don Hee Ahn1, Je Geun Chi2
1Department of Pediatrics, National Medical Center, Seoul, Korea
2Department of Pathology, Seoul National University College of Medicine, Seoul, Korea
Kugelberg-Welander 증후군 1례
이선영1, 이명익1, 김순화1, 안돈희1, 지제근2
1국립의료원 소아과
2서울대학교 의과대학 병리학교실
Abstract
We have experienced a 6 year-old female patient who had suffered from progressive muscular atrophy with weakness and loss of voluntary motor action since 3 years of age. She was diagnosed as Kugelberg-Welander syndrome by EMG and muscle bio. Multiple polyphasic motor unit potentials were revealed on EMG and grouped muscle atrophy without fibrosis or degenerative change was found on muscle biopsy. So we report this case with related literature review.
Key Words: Kugelberg-Welander Syndrome


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