All issues > Volume 35(9); 1992

Case Report

J Korean Pediatr Soc. 1992;35(9):1263-1266. Published online September 15, 1992.

A Case of Kugelberg-Welander Syndrome

Sun Young SY Lee¹, Myung Ik MI Lee¹, Soon Wha SW Kim¹, Don Hee DH Ahn¹, Je Geun JG Chi²

¹Department of Pediatrics, National Medical Center, Seoul, Korea
²Department of Pathology, Seoul National University College of Medicine, Seoul, Korea

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.

Keywords :Kugelberg-Welander Syndrome