All issues > Volume 30(10); 1987

Case Report

J Korean Pediatr Soc. 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

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.

Keywords :Werdnig- Hoffmann disease; Kugelberg-Welander disease.