All issues > Volume 32(2); 1989

Original Article

J Korean Pediatr Soc. 1989;32(2):289-296. Published online February 28, 1989.

A Case of Werdnig-Hoffmann Disease.

Ok Ji Hwang¹, Kon Hee Lee¹, Ha Joo Choi¹, Woo Kap Chung¹

¹Department of Pediatrics, College of Medicine, Hallym University, Seoul, Korea

Received: June 8, 1988;  Accepted: August 6, 1988.

Abstract

Acute Werdnig-Hoffmann disease is heredofamilial degeneration of anterior horn cells in the spinal cord and of motor nuclei in the brain stem, which is clinically characterized by fasciculations of the tongue, paradoxical respiration, bell shaped thorax, frog-leg posture and intact mentality. We experienced an 8 month old female infant with acute Werdnig-Hoffmann disease. We report the case with brief review of some related literatures.

Keywords :Acute Werdnig-Hoffmann Disease