Kennedy Disease Showing Prominent Remyelinating Features of
Sural Nerve

J Korean Neurol Assoc > Volume 28(2); 2010 > Article

Journal of the Korean Neurological Association 2010;28(2): 115-118.

장딴지신경 조직검사에서 재수초화 신경섬유가 현저하게 증가한 케네디병

김지영, 김승민 a 박기덕 김태승 b 선우일남 a

이화여자대학교 의학전문대학원 신경과학교실, 연세대학교 의과대학 신경과학교실 a , 병리학교실 b

Kennedy Disease Showing Prominent Remyelinating Features of Sural Nerve

Jee Young Kim

Department of Neurology, Ewha Womans University School of Medicine, Department of Neurology a and Pathology b , Yonsei University School of Medicine, Seoul, Korea

Abstract

We report a 55-year-old man with chronic weakness of both legs with recently experienced nasal voice. Despite the absence of sensory symptoms, electrophysiologic studies revealed the presence of sensorimotor polyneuropathy. A sural-nerve biopsy showed remarkable reduction of large myelinated fibers with prominent remyelination. Intravenous immunoglobulin was administered due to suspected chronic demyelinating neuropathy, but had no effect. Abnormal trinucleotide-repeat expansion of the androgen receptor gene was subsequently detected in both the patient and his family. These observation indicate that prominent remyelinating features are not necessarily indicative of demyelinating neuropathy. Key Words: Bulbospinal atrophy, X-linked disease, Sural nerve