Kennedy Disease Showing Prominent Remyelinating Features of Sural Nerve.
- Author:
Jee Young KIM
1
;
Seung Min KIM
;
Kee Duk PARK
;
Tai Seung KIM
;
Il Nam SUNWOO
Author Information
1. Department of Neurology, Ewha Womans University School of Medicine, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Bulbospinal atrophy;
X-linked disease;
Sural nerve
- MeSH:
Biopsy;
Bulbo-Spinal Atrophy, X-Linked;
Humans;
Immunoglobulins;
Leg;
Middle Aged;
Myelin Sheath;
Organic Chemicals;
Polyneuropathies;
Receptors, Androgen;
Sural Nerve;
Voice
- From:Journal of the Korean Neurological Association
2010;28(2):115-118
- CountryRepublic of Korea
- Language:Korean
-
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.
