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KMID : 0359720100280020115
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Journal of the Korean Neurological Association
2010 Volume.28 No. 2 p.115 ~ p.118
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Kennedy Disease Showing Prominent Remyelinating Features of Sural Nerve
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Kim Jee-Young
Kim Seung-Min
Park Kee-Duk
Kim Tae-Seung
Sunwoo Il-Nam
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Abstract
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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.
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KEYWORD
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Bulbospinal atrophy, X-linked disease, Sural nerve
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