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KMID : 0860920050070010008
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Journal of the Korean Association EMG-Electrodiagnostic Medicine
2005 Volume.7 No. 1 p.8 ~ p.12
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Nerve Conduction Studies in Kennedy’s Disease
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Cho Jeong-Hee
Kim Seung-Min
Kim Ji-Hyun
Jung Yeon-Kyung
Kang Sa-Yoon
Lee Jun-Hong
Sunwoo Il-Nam
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Abstract
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Objectives: The aim of this study is to define the characteristics of the nerve conduction studies(NCS)
in Kennedy’s disease.
Methods: We reviewed the NCS of 21 patients in 18 families with genetically confirmed Kennedy’s disease.
We analyzed terminal latencies(TL), nerve conduction velocities(NCV) and amplitudes of compound
action potentials of the median, ulnar, peroneal, posterior tibial and sural nerves.
Results: Abnormal NCS was noticed in 13(61.9%) of 21 patients ; reduced or absent sensory nerve action
potentials(SNAPs) in 12(57.1%), reduced amplitudes of compound muscle action potentials(CMAPs) in
7(33.3%), slow motor or sensory NCVs in 5(23.8%) and prolonged TL in 3(14.3%). Sensory
symptoms/signs were noticed only in 4 patients; 3 with abnormal sensory NCS and one with normal sensory
NCS. In 4 patients with serial NCS, significant reduction of CMAPs was seen in 9 and 13 years later,
but not in 2 and 3 years intervals. There was no significant change of SNAPs in these 4 cases.
Conclusion: We found that the abnormal sensory NCS was common in Kennedy disease, but not essential
for the diagnosis of this disease. There was no close relationship between sensory NCS and clinical
findings. Follow-up NCS showed slowly progressive reduction of CMAPs but not SNAPs.
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KEYWORD
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Kennedy’s disease, Nerve conduction study
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