Kennedy 병의 신경전도검사
Nerve Conduction Studies in Kennedy’s Disease
조정희, 김승민, 김지현, 정연경, 강사윤, 이준홍, 선우일남,
소속 상세정보
조정희 ( Cho Jeong-Hee )
국민건강보험공단 일산병원 신경과
김승민 ( Kim Seung-Min )
연세대학교 의과대학 신경과학교실
김지현 ( Kim Ji-Hyun )
연세노블병원
정연경 ( Jung Yeon-Kyung )
국립의료원 신경과
강사윤 ( Kang Sa-Yoon )
제주대학교 의학전문대학원 신경과학교실
이준홍 ( Lee Jun-Hong )
국민건강보험공단 일산병원 신경과
선우일남 ( Sunwoo Il-Nam )
연세대학교 의과대학 신경과학교실
KMID : 0860920050070010008
Abstract
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.
키워드
Kennedy’s disease;Nerve conduction study
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