Kennedy 병의 신경전도검사

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.