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Hereditary neuropathy with liability to pressure palsies와 Charcot-Marie-Tooth1A 환자군의 신경전도검사 소견의 비교

Other Titles
 Comparison of Nerve Conduction Studies between the Patients with Hereditary Neuropathy with Liability to Pressure Palsies and Charcot-Marie-Tooth1A 
 이종윤  ;  선우일남  ;  김승민  ;  김상운  ;  정연경  ;  김지현  ;  조정희  ;  최병옥 
 Journal of the Korean Neurological Association, Vol.22(4) : 345-351, 2004 
Journal Title
Journal of the Korean Neurological Association
Issue Date
Nerve conduction study ; Hereditary neuropathy with liability to pressure palsy ; Charcot-Marie-Tooth1A
Background: Hereditary neuropathy with liability to pressure palsy (HNPP) and Charcot-Marie-Tooth1A (CMT1A) are autosomal dominant inherited demyelinating neuropathy associated with the deletion or duplication of 17p11.2-p12 segments including the gene for peripheral myelin protein 22 (PMP22). The aim of this study is to compare the
electrophysiological features of these two disorders genetically related.

Methods: We studied the nerve conduction studies of genetically confirmed 43 HNPP in 23 families and 31 CMT1A patients in 13 families.

Results: Nerve conduction studies presented prolonged terminal latency, slow nerve conduction velocity and low amplitude of compound muscle or nerve action potentials in both diseases, but more severe in CMT1A. The incidence of conduction block with or without dispersion was higher in CMT1A than HNPP, with 23.6% and 5.7% respectively. CMT1A revealed a rather even distribution of abnormalities in segmental nerve conduction studies than HNPP. HNPP showed frequent involvements at the segment of entrapment-prone site such as peroneal and ulnar nerves. The characteristical differences of these diseases could be defined by the quantitative correlation study. The correlation coefficients of nerve conduction velocities between
tested nerves in the same or different limbs and between tested segments of the same nerve was much higher in CMT1A than HNPP.

Conclusions: We conclude that CMT1A presents with more severe diffuse and uniform slowing, whereas HNPP presents with focal electrophysiological abnormalities.
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1. College of Medicine (의과대학) > Dept. of Neurology (신경과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Seung Min(김승민) ORCID logo https://orcid.org/0000-0002-4384-9640
Kim, Jee Heun(김지현)
Sunwoo, Il Nam(선우일남)
Lee, Jong Yun(이종윤)
Cho, Jeong Hee(조정희)
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