대한임상검사과학회지 (Korean Journal of Clinical Laboratory Science)

  • 제53권2호
  • /
  • Pages.151-157
  • /
  • 2021
  • /
  • 1738-3544(pISSN)
  • /
  • 2288-1662(eISSN)
대한임상검사과학회 (Korean Society for Clinical Laboratory Science)
권호 표지
DOI QR코드

DOI QR Code

CIDP와 CMT 1형의 전기생리학적 특성에 대한 정량 분석: 감각신경연구

Quantitative Analysis of Electrophysiological Characteristics of CIDP and CMT Type 1: Sensory Nerve Research

  • 강지혁 (대전대학교 보건의료과학대학 임상병리학과)
  • Kang, Ji-Hyuk (Department of Biomedical Laboratory Science, College of Health and Medical Science, Daejeon University)
  • 투고 : 2021.03.29
  • 심사 : 2021.04.14
  • 발행 : 2021.06.30
PDF 다운로드
⟨ 이전 논문 다음 논문 ⟩

초록

선천성 유전질환인 CMT와 후천성 면역 매개 질환인 CIDP는 임상적 증상이 유사하므로 두 질환의 감별진단을 위해서는 말초신경의 전기생리학적 특징을 비교하는 것이 도움이 될 수 있다. 본 연구는 CIDP와 CMT 1형으로 확진된 환자의 신경전도검사 결과 중 감각신경전도검사의 주요 지표별 결과를 후향적으로 정량분석하여 두 질환군의 전기생리학적 특징을 규명하고자 하였다. CIDP (N=35)와 CMT1 (N=30)로 확진된 환자의 dCNAP와 SNCV를 이용하여 두 질환군의 중증도 분석, 유의성 검정, 비정상 범위별 비율분석 및 상관분석을 실시하여 통계적 차이를 기반으로 특징을 비교하였다. 두 질환 모두 전신성 말초신경다발신경병증의 특징이 관찰되었고, 장딴지신경의 손상이 가장 심한 것으로 확인되었다. CMT1군은 탈수초성 및 축삭성 신경병증을 동반하는 전신성 신경병증이고, CIDP보다 더 중증의 신경병증임이 확인되었다. 또한, 상관계수 및 산점도 분석에서 CMT1은 신경 손상 범위가 전체 신경에서 균등한 전기생리학적 특징이 관찰되었다. 감각신경전도검사의 결과를 기반으로한 본 연구결과가 CIDP와 CMT 1형의 감별진단 및 연구에 도움이 될 것으로 사료된다.

Charcot-Marie-Tooth disease (CMT) is a slowly progressive hereditary degenerative disease and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired immune-mediated disorder characterized by weakness and sensory deficits. The purpose of this study was to analyze and compare the electrophysiological characteristics observed in sensory nerve conduction studies (SNCS) of both diseases. A retrospective study of 65 patients with a diagnosis of CIDP (N=35) and CMT type I (N=30) was performed. This study analyzed No potentials ratio, distal compound nerve action potential (dCNAP) of various nerve types, and a correlation coefficient analysis of the sensory nerve conduction velocity (SNCV). As a result, I found that CMT 1 was more severe systemic demyelinating and axonal polyneuropathy better than CIDP (P<0.05). In a quantitative analysis of dCNAP and SNCV, especially sural nerve was the most severe nerve injury observed in both diseases. In correlation and scatter plot analysis, CMT 1 showed relatively high correlations compared to CIDP based on the correlation coefficient analysis (Fisher's Z test) of SNCV. The results of this study suggested that CMT 1 showed the slowness in SNCV, one of the characteristics of demyelinating polyneuropathy, and this slowing had a uniform pattern. In conclusion, electrophysiological characteristic of SNCS may be useful in the diagnosis and research between patients with CMT 1 and CIDP.

키워드

과제정보

This research was supported by the Daejeon University fund (2020).

참고문헌

  1. Mary MR. Axonal charcot-Marie-Tooth disease. Neurology. 2005; 65:186-187. https://doi.org/10.1212/01.wnl.0000173904.97549.94
  2. Latour P, Gonnaud PM, Ollagnon E, Chan V, Perelman S, Stojkovic T, et al. Simple mutation analysis in dominant demyelinating Charcot-Marie-Tooth disease three novel mutations. J Peripher Nerv Syst. 2006;11:148-155. https://doi.org/10.1111/j.1085-9489.2006.00080.x
  3. Jeng LJ, Balice-Gordon RJ, Messing A, Fischbeck KH, Scherer SS. The effects of a dominant connexin32 mutant in myelinating Schwann cells. Mol Cell Neurosci. 2006;32:283-298. https://doi.org/10.1016/j.mcn.2006.05.001
  4. Harding AE, Thomas PK. Genetic aspects of hereditary motor and sensory neuropathy (types I and II). J Med Genet. 1980; 17:329-336. https://doi.org/10.1136/jmg.17.5.329
  5. Kim SB, Park KD, Choi BO. Diagnosis and treatment in Charcot-Marie-Tooth disease. Ann Clin Neurophysiol. 2005;7: 65-74.
  6. Berciano J, Garcia A, Gallardo E, Peeters K, Pelayo-Negro AL, Alvarez-Paradelo S, et al. Intermediate Charcot-Marie-Tooth disease: an electrophysiological reappraisal and systematic review. J Neurol. 2017;264:1655-1677. https://doi.org/10.1007/s00415-017-8474-3
  7. Manganelli F, Pisciotta C, Reilly MM, Tozza S, Schenone A, et al. Nerve conduction velocity in CMT1A: what else can we tell?. Eur J Neurol. 2016;23:1566-1571. https://doi.org/10.1111/ene.13079
  8. Pareyson D, Scaioli V. Clinical and electrophysiological aspects of Charcot-Marie-Tooth disease. Neuromolecular Med. 2006;8: 3-22. https://doi.org/10.1385/nmm:8:1-2:3
  9. Stanton M, Pannoni V, Lewis RA, Logigian EL, Naguib D, Shy ME, et al. Dispersion of compound muscle action potential in hereditary neuropathies and chronic inflammatory demyelinating polyneuropathy. Muscle Nerve. 2006;34:417-422. https://doi.org/10.1002/mus.20600
  10. Kuwabara S, Misawa S. Chronic inflammatory demyelinating polyneuropathy. Adv Exp Med Biol. 2019;1190:333-343. https://doi.org/10.1007/978-981-32-9636-7_21
  11. Lehmann HC, Burke D, Kuwabara S. Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment. J Neurol Neurosurg Psychiatry. 2019;90:981-987. https://doi.org/10.1136/jnnp-2019-320314
  12. Bunschoten C, Jacobs BC, Van den Bergh PYK, Cornblath DR, van Doorn PA. Progress in diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy. Lancet Neurol. 2019;18:784-794. https://doi.org/10.1016/S1474-4422(19)30144-9
  13. Staudt M, Diederich JM, Meisel C, Meisel A, Klehmet J. Differences in peripheral myelin antigen-specific T cell responses and T memory subsets in atypical versus typical CIDP. BMC Neurol. 2017;17:81. https://doi.org/10.1186/s12883-017-0860-z
  14. Stino AM, Naddaf E, Dyck PJ, Dyck PJB. Chronic inflammatory demyelinating polyradiculoneuropathy-Diagnostic pitfalls and treatment approach. Muscle Nerve. 2021;63:157-169. https://doi.org/10.1002/mus.27046
  15. Escorcio-Bezerra ML, Pinto WBVR, Bichuetti DB, Souza PVS, Nunes RM, Silva LHL, et al. Immune-mediated inflammatory polyneuropathy overlapping Charcot-Marie-Tooth 1B. J Clin Neurosci. 2020;75:228-231. https://doi.org/10.1016/j.jocn.2020.03.014
  16. Oh SJ. Clinical electromyography. Nerve conduction studies, third edition. Philadelphia: Lippincott Williams & Wilkins, 2003;86-135.
  17. Kang JH, Lee YS. Sensory nerve conduction studies in the diagnosis of diabetic sensorimotor polyneuropathy: electrophysiological features. J Phys Ther Sci. 2012;24:139-142. https://doi.org/10.1589/jpts.24.139
  18. Garg N, Howells J, Yiannikas C, Vucic S, Krishnan AV, Spies J, et al. Motor unit remodelling in multifocal motor neuropathy: The importance of axonal loss. Clin Neurophysiol. 2017;128:2022-2028. https://doi.org/10.1016/j.clinph.2017.07.414
  19. Kang JH, Kim HJ, Lee ER. Electrophysiological evaluation of chronic inflammatory demyelinating polyneuropathy and charcot-marie-tooth type 1: dispersion and correlation analysis. J Phys Ther Sci. 2013;25:1265-1268. https://doi.org/10.1589/jpts.25.1265