CIDP와 CMT 1형의 전기생리학적 특성에 대한 정량 분석 : 감각신경연구
Quantitative Analysis of Electrophysiological Characteristics of CIDP and CMT Type 1: Sensory Nerve Research
1
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
선천성 유전질환인 CMT와 후천성 면역 매개 질환인 CIDP 는 임상적 증상이 유사하므로 두 질환의 감별진단을 위해서는말초신경의 전기생리학적 특징을 비교하는 것이 도움이 될 수있다. 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 환자의dCNAP와 SNCV를 이용하여 두 질환군의 중증도 분석, 유의성검정, 비정상 범위별 비율분석 및 상관분석을 실시하여 통계적차이를 기반으로 특징을 비교하였다. 두 질환 모두 전신성 말초신경다발신경병증의 특징이 관찰되었고, 장딴지신경의 손상이가장 심한 것으로 확인되었다. CMT1군은 탈수초성 및 축삭성신경병증을 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.
Abstract
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.
참고문헌 (19)
- Mary MR, "Axonal charcot-Marie-Tooth disease", Neurology, 65, pp.186~187 (2005)[DOI]
- Latour P, "Simple mutation analysis in dominant demyeli-nating Charcot-Marie-Tooth disease three novel mutations", J Peripher Nerv Syst, 11, pp.148~155 (2006)[DOI]
- Linda Jo Bone Jeng, "The effects of a dominant connexin32 mutant in myelinating Schwann cells", Molecular and Cellular Neuroscience, 32(3), pp.283~298 (2006), Elsevier BV[DOI]
- Harding AE, "Genetic aspects of hereditary motor and sensory neuropathy(types I and II)", J Med Genet, 17, pp.329~336 (1980)[DOI]
- 김상범, "샤르코-마리-투스 질환의 진단 및 치료", Annals of Clinical Neurophysiology, 7(2), pp.65~74 (2005), 대한임상신경생리학회
- Berciano J, "Intermediate Charcot-Marie-Tooth disease : an electrophysiological reappraisal and systematic review", J Neurol, 264, pp.1655~1677 (2017)[DOI]
- Manganelli F, "Nerve conduction velocity in CMT1A : what else can we tell?", Eur J Neurol, 23, pp.1566~1571 (2016)
- Pareyson D, "Clinical and electrophysiological aspects of Charcot-Marie-Tooth disease", Neuromolecular Med, 8, pp.3~22 (2006)[DOI]
- Stanton M, "Dispersion of compound muscle action potential in heredi-tary neuropathies and chronic inflammatory demyelinating poly-neuropathy", Muscle Nerve, 34, pp.417~422 (2006)[DOI]
- Kuwabara S, "Chronic inflammatory demyelinating polyneuropathy", Adv Exp Med Biol, 1190, pp.333~343 (2019)[DOI]
- Lehmann HC, "Chronic inflammatory de-myelinating polyneuropathy : update on diagnosis, immunopa-thogenesis and treatment", J Neurol Neurosurg Psychiatry, 90, pp.981~987 (2019)[DOI]
- Bunschoten C, "Progress in diagnosis and treatment of chronic in-flammatory demyelinating polyradiculoneuropathy", Lancet Neurol, 18, pp.784~794 (2019)[DOI]
- Staudt M, "Differences in peripheral myelin antigen-specific T cell responses and T memory subsets in atypical versus typical CIDP", BMC Neurol, 17, pp.81~ (2017)[DOI]
- Stino AM, "Chronic inflammatory demyelinating polyradiculoneuropathy-Diagnostic pitfalls and treatment approach", Muscle Nerve, 63, pp.157~169 (2021)[DOI]
- Escorcio-Bezerra ML, "Immune-mediated inflammatory pol-yneuropathy overlapping Charcot-Marie-Tooth 1B", J Clin Neurosci, 75, pp.228~231 (2020)[DOI]
- Oh SJ, "Clinical electromyography. Nerve conduction studies, third edition", pp.86~135 (2003), Lippincott Williams & Wilkins
- Kang JH, "Sensory nerve conduction studies in the diag-nosis of diabetic sensorimotor polyneuropathy : electrophysio-logical features", J Phys Ther Sci, 24, pp.139~142 (2012)
- Garg N, "Motor unit remodelling in multifocal motor neuropathy : The importance of axonal loss", Clin Neurophysiol, 128, pp.2022~2028 (2017)[DOI]
- Kang JH, "Electrophysiological evaluation of chronic inflammatory demyelinating polyneuropathy and char-cot-marie-tooth type 1 : dispersion and correlation analysis", J Phys Ther Sci, 25, pp.1265~1268 (2013)[DOI]