Upper Limbs Muscle Co-Contraction Changes Correlate With The Physical Motor Impairments in CMT.

IF 3.2 4区 医学 Q2 CLINICAL NEUROLOGY
Tiziana Lencioni, Virginia Bandini, Cristina Schenone, Maria Lagostina, Alessia Aiello, Angelo Schenone, Maurizio Ferrarin, Carlo Trompetto, Laura Mori
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Abstract

Background: Subjects with Charcot-Marie-Tooth (CMT) disease show hands impairment which is a relevant problem affecting the quality of life. This symptom is related to muscle weakness and reduced motor coordination of the upper limb. However, most studies focus on lower limb impairment, therefore the investigation of upper limb disability is necessary to identify biomarkers able to monitor disease-specific features and to tailor rehabilitation.

Objective: This study aimed at characterizing upper limb muscle co-contraction using the co-contraction index (CCI) in CMT population.

Methods: Upper limb kinematic and electromyography (EMG) data were collected from fourteen CMT subjects (6-CMT1A and 8-CMT1X) during motor tasks typical of daily living activities. Rudolph's CCI was used to quantify muscle co-contraction of four muscle pairs acting on shoulder, elbow and wrist. All CMT subjects underwent clinical examination. Thirteen healthy subjects served as the normative reference (HC).

Results: CMT1X and CMT1A showed a significant reduction in CCI for distal and proximal muscle pairs compared to HC. Furthermore, CMT1A showed greater values of CCI compared to CMT1X mainly for the axial and axial-to-proximal muscle pairs. Movement speed and smoothness were not altered compared to HC. In addition, EMG metrics showed moderate-to-strong significant correlations with clinical outcomes.

Conclusions: CCI was able to quantify disease-specific deficits with respect to the normative reference, highlighting motor control alterations even before motor output impairment. CCI was also sensitive in detecting CMT subtypes-based differences and adopted compensatory strategies. Our findings suggest that CCI can be an outcome measure for CMT disease monitoring and interventional studies.

上肢肌肉共收缩变化与 CMT 患者的身体运动障碍有关。
背景:患有 Charcot-Marie-Tooth (CMT) 疾病的患者会出现手部功能障碍,这是影响生活质量的一个相关问题。这种症状与上肢肌肉无力和运动协调能力下降有关。然而,大多数研究都集中在下肢功能障碍方面,因此有必要对上肢残疾进行调查,以确定能够监测疾病特异性特征的生物标记物,并为康复量身定制:本研究旨在利用共收缩指数(CCI)描述 CMT 患者上肢肌肉共收缩的特征:方法:收集了 14 名 CMT 受试者(6 名 CMT1A 和 8 名 CMT1X)在典型的日常生活运动任务中的上肢运动学和肌电图(EMG)数据。鲁道夫 CCI 用于量化作用于肩、肘和腕的四对肌肉的肌肉协同收缩。所有 CMT 受试者均接受了临床检查。13名健康受试者作为常模参照(HC):结果:与 HC 相比,CMT1X 和 CMT1A 远端和近端肌肉对的 CCI 显著降低。此外,与 CMT1X 相比,CMT1A 的 CCI 值更高,主要体现在轴向和轴向至近端肌肉对上。与 HC 相比,运动速度和平稳性没有改变。此外,EMG指标与临床结果显示出中度到高度的显著相关性:结论:CCI 能够量化与常模参照物相比的疾病特异性缺陷,甚至在运动输出受损之前就能突出运动控制的改变。CCI 还能灵敏地检测出基于 CMT 亚型的差异和所采取的补偿策略。我们的研究结果表明,CCI 可以作为 CMT 疾病监测和干预研究的结果测量指标。
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来源期刊
Journal of neuromuscular diseases
Journal of neuromuscular diseases Medicine-Neurology (clinical)
CiteScore
5.10
自引率
6.10%
发文量
102
期刊介绍: The Journal of Neuromuscular Diseases aims to facilitate progress in understanding the molecular genetics/correlates, pathogenesis, pharmacology, diagnosis and treatment of acquired and genetic neuromuscular diseases (including muscular dystrophy, myasthenia gravis, spinal muscular atrophy, neuropathies, myopathies, myotonias and myositis). The journal publishes research reports, reviews, short communications, letters-to-the-editor, and will consider research that has negative findings. The journal is dedicated to providing an open forum for original research in basic science, translational and clinical research that will improve our fundamental understanding and lead to effective treatments of neuromuscular diseases.
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