Klehmet Juliane, Beutner Svenja, Hoffmann Sarah, Dornauer Matthias, Paul Friedemann, Reilmann Ralf, Brandt Alexander U, Meisel Andreas
NeuroCure Clinical Research Center, Charité - University Medicine Berlin, Charitéplatz 1, 10117, Berlin, Germany.
Department of Neurology, Jüdisches Krankenhaus Berlin, Heinz-Galinski-Strasse 1, 13347, Berlin, Germany.
BMC Neurol. 2019 Jun 8;19(1):118. doi: 10.1186/s12883-019-1339-x.
In patients suffering from Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) disease severity is assessed by Medical Research Counsil (MRC) Scale or Inflammatory Neuropathy Cause and Treatment (INCAT) disability score. However, none of these methods is appropriate to objectively assess muscle weakness or to detect very small subclinical changes. More objective and quantitative measures are needed in order to evaluate treatment efficiency or to detect subclinical affection of upper limps for early diagnosis. The goal of our study was to objectively quantify muscular weakness in CIDP patients with the non-invasive Quantitative Motor (Q-Motor) test of Grip Force Assessment (QGFA) as well as the Involuntary Movement Assessment (QIMA) and to search for differences between typical and atypical CIDP variants. In addition, we hypothesized that Q-Motor findings correlate with disease severity scales such as MRC or INCAT score.
In this cross-sectional exploratory proof-of-concept study subjects with confirmed diagnosis of typical or atypical CIDP were examined and compared to healthy controls (HC). For Q-Motor tests all subjects had to lift a device (250 g and 500 g) equipped with an electromagnetic sensor that measured grip force (GF) and three-dimensional changes in position and orientation. The measures "grip force variability" (GFV), "position index" (PI) and "orientation index" (OI) were provided to assess involuntary movements due to muscular weakness.
33 patients with CIDP and 28 HC were included. All measures were significantly elevated in CIDP patients for both devices in the right and left hand compared to healthy controls. Subgroup analysis revealed no differences between typical and atypical CIDP variants. INCAT score only weakly correlated with OI and PI. However, there was a stronger correlation between MRC and QIMA parameters in both hands.
Q-Motor assessments were capable to objectively assess muscular weakness in CIDP. In particular, QIMA measures detected subclinical generalized muscle weakness even in patients with milder disability. Sensitivity and rater-independence of Q-Motor assessments support a further exploration of QIMA measures as potential endpoints for future clinical trials in CIDP.
对于慢性炎症性脱髓鞘性多发性神经病(CIDP)患者,疾病严重程度通过医学研究委员会(MRC)量表或炎症性神经病病因与治疗(INCAT)残疾评分进行评估。然而,这些方法均不适用于客观评估肌肉无力或检测非常微小的亚临床变化。为了评估治疗效果或检测上肢的亚临床病变以进行早期诊断,需要更客观和定量的测量方法。我们研究的目的是通过握力评估的非侵入性定量运动(Q-Motor)测试以及不自主运动评估(QIMA)来客观量化CIDP患者的肌肉无力,并寻找典型和非典型CIDP变体之间的差异。此外,我们假设Q-Motor检查结果与MRC或INCAT评分等疾病严重程度量表相关。
在这项横断面探索性概念验证研究中,对确诊为典型或非典型CIDP的受试者进行检查,并与健康对照(HC)进行比较。对于Q-Motor测试,所有受试者都必须拿起一个配备电磁传感器的装置(250克和500克),该传感器可测量握力(GF)以及位置和方向的三维变化。提供“握力变异性”(GFV)、“位置指数”(PI)和“方向指数”(OI)等测量指标以评估由于肌肉无力导致的不自主运动。
纳入了33例CIDP患者和28例健康对照。与健康对照相比,CIDP患者双手使用两种装置时的所有测量指标均显著升高。亚组分析显示典型和非典型CIDP变体之间没有差异。INCAT评分仅与OI和PI弱相关。然而,双手的MRC与QIMA参数之间存在更强的相关性。
Q-Motor评估能够客观评估CIDP患者的肌肉无力。特别是,QIMA测量即使在残疾较轻的患者中也能检测到亚临床全身性肌肉无力。Q-Motor评估的敏感性和评分者独立性支持进一步探索QIMA测量作为CIDP未来临床试验潜在终点的可能性。