Neurology Clinic, Clinical Center of Serbia, Faculty of Medicine, University of Belgrade, 6, Dr Subotic Street, Belgrade, 11 000, Serbia.
Institute of Human Genetics, School of Medicine, University of Belgrade, Belgrade, Serbia.
Neurol Sci. 2020 Mar;41(3):625-630. doi: 10.1007/s10072-019-04142-5. Epub 2019 Nov 15.
Only several studies analyzed the characteristics of neuropathic pain (NeP) more extensively in patients with Charcot-Marie-Tooth type 1A (CMT1A). Therefore, we sought to determine the frequency and features of NeP in CMT1A patients and to assess the association between NeP and sociodemographic and clinical characteristics of patients with CMT1A.
Our research included 51 genetically diagnosed CMT1A patients. The International Association for the Study of Pain (IASP) criteria were used for diagnosis of NeP. PainDETECT questionnaire (PD-Q) was used to assess NeP features. The Medical Research Council (MRC) Sum Score, CMT Neuropathy Score (CMTNS), Overall Neuropathy Limitation Scale (ONLS) score, and Beck Depression Inventory were also used.
NeP was present in 15 (29.4%) patients with CMT1A. The average intensity of pain was 5.7 ± 2.2 out of 10. The most sensitive neuropathic symptoms were numbness, then tingling, and burning sensations, while the most specific symptom was allodynia. Patients with NeP more frequently reported pain in the back (p < 0.01) and the trunk (p < 0.05). Patients with NeP had more pronounced disability of the upper extremities and overall disability, as assessed by the ONLS score (p < 0.05). Depression was more frequent in patients with NeP compared with patients without NeP (66.7 to 13.9%, p < 0.01).
NeP was present in almost one-third of the patients with CMT1A and it was moderate on average. Presence of NeP was associated with worse functional disability and depression.
仅有少数研究更广泛地分析了 1A 型腓骨肌萎缩症(CMT1A)患者的神经病理性疼痛(NeP)特征。因此,我们旨在确定 CMT1A 患者中 NeP 的频率和特征,并评估 NeP 与 CMT1A 患者的社会人口学和临床特征之间的关联。
我们的研究纳入了 51 例经基因诊断的 CMT1A 患者。采用国际疼痛研究协会(IASP)标准诊断 NeP。采用疼痛 DETECT 问卷(PD-Q)评估 NeP 特征。还使用了医学研究委员会(MRC)总和评分、CMT 神经病变评分(CMTNS)、总体神经病变限制量表(ONLS)评分和贝克抑郁量表。
15 例(29.4%)CMT1A 患者存在 NeP。疼痛平均强度为 10 分的 5.7±2.2 分。最敏感的神经病理性症状是麻木,其次是刺痛和烧灼感,而最特异的症状是感觉异常。有 NeP 的患者更频繁地报告背部(p<0.01)和躯干(p<0.05)疼痛。与无 NeP 的患者相比,有 NeP 的患者上肢和总体残疾更严重,ONLS 评分更高(p<0.05)。与无 NeP 的患者相比,有 NeP 的患者抑郁更常见(66.7%比 13.9%,p<0.01)。
CMT1A 患者中近三分之一存在 NeP,平均程度为中度。存在 NeP 与更严重的功能残疾和抑郁有关。