Department of Clinical Neurophysiology, Great Ormond Street Hospital for Children NHS Foundation Trust, Great Ormond Street, London, UK; Division of Pediatric Neurology, University Children's Hospital Basel (UKBB), University of Basel, Spitalstrasse 33, 4056 Basel, Switzerland; Division of Neurology, Medical University Clinic, Kantonsspital Baselland, Switzerland.
Division of Neuropathology, UCL Institute of Neurology, London, UK; Dubowitz Neuromuscular Centre, MRC Centre for Neuromuscular Diseases, UCL Great Ormond Street Institute of Child Health, London, UK.
Neuromuscul Disord. 2019 Jan;29(1):14-20. doi: 10.1016/j.nmd.2018.10.003. Epub 2018 Oct 31.
The conduct and interpretation of electromyography in children is considered difficult and therefore often avoided. We assessed the diagnostic accuracy of the paediatric electromyography protocol used in our tertiary reference centre and compared it to muscle biopsy results and clinical diagnosis. Electromyography was performed in unsedated children with suspected neuromuscular diseases between January 2010 and September 2017 and was analysed quantitatively. Muscle pathology was classified into seven groups based on existing histopathology reports. The clinical diagnosis, including myopathic, neurogenic and non-neuromuscular categories was used as the gold standard. 171 children between the age of 12 days to 17.4 years were included in the analysis. 41 children (24%) were under the age of 2 years at the time of electromyography. 98 (57%) children were diagnosed with a myopathic disorder, 18 (11%) with a neurogenic disease and 55 (32%) as not having a primary neuromuscular disorder. In detecting myopathic disease, electromyography performed as well as muscle biopsy (sensitivity 87.8% for electromyography vs. 84.5% for muscle biopsy; specificity 75.7% vs. 86.4%). This also applied to children under the age of 2 years (sensitivity 81.8% vs. 86.4%). Quantitative analysis of a limited electromyography protocol performed in unsedated children is a very valuable diagnostic tool.
在儿童中进行肌电图检查和解释被认为具有一定难度，因此往往被回避。我们评估了我院参考中心使用的儿科肌电图方案的诊断准确性，并将其与肌肉活检结果和临床诊断进行了比较。2010 年 1 月至 2017 年 9 月，对疑似神经肌肉疾病的未镇静儿童进行了肌电图检查，并进行了定量分析。肌肉病理学根据现有的组织病理学报告分为七组。临床诊断包括肌病、神经病和非神经肌肉疾病类别，作为金标准。共纳入 171 名年龄在 12 天至 17.4 岁之间的儿童进行分析。41 名儿童（24%）在进行肌电图检查时年龄小于 2 岁。98 名（57%）儿童被诊断为肌病，18 名（11%）为神经病，55 名（32%）未诊断为原发性神经肌肉疾病。在检测肌病方面，肌电图检查与肌肉活检一样敏感（肌电图检查的敏感性为 87.8%，肌肉活检的敏感性为 84.5%；特异性为 75.7%，肌肉活检的特异性为 86.4%）。这也适用于 2 岁以下的儿童（敏感性为 81.8%，肌肉活检的敏感性为 86.4%）。对未镇静儿童进行有限的肌电图方案的定量分析是一种非常有价值的诊断工具。
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