肌电图检查在儿童神经系统疾病诊断中的意义

Diagnostic Significance of Electricomyogrphy in Children with Nervous System Diseases

目的探讨肌电图检查在儿童神经系统疾病诊断中的意义。方法对354例神经系统疾病患儿进行肌电图检查,包括周围神经传导速度、针极肌电图及重复频率刺激,并对结果进行分析。结果肌电图检查结果异常166例,其中肌源性损害36例,神经源性损害47例,神经性损害69例,重复频率刺激异常3例,界线性改变11例。36例肌源性损害中,确诊为进行性肌营养不良18例,多发性肌炎2例,线粒体脑肌病1例,未确诊15例。47例神经源性损害中,脊肌萎缩症29例,急性脊髓炎4例,脊髓灰质炎后遗症2例,12例诊断不明。69例神经性损害中,臂丛神经损伤23例,格林-巴利综合征9例,腓总神经损伤6例,面神经损伤4例,遗传性运动感觉神经病2例,慢性炎性脱髓鞘性多神经病、异染性脑白质营养不良各1例,23例诊断不明。3例重复频率刺激异常者均诊断为重症肌无力,其中全身型2例,眼肌型1例。界线性异常11例均未确诊。肌电图检查正常188例,其中临床诊断为重症肌无力眼肌型21例,脑性瘫痪5例,急性脊髓炎2例,多发性肌炎1例,神经系统其他疾病21例,余138例诊断不明。结论1.肌电图检查有助于进行性肌营养不良、脊髓性肌萎缩、神经损伤等疾病明确诊断;2.为侵犯下运动单位的疾病的诊断及鉴别诊断可提供重要依据;3.重症肌无力患儿重复频率刺激检查阳性率不高;4.对主要侵犯上运动神经元的疾病诊断意义不大。

Objective To investigate the role of electrornyography （EMG） in diagnosis of nervous system diseases in children. Methods EMG tests were carried out in 354 patients with nervous system diseases, and the data and results of EMG tests were analyzed. Resuits One hundred and sixty- six patients＇ results of EMG were abnormal. Among these abnormalities, 36 cases were myogenic, 47 cases were neurogenic, abnormalities of 69 cases were located in peripheral nerves, 3 cases got positive in repetitive nerve stimulation （RNS）, and 11 cases were on the borderline. In 36 myogenic patients, clinical diagnosis were as follow： progressive muscular dystrophy （PMD, 18 cases）, polymyositis （2 cases）, mitochondrial encephalomyopathy （ 1 case）, and the other 15 cases had no definite diagnosis. In 47 neurogenic patients, the diagnosis were spinal muscular atrophy （SMA,29 cases）, sequela of poliomyelitis （2 cases）, acute transverse myelitis （ATM,4 canes）, and the other 12 patients had no definite diagnosis. In 69 cases of peripheral nerve abnormality, diagnosis were injury of brachlal nerve （23 cases）, hereditary motor sensory ncuropathy （HMSN, 2 cases）, Guillain- Bane syndrome （GBS, 9 cases）, chronic inflammatory demyelinating polyradiculoneuropathy （CIDP, 1 case）, injury of facial nerve （4 case.s）, injury of common peroneal nerve （6 cases）, metachrornatic leukodystrophy （MLD, 1 case）, and the other 23 patients had no definite diagnosis. Three patients who got RNS positive were all diagnosed myasthenia gravis （MG）, and ocular type （ 1 case）, general type （2 canes）. Eleven patients whose EMG results were borderline were all diagnosed indefinitely. One hundred and eighty - eight patients had normaol results of EMG test. The diagnosis of these patients were included ocular MG （21 cases）, cerebral palsy （CP, 5 cases）, ATM （2 case.s）, polymyositis （ 1 case＇）, and some other nervous system diseases （21 cases）, and the other 138 were diagnosed indefinitdy. Conclusions 1. EMG plays an important role in definite diagnosis of PMD, SMA, poliomyelitis and nerve injury; 2. EMG can provide clue or basis in the differential diagnosis of nervous system diseases which involved lower motor unit; 3. EMG test has very low positive results in children with MG; 4. EMG has little help in diagnosis of diseases involved upper motor unit only.