摘要：

目的: 总结周围神经过度兴奋综合征临床特征。方法与结果: 纳入河南省人民医院2018年10月至2022年4月收治的10例周围神经过度兴奋综合征患者，临床表型为Morvan综合征5例、Issacs综合征4例、痉挛-束颤综合征1例；临床表现为肌颤搐10例、自主神经功能障碍8例、中枢神经系统症状7例、肢体无力7例、感觉障碍6例、痉挛和肌肉僵硬2例；5例自身抗体阳性；2例MRI异常，分别位于脑干、左侧枕叶和右侧丘脑；10例肌电图异常，表现为F波后发放电位10例、异常自发运动单位电位10例、束颤电位10例、肌颤搐电位8例、神经性肌强直电位3例；3例仅行对症治疗，7例行对症治疗及免疫调节治疗；1例失访，5例预后良好，2例预后不良，2例死亡。结论: 周围神经过度兴奋综合征临床表现具有异质性，神经电生理检出后发放电位可能是诊断周围神经过度兴奋综合征的敏感性指标。

关键词: 周围神经系统疾病, 神经系统自身免疫疾病, 肌纤维颤搐, 肌电描记术

Abstract:

Objective: To summarize the clinical characteristics of peripheral nerve hyperexcitability syndrome (PNHS). Methods and Results: Ten patients with PNHS were admitted to He'nan Provincial People's Hospital from October 2018 to April 2022, with the clinical phenotypes of Morvan syndrome in 5 cases, Issacs syndrome in 4 cases, and cramp-fasciculation syndrome (CFS) in one case. There were 10 cases with clinical manifestations of muscle twitching, 8 of dysautonomia, 7 of central nervous system symptoms, 7 of limb weakness, 6 of sensory deficits, and 2 of cramping and muscle stiffness. Five cases were positive for autoimmune antibodies. Two cases had MRI abnormalities located in the brainstem, left occipital lobe and right thalamus. Ten cases had EMG abnormalities, including afterdischarges of F-wave in 10 cases, unit potential of spontaneous motion abnormalities in 10 cases, fasciculation potentials in 10 cases, myokymic discharges in 8 cases, and neuromyotonic and myokymic discharges in 3 cases. Three cases underwent only symptomatic therapy, and 7 cases underwent symptomatic therapy and immunomodulatory treatments. Except for one case who lost of follow-up, 5 cases had a good prognosis, 2 cases with poor prognosis, and 2 cases died. Conclusions: PNHS is a heterogeneous group of clinical manifestations. Electrophysiological appearance of afterdischarges may be a sensitive indicator for the diagnosis of PNHS.

Key words: Peripheral nervous system diseases, Autoimmune diseases of the nervous system, Myokymia, Electromyography

中图分类号: 

• 周围神经系统疾病

• 神经系统自身免疫疾病

• 肌纤维颤搐

• 肌电描记术

• Peripheral nervous system diseases

• Autoimmune diseases of the nervous system

• Myokymia

• Electromyography