Department of Neurology, Mayo Clinic Florida, 4500 San Pablo Rd S, Jacksonville, Florida, 32224, USA.
Department of Neurology, Mayo Clinic Arizona, 13400 East Shea Blvd., Scottsdale, Arizona, 85259, USA.
Neurotherapeutics. 2020 Oct;17(4):1665-1680. doi: 10.1007/s13311-020-00922-6.
Myoclonus can cause significant disability for patients. Myoclonus has a strikingly diverse array of underlying etiologies, clinical presentations, and pathophysiological mechanisms. Treatment of myoclonus is vital to improving the quality of life of patients with these disorders. The optimal treatment strategy for myoclonus is best determined based upon careful evaluation and consideration of the underlying etiology and neurophysiological classification. Electrophysiological testing including EEG (electroencephalogram) and EMG (electromyogram) data is helpful in determining the neurophysiological classification of myoclonus. The neurophysiological subtypes of myoclonus include cortical, cortical-subcortical, subcortical-nonsegmental, segmental, and peripheral. Levetiracetam, valproic acid, and clonazepam are often used to treat cortical myoclonus. In cortical-subcortical myoclonus, treatment of myoclonic seizures is prioritized, valproic acid being the mainstay of therapy. Subcortical-nonsegmental myoclonus may be treated with clonazepam, though numerous agents have been used depending on the etiology. Segmental and peripheral myoclonus are often resistant to treatment, but anticonvulsants and botulinum toxin injections may be of utility depending upon the case. Pharmacological treatments are often hampered by scarce evidence-based knowledge, adverse effects, and variable efficacy of medications.
肌阵挛可导致患者明显残疾。肌阵挛的潜在病因、临床表现和病理生理机制多种多样。治疗肌阵挛对于改善这些疾病患者的生活质量至关重要。肌阵挛的最佳治疗策略最好根据仔细的评估和对潜在病因和神经生理学分类的考虑来确定。包括脑电图 (EEG) 和肌电图 (EMG) 数据在内的电生理测试有助于确定肌阵挛的神经生理学分类。肌阵挛的神经生理学亚型包括皮质、皮质下、皮质下非节段性、节段性和周围性。左乙拉西坦、丙戊酸和氯硝西泮常用于治疗皮质肌阵挛。在皮质下肌阵挛中,优先治疗肌阵挛发作,丙戊酸是主要的治疗方法。皮质下非节段性肌阵挛可使用氯硝西泮治疗,但根据病因,已有多种药物被使用。节段性和周围性肌阵挛通常对治疗有抗性,但根据具体情况,抗癫痫药物和肉毒毒素注射可能会有效果。由于缺乏基于证据的知识、药物的不良反应和疗效的变异性,药物治疗往往受到阻碍。