肉毒杆菌神经毒素对持续性部分性癫痫所致肌阵挛的持续疗效。
Sustained Effect of Botulinum Neurotoxin in Myoclonus Owing to Epilepsia Partialis Continua.
作者信息
Bedarf Janis Rebecca, Marek Milena, Bien Christian G, Elger Christian E, Paus Sebastian
机构信息
Department of Neurology University of Bonn Bonn Germany.
Epilepsy Center Bethel Krankenhaus Mara Bielefeld Germany.
出版信息
Mov Disord Clin Pract. 2015 Jul 15;2(4):402-406. doi: 10.1002/mdc3.12216. eCollection 2015 Dec.
BACKGROUND
Epilepsia partialis continua (EPC) is defined as continuous myoclonic jerking of a body part of cortical origin and often resembles a movement disorder. Unfortunately, anti-epileptic therapy is frequently ineffective. Currently, the effect of botulinum neurotoxin (BoNT) therapy in EPC is controversial.
METHODS
We analyzed case histories, treatment protocols, and video documentation of 5 patients with EPC, who received BoNT therapy in our movement disorders unit. The Unified Myoclonus Rating Scale was used to document treatment effects.
RESULTS
In all patients, BoNT treatment significantly reduced severity and frequency of myoclonus and led to pronounced improvement of speech and arm utilization, regardless of etiology or duration of EPC.
CONCLUSION
BoNT represents a safe, effective treatment in EPC, offering long-term abatement of myoclonus. The substantial functional profit may hint at mechanisms beyond local impairment of neuromuscular transmission, such as modulation of maladaptive cortical plasticity, as observed in dystonia and poststroke spasticity.
背景
持续性部分性癫痫（EPC）被定义为源于皮质的身体某一部位的持续性肌阵挛抽搐，常类似运动障碍。不幸的是，抗癫痫治疗常常无效。目前，肉毒杆菌神经毒素（BoNT）治疗EPC的效果存在争议。
方法
我们分析了5例在我们运动障碍科接受BoNT治疗的EPC患者的病历、治疗方案和视频资料。采用统一肌阵挛评分量表记录治疗效果。
结果
在所有患者中，无论EPC的病因或病程如何，BoNT治疗均显著降低了肌阵挛的严重程度和频率，并使言语和手臂使用情况得到明显改善。
结论
BoNT是EPC的一种安全、有效的治疗方法，可长期减轻肌阵挛。显著的功能改善可能暗示了除神经肌肉传递局部受损之外的机制，如在肌张力障碍和中风后痉挛中观察到的适应性不良皮质可塑性的调节。
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