Department of Neurology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Department of Neurology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
J Neuroimmunol. 2021 Jun 15;355:577569. doi: 10.1016/j.jneuroim.2021.577569. Epub 2021 Apr 7.
To analyze the clinical characteristics and outcomes of patients diagnosed with acquired neuromyotonia and who were treated with tacrolimus.
A single center, retrospective study was performed on patients with acquired meuromyotonia whose treatment included tacrolimus. The clinical information, antibody tests, and electromyography results were reviewed. The Numeric Rating Scale for pain and modified Rankin scale were used to quantify outcomes.
This study included four patients who presented with fasciculation or myokymia in their limbs. Electromyography suggested peripheral nerve hyperexcitability. Autoantibodies including contactin-associated protein 2 (CASPR2), leucine-rich glioma inactivated protein 1 (LGl1) or IgLON5 antibody were detected in three patients, and another patient had Sjogren's syndrome. Initial treatment included membrane-stabilizing drugs and/or corticosteroids. Tacrolimus was administered at a dose of 3 mg once daily to all patients. All patients showed clinical improvement after the treatment. No recurrence was observed after gradual tapering or discontinuation of therapy during follow-up.
Tacrolimus may be a therapeutic option for acquired neuromyotonia. Further studies on tacrolimus in larger patient cohort should be performed.
分析诊断为获得性肌强直性神经病并接受他克莫司治疗的患者的临床特征和结局。
对接受他克莫司治疗的获得性肌强直性神经病患者进行单中心回顾性研究。回顾患者的临床资料、抗体检测和肌电图结果。采用数字评分量表评估疼痛，采用改良 Rankin 量表评估结局。
本研究纳入了 4 例出现四肢肌束颤动或肌纤维抽搐的患者。肌电图提示周围神经兴奋性过高。3 例患者检测到抗接触蛋白相关蛋白 2（CASPR2）、富亮氨酸胶质瘤失活蛋白 1（LGl1）或 IgLON5 抗体等自身抗体，另 1 例患者患有干燥综合征。初始治疗包括膜稳定剂和/或皮质类固醇。所有患者均接受他克莫司 3mg/d 治疗。治疗后所有患者的临床症状均改善。随访期间逐渐减药或停药后未见复发。
他克莫司可能是获得性肌强直性神经病的一种治疗选择。应在更大的患者队列中开展他克莫司治疗的研究。
