Supplementary Material for: Lack of Therapeutic Benefit of Vim Thalamotomy for Tremor-like Myoclonus in Two Cases of Benign Adult Familial Myoclonus Epilepsy

Introduction: Benign adult familial myoclonus epilepsy (BAFME) is an autosomal dominant disorder characterized by adult-onset cortical tremor and infrequent generalized seizures. Treatment options for managing involuntary movements in BAFME remain limited.
Case Presentation: Here, we present two cases involving individuals with BAFME who underwent Vim thalamotomy for tremor-like myoclonus. Despite the intervention, neither patient experienced any improvement in their symptoms.
Conclusion: These findings suggest that the Vim nucleus may not contribute to the pathophysiology of tremor-like myoclonus in BAFME, despite the established efficacy of Vim thalamotomy in the treatment of essential tremor.

引言：良性成人家族性肌阵挛癫痫（Benign adult familial myoclonus epilepsy，BAFME）是一种常染色体显性遗传病，以成人起病的皮层震颤及偶发全面性发作为特征。目前针对BAFME患者不自主运动的治疗选择仍较为有限。
案例陈述：本文报告2例BAFME患者，其因震颤样肌阵挛接受了丘脑腹中间核（Vim）丘脑切开术。尽管实施了该手术干预，两名患者的症状均未获得任何改善。
结论：上述研究结果提示，尽管Vim丘脑切开术在原发性震颤（essential tremor）的治疗中已被证实具有确切疗效，但Vim核团可能并未参与BAFME患者震颤样肌阵挛的病理生理过程。