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

IF 1.9 4区医学 Q3 NEUROIMAGING

Stereotactic and Functional Neurosurgery Pub Date : 2025-05-09 DOI:10.1159/000546301

Taku Nonaka, Takashi Asahi, Shiro Horisawa, Ichiro Takumi, Kiyonobu Ikeda, Akikazu Nakamura, Kenko Azuma, Hiroyuki Akagawa, Jiro Yamamoto, Nobutaka Yamamoto, Takaomi Taira

引用次数: 0

Abstract

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.

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两例良性成人家族性肌阵挛性癫痫的震颤样肌阵挛行维姆丘脑切开术疗效不佳。

良性成人家族性肌阵挛性癫痫（BAFME）是一种常染色体显性遗传病，以成人发病的皮质震颤和罕见的全身性癫痫发作为特征。治疗BAFME患者不自主运动的选择仍然有限。病例介绍：在这里，我们报告了两例涉及BAFME患者的病例，他们因震颤样肌阵挛而接受了Vim丘脑切开术。尽管进行了干预，但两名患者的症状都没有任何改善。结论：这些发现表明，尽管Vim丘脑切开术治疗特发性震颤的疗效已得到证实，但Vim核可能与BAFME震颤样肌颤的病理生理无关。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Stereotactic and Functional Neurosurgery 医学-神经成像

CiteScore

3.80

自引率

0.00%

发文量

审稿时长

3 months

期刊介绍： ''Stereotactic and Functional Neurosurgery'' provides a single source for the reader to keep abreast of developments in the most rapidly advancing subspecialty within neurosurgery. Technological advances in computer-assisted surgery, robotics, imaging and neurophysiology are being applied to clinical problems with ever-increasing rapidity in stereotaxis more than any other field, providing opportunities for new approaches to surgical and radiotherapeutic management of diseases of the brain, spinal cord, and spine. Issues feature advances in the use of deep-brain stimulation, imaging-guided techniques in stereotactic biopsy and craniotomy, stereotactic radiosurgery, and stereotactically implanted and guided radiotherapeutics and biologicals in the treatment of functional and movement disorders, brain tumors, and other diseases of the brain. Background information from basic science laboratories related to such clinical advances provides the reader with an overall perspective of this field. Proceedings and abstracts from many of the key international meetings furnish an overview of this specialty available nowhere else. ''Stereotactic and Functional Neurosurgery'' meets the information needs of both investigators and clinicians in this rapidly advancing field.