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特发性震颤患者聚焦超声丘脑毁损术后手部震颤

Hand Dystonia after Focused Ultrasound Thalamotomy in Essential Tremor.

机构信息

Department of Neurology, Geneva University Hospital, Geneva, Switzerland.

Department of Neurosurgery, Geneva University Hospital, Geneva, Switzerland.

出版信息

Stereotact Funct Neurosurg. 2024;102(4):203-208. doi: 10.1159/000538931. Epub 2024 Jun 4.

Abstract

INTRODUCTION

Magnetic resonance guided focused ultrasound (MRgFUS) thalamotomy is an effective treatment for drug-resistant tremor. The most frequent side effects are ataxia, gait disturbance, paresthesias, dysgeusia, and hemiparesis. Here, we report the first case of thalamic hand dystonia rapidly occurring after MRgFUS thalamotomy of the ventral intermediate nucleus (V.im).

CASE PRESENTATION

MRgFUS thalamotomy was performed in a 60-year-old left-handed patient for his disabling medically refractory essential tremor. The intervention resulted in a marked reduction of his action tremor. However, the patient developed an unvoluntary abnormal posture in his left hand a few days after the procedure with difficulty holding a cigarette between his fingers. Brain MRI revealed the expected MRgFUS lesion within the right V.im as well as an extension of the lesion anteriorly to the V.im in the ventro-oralis nucleus. Tractography showed that the lesion disrupted the dentato-rubro-thalamic tract as expected with a lesion suppressing tremor. However, the lesion also was interrupted fibers connecting to the superior frontal and pre-central cortices (primary motor cortex, premotor cortex, and supplementary area). We hypothesized that the interventional MRgFUS thalamotomy was slightly off target, which induced a dysfunction within the cortico-striato-thalamo-cortical network and the cerebello-thalamo-cortical pathway reaching a sufficient threshold of basal ganglia/cerebellum circuitry interference to induce dystonia.

CONCLUSION

This rare side effect emphasizes the risk of imbalance within the dystonia network (i.e., basal ganglia-cerebello-thalamo-cortical circuit) secondary to V.im thalamotomy.

摘要

简介

磁共振引导聚焦超声(MRgFUS)丘脑切开术是治疗耐药性震颤的有效方法。最常见的副作用是共济失调、步态障碍、感觉异常、味觉障碍和偏瘫。在这里,我们报告了首例 V.im 腹侧中间核(V.im)MRgFUS 丘脑切开术后迅速出现丘脑手肌张力障碍的病例。

病例介绍

一名 60 岁的左利手患者因药物难治性特发性震颤接受了 MRgFUS 丘脑切开术,以减轻其致残性震颤。干预后,他的动作性震颤明显减轻。然而,患者在手术后几天出现左手不由自主的异常姿势,手指难以夹住香烟。脑 MRI 显示右侧 V.im 内预期的 MRgFUS 病变以及病变在腹侧口核(ventro-oralis nucleus)内向前延伸到 V.im。示踪显示,病变如预期的那样破坏了齿状核-红核-丘脑束,导致震颤抑制。然而,病变也中断了与额上和中央前皮质(初级运动皮质、运动前皮质和补充区)连接的纤维。我们假设介入性 MRgFUS 丘脑切开术有点偏离目标,导致皮质-纹状体-丘脑-皮质网络和小脑-丘脑-皮质通路的功能障碍,达到基底节/小脑回路干扰的足够阈值,从而引发肌张力障碍。

结论

这种罕见的副作用强调了 V.im 丘脑切开术后,与肌张力障碍网络(即基底节-小脑-丘脑-皮质回路)相关的平衡失调风险。

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