The striking effects of deep cerebellar stimulation on generalized fixed dystonia: case report

The striking effects of deep cerebellar stimulation on generalized fixed dystonia: case report
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DOI:
10.3171/2018.11.jns182180
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发表时间:
2020-03-01
影响因子:
4.1
通讯作者:
Taira, Takaomi
Taira, Takaomi
中科院分区:
医学1区
文献类型:
--
作者:
Horisawa, Shiro;Arai, Takashi;Taira, Takaomi

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小脑的神经调节可以通过小脑与基底节或其他皮质之间的几种联系影响运动障碍的病理异常。在本报告中,作者证明了小脑深部脑刺激(DBS)对双侧苍白球切开术和鞘内巴氯芬治疗无效的重度全身性固定性肌张力障碍(FD)患者的影响。一个以前健康的16岁女孩提出了广泛的FD。双侧苍白球切开术和鞘内巴氯芬治疗未能解决她的病情,随后她通过双侧小脑上级脚(SCP)和齿状核(DN)接受了DBS。刺激同侧SCP或DN可改善FD,经SCP给予DBS的肌肉松弛能力优于DN DBS。通过SCP进行6个月的慢性双侧DBS后,观察到患者的全身FD从卧床不起状态到轮椅束缚状态有相当大的改善;此外,患者能够移动她的手臂和腿。本病例的研究结果表明,小脑深部结构的神经调节是一种有前途的治疗FD的方法,这是传统治疗难治性的。
Cerebellar neuromodulation could influence the pathological abnormalities of movement disorders through several connections between the cerebellum and the basal ganglia or other cortices. In the present report, the authors demonstrate the effects of cerebellar deep brain stimulation (DBS) on a patient with severe generalized fixed dystonia (FD) that was refractory to bilateral pallidotomy and intrathecal baclofen therapy. A previously healthy 16-year-old girl presented with generalized FD. Bilateral pallidotomy and intrathecal baclofen therapy had failed to resolve her condition, following which she received DBS through the bilateral superior cerebellar peduncle (SCP) and dentate nucleus (DN). Ipsilateral stimulation of the SCP or DN improved the FD, and the ability of DBS administered via the SCP to relax muscles was better than that of DN DBS. A considerable improvement of generalized FD, from a bedridden state to a wheelchair-bound state, was observed in the patient following 6 months of chronic bilateral DBS via the SCP; moreover, the patient was able to move her arms and legs. The findings in the present case suggest that neuromodulation of deep cerebellar structures is a promising treatment for FD that is refractory to conventional treatments.