Abstract:
Cerebral palsy (CP) is a common cause of acquired dystonia, which can lead to significant interference with quality of life and societal participation. In the last two decades, the surgical treatment of dystonia has primarily focused on deep brain stimulation targeting the basal ganglia and thalamic circuits. However, stimulation of the basal ganglia has generally been less effective in acquired combined forms of dystonia, including dystonic CP. These limitations, along with growing evidence for the role of the cerebellum in the pathophysiology of dystonia, have led to renewed interest in the cerebellum as a target for therapeutic stimulation in dystonia. Nevertheless, there are very few contemporary studies demonstrating its use. We present the case of a patient with generalized dystonia due to dyskinetic CP who was successfully treated with stimulation of the cerebellar cortex in the modern era. We also review the evidence underpinning targeting of the cerebellum in surgical therapy for dystonia and examine the latest reports of this approach in the surgical literature.
The patient derived significant improvement in the control of her dystonic symptoms, with a reduction in her BFMDRS score from 83 to 25. No complications were observed during more than 3 years of postoperative follow-up. Since the turn of the 21st century, there have been only 7 reports of cerebellar stimulation for dystonia, recruiting a total of 18 patients. These studies have exclusively targeted deep brain structures, making the present report of cortical cerebellar stimulation particularly unique.
In the 21st century, cerebellar stimulation has predominantly been a second-line treatment for dystonia, after the failure of DBS targeting more mainstream loci within the thalamus and globus pallidus. However, there is increasing recognition of the role of the cerebellum in movement disorders, with multiple convergent lines of evidence supporting its involvement in dystonia pathophysiology. The cerebellum is worthy of greater consideration as a target for neurostimulation in dystonia, particularly in cases of acquired etiology.
摘要:
背景:脑瘫(CP)是获得性肌张力障碍的常见原因,这可能会严重干扰生活质量和社会参与。在过去的二十年里,肌张力障碍的外科治疗主要集中于针对基底神经节和丘脑回路的深部脑刺激。然而,对基底神经节的刺激通常在获得性联合形式的肌张力障碍中效果较差,包括肌张力障碍CP。这些限制,随着越来越多的证据表明小脑在肌张力障碍的病理生理学中的作用,引起了人们对小脑作为肌张力障碍治疗刺激目标的新兴趣。然而,很少有当代研究证明它的用途。我们介绍了一例由于运动障碍CP引起的全身性肌张力障碍患者,该患者在现代已成功通过小脑皮层刺激治疗。我们还回顾了在肌张力障碍的手术治疗中支持小脑靶向的证据,并检查了手术文献中这种方法的最新报道。
结论:患者的肌张力障碍症状得到显著改善,她的BFMDRS评分从83分降低到25分。术后随访3年以上无并发症发生。自21世纪之交以来,只有7例小脑刺激肌张力障碍的报道,共招募18名患者。这些研究只针对大脑深层结构,使得目前报道的皮质小脑刺激特别独特。
结论:在21世纪,小脑刺激主要是肌张力障碍的二线治疗,在针对丘脑和苍白球内更多主流基因座的DBS失败后。然而,人们越来越认识到小脑在运动障碍中的作用,有多个一致的证据支持其参与肌张力障碍病理生理学。小脑作为肌张力障碍神经刺激的目标值得更多考虑,特别是在获得性病因的情况下。