PDF(Pubmed)
Abstract：
Prader-Willi syndrome (PWS) is a complex, genetic disorder characterized by multisystem involvement, including hyperphagia, maladaptive behaviors and endocrinological derangements. Recent developments in advanced neuroimaging have led to a growing understanding of PWS as a neural circuit disorder, as well as subsequent interests in the application of neuromodulatory therapies. Various non-invasive and invasive device-based neuromodulation methods, including vagus nerve stimulation (VNS), transcranial direct current stimulation (tDCS), repetitive transcranial magnetic stimulation (rTMS), and deep brain stimulation (DBS) have all been reported to be potentially promising treatments for addressing the major symptoms of PWS. In this systematic literature review, we summarize the recent literature that investigated these therapies, discuss the underlying circuits which may underpin symptom manifestations, and cover future directions of the field. Through our comprehensive search, there were a total of 47 patients who had undergone device-based neuromodulation therapy for PWS. Two articles described VNS, 4 tDCS, 1 rTMS and 2 DBS, targeting different symptoms of PWS, including aberrant behavior, hyperphagia and weight. Multi-center and multi-country efforts will be required to advance the field given the low prevalence of PWS. Finally, given the potentially vulnerable population, neuroethical considerations and dialogue should guide the field.
摘要：
普拉德-威利综合征（PWS）是一个复杂的，以多系统参与为特征的遗传性疾病，包括饮食亢进,适应不良的行为和内分泌紊乱。先进的神经影像学的最新发展使人们越来越了解PWS是一种神经回路疾病，以及随后对神经调节疗法应用的兴趣。各种非侵入性和基于侵入性设备的神经调节方法，包括迷走神经刺激（VNS），经颅直流电刺激（tDCS），重复经颅磁刺激（rTMS），和深部脑刺激（DBS）都被报道为解决PWS主要症状的潜在有希望的治疗方法。在这篇系统的文献综述中，我们总结了最近研究这些疗法的文献，讨论可能支撑症状表现的潜在电路，并涵盖该领域的未来方向。通过我们全面的搜索,共有47例患者接受了基于器械的PWS神经调节治疗.两篇文章描述了VNS，4tDCS,1个rTMS和2个DBS，针对PWS的不同症状，包括异常行为，饮食亢进和体重.鉴于PWS的患病率较低，将需要多中心和多国家的努力来推进这一领域。最后,考虑到潜在的弱势群体，神经伦理学的考虑和对话应该指导这一领域。
