Clinical Outcome and Intraoperative Neurophysiology of the Lance-Adams Syndrome Treated with Bilateral Deep Brain Stimulation of the Globus Pallidus Internus: A Case Report and Review of the Literature
Clinical Outcome and Intraoperative Neurophysiology of the Lance-Adams Syndrome Treated with Bilateral Deep Brain Stimulation of the Globus Pallidus Internus: A Case Report and Review of the Literature
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双侧苍白球内部深部脑刺激治疗兰斯-亚当斯综合征的临床结果和术中神经生理学：一例报告及文献复习
DOI：
10.1159/000509318
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发表时间：
2020
影响因子：
1.7
通讯作者：
Takagi Yasushi
中科院分区：
文献类型：
--
作者：
Mure Hideo;Toyoda Naoto;Morigaki Ryoma;Fujita Koji;Takagi Yasushi
Lance-Adams综合征(LAS)是一种由缺氧缺血性脑病引起的肌阵挛综合征。LAS患者可能对一线药物治疗无效，其发病机制尚不清楚。目的描述一例接受双侧苍白球内侧核(GPI)刺激的LAS患者，并结合术中电生理检查探讨LAS的病理生理学改变。然而，在1个月内，患者出现了感官刺激诱发的面部和四肢肌阵挛。由于她的肌阵挛症状对药物治疗无效，在缺氧发作1年后，进行了GPI的脑深部刺激。结果通过优化参数设置，持续的双侧GPI刺激显著改善了患者的肌阵挛症状。在2年的随访中，她的统一肌阵挛评定量表评分从90分下降到24分。此外，我们还观察了术中双侧GPI微电极记录的阵发性放电和阵发性间歇模式，并将该区域作为治疗靶点进行刺激。结论LAS患者肌阵挛的发生可能与基础神经节环路受损有关。
BackgroundThe Lance-Adams syndrome (LAS) is a myoclonus syndrome caused by hypoxic-ischemic encephalopathy. LAS cases could be refractory to first-line medications, and the neuronal mechanism underlying LAS pathology remains unknown.ObjectivesTo describe a patient with LAS who underwent bilateral globus pallidus internus (GPi) stimulation and discuss the pathophysiology of LAS with intraoperative electrophysiological findings.PatientsA 79-year-old woman presented with a history of cardiopulmonary arrest due to internal carotid artery rupture following carotid endarterectomy after successful cardiopulmonary resuscitation. However, within 1 month, the patient developed sensory stimulation-induced myoclonus in her face and extremities. Because her myoclonic symptoms were refractory to pharmacotherapy, deep brain stimulation of the GPi was performed 1 year after the hypoxic attack.ResultsContinuous bilateral GPi stimulation with optimal parameter settings remarkably improved the patient’s myoclonic symptoms. At the 2-year follow-up, her Unified Myoclonus Rating Scale score decreased from 90 to 24. In addition, we observed burst firing and interburst pause patterns on intraoperative microelectrode recordings of the bilateral GPi and stimulated this area as the therapeutic target.ConclusionOur results show that impairment in the basal ganglion circuitry might be involved in the pathogenesis of myoclonus in patients with LAS.
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影响因子：
14.5
作者：
CHADWICK, D;HALLETT, M;MARSDEN, CD
通讯作者：
MARSDEN, CD
皮质反射性肌阵挛巨体感诱发电位的发生器机制。
DOI：
10.1093/brain/110.5.1359
发表时间：
1987
期刊：
Brain : a journal of neurology
影响因子：
--
作者：
R. Kakigi;Hiroshi Shibasaki
通讯作者：
Hiroshi Shibasaki
DOI：
10.7916/d8nz8dxp
发表时间：
2017
期刊：
Tremor and other hyperkinetic movements (New York, N.Y.)
影响因子：
--
作者：
Ramdhani RA;Frucht SJ;Kopell BH
通讯作者：
Kopell BH
肌阵挛肌张力障碍的苍白球活动与运动体征相关
影响因子：
8.6
作者：
M. Welter;D. Grabli;C. Karachi;N. Jodoin;S. Fernández;Y. Brun;S. Navarro;Alister Rogers;P. Cornu;B. Pidoux;J. Yelnik;E. Roze;É. Bardinet;M. Vidailhet
通讯作者：
M. Vidailhet
影响因子：
14.5
作者：
LANCE, JW;ADAMS, RD
通讯作者：
ADAMS, RD