Department of Neurosurgery, Graduate School of Biomedical Sciences, Tokushima University, Tokushima, Japan,
Parkinson's Disease and Dystonia Research Center, Tokushima University Hospital, Tokushima, Japan,
Stereotact Funct Neurosurg. 2020;98(6):399-403. doi: 10.1159/000509318. Epub 2020 Sep 7.
The 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.
To describe a patient with LAS who underwent bilateral globus pallidus internus (GPi) stimulation and discuss the pathophysiology of LAS with intraoperative electrophysiological findings.
A 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.
Continuous 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.
Our results show that impairment in the basal ganglion circuitry might be involved in the pathogenesis of myoclonus in patients with LAS.
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 病例可能对一线药物治疗有抗性，且 LAS 病理的神经元机制仍不清楚。
描述一位接受双侧苍白球内侧部（GPi）刺激的 LAS 患者，并结合术中电生理发现讨论 LAS 的病理生理学。
一位 79 岁女性，因颈动脉内膜切除术中心颈动脉破裂导致心肺骤停后成功心肺复苏，但在 1 个月内出现感觉刺激诱导的面和肢体肌阵挛。由于她的肌阵挛症状对药物治疗有抗性，在缺氧发作 1 年后进行了 GPi 的深部脑刺激。
双侧 GPi 以最佳参数设置进行持续刺激，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 分降至 24 分。此外，我们在双侧 GPi 的术中微电极记录中观察到爆发放电和爆发间停顿模式，并将该区域作为治疗靶点进行刺激。
我们的结果表明，基底节回路的损伤可能与 LAS 患者肌阵挛的发病机制有关。
