Ramdhani Ritesh A, Frucht Steven J, Kopell Brian H
Division of Movement Disorders, Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
Tremor Other Hyperkinet Mov (N Y). 2017 May 19;7:461. doi: 10.7916/D8NZ8DXP. eCollection 2017.
Post-hypoxic myoclonus (PHM) is a syndrome that occurs when a patient has suffered hypoxic brain injury. The myoclonus is usually multifocal and generalized, often stemming from both cortical and subcortical origins. In severe cases, pharmacological treatments with antiepileptic medications may not satisfactorily control the myoclonus.
We present a case of a 23-year-old male with chronic medication refractory PHM following a cardiopulmonary arrest related to an asthmatic attack who improved with bilateral globus pallidus internus (GPi) deep brain stimulation (DBS). We review the clinical features of PHM, as well as the preoperative and postoperative Unified Myoclonus Rating Scale scores and DBS programming parameters in this patient and compare them with the three other published PHM-DBS cases in the literature.
This patient experienced an alleviation of myoclonic jerks at rest and a 39% reduction in action myoclonus with improvement in both positive and negative myoclonus with bilateral GPi-DBS. High frequency stimulation (130 Hz) with amplitudes >2.5 V were needed for the therapeutic response.
We demonstrate a robust improvement in a medication refractory PHM patient with bilateral GPi-DBS, and suggest that it is a viable therapeutic option for debilitating post-hypoxic myoclonus.
缺氧后肌阵挛(PHM)是一种在患者遭受缺氧性脑损伤时出现的综合征。肌阵挛通常是多灶性和全身性的,常常源于皮质和皮质下区域。在严重情况下,使用抗癫痫药物进行药物治疗可能无法令人满意地控制肌阵挛。
我们报告一例23岁男性患者,其因哮喘发作导致心肺骤停后出现慢性药物难治性PHM,经双侧内侧苍白球(GPi)深部脑刺激(DBS)后病情改善。我们回顾了该患者PHM的临床特征,以及术前和术后统一肌阵挛评定量表评分和DBS程控参数,并将其与文献中其他三例已发表的PHM-DBS病例进行比较。
该患者静息时肌阵挛性抽搐减轻,动作性肌阵挛减少39%,双侧GPi-DBS使正负肌阵挛均得到改善。治疗反应需要频率>2.5 V的高频刺激(130 Hz)。
我们证明双侧GPi-DBS可使药物难治性PHM患者显著改善,并表明它是治疗衰弱性缺氧后肌阵挛的一种可行治疗选择。