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Deep Brain Stimulation for Dystonia 深部脑刺激治疗肌张力障碍
Pub Date : 2020-06-01 DOI: 10.1093/med/9780190647209.003.0036
Mitesh Lotia
The past two decades have revealed substantial benefits of bilateral pallidal deep brain stimulation (DBS) in patients with medication-refractory primary dystonia. There is a growing body of evidence now describing not only short-term but also long-term benefits up to 10 years following DBS. These benefits are often sustained, requiring minimal long-term modification. Pallidal programming for dystonia may be complex owing to the gradual onset of benefits and often delayed development of side effects. There is a relative scarcity of evidence-based recommendations for standardized programming methods. This chapter reviews essential factors to consider for appropriate patient selection and discusses strategies for initial and follow-up programming. Finally, the chapter describes the potential short-term and long-term adverse effects, while considering various strategies to mitigate them.
在过去的二十年中,双侧白斑深部脑刺激(DBS)对药物难治性原发性肌张力障碍患者有显著的益处。现在有越来越多的证据表明,星展计划不仅能带来短期效益,还能带来长达10年的长期效益。这些好处通常是持续的,需要最小的长期修改。肌张力障碍的苍白质规划可能是复杂的,因为益处逐渐开始,副作用往往延迟发展。标准化规划方法的循证建议相对缺乏。本章回顾了适当患者选择的基本因素,并讨论了初始和后续规划的策略。最后,本章描述了潜在的短期和长期的不利影响,同时考虑了各种策略来减轻它们。
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引用次数: 0
Steroid-Responsive Edema Interfering With Deep Brain Stimulation Programming 类固醇反应性水肿干扰深部脑刺激程序
Pub Date : 2020-06-01 DOI: 10.1093/med/9780190647209.003.0026
Pravin Khemani, Shilpa Chitnis
Deep brain stimulation (DBS) surgery–associated complications are most commonly due to infections and issues with surgical hardware. An uncommon postsurgical complication hindering effective programming is noninfectious edema appearing as an abnormal peri-electrode T2 signal hyperintensity on brain magnetic resonance imaging (MRI). A 72-year-old woman with Parkinson disease underwent bilateral subthalamic nucleus (STN) implantation for medication-refractory motor symptoms. Worsening balance shortly after surgery and suboptimal response to DBS programming prompted brain MRI, which showed an abnormal T2 hyperintensity around the left STN electrode. Although intravenous antibiotics were considered, the absence of clinical signs and lack of postcontrast T2 signal enhancement argued against infection. Suspecting vasogenic edema, a short course of oral dexamethasone steroids was initiated, with gradual improvement in balance and resolution of the abnormal MRI signal. The mechanism of steroid-responsive vasogenic edema after DBS surgery is not well-understood. This case underscores the importance of being vigilant about this rare complication, differentiating it from infection based on clinical presentation and radiographic characteristics, and treating it appropriately.
深部脑刺激(DBS)手术相关并发症最常见的原因是感染和手术硬件问题。在脑磁共振成像(MRI)上,非感染性水肿表现为电极周围异常的T2信号高信号,这是一种不常见的术后并发症,阻碍了有效的编程。一名72岁女性帕金森病患者接受了双侧丘脑下核(STN)植入治疗药物难治性运动症状。术后平衡性恶化和DBS编程反应不佳提示脑MRI,显示左侧STN电极周围异常T2高信号。虽然考虑静脉注射抗生素,但缺乏临床体征和造影后T2信号增强,不利于感染。怀疑血管源性水肿,开始口服地塞米松类固醇短期疗程,逐渐改善平衡和异常MRI信号的分辨率。DBS手术后类固醇反应性血管源性水肿的机制尚不清楚。本病例强调了警惕这种罕见并发症的重要性,根据临床表现和影像学特征将其与感染区分开来,并适当治疗。
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引用次数: 0
Symptomatic Cystic Lesion Following Deep Brain Stimulation Surgery 脑深部刺激手术后的症状性囊性病变
Pub Date : 2020-06-01 DOI: 10.1093/med/9780190647209.003.0007
Vibhash D. Sharma, Shilpa Chitnis
Deep brain stimulation therapy is an effective therapy for selected patients with movement disorders. The procedure is relatively safe, but complications related to the surgical procedure or implanted hardware can occur. The common complications include hemorrhage, infarct, infection, and confusion. Noninfectious cyst formation around the DBS lead is a rare but potential complication of this procedure, which can occur several weeks to months after DBS lead implantation. This chapter describes a case of noninfectious cyst formation at the tip of DBS lead in a patient with essential tremor. Clinical presentation, role of imaging, and the management options for this rare complication are discussed. This case also illustrates the importance of post-DBS imaging in suspected cases with new or unexplained symptoms.
脑深部电刺激治疗是一种有效的治疗运动障碍的患者。该手术相对安全,但可能发生与外科手术或植入硬件相关的并发症。常见的并发症包括出血、梗塞、感染和意识不清。DBS导线周围的非感染性囊肿形成是该手术的罕见但潜在的并发症,可在DBS导线植入后数周至数月发生。本章描述了一例原发性震颤患者在DBS导联尖端形成的非感染性囊肿。临床表现,影像的作用,和管理的选择,这种罕见的并发症进行了讨论。该病例也说明了在有新症状或无法解释的疑似病例中进行dbs后成像的重要性。
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引用次数: 0
Deep Brain Stimulation for Essential Tremor 脑深部电刺激治疗特发性震颤
Pub Date : 2020-06-01 DOI: 10.1093/med/9780190647209.003.0003
B. Bluett
Essential tremor (ET) is one of the most common movement disorders and can result in disabling symptoms that are refractory to standard medications. In 1997, the US Food and Drug Administration approved deep brain stimulation (DBS) of the ventral intermedius nucleus of the thalamus for treatment of ET. DBS often reduces or eliminates the need for medications to treat ET. Proper patient selection and expertise in DBS programming are critical to ensure optimal outcomes. Recent advances in DBS include directional current steering and multisource independent constant current to program each electrode with its own power source. This chapter discusses proper patient selection, programming guidelines, and clinical pearls in DBS for ET.
特发性震颤(ET)是最常见的运动障碍之一,可导致标准药物难以治疗的致残症状。1997年,美国食品和药物管理局批准对丘脑腹侧中间核进行深部脑刺激(DBS)治疗ET。DBS通常可以减少或消除治疗ET的药物需求。正确的患者选择和DBS编程的专业知识对于确保最佳结果至关重要。DBS的最新进展包括定向电流控制和多源独立恒流,以使每个电极具有自己的电源。本章讨论了脑起搏术治疗ET的正确患者选择、程序设计指南和临床要点。
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引用次数: 3
Stimulation-Induced Dyskinesia, Interleaving Settings, and Management of Subthalamic Nucleus Deep Brain Stimulation in DYT1 Dystonia DYT1肌张力障碍中刺激诱导的运动障碍,交错设置和丘脑下核深部脑刺激的管理
Pub Date : 2020-06-01 DOI: 10.1093/med/9780190647209.003.0038
Kyle T. Mitchell, Kristen Dodenhoff, P. Starr, J. Ostrem
DYT1 dystonia is a primary dystonia with potential for significant symptomatic improvement after bilateral deep brain stimulation (DBS) of the globus pallidus interna (GPi). GPi is the historical target of choice for this disease. This chapter presents a case of an adolescent with disabling generalized DYT1 dystonia who underwent bilateral subthalamic nucleus (STN) DBS as part of a prospective clinical trial. While limb and cervical dystonia dramatically improved with DBS, programming was limited by stimulation-induced bilateral limb dyskinesia, including in the left arm, which was previously unaffected by dystonia. After years of evolving symptoms and complex programming, bilateral interleaved settings using both a contact in motor STN and the most dorsal DBS contact in the zona incerta resulted in sustained, near-complete resolution of dystonia without side effects. This case illustrates the use of the STN as an effective DBS target for primary dystonia, although complex programming was necessary to mitigate stimulation-induced dyskinesia.
DYT1肌张力障碍是一种原发性肌张力障碍,在双侧深部脑刺激(DBS)内苍白球(GPi)后具有显著的症状改善潜力。GPi是这种疾病的历史目标。本章提出了一个青少年致残的全身性DYT1肌张力障碍的病例,他接受了双侧丘脑下核(STN) DBS作为前瞻性临床试验的一部分。虽然DBS显著改善了肢体和颈部肌张力障碍,但由于刺激引起的双侧肢体运动障碍(包括左臂),编程受到限制,而左臂先前未受肌张力障碍的影响。经过多年的症状演变和复杂的程序设计,双侧交错设置使用运动STN接触和最背侧的起搏器接触在无副作用的情况下持续,几乎完全解决了肌张力障碍。尽管复杂的编程对于减轻刺激引起的运动障碍是必要的,但该病例说明了将STN作为原发性肌张力障碍的有效DBS靶点的使用。
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引用次数: 1
Rescue Ventral Intermediate Thalamus Deep Brain Stimulation to Address Refractory Tremor Following Subthalamic Nucleus Deep Brain Stimulation With Brittle Dyskinesia 救援腹侧中间丘脑深部脑刺激治疗丘脑下核深部脑刺激后伴脆性运动障碍的难治性震颤
Pub Date : 2020-06-01 DOI: 10.1093/med/9780190647209.003.0023
Mitra Afshari, J. Ostrem, M. S. Luciano, P. Larson
This chapter discusses a case in which a “rescue” deep brain stimulation (DBS) lead was implanted to address suboptimal tremor control. The patient was a 52-year-old woman with disabling bilateral postural and action hand tremor who also had mild parkinsonian signs. An essential tremor (ET)–Parkinson disease (PD) syndrome was suspected, and subthalamic nucleus (STN) DBS was pursued. Attempts at optimizing tremor control by reprogramming were limited by the induction of brittle dyskinesia even with small amounts of stimulation. Bilateral ventral intermediate thalamus DBS leads were then implanted, and the tremors improved significantly. Troubleshooting strategies for optimizing tremor control and reducing STN DBS–induced brittle dyskinesia are discussed. The chapter reviews important learning points on DBS target selection for ET, PD, and ET-PD spectrum syndromes.
本章讨论了一个病例,其中“抢救”脑深部刺激(DBS)铅植入解决次优震颤控制。患者是一名52岁的女性,患有致残性双侧姿势性和行动性手部震颤,并伴有轻度帕金森症状。怀疑为特发性震颤(ET) -帕金森病(PD)综合征,并进行丘脑下核(STN) DBS检查。通过重新编程来优化震颤控制的尝试受到了即使少量刺激也会诱发脆性运动障碍的限制。然后植入双侧腹侧丘脑中间DBS导联,震颤明显改善。讨论了优化震颤控制和减少STN - dbs引起的脆性运动障碍的故障排除策略。本章回顾了ET、PD和ET-PD谱系综合征的DBS靶点选择的重要学习要点。
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引用次数: 0
Tardive Dystonia and Dyskinesia Responsive to Deep Brain Stimulation 迟发性肌张力障碍和运动障碍对深部脑刺激的反应
Pub Date : 2020-06-01 DOI: 10.1093/med/9780190647209.003.0044
Shannon Y. Chiu, I. Malaty
Tardive disorders encompass phenomenologically diverse delayed-onset persistent involuntary motor symptoms associated with exposure to dopamine receptor blocking agents. Two common tardive disorders encountered in the clinical setting include tardive dyskinesia and tardive dystonia. This chapter presents a patient with severe refractory tardive dyskinesia and also tardive dystonia, manifesting as frequent and disabling retropulsion. He initially underwent bilateral globus pallidus interna (GPi) deep brain stimulation (DBS) but was found to have lead migration secondary to his severe hyperkinetic movements. He had persistent symptoms despite lead revision and ultimately required bilateral subthalamic nucleus (STN) rescue DBS implantation. The rescue procedure was synergistic with the initial GPi DBS and markedly improved his symptoms. Severe tardive dyskinesia and dystonia may respond to bilateral GPi DBS, and if necessary, rescue STN DBS can be added.
迟发性疾病包括与暴露于多巴胺受体阻滞剂有关的现象上多样的迟发性持续性不自主运动症状。临床中遇到的两种常见的迟发性疾病包括迟发性运动障碍和迟发性肌张力障碍。本章介绍了一位患有严重难治性迟发性运动障碍和迟发性肌张力障碍的患者,表现为频繁和致残的反冲。患者最初接受了双侧内苍白球(GPi)深部脑刺激(DBS),但发现严重的多动运动导致铅迁移。尽管进行了导联翻修,但他的症状仍持续存在,最终需要双侧丘脑下核(STN)抢救DBS植入。抢救程序与初始GPi DBS具有协同作用,并显着改善了他的症状。严重的迟发性运动障碍和肌张力障碍可能对双侧GPi DBS有反应,必要时可增加STN DBS抢救。
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引用次数: 0
Choosing a Target for Deep Brain Stimulation in Dystonia-Associated Tremor 肌张力障碍相关震颤的深部脑刺激靶点选择
Pub Date : 2020-06-01 DOI: 10.1093/med/9780190647209.003.0037
M. Siddiqui, S. Tatter
This chapter presents a case of medication-refractory primary generalized dystonia presenting with disabling symptoms of cervical dystonia and dystonic bilateral arm tremors. This case posed a challenge in the choice of the appropriate target for deep brain stimulation (DBS) because the targeting of the globus pallidus interna improves dystonia symptoms but may have a less predictable response to tremor. DBS targeting the ventral intermedius may not in many cases improve dystonia symptoms but can effectively control tremor. The authors describe a case in which they employed a systematic approach including implanting multiple leads in different DBS targets. This approach resulted in a favorable outcome for this patient in dystonia and in dystonic tremor.
本章报告一例药物难治性原发性全身性肌张力障碍,表现为颈肌张力障碍和双侧手臂肌张力障碍震颤等致残症状。该病例对深部脑刺激(DBS)的合适靶点的选择提出了挑战,因为针对内苍白球可改善肌张力障碍症状,但可能对震颤的反应不太可预测。针对腹正中肌的DBS可能在许多情况下不能改善肌张力障碍症状,但可以有效地控制震颤。作者描述了一个案例,他们采用了一种系统的方法,包括在不同的DBS目标中植入多个导联。这种方法对肌张力障碍和肌张力障碍震颤患者有良好的疗效。
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引用次数: 0
Bilateral Globus Pallidus Deep Brain Stimulation for Dystonia and Dystonic Tremor in Spinocerebellar Ataxia 17 双侧苍白球深部脑刺激治疗脊髓小脑共济失调患者肌张力障碍和肌张力障碍震颤17
Pub Date : 2020-06-01 DOI: 10.1093/med/9780190647209.003.0042
A. Shukla, Pamela Zeilman
Spinocerebellar ataxia 17 (SCA17) is a rare form of autosomal dominant cerebellar ataxia. SCA17 results from trinucleotide repeat expansions and has no effective symptomatic treatments to date. This chapter describes a case of SCA17 with dystonic symptoms that presented during adolescence. Symptoms were generalized in distribution and were accompanied with a dystonic tremor of the arms. Because many medication trials did not alleviate the patient’s symptoms, he underwent bilateral globus pallidus interna (GPi) deep brain stimulation (DBS) surgery. Several DBS programming sessions were performed. Dystonia and dystonic tremor were controlled with the settings adjusted at high pulse widths and low frequencies. Furthermore, the cyclic mode of stimulation was employed to mitigate the capsular side effects. Two years after DBS surgery, the patient presented with cerebellar ataxia that prompted an extensive workup, including genetic testing and revision of his diagnosis from generalized dystonia to a dystonia syndrome. The patient continues to endorse symptomatic benefits with DBS for dystonia and dystonic tremor 11 years after surgery, despite a diagnosis of dystonia syndrome. The current case of SCA17 cerebellar ataxia indicates that bilateral GPi DBS is a potential option for the long-term symptomatic treatment of dystonia and tremor symptoms manifesting as a part of a clinical syndrome.
脊髓小脑性共济失调17 (SCA17)是一种罕见的常染色体显性小脑性共济失调。SCA17是由三核苷酸重复扩增引起的,迄今尚无有效的对症治疗方法。本章描述了一例在青春期出现张力障碍症状的SCA17。症状在分布上具有广泛性,并伴有手臂肌张力障碍震颤。由于许多药物试验并没有减轻患者的症状,他接受了双侧内白球(GPi)深部脑刺激(DBS)手术。进行了几次DBS编程。肌张力障碍和肌张力障碍震颤控制设置调整在高脉宽和低频率。此外,循环刺激模式被用来减轻胶囊的副作用。DBS手术两年后,患者出现小脑性共济失调,进行了广泛的检查,包括基因检测和将其诊断从全身性肌张力障碍修改为肌张力障碍综合征。尽管患者被诊断为肌张力障碍综合征,但手术后11年,DBS治疗肌张力障碍和肌张力障碍震颤仍有症状性获益。目前的sc17小脑共济失调病例表明,双侧GPi DBS是作为临床综合征一部分表现的肌张力障碍和震颤症状的长期对症治疗的潜在选择。
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引用次数: 0
Cerebral Venous Infarction After Deep Brain Stimulation Surgery 脑深部刺激手术后脑静脉梗死
Pub Date : 2020-06-01 DOI: 10.1093/med/9780190647209.003.0029
Andi N. Sendjaja, T. Morishita, Tooru Inoue
Venous infarction is an important adverse event potentially encountered in the practice of deep brain stimulation (DBS). This adverse event is considered to result from damage to the cortical vein during surgery and is noted on postoperative day 1 or 2 owing to neurologic symptoms such as confusion and headache. The imaging findings are characterized by edema surrounding the trajectory of the DBS lead and flame-shaped hemorrhage in some cases. Venous infarction can be managed conservatively with careful observation, and the prognosis is usually benign. The neurologic condition of patients can in many cases return to baseline after days to weeks, as observed in this case. The prognostic information is valuable for reassuring afflicted patients and their families.
静脉梗死是脑深部电刺激(DBS)治疗过程中可能遇到的重要不良事件。这种不良事件被认为是由于手术过程中皮质静脉的损伤引起的,并且在术后第1天或第2天由于神经系统症状如精神错乱和头痛而被注意到。影像学表现为脑起搏器导联轨迹周围水肿,部分病例呈火焰状出血。静脉梗死可以保守治疗并仔细观察,预后通常是良性的。正如本例所观察到的,在许多情况下,患者的神经系统状况可以在几天到几周后恢复到基线。预后信息是有价值的,使患者和他们的家人放心。
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引用次数: 0
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Deep Brain Stimulation
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