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Patterns of neural activity and clinical outcomes in a juvenile Huntington's Disease patient undergoing Deep Brain Stimulation of the subthalamic nucleus 青少年亨廷顿氏病患者接受丘脑下核深部脑刺激的神经活动模式和临床结果
Deep Brain Stimulation Pub Date : 2023-06-01 Epub Date: 2023-03-12 DOI: 10.1016/j.jdbs.2023.03.001
Ahmet Kaymak , Matteo Vissani , Matteo Lenge , Federico Melani , Edoardo Fino , Pietro Cappelletto , Germana Tuccinardi , Michele Alessandro Cavallo , Flavio Giordano , Alberto Mazzoni
{"title":"Patterns of neural activity and clinical outcomes in a juvenile Huntington's Disease patient undergoing Deep Brain Stimulation of the subthalamic nucleus","authors":"Ahmet Kaymak ,&nbsp;Matteo Vissani ,&nbsp;Matteo Lenge ,&nbsp;Federico Melani ,&nbsp;Edoardo Fino ,&nbsp;Pietro Cappelletto ,&nbsp;Germana Tuccinardi ,&nbsp;Michele Alessandro Cavallo ,&nbsp;Flavio Giordano ,&nbsp;Alberto Mazzoni","doi":"10.1016/j.jdbs.2023.03.001","DOIUrl":"https://doi.org/10.1016/j.jdbs.2023.03.001","url":null,"abstract":"<div><h3>Background</h3><p>Huntington's disease (HD) is a hereditary neurodegenerative disease leading to cognitive and motor impairment. HD depends on basal ganglia dysfunctions, but the role of subthalamic nucleus (STN) neurons is not completely known. Drug-resistant motor symptoms of HD can be alleviated by neuromodulation of the basal ganglia through Deep Brain Stimulation (DBS) of STN. DBS target selection is supported by intra-operative microelectrode recordings (MER). MER have been previously used to characterize neural dynamics of STN in several movement disorders and can provide information on firing patterns underlying HD.</p></div><div><h3>Methods</h3><p>We analyzed MER data acquired during bilateral DBS of STN in a juvenile HD female patient with hypokinetic motor symptoms (generalized dystonia, stiffness, and severe gait impairment). Firing patterns of STN in HD were characterized by isolating single neuron activities (n = 23) and measuring their regularity, bursting, and oscillatory behavior. Multi-unit activity recordings spectrum was used to estimate the presence of network oscillations.</p></div><div><h3>Results</h3><p>STN neurons displayed irregular dynamics and intense and sparse bursting. Only 3/23 neurons presented oscillatory activity. However, network oscillations were detected, in particular in the beta (12–30 Hz) band. After bilateral STN-DBS surgery, the Unified Huntington's Disease Rating Scale decreased from 60 to 54.</p></div><div><h3>Conclusions</h3><p>The most salient difference between HD and other movement disorders in STN activity is the presence of a weakly synchronized oscillatory mode, in which oscillations are evident at the network level but not at the single neuron level.</p></div>","PeriodicalId":100359,"journal":{"name":"Deep Brain Stimulation","volume":"1 ","pages":"Pages 15-19"},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49749642","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 4
Issues to Consider When Mild Cognitive Impairment Is Revealed in Preoperative Screening 术前筛查发现轻度认知障碍时应考虑的问题
Deep Brain Stimulation Pub Date : 2020-06-01 DOI: 10.1093/med/9780190647209.003.0012
Sushma Kola, Anhar Hassan
{"title":"Issues to Consider When Mild Cognitive Impairment Is Revealed in Preoperative Screening","authors":"Sushma Kola, Anhar Hassan","doi":"10.1093/med/9780190647209.003.0012","DOIUrl":"https://doi.org/10.1093/med/9780190647209.003.0012","url":null,"abstract":"A 72-year-old man with Parkinson disease of 7 years duration, multifactorial gait disorder, and minor memory complaints underwent deep brain stimulation (DBS) evaluation to treat motor fluctuations and dyskinesia. Neuropsychometric evaluation revealed multidomain mild cognitive impairment, which was stable to improved on repeat testing 6 months later. He underwent bilateral subthalamic nucleus DBS with improvement in dyskinesia, and residual motor fluctuations were medically managed. He denied postoperative cognitive changes, although repeat neuropsychometric evaluation 9 months later showed decline in cognition. The patient moved to an assisted living facility shortly afterward because of progressive gait disability. One year later, he developed florid Parkinson disease psychosis, with partial improvement after cessation of entacapone. We discuss the rationale for the assessment of mild cognitive impairment before DBS, DBS target selection, risk factors associated with postoperative cognitive decline, and strategies to minimize this.","PeriodicalId":100359,"journal":{"name":"Deep Brain Stimulation","volume":"61 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75821738","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Deep Brain Stimulation for Medication-Refractory Aggressive and Injurious Behavior 深部脑刺激治疗药物难治性攻击和伤害行为
Deep Brain Stimulation Pub Date : 2020-06-01 DOI: 10.1093/med/9780190647209.003.0041
O. Bernal-Pacheco, Adriana Martinez Perez, Mary Fonseca-Ramos
{"title":"Deep Brain Stimulation for Medication-Refractory Aggressive and Injurious Behavior","authors":"O. Bernal-Pacheco, Adriana Martinez Perez, Mary Fonseca-Ramos","doi":"10.1093/med/9780190647209.003.0041","DOIUrl":"https://doi.org/10.1093/med/9780190647209.003.0041","url":null,"abstract":"Aggressiveness, defined as violent and angry behavior, is a special and challenging condition that is encountered in medicine. There is frequently involvement of the patient with self-aggressiveness and automutilation, and there may be a history in relatives. Allied health personal and others may be affected by injuries and trauma caused by the patient. Lesioning of the posterior hypothalamus (PHyp) is a technique that was used successfully to control aggressiveness 40 years ago. Deep brain stimulation (DBS) has been recently used for treatment of motor symptoms of movement disorders as well as for some psychiatric diseases, and groups have also suggested that it may be useful for aggressive behavior in select circumstances. This chapter reports the case of a patient with uncontrollable aggressiveness and the implantation of DBS in the PHyp. In this case, the DBS also helped to control epilepsy and to focus attention.","PeriodicalId":100359,"journal":{"name":"Deep Brain Stimulation","volume":"14 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89779648","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Deep Brain Stimulation for Dystonia 深部脑刺激治疗肌张力障碍
Deep Brain Stimulation Pub Date : 2020-06-01 DOI: 10.1093/med/9780190647209.003.0036
Mitesh Lotia
{"title":"Deep Brain Stimulation for Dystonia","authors":"Mitesh Lotia","doi":"10.1093/med/9780190647209.003.0036","DOIUrl":"https://doi.org/10.1093/med/9780190647209.003.0036","url":null,"abstract":"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.","PeriodicalId":100359,"journal":{"name":"Deep Brain Stimulation","volume":"32 6","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72592294","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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
Deep Brain Stimulation Pub Date : 2020-06-01 DOI: 10.1093/med/9780190647209.003.0026
Pravin Khemani, Shilpa Chitnis
{"title":"Steroid-Responsive Edema Interfering With Deep Brain Stimulation Programming","authors":"Pravin Khemani, Shilpa Chitnis","doi":"10.1093/med/9780190647209.003.0026","DOIUrl":"https://doi.org/10.1093/med/9780190647209.003.0026","url":null,"abstract":"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.","PeriodicalId":100359,"journal":{"name":"Deep Brain Stimulation","volume":"289 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74388986","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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
Deep Brain Stimulation Pub Date : 2020-06-01 DOI: 10.1093/med/9780190647209.003.0007
Vibhash D. Sharma, Shilpa Chitnis
{"title":"Symptomatic Cystic Lesion Following Deep Brain Stimulation Surgery","authors":"Vibhash D. Sharma, Shilpa Chitnis","doi":"10.1093/med/9780190647209.003.0007","DOIUrl":"https://doi.org/10.1093/med/9780190647209.003.0007","url":null,"abstract":"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.","PeriodicalId":100359,"journal":{"name":"Deep Brain Stimulation","volume":"54 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81345261","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Deep Brain Stimulation for Essential Tremor 脑深部电刺激治疗特发性震颤
Deep Brain Stimulation Pub Date : 2020-06-01 DOI: 10.1093/med/9780190647209.003.0003
B. Bluett
{"title":"Deep Brain Stimulation for Essential Tremor","authors":"B. Bluett","doi":"10.1093/med/9780190647209.003.0003","DOIUrl":"https://doi.org/10.1093/med/9780190647209.003.0003","url":null,"abstract":"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.","PeriodicalId":100359,"journal":{"name":"Deep Brain Stimulation","volume":"13 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90437363","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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
Deep Brain Stimulation Pub Date : 2020-06-01 DOI: 10.1093/med/9780190647209.003.0038
Kyle T. Mitchell, Kristen Dodenhoff, P. Starr, J. Ostrem
{"title":"Stimulation-Induced Dyskinesia, Interleaving Settings, and Management of Subthalamic Nucleus Deep Brain Stimulation in DYT1 Dystonia","authors":"Kyle T. Mitchell, Kristen Dodenhoff, P. Starr, J. Ostrem","doi":"10.1093/med/9780190647209.003.0038","DOIUrl":"https://doi.org/10.1093/med/9780190647209.003.0038","url":null,"abstract":"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.","PeriodicalId":100359,"journal":{"name":"Deep Brain Stimulation","volume":"20 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76904830","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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
Deep Brain Stimulation Pub Date : 2020-06-01 DOI: 10.1093/med/9780190647209.003.0023
Mitra Afshari, J. Ostrem, M. S. Luciano, P. Larson
{"title":"Rescue Ventral Intermediate Thalamus Deep Brain Stimulation to Address Refractory Tremor Following Subthalamic Nucleus Deep Brain Stimulation With Brittle Dyskinesia","authors":"Mitra Afshari, J. Ostrem, M. S. Luciano, P. Larson","doi":"10.1093/med/9780190647209.003.0023","DOIUrl":"https://doi.org/10.1093/med/9780190647209.003.0023","url":null,"abstract":"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.","PeriodicalId":100359,"journal":{"name":"Deep Brain Stimulation","volume":"108 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81577051","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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
Deep Brain Stimulation Pub Date : 2020-06-01 DOI: 10.1093/med/9780190647209.003.0037
M. Siddiqui, S. Tatter
{"title":"Choosing a Target for Deep Brain Stimulation in Dystonia-Associated Tremor","authors":"M. Siddiqui, S. Tatter","doi":"10.1093/med/9780190647209.003.0037","DOIUrl":"https://doi.org/10.1093/med/9780190647209.003.0037","url":null,"abstract":"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.","PeriodicalId":100359,"journal":{"name":"Deep Brain Stimulation","volume":"12 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83225959","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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