脑深部电刺激治疗肌张力障碍

目的 探讨脑深部电刺激术(deep brain stimulation, DBS)治疗全身性、偏身性和节段性肌张力障碍的有效性和安全性,肌张力障碍患者治疗和术后程控的策略.方法 采用微电极记录下丘脑底核(subthalamic nucleus, STN)、苍白球内侧部(globus pallidus intemus, GPi)和丘脑腹中间核(ventrointermediate nucleus, Vim)埋置脑深部刺激器治疗肌张力障碍患者15例,同时记录患者对侧受累肌肉的电活动.其中13例患者的刺激靶点为STN(11例为双侧,2例为单侧),1例刺激靶点为单侧GPi,1例为Vim.结果 除1例严重全身肌张力障碍患者外,其余患者因随访时间长短,均有不同程度改善,改善率从22.0%～95.8%不等.其中随访时间超过12个月的患者症状改善率均大于48.6%.1例因全身扭动造成电极外露,颈、胸腹部切口感染,最终将DBS装置取出.另有1例患者因双侧上端的两个触点断路,再次手术将电极位置上移.所有患者均未出现因穿刺造成的颅内出血的永久并发症.结论 通过对现有DBS治疗肌张力障碍病例资料有效性和安全性的总结,DBS成为治疗肌张力障碍的一种新方法.     

Deep brain stimulation in dystonia

Renewed interest in stereotaxy for dystonia followed the introduction of deep brain stimulation (DBS) in Parkinson's disease and essential tremor in the 1990s. DBS evolved from ablative surgery, which was applied with varying results in the 1950s in patients with movement disorders such as Parkinson's disease, essential tremor and dystonia. The present review summarizes the current knowledge on clinical aspects of DBS in dystonia (Dec. 2002). Excellent results have been achieved in dystonic patients carrying a mutation in the DYT1 gene with improvements up to 90 %. Similar results may also be obtained in patients with idiopathic generalized dystonia, myoclonus-dystonia syndrome, and tardive dystonia. Substantial improvement has been observed in patients with focal dystonia (for instance cervical dystonia). Patients with secondary dystonia often display a lesser and more variable degree of improvement. Long-term studies are warranted to assess both motor and neuropsychological sequelae of DBS in dystonia. Furthermore, the optimal target for different dystonic disorders remains to be determined, although the globus pallidus internus has currently emerged as the most promising target for dystonia.     

Deep brain stimulation in dystonia

Journal of Neurology - Renewed interest in stereotaxy for dystonia followed the introduction of deep brain stimulation (DBS) in Parkinson's disease and essential tremor in the 1990s. DBS...     

脑深部电刺激苍白球内侧部治疗肌张力障碍

目的 探讨脑深部电刺激(DBS)苍白球内侧部(GPi)治疗肌张力障碍的有效性和安全性,以及术后程控策略.方法 采用微电极记录GPi的电生理信号,埋置脑深部刺激器治疗肌张力障碍患者8例,同时记录患者对侧受累肌肉的电活动.所有患者的刺激靶点均为双侧GPi.结果 除1例继发性全身肌张力障碍患者外,其余患者因随访时间长短,均有不同程度改善,随访时间12-36个月,改善率35.5％～87.5％.其中随访超过18个月的患者症状改善率均＞59.6％.1例因全身扭动造成电极外露,锁骨下切口感染,最终将DBS装置取出.另有1例术后靶点区域少量出血,血肿吸收后原有症状仍有改善.结论 通过对现有DBS刺激GPi治疗肌张力障碍病例资料有效性和安全性的总结,DBS成为治疗肌张力障碍的一种有效方法.GPi是治疗肌张力障碍较为有效的靶点.     

Deep brain stimulation for generalised dystonia and spasmodic torticollis.

Dystonia appears distinct from the other tremulous disorders in that improvement following deep brain stimulation frequently appears in a delayed and progressive manner. The rate of this improvement and the point at which no further progress can be expected are presently unknown. The establishment of these parameters is important in the provision of accurate and relevant prognostic information to these patients, their carers, and their treating physicians. We studied 12 consecutive patients with generalised dystonia (n=6) and spasmodic torticollis (n=6) who underwent bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) and were followed up for a minimum of 2 years postoperatively. Standard rating scales were used to quantify their neurological improvement. Both groups experienced a statistically significant improvement in their rating scores at both one and two years following surgery. At 2 years follow-up, the spasmodic torticollis group exhibited a 59% improvement in their total Toronto Western Spasmodic Torticoilis Rating Scale (TWSTRS) rating score and the generalised dystonia group attained a 46% improvement in their overall Burke, Fahn and Marsden Dystonia Rating Scale (BFMDRS) evaluation. Ninety-five percent of the final improvement was attained by 6.4 months in the generalised dystonia group and by 6.6 months in those with spasmodic torticollis. There was no significant improvement after one year postoperatively. These findings add further support to GPi DBS as an effective treatment for generalised dystonia and spasmodic torticollis, and furnish important information as to the expected rate of improvement and the point at which no further gains can be reasonably anticipated.     

Deep brain stimulation for torsion dystonia in children

Introduction Deep brain stimulation (DBS) at the internal globus pallidus (GPi) is an effective treatment for some patients with medically refractory torsion dystonia. In this article, we review the results of pallidal DBS surgery in children with dystonia. Details of the DBS procedure and programming of the DBS devices are discussed. Discussion Pallidal DBS is most effective in patients with primary generalized dystonia. Children and adolescents possessing the DYT1 gene mutation may respond best of all. The presence of static dystonic postures and/or fixed orthopedic contractures may limit the functional response to DBS and may require additional surgery. Conclusion As a group, patients with secondary dystonias respond less well to DBS than patients with primary dystonia. However, patients with dystonia secondary to anoxic brain injury who have grossly intact basal ganglia anatomy may represent a subpopulation for whom pallidal DBS is a viable option.     

Deep brain stimulation of globus pallidus internus for dystonia

Neuromodulation is the functional modification of neural structures through the use of electrical stimulation. Its most clinically applicable use is deep brain stimulation (DBS) of basal ganglia structures in Parkinson's disease (PD) and essential tremor (ET). More recently, it has been used as a means of treating dystonic movement disorders. The main target of DBS for dystonia is the posteroventral globus pallidus internus (GPi), although the thalamus has been used as an alternate target in a minority of cases. In comparison to the effects seen in PD, the improvement in dystonic postures appear to differ in several ways--delay of clinical benefit, higher voltage requirements, and varied stimulator settings. In this review, the authors discuss the clinical characteristics, pathophysiology, microelectrode recording (MER) signatures, optimal surgical targets, programming parameters and outcomes in dystonia.     

Deep brain stimulation in the treatment of dystonia

Dystonia refers to movement disorders characterized by sustained muscle contractions that produce abnormal postures and twisting movements. First-line therapy for dystonia includes several classes of pharmacologic agents. Botulinum toxin injections are the treatment of choice for several forms of focal dystonia. Many patients with dystonia do not benefit from these treatments, and for those patients whose symptoms are sufficiently troublesome, surgical treatment can be used to reduce symptoms and to improve function. Formerly the ablative procedures of thalamotomy and pallidotomy were used. More recently, deep brain stimulation (DBS) has emerged not only as the preferred surgical treatment for advanced idiopathic form of Parkinson's disease and severe forms of essential tremor but also for dystonia. For dystonia, stimulation directed at the globus pallidus internus has been the most thoroughly studied to date. Advantages of DBS include its relatively non-destructive nature, its adjustability and reversibility, and its capacity to be used bilaterally in a single surgical session. Use of DBS to treat dystonia is a rapidly evolving area, and preliminary evidence suggests that primary dystonia linked to genetic mutation, especially DYT-1 positive generalized dystonia, and other primary dystonias respond most dramatically to treatment with DBS, whereas secondary dystonia tends to be less responsive.     

Deep brain stimulation for dystonia: outcome at long-term follow-up

OBJECTIVE: Deep brain stimulation (DBS) has emerged as a useful therapeutic option for patients with insufficient benefit from conservative treatment. METHODS: Nine patients with chronic DBS who suffered from cervical dystonia (4), generalized dystonia (2), hemidystonia (1), paroxysmal dystonia (1) and Meige syndrome (1) were available for formal follow-up at three years postoperatively, and beyond up to 10 years. All patients had undergone pallidal stimulation except one patient with paroxysmal dystonia who underwent thalamic stimulation. RESULTS: Maintained improvement was seen in all patients with pallidal stimulation up to 10 years after surgery except in one patient who had a relative loss of benefit in dystonia ratings but continued to have improved disability scores. After nine years of chronic thalamic stimulation there was a mild loss of efficacy which was regained when the target was changed to the pallidum in the patient with paroxysmal dystonia. There were no major complications related to surgery or to chronic stimulation. Pacemakers had to be replaced within 1.5 to 2 years, in general. CONCLUSION: DBS maintains marked long-term symptomatic and functional improvement in the majority of patients with dystonia.     

Deep brain stimulation in the treatment of severe dystonia

A retrospective study of a consecutive series of 19 patients with medically intractable dystonia treated with uni- or bilateral deep brain stimulation (DBS) is reported. A minimal follow-up of 6 months was available, up to eleven years in one patient. The first twelve consecutive patients (4 with primary and 8 with secondary dystonia) were treated with chronic stimulation of the posterior part of the ventrolateral thalamic nucleus (VLp). In this group global functional outcome was improved in 8 patients, although dystonia movement and disability scale scores did not show significant improvement. Of the 12 patients treated first by VLp DBS, three (1 primary and 2 secondary dystonia) underwent pallidal (GPi) DBS after the VLp DBS failed to improve their symptoms. The last seven consecutive patients (5 primary and 2 secondary dystonia) were treated directly with GPi DBS. Extracranial infection prevented chronic GPi DBS in one patient. In another GPi patient, preliminary negative tests with the electrodes discouraged implantation of the stimulators, and the patient was not treated with chronic DBS. In the remaining group of eight patients including those previously treated with VLp DBS, chronic GPi DBS resulted in a significant improvement in the dystonia movement scale and disability scores. Although this is a retrospective study dealing with dystonia of heterogeneous etiology, the results strongly suggest that GPi DBS has a better outcome than VLp DBS Received: 22 January 2001 / Received in revised form: 28 February 2001 / Accepted: 1 March 2001     

脑深部电刺激术治疗原发性全身性肌张力障碍(附2例报告)

目的 探讨关于脑深部电刺激术治疗原发性全身性肌张力障碍疗效的影响因素.方法 评估病程、病情相似的2例原发性全身性肌张力障碍患者接受脑深部电刺激术治疗的术后疗效.病例1:8岁发病,BFMDRS评分100,运动评分80,功能障碍评分20,DYT1基因突变阳性.病例2:18岁发病,BFMDRS评分98,运动评分71,功能障碍评分27,DYT1基因突变阴性.结果 2例患者术后症状均得到了改善,给予刺激治疗5个月时病例1的BFMDRS/运动评分/功能评分分别为38/32/6,病例2分别为66/44/22.病例1术后肌张力障碍症状改善的速度和程度均优于病例2,且病例2术后功能障碍改善较差.结论 脑深部电刺激术治疗原发性全身性肌张力障碍的术后疗效主要与手术时机有很大关系,应早期手术.     

Deep brain stimulation of the internal globus pallidus for refractory tardive dystonia

We report a rapid, dramatic and sustained improvement following bilateral pallidal stimulation in two patients affected by intractable generalized tardive dystonia. Both patients had a chronic psychiatric disorder and developed chronic disabling generalized dystonic symptoms persisting despite prolonged withdrawal of neuroleptics and all available symptomatic treatment. The clinical benefit in both patients persisted throughout all the follow up period of 13 and 7 months. The favorable and prolonged response in our two patients suggests that deep brain stimulation may be an effective treatment for medically refractory tardive dystonia.     

Deep brain stimulation of the GPi treats restless legs syndrome associated with dystonia.

We report on the case of a woman with generalized dystonia and restless legs syndrome whose restless legs resolved after bilateral internal globus pallidus deep brain stimulation, and recurred unilaterally after an infection required removal of one of the devices.     

Deep brain stimulation for psychiatric disorders.

Over the last decade, deep brain stimulation (DBS) has revolutionized the practice of neurosurgery, particularly in the realm of movement disorders. It is no surprise that DBS is now being studied in the treatment of refractory psychiatric disease. Deep brain stimulation has inherent advantages over previous lesioning procedures. It is fully reversible, and stimulation can be adjusted according to a patient's changing symptoms and disease progression. Coupled with the fact that the stimulation can generally be turned on or off without the patient's awareness, DBS provides a unique opportunity for double-blinding studies. To undertake DBS for psychiatric conditions, appropriate surgical targets must be chosen. What is most strongly supported is the role of cortico-striato-thalamocortical (CSTC) loops in the pathophysiology of psychiatric symptoms. Recent functional imaging studies have consistently found evidence that corroborate this model of psychiatric symptom pathogenesis. Based on the psychiatric and cognitive effects seen in recent movement disorder surgery, it is apparent that modulation of neural systems subserving psychiatric phenomenon can be accomplished by DBS. The few published studies on DBS for obsessive-compulsive disorder (OCD) suggest that this can be done safely. While efficacy data are still uncertain, initial data are promising.     

Globus pallidus deep brain stimulation in dystonia.

Globus pallidus deep brain stimulation (GPi-DBS) is a useful alternative in the treatment of dystonia. Patients selected for GPi-DBS were prospectively rated with the Unified Dystonia Rating Scale (UDRS). Also, "blinded" videotape assessments were performed. Eleven patients were identified. Compared with pre-DBS scores, there were improvements in mean total UDRS score (15.3%) and in the following subscores: neck (18.18%), trunk (32.9%), arm (17.9%), and leg (19.9%). One patient developed a skin infection and erosion requiring surgical debridement. GPi-DBS is a safe and effective treatment for generalized dystonia in patients who remained impaired, despite optimal medical therapy.