成人型肌张力障碍的手术治疗(附16例分析)

目的探讨立体定向核团毁损手术及脑深部电刺激手术治疗成人型肌张力障碍的疗效。方法将16例成人型肌张力障碍病人按受累部位分成3组：A组：书写痉挛．12例；B组：节段性肌张力障碍，3例，A、B组采用立体定向核团毁损手术治疗；C组：痉挛性斜颈，1例，采用脑深部电刺激手术治疗，并根据颈部痉挛肌群肌电活动强度．进行脑深部电刺激参数的调整。随访1年．观察运动功能改善情况。结果A组均治愈；B组平均改善率59、7％；C组在肌电监测之前．临床改善率为23．1％．根据肌电活动强度调整双侧脑深部电极刺激参数后临床改善率为67．3％。结论立体定向丘脑腹外侧核毁损手术是治疗书写痉挛的有效手段。肌电活动强度是设定和调整脑深部电刺激参数的量化指标，可以缩短反复调整的时间．减少电池耗费。     

混合性脑瘫的外科治疗

目的探讨立体定向核团毁损加选择性脊神经后根切断术治疗混合性脑瘫的手术疗效。方法对6例混合性脑瘫病人行立体定向苍白球腹后内侧部（Gpi，3例）或丘脑底核（STN，3例）毁损，加选择性脊神经后根切断术（L2-S1 5例，C5～C8 1例），手术同期进行2例，间隔约2周后分期进行4例。随访24个月，观察手术肢体痉挛、肌张力障碍及运动功能改善情况。结果6例病人Ashworth痉挛评分及Fahn肌张力障碍评分降低，运动功能明显改善。结论应用立体定向核团毁损加选择性脊神经后根切断术治疗混合性脑瘫疗效可靠。     

MRI导向丘脑腹中间核立体定向毁损术治疗书写痉挛二例并文献复习

目的探讨立体定向手术治疗书写痉挛的方法及疗效。方法与结果采用MRI导向丘脑腹中间核立体定向毁损术治疗2例书写痉挛(震颤型)患者,例1术后书写时震颤即消失,写字较术前工整;例2术后右上肢震颤明显改善,可正常工作和生活。2例患者均未出现明显并发症,术后随访至今均未复发。结论书写痉挛的外科手术疗效明确,MRI导向丘脑腹中间核立体定向毁损术可以精确定位神经核团,有效避免术中并发症。文献复习两种外科手术方法(脑深部电刺激术和脑深部神经核团毁损术)的优劣和适应证,尚待进一步探讨。     

立体定向脑内核团毁损及深部电刺激术治疗帕金森病54例临床分析

目的探讨立体定向脑内核团毁损术及深部电刺激术（DBS）治疗帕金森病的疗效。方法对长期随访的45例接受立体定向核团毁损术和9例接受脑深部电刺激丘脑底核（STN）治疗的帕金森病患者进行疗效评估和临床分析。结果1例DBS病人术后无效,调整电极后效果满意,射频毁损术及DBS术后短期效果均满意,本组显效47例（87％）,有效7例（13％）,总有效率100％。日常生活能力（ADL）评分：“开”状态下提高38％,“关”状态下提高49％。统一帕金森病评分量表（UPDRS）评分：“开”状态下症状改善率52％,“关”状态下改善率72％。随访结果：射频毁损者复发率为17．8％,DBS者无复发。结论立体定向脑内核团毁损及深部电刺激术治疗帕金森病疗效满意,精确定位是手术成功的关键,微电极记录可提高手术的准确性。DBS具有非破坏性、可双侧同期手术、术后可调节等优点,但价格昂贵。     

继发性全身性肌张力障碍的手术治疗分析

目的探讨采用立体定向核团毁损和核团毁损加选择性脊神经后根切断术治疗兼有锥体系统损害的继发性全身性肌张力障碍的手术疗效。方法18例病人随机分成两组:a组12例,单纯采用立体定向核团毁损手术治疗;b组6例,采用核团毁损加选择性脊神经后根切断术。随访3个月,观察并对比术后运动功能改善情况。结果单纯立体定向核团毁损手术治疗效果不明显,核团毁损手术加选择性脊神经后根切断术能使病人运动功能得到明显提高。结论联合采用核团毁损手术与选择性脊神经后根切断术,对兼有锥体系统损害的继发性全身性肌张力障碍病人近期疗效较好。     

脑立体定向手术治疗Meige综合征二例报告并文献复习

目的 探讨立体定向深部脑刺激术( DBS)和脑内核团毁损术在Meige综合征(MS)中的临床应用.方法 2例患者术前均采用Burke - Fahn - Marsden肌张力障碍运动评分(BFMDRS),1例MS患者行双侧苍白球内侧核(GPi) DBS手术治疗,1例患者行单侧GPi射频毁损术治疗.结果 行双侧GPi - DBS患者术后随访1年改善达83.7％(BFMDRS评分由术前的22.5分减少为4分),随访2年改善率达69.1％左右(BFMDRS评分由22.5分减少为6分),疗效较为稳定.1例行立体定向右侧GPi毁损术,术后1周疗效明显,BFMDRS从28分减少到6分,改善达78.6％;术后3个月BFMDRS评分为15分,改善率为46.2％,术后6个月随访,所有症状恢复到术前水平.结论 双侧GPi - DBS对于传统治疗无效的Meige综合征患者是一种安全有效的治疗方法;对于不能接受DBS手术的患者,行苍白球或丘脑毁损手术也是值得推荐的一种方法.     

微电极引导立体定向核团毁损和脑深部电刺激治疗帕金森病

目的研究微电极引导立体定向颅内核团毁损和脑深部电刺激手术(deep brain stimulation,DBS)治疗帕金森病的临床疗效。方法分析我院116例应用微电极引导立体定向核团毁损术和85例应用脑深部电刺激术治疗的帕金森病患者的临床资料,获得术前、术后和DBS开启后6个月、1年、3年及5年的不同服药状态下帕金森病联合评分量表(UPDRS)的评分,比较手术前后UPDRS运动评分的差异。结果核团毁损术和DBS在术后6个月、1年和3年的随访中均能显著改善患者术前UPDRS运动评分,在第5年仅DBS组UPDRS运动评分较术前有改善,同时DBS组患者术后抗帕金森病药物用量较术前减少。结论核团毁损和脑深部电刺激手术均能显著改善帕金森病患者的UPDRS运动评分,DBS疗效更为长久。     

立体定向脑内核团毁损术治疗帕金森病(附52例报告)

目的探讨立体定向脑内核团毁损术治疗帕金森病的安全性及有效性。方法本组男29例,女23例;年龄41~77岁,病程3~15年,其中震颤型40例,肌强直型8例,混合型4例。在局麻下对这些患者行立体定向脑内核团射频毁损术,术后观察治疗的效果。结果本组44例行Vim核毁损术,8例行Gpi毁损术,手术有效率100%,术后出现毁损部位少量出血1例,出现术侧肢体偏瘫1例,经治疗1个月后症状均缓解。结论立体定向脑内核团毁损术治疗帕金森病疗效显著,且靶点选择、毁损范围及程度与手术疗效及并发症密切相关。     

微电极导向核团毁损术和脑深部电刺激术治疗帕金森病的疗效分析

目的 观察微电极导向核团毁损术和脑深部电刺激术（DBS）治疗帕金森病的临床疗效。方法 对380例接受微电极导向立体定向核团毁损术和25例脑深部电刺激丘脑底核（STN—DBS）治疗的帕金森病患者进行随访和神经功能评估，分别获得术前、术后和DBS开启后1周、6个月、2年及5年的不同服药状态下统一帕金森病量表（UPDRS）评分资料，采用威尔科克森检验（Wilcoxontest），比较不同术后时间点UPDRS运动评分与术前评分的差异。结果 核团毁损术和DBS在术后1周、6个月及2年随访中均能明显改善术前帕金森病患者的UPDRS运动评分，减轻左旋多巴诱发的运动波动及异动症。在5年随访时间点上仅DBS治疗组较术前比较仍显示差异性。而且DBS组患者术后左旋多巴服药的剂量较术前减少。核团毁损组总体并发症的发生率为5．8％，永久性并发症的发生率为1．2％。DBS组未发生严重并发症。结论 核团毁损术和DBS两者被证实是中晚期帕金森病安全、有效的治疗方法，能显著改善术前帕金森病患者的UPDRS运动评分，减轻左旋多巴诱发的运动波动及异动症。STN—DBS较毁损术更具有独特的可控性、安全性和长效性。     

立体定向下核团毁损术及脑深部电刺激术治疗难治性强迫症

目的探讨MRI定位下核团毁损术及脑深部电刺激术治疗难治性强迫症的疗效。方法应用MRI定位下射频热凝核团毁损术及脑深部电刺激术治疗难治性强迫症49例，其中30例行双侧内囊前肢毁损术，13例行双侧扣带回加双侧内囊前肢毁损术,6例行右侧伏隔核脑深部电刺激术加对侧内囊前肢毁损术。手术前后应用YALE-BROWN强迫症状量表、汉密尔顿抑郁量表、汉密尔顿焦虑量表对手术效果进行评价。结果术后6个月均获随访，其中临床痊愈28例，显著改善9例，轻度改善7例，无明显变化5例。22例术后早期出现一过性并发症，均在术后2周内恢复。术后焦虑，强迫，抑郁症状量表分数均明显下降（P〈0．01）。结论MRI定位下核团毁损术及脑深部电刺激术治疗难治性强迫症有显著疗效，并发症较轻，可以显著改善病人强迫、焦虑、抑郁症状。     

Stereotactic selective thalamotomy for the treatment of tremor type cerebral palsy in adolescence

6 cases with tremor-athetotic type cerebral palsy and 2 cases with moderate dystonia-tremor type cerebral palsy were treated by selective stereotactic thalamotomy. In the former group, postural-movement type tremor in the upper limb gradually progressed with age while athetosis remained unchanged. In the latter group, dystonia in the truncal muscles predominated over the irregular tremulous movement of the upper limbs. In all cases, the intelligence was almost normal. Stereotactic selective thalamotomy (Vim for tremor athetosis, VL-Vim for dystonia tremor) was performed under local anesthesia with the aid of radiological and neurophysiological control methods. The results of the operations were satisfactory in regard to the tremor relief and concomitant improvement of motor performances in most of the cases. Stereotactic treatment might be an effective way to make possible a one-step progress in these handicapped cases. The importance of postoperative physical therapy is also emphasized.     

Effects of deep brain stimulation in dyskinetic cerebral palsy: A meta‐analysis

Secondary dystonia encompasses a heterogeneous group with different etiologies. Cerebral palsy is the most common cause. Pharmacological treatment is often unsatisfactory. There are only limited data on the therapeutic outcomes of deep brain stimulation in dyskinetic cerebral palsy. The published literature regarding deep brain stimulation and secondary dystonia was reviewed in a meta‐analysis to reevaluate the effect on cerebral palsy. The Burke‐Fahn‐Marsden Dystonia Rating Scale movement score was chosen as the primary outcome measure. Outcome over time was evaluated and summarized by mixed‐model repeated‐measures analysis, paired Student t test, and Pearson's correlation coefficient. Twenty articles comprising 68 patients with cerebral palsy undergoing deep brain stimulation assessed by the Burke‐Fahn‐Marsden Dystonia Rating Scale were identified. Most articles were case reports reflecting great variability in the score and duration of follow‐up. The mean Burke‐Fahn‐Marsden Dystonia Rating Scale movement score was 64.94 ± 25.40 preoperatively and dropped to 50.5 ± 26.77 postoperatively, with a mean improvement of 23.6% (P < .001) at a median follow‐up of 12 months. The mean Burke‐Fahn‐Marsden Dystonia Rating Scale disability score was 18.54 ± 6.15 preoperatively and 16.83 ± 6.42 postoperatively, with a mean improvement of 9.2% (P < .001). There was a significant negative correlation between severity of dystonia and clinical outcome (P < .05). Deep brain stimulation can be an effective treatment option for dyskinetic cerebral palsy. In view of the heterogeneous data, a prospective study with a large cohort of patients in a standardized setting with a multidisciplinary approach would be helpful in further evaluating the role of deep brain stimulation in cerebral palsy. © 2013 Movement Disorder Society     

Surgery for Dystonia and Tremor

Surgical procedures for dystonia and tremor have evolved over the past few decades, and our understanding of risk, benefit, and predictive factors has increased substantially in that time. Deep brain stimulation (DBS) is the most utilized surgical treatment for dystonia and tremor, though lesioning remains an effective option in appropriate patients. Dystonic syndromes that have shown a substantial reduction in severity secondary to DBS are isolated dystonia, including generalized, cervical, and segmental, as well as acquired dystonia such as tardive dystonia. Essential tremor is quite amenable to DBS, though the response of other forms of postural and kinetic tremor is not nearly as robust or consistent based on available evidence. Regarding targeting, DBS lead placement in the globus pallidus internus has shown marked efficacy in dystonia reduction. The subthalamic nucleus is an emerging target, and increasing evidence suggests that this may be a viable target in dystonia as well. The ventralis intermedius nucleus of the thalamus is the preferred target for essential tremor, though targeting the subthalamic zone/caudal zona incerta has shown promise and may emerge as another option in essential tremor and possibly other tremor disorders. In the carefully selected patient, DBS and lesioning procedures are relatively safe and effective for the management of dystonia and tremor.     

Localization of Basal Ganglia and Thalamic Damage in Dyskinetic Cerebral Palsy

BackgroundDyskinetic cerebral palsy affects 15%-20% of patients with cerebral palsy. Basal ganglia injury is associated with dyskinetic cerebral palsy, but the patterns of injury within the basal ganglia predisposing to dyskinetic cerebral palsy are unknown, making treatment difficult. For example, deep brain stimulation of the globus pallidus interna improves dystonia in only 40% of patients with dyskinetic cerebral palsy. Basal ganglia injury heterogeneity may explain this variability.MethodsTo investigate this, we conducted a qualitative systematic review of basal ganglia and thalamic damage in dyskinetic cerebral palsy. Reviews and articles primarily addressing genetic or toxic causes of cerebral palsy were excluded yielding 22 studies (304 subjects).ResultsThirteen studies specified the involved basal ganglia nuclei (subthalamic nucleus, caudate, putamen, globus pallidus, or lentiform nuclei, comprised by the putamen and globus pallidus). Studies investigating the lentiform nuclei (without distinguishing between the putamen and globus pallidus) showed that all subjects (19 of 19) had lentiform nuclei damage. Studies simultaneously but independently investigating the putamen and globus pallidus also showed that all subjects (35 of 35) had lentiform nuclei damage (i.e., putamen or globus pallidus damage); this was followed in frequency by damage to the putamen alone (70 of 101, 69%), the subthalamic nucleus (17 of 25, 68%), the thalamus (88 of 142, 62%), the globus pallidus (7/35, 20%), and the caudate (6 of 47, 13%). Globus pallidus damage was almost always coincident with putaminal damage.ConclusionsNoting consistent involvement of the lentiform nuclei in dyskinetic cerebral palsy, these results could suggest two groups of patients with dyskinetic cerebral palsy: those with putamen-predominant damage and those with panlenticular damage involving both the putamen and the globus pallidus. Differentiating between these groups could help predict response to therapies such as deep brain stimulation.     

Surgical treatments for tremors

Stereotactic surgery is an increasingly popular option for disabling tremors whenever it is insufficiently improved by drug treatment. Surgical approaches are expanding. Thalamic deep brain stimulation is one of the most efficacious treatments. Its recent technological advances with adaptive stimulation and new electrodes configuration will allow a more physiological stimulation. However, a reappraisal of less invasive, new lesioning procedures is underway. Gamma Knife thalamotomy and magnetic resonance-guided focused ultrasounds encounter very few contraindications. Recent studies reported their efficacy on tremor control and safety profile. Besides the ventralis intermedius nucleus of the thalamus, alternative targets are also emerging. The effectiveness of surgical therapies on essential tremor and Parkinson's disease tremor is well established. For more uncommon tremors, preliminary studies are encouraging. All these surgical therapies can be proposed as treatment option for medically refractory tremors.     

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.