肌电引导局部注射A型肉毒素治疗颈部肌张力障碍交叉对照研究

目的探讨肌电引导对提高Ａ型肉毒素局部注射治疗颈部肌张力障碍的有效性和安全性。方法对３２例颈部肌张力障碍患者采用交叉对照设计,分成肌电引导和非肌电引导注射组。注射Ａ型肉毒素的部位和剂量,根据受累肌肉、大小及痉挛、肥厚程度。按Ｔｓｕｉ量表评价疗效。结果肌电引导组和非肌电引导组比较,治疗前后评分减少分别为１１．５３±３．４１和１０．８４±３．２５,ｔ＝２．６４,Ｐ＜０．０５;明显和基本缓解率分别为８４％和７５％;颈肌无力和吞咽困难副作用分别为３例（９．３６％）和９例（２８．１３％）,χ２＝４．１７,Ｐ＜０．０５;２～７周完全恢复。结论该方法可明显提高颈部肌张力障碍的疗效及减少副作用。     

丘脑底核电刺激治疗继发性肌张力障碍

目的 探讨丘脑底核（STN）的脑深部电剌激（DBS）治疗继发性肌张力障碍的可行性、适应证和并发症。方法 5例行双侧STN—DBS，1例行单侧STN—DBS。结果 术中利用微电极记录的电信号获得STN的准确靶点定位，电刺激后患者肌张力有不同程度下降，但扭转改善不明显。随访半年至3年，6例患者中，药物引起的迟发性肌张力障碍及外伤性肌张力障碍的患者疗效理想，BFMDRS评分改善均在90％以上，且随着随访时间的延长，效果持续不断改善；其余4例患者疗效不佳，4例均肌张力略有改善，其中1例扭转略改善，1例语言及步态略有改善。手术后患者均无明显合并症，但1例术后16个月发现左侧电极折断，后取出。结论 DBS治疗迟发性和外伤性继发性肌张力障碍效果理想，而对于缺氧或脑基底节区弥漫性损害的继发性肌张力障碍效果不佳；STN可以成为治疗本病的理想靶点；术中应根据电生理记录结果和肌张力的轻度改善作为靶点定位的指标；手术无明显合并症。     

肉毒杆菌毒素A对痉挛性肌张力障碍的治疗进展

应用肉毒杆菌毒素A 治疗痉挛性肌张力障碍是神经科治疗学方面的一个进展,已被临床证明是一种安全有效的药物。本文就其生物特性、药理作用、临床应用及副作用等方面进行综述。     

丘脑底核电刺激术治疗肌张力障碍3例并文献复习

目的 总结丘脑底核（STN）脑深部电刺激术（DBS）治疗肌张力障碍的经验。方法 2012年6月至2014年4月收治3例肌张力障碍患者,其中2例行单侧STN-DBS,1例行双侧STN-DBS。3例患者术后随访3~22个月。采用BFM肌张力障碍评分量表（BFMDBS）进行评分,症状改善率=（术前BFMDBS评分-术后BFMDBS评分）/术前BFMDBS评分×100%。结果 3例患者开机后症状都有不同程度的改善,1例随访22个月,症状改善率为97%;1例随访3个月,症状改善率为63%;1例随访3个月,症状改善率为37%。3例患者均未出现手术相关并发症。结论 STN是治疗肌张力障碍有效的靶点,STN-DBS可明显、长期改善肌张力障碍症状。     

肌张力障碍的功能外科治疗

肌张力障碍(dystonia),是一组以持续肌肉收缩．频繁扭转和重复动作的异常姿势为特征的症候群。肌张力障碍早期采用射频毁损术治疗．包括丘脑腹外侧核毁损术和苍白球腹后部毁损术。单侧毁损术对于肌张力障碍的治疗有一定的疗效。因为并发构音障碍和认知功能障碍的概率很高,所以双侧毁损术现在已经很少应用于临床。随着脑深部电刺激术(deep brain stimulation,DBS)治疗帕金森病取得满意疗效DBS成为治疗肌张力障碍的首选方法。因为对于中线的症状的改善有限,丘脑DBS手术现已经很少用于治疗肌张力障碍。苍白球DBS治疗肌张力障碍国内外有大量的文献报道,结果均显示苍白球DBS对于缓解肌张力障碍症状、提高肌张力障碍病人生活质量有明显疗效。但是,对于丘脑底核DBS治疗肌张力障碍的报道还很少见,需要进一步探索。     

脑深部电刺激治疗肌张力障碍研究进展

肌张力障碍是由于多种原因造成的脑内一些神经递质的代谢及传导出现异常，从而直接或间接增加了运动神经突触间的兴奋性，削弱了其抑制性，最终产生的以全身或局部的异常动作和（或）姿势为主要特征的综合征。其病因及发病机理尚不明确，故临床对其处理的重点主要为对症治疗。随着立体定向功能神经外科的深入发展，脑深部电刺激治疗肌张力障碍已显示出良好的应用前景。本文综述肌张力障碍的发病机制、治疗方法、疗效及相关并发症。     

脑深部电刺激治疗迟发性肌张力障碍一例报道并文献复习

目的观察脑深部电刺激治疗1例迟发性肌张力障碍的手术效果。方法在微电极的引导下,将刺激电极植入双侧丘脑底核,分别于术前、术后1个月、术后3个月为患者进行UDRS和BFMS评分,评价治疗效果。结果术前、术后1个月、术后3个月的UDRS评分分别为94、38和7.5,BFMS评分分别为98.5、42.5和8,随访3个月的症状缓解率在90%以上,无手术并发症。结论双侧电刺激丘脑底核治疗迟发性肌张力障碍初步显示出良好的效果。     

原发性肌张力障碍和继发性肌张力障碍苍白球内侧部局部场电位信号特点比较

目的 原发性和继发性肌张力障碍均可以使用苍白球内侧部脑深部电刺激治疗,两种病因导致的疾病可能存在相同的病理基础.运动障碍疾病发生和脑深部电刺激治疗均与局部场电位有关.本文拟探索原发性肌张力障碍和继发性肌张力障碍患者的苍白球内侧部局部场电位特点是否存在差异.方法 本文纳入3例继发性肌张力障碍和4例原发性肌张力障碍患者,均...     

丘脑底核电刺激术治疗原发性肌张力障碍

目的观察双侧丘脑底核(STN)脑深部电刺激(DBS)治疗原发性肌张力障碍的疗效。方法回顾性分析8例接受双侧STN-DBS的原发性肌张力障碍病人的临床资料。分别在术前和术后1、3、6个月进行Burke-Fahn-Marsden肌张力障碍评分(BFMDRS)和帕金森病综合评分量表(UPDRS),计算两项评分改善率,评价治疗效果。结果 BFMDRS和UPDRS评分的改善率:术后1个月其均值分别为47%和48%,术后3个月为69%和73%,术后6个月为75%和79%。结论 STN-DBS可有效改善原发性肌张力障碍病人的症状,术后疗效稳定。STN是DBS治疗原发性肌张力障碍的理想靶点。     

Dystonia

Dystonia, a hyperkinetic movement disorder, is characterized by involuntary muscle spasms leading to abnormal postures. Dystonic syndromes are classified by etiology (primary vs. secondary), age of onset (early vs. late onset) or spread of symptoms (focal, segmental, generalized). Clinically, young-onset dystonia is rare, often inherited and tends to spread to become generalized. In contrast, adult-onset dystonia is frequent, typically sporadic and remains focal. In recent years, 15 genes associated with dystonia have been identified and classified as DYT loci. Of these, DYT1 is the most frequent, causing early-onset generalized dystonia. Pathophysiology remains ill understood but basal ganglia dysfunction is thought to play an important role. Treatment remains symptom-oriented. A trial of levodopa is recommended in young-onset cases. In focal forms, botulinum toxin injections are helpful. Anticholinergics may be beneficial. In severe cases deep brain stimulation may be considered.     

Dystonia update

PURPOSE OF REVIEW: Dystonia is a movement disorder with a complex and not fully understood pathophysiology. Its better understanding would enable more focused treatment for the disorder. In this review, we provide an overview of recent studies of the pathophysiology of primary and secondary dystonia, with an emphasis on functional brain imaging. Potential mechanisms underlying the beneficial effects of deep brain stimulation for dystonia are also summarized. RECENT FINDINGS: The recognition of dysfunction at different levels of the nervous system has extended the classical notions of localized striatal abnormalities in primary dystonia. Recent biochemical studies have revealed evidence of abnormal torsion activity in DYT1 dystonia. Abnormal patterns of brain metabolism have also been identified using functional brain imaging in different dystonia genotypes. These findings, in conjunction with new electrophysiological techniques, can be utilized to help define a common mechanism for the neural dysfunction in dystonia. SUMMARY: New insights into the pathophysiology of dystonia have been provided by recent studies using electrophysiology, biochemistry and human genetics, as well as functional brain imaging studies. These advances together may create the basis for new therapies for this disorder.     

Tardive Dystonia

In a population of 200 consecutive inpatients with a history of at least 3 months' total cumulative neuroleptic exposure, the prevalence of tardive dystonia (TDt) was 4%, higher than previously reported. The prevalence of tardive dyskinesia (TDk) was 22%. Patients with TDt did not differ in demographic or clinical variables from nondyskinetic patients. In comparison with patients with TDk, patients with TDt were significantly younger, had a more severe movement disorder, and had received neuroleptics for the first time fewer years before. Patients with TDk were significantly older than patients without tardive disorders, both when they were examined and when they had started their first neuroleptic treatment. Furthermore, they had started their first neuroleptic treatment more years before. These results support the distinction between TDt and TDk, and suggest that the previously reported prevalence of TDt might have been underestimated.     

特殊工作性肌张力障碍

特殊工作性肌张力障碍是一种特殊形式的局灶性肌张力障碍。常见症状有书写痉挛和音乐家手部痉挛等，症状的出现多与职业行为相关。病因尚不明，目前的研究认为过度的活动、基底节功能障碍、大脑皮质运动和感觉功能异常及一定的遗传背景可能与之有关。在治疗方面，除了常规的口服药物治疗外，肉毒毒素注射、支具固定制动和行为治疗等特殊方法有较好的疗效。     

Dystonia update

Dystonia is a syndrome of sustained muscle spasms of presumed central nervous system origin. Recent advances in molecular biology have permitted clearer understanding of the genetics of various forms of dystonia and suggest pathophysiological deficits at the origin of the clinical signs. Treatment has involved centrally-acting drugs, specifically the anticholinergic medications, as well as peripherally acting agents that block neuromuscular transmission (botulinum toxin). Some forms of dystonia are particularly responsive to levodopa. A systematic approach to the diagnostic and treatment evaluation of dystonic patients permits optimal care for long-term management.     

Dystonia: phenomenology

In 1984, dystonia was defined by an ad hoc committee of the Dystonia Medical Research Foundation as a syndrome of involuntary, sustained muscle contractions affecting one or more sites of the body, frequently causing twisting and repetitive movements, or abnormal postures. In 2011, dystonia remains a purely clinical diagnosis. Primary dystonia includes syndromes in which dystonia is the sole phenotypic manifestation with the exception that tremor can be present as well. Primary dystonias are typically mobile and may show task specificity. Fixed dystonias are often psychogenic or associated with complex regional pain syndrome. Fixed dystonia may also be the terminal consequence of long-standing, inadequately-treated, severe appendicular or cervical dystonia. The vast majority of primary dystonias have their onset in adults. Late-onset, primary, focal dystonia, particularly blepharospasm, may spread to affect other anatomical segments. Patients with focal dystonia may also exhibit spontaneous remissions that last for years. Although sensory tricks are commonly reported by patients with primary dystonia, they have also been described in subjects with secondary dystonia. Another important sensory aspect of dystonia is pain which is relatively common in cervical dystonia but also reported by many patients with masticatory dystonia, hand-forearm dystonia and blepharospasm. In conclusion, "dystonia" can be used to delimit a clinical sign or loosely define a neuropsychiatric sensorimotor syndrome.