Dyskinesias

运动障碍
  • 文章类型: Journal Article
    背景:运动障碍是中风最常见的并发症之一。针刺疗法(AT)和镜像疗法(MT)是治疗中风后运动障碍的有希望的康复措施。尽管一些研究表明AT和MT对运动障碍是有效和安全的,的影响,由于缺乏强有力的证据,安全性仍然不确定。目的探讨AT联合MT治疗脑卒中后运动障碍的疗效和安全性。
    方法:我们搜索了以下数据库:PubMed,WebofScience,科克伦图书馆,EMBASE,Medline,中国知网,万方,和中国生物医学文献数据库,从开始到2023年1月1日,以确定符合条件的研究。总有效率,Fugl-Meyer评估量表(FMA)上肢和下肢评分,修改后的Barthel指数得分,伯格平衡量表,改良的Ashworth秤,并以不良反应作为结局指标。2名独立评审员使用建议评估开发和评估系统来评估研究中包含的结果指标的证据质量。采用RevManV.5.4软件进行统计分析。
    结果:共纳入24项随机对照研究,包括2133例脑卒中后运动障碍患者。AT联合MT治疗脑卒中后运动障碍的总有效率更有优势(相对危险度=1.31,95%置信区间[CI][1.22-1.42],Z=6.96,P<.0001)。AT联合MT对FMA上肢评分更有利(平均差[MD]=6.67,95%CI[5.21-8.13],Z=8.97,P<.00001)和FMA下肢评分(MD=3.72,95%CI[2.81-4.63],Z=7.98,P<.00001)。Meta分析显示,AT联合MT治疗脑卒中后运动障碍的改良Barthel指数评分更有优势(MD=9.51,95%CI[7.44-11.58],Z=9.01,P<.00001)。
    结论:AT联合MT可有效改善患者的运动功能和日常生活能力。尤其是改善肌肉痉挛.然而,鉴于评估结果的证据质量较低,因此应谨慎对待这些结果。
    BACKGROUND: Dyskinesia is one of the most common complications of stroke. Acupuncture therapy (AT) and mirror therapy (MT) are promising rehabilitation measures for the treatment of post-stroke dyskinesia. Although some studies suggested that AT and MT are effective and safe for dyskinesia, the effects, and safety remain uncertain due to lacking strong evidence. The purpose of this study is to investigate the efficacy and safety of AT combined with MT in the treatment of post-stroke dyskinesia.
    METHODS: We searched the following databases: PubMed, Web of Science, Cochrane Library, EMBASE, Medline, China Knowledge Network, WANFANG, and China Biomedical Literature Database, from inception to 1 January 2023 to identify eligible studies. Total effective rate, the Fugl-Meyer assessment scale (FMA) upper and lower limb scores, modified Barthel index scores, Berg balance scale, modified Ashworth scale, and adverse reactions were adopted as outcome indicators. The Grading of Recommendations Assessment Development and Evaluation system was used by 2 independent reviewers to assess the quality of evidence for the outcome indicators included in the study. The statistical analysis was conducted by RevMan V.5.4 software.
    RESULTS: A total of 24 randomized controlled studies included 2133 patients with post-stroke dyskinesia were included. The total effective rate of AT combined with MT was more advantageous in the treatment of post-stroke dyskinesia (relative risk = 1.31, 95% confidence interval [CI] [1.22-1.42], Z = 6.96, P < .0001). AT combined with MT was more advantageous for FMA upper limb score (mean difference [MD] = 6.67, 95% CI [5.21-8.13], Z = 8.97, P < .00001) and FMA lower limb score (MD = 3.72, 95% CI [2.81-4.63], Z = 7.98, P < .00001). Meta-analysis showed that AT combined with MT for post-stroke dyskinesia had a more advantageous modified Barthel index score (MD = 9.51, 95% CI [7.44-11.58], Z = 9.01, P < .00001).
    CONCLUSIONS: AT combined with MT is effective in improving motor function and daily living ability of patients, especially in improving muscle spasms. However, these results should be regarded with caution given the low quality of evidence for the evaluation results.
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  • 文章类型: Systematic Review
    目的:了解肩胛骨肌肉在肩胛骨发育不良(SD)的头顶运动员中的主要表现。
    方法:系统评价。
    方法:在Pubmed(MedLine)中进行电子搜索,Embase,CINAHL,和SPORTDiscus数据库。
    方法:具有SD的高架运动员。
    方法:上部(UT)的肌电图活动,中间(MT),和下(LT)斜方肌,和前锯肌(SA)。
    结果:本综述纳入了8项研究。与45°相比,UT活性显示出主要在90°以上的任务期间倾向于增加其活性。SA活性也有类似的行为,主要是在等距任务期间。与较低的角度相比,MT还主要在具有高架角度的任务中增加了其活动。在患有SD的高架运动员中,LT激活倾向于在60°以下的角度降低其EMG活性。
    结论:非运动员的UT和SA的EMG行为似乎与文献中已经描述的不同。在患有SD的头顶运动员中,MT似乎是肩胛骨稳定最被忽视的肌肉。LT活动的减少表明这可能对这些运动员的表现产生影响。
    OBJECTIVE: Understanding how the main scapular muscles behave in overhead athletes with scapular dyskinesis (SD).
    METHODS: Systematic Review.
    METHODS: Electronic searches were performed in Pubmed (MedLine), Embase, CINAHL, and SPORTDiscus databases.
    METHODS: Overhead athletes with SD.
    METHODS: Electromyographic activity of the upper (UT), middle (MT), and lower (LT) trapezius, and serratus anterior (SA).
    RESULTS: Eight studies were included in this review. The UT activity showed a tended to increase its activity mainly during tasks over 90° compared to 45°. SA activity had similar behavior, mainly during isometric tasks. The MT also increased its activity mainly in tasks with overhead angulations when compared to lower angulations. The LT activation tended to decrease its EMG activity at angulations below 60° in overhead athletes with SD.
    CONCLUSIONS: The EMG behaviour of UT and SA for non-athletes appears to differ from what has already been described in the literature. The MT seems to be the most neglected muscle for scapular stabilization in overhead athletes with SD. The decrease in LT activity suggests that this may have implications for the performance of these athletes.
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  • 文章类型: Journal Article
    Asterixis是一种阴性肌阵挛症的亚型,其特征是短暂的,由于肌肉收缩的不自主停顿,持续姿势的心律失常失误。我们进行了叙述性审查,以描述关于命名法的进一步星号,历史方面,病因学,病理生理学,分类,诊断,和治疗。在文献和以前的文章中,Asterixis已被经典地用作负肌阵挛症的同义词。然而,区分星号和其他亚型的负型肌阵挛症是很重要的,例如,癫痫阴性肌阵鸣,因为管理可以改变。Asterixis不是特定于任何病理生理过程,但更常见于肝性脑病,肾功能衰竭和呼吸衰竭,脑血管疾病,以及可能导致高氨血症的药物,如丙戊酸,卡马西平,还有苯妥英.Asterixis通常无症状,患者不会自发报告。这突出了在脑病患者的身体检查中积极寻找这种体征的重要性,因为它可能表明潜在的毒性或代谢原因。Asterixis通常在治疗潜在原因后是可逆的。
    Asterixis is a subtype of negative myoclonus characterized by brief, arrhythmic lapses of sustained posture due to involuntary pauses in muscle contraction. We performed a narrative review to characterize further asterixis regarding nomenclature, historical aspects, etiology, pathophysiology, classification, diagnosis, and treatment. Asterixis has been classically used as a synonym for negative myoclonus across the literature and in previous articles. However, it is important to distinguish asterixis from other subtypes of negative myoclonus, for example, epileptic negative myoclonus, because management could change. Asterixis is not specific to any pathophysiological process, but it is more commonly reported in hepatic encephalopathy, renal and respiratory failure, cerebrovascular diseases, as well as associated with drugs that could potentially lead to hyperammonemia, such as valproic acid, carbamazepine, and phenytoin. Asterixis is usually asymptomatic and not spontaneously reported by patients. This highlights the importance of actively searching for this sign in the physical exam of encephalopathic patients because it could indicate an underlying toxic or metabolic cause. Asterixis is usually reversible upon treatment of the underlying cause.
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  • 文章类型: Systematic Review
    在慢性失代偿性糖尿病患者中,非酮症性高血糖可能是舞蹈症的原因。因为它很少见,因此研究不足,诊断和治疗可能会延迟。因此,我们的目标是总结临床和放射学特征,以及进行的治疗,从以前报告的病例中提取,以促进临床实践中的适当管理。我们搜索了MEDLINE/PubMed,EMBASE,科克伦,CINAHL,WebofScience,Scopus,和LILACS数据库,用于2021年4月23日之前发表的研究。我们纳入了病例报告和成人(年龄≥18岁)的病例系列,这些病例系列描述了高血糖性舞蹈症,并测量了糖化血红蛋白(HbA1c)和头颅磁共振成像(MRI)。如果参与者是孕妇,研究被排除在外,年龄<18岁,并且没有关于舞蹈症和/或体格检查的描述。我们发现121项符合纳入标准的研究,共214例。大多数纳入的研究发表在亚洲(67.3%)。大多数患者是年龄>65岁(67.3%)的女性(65.3%)。几乎所有患者在到达急诊科时都患有失代偿性糖尿病(97.2%)。最常见的MRI发现是基底神经节异常(89.2%)。单独使用胰岛素和与其他药物组合治疗之间的患者恢复没有差异。虽然罕见,高血糖性舞蹈病是这种综合征的可逆原因;因此,在这些病例中,应始终考虑高血糖.
    Nonketotic hyperglycemia may occur as a cause of chorea in patients with chronic decompensated diabetes. Because it is rare and consequently poorly studied, diagnosis and treatment can be delayed. Therefore, our objective was to summarize clinical and radiological features, as well as treatments performed, from previously reported cases to facilitate adequate management in clinical practice. We searched MEDLINE/PubMed, EMBASE, Cochrane, CINAHL, Web of Science, Scopus, and LILACS databases for studies published before April 23, 2021. We included case reports and case series of adults (aged ≥ 18 years) that described hyperglycemic chorea with measurement ofglycated hemoglobin (HbA1c) and cranial magnetic resonance imaging (MRI). Studies were excluded if participants were pregnant women, aged < 18 years, and had no description of chorea and/or physical examination. We found 121 studies that met the inclusion criteria, for a total of 214 cases. The majority of the included studies were published in Asia (67.3%). Most patients were women(65.3%) aged > 65 years (67.3%). Almost all patients had decompensated diabetes upon arrival at the emergency department (97.2%). The most common MRI finding was abnormalities of the basal ganglia (89.2%). There was no difference in patient recovery between treatment with insulin alone and in combination with other medications. Although rare, hyperglycemic chorea is a reversible cause of this syndrome; therefore, hyperglycemia should always be considered in these cases.
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  • 文章类型: Review
    内分泌功能障碍可显著影响神经系统。各种非特异性神经症状可以表现出来,包括头痛,肌肉无力,非自愿运动,和意识受损。由于它们缺乏特异性,神经系统症状的内分泌异常可能无法被识别,做出诊断可能具有挑战性.临床医生在诊断神经系统症状时不要忽视内分泌紊乱,这一点至关重要。作为及时和适当的治疗,比如激素替代疗法,可以改善患者的一般状况和神经症状。
    Endocrine dysfunction can considerably impact the nervous system. Various nonspecific neurological symptoms can manifest, including headache, muscle weakness, involuntary movements, and impaired consciousness. Because of their lack of specificity, endocrine abnormalities underlying neurological symptoms may not be recognized and making the diagnosis may be challenging. It is essential that clinicians not overlook endocrine disorders when diagnosing neurological symptoms, as prompt and appropriate treatment, such as hormone replacement therapy, can improve a patient\'s general condition and neurological symptoms.
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  • 文章类型: Review
    目的:参加本次CME活动后,精神科医生应该能够更好地:•分类和描述不同类型的异常非自主运动(AIM)。•确定AIM的评估工具和治疗方案。
    异常不自主运动(AIM)包括一组不同的运动障碍,其特征是不受控制和意外运动(例如,震颤,Tics,肌张力障碍)。AIM可以发生在生命的任何阶段,并对临床医生构成重大挑战。由于涉及复杂的神经生物学机制,很难确定其根本原因。因此,使用经过充分验证的测量量表量化AIM的严重程度和进展至关重要,如异常非自愿运动量表(AIMS)。通过采用可靠的评估方法,临床医生可以客观地评估AIM的运动表现,并随着时间的推移进行跟踪。AIM的治疗根据其性质和病因而变化。虽然AIM经常对治疗有反应,严重的副作用会破坏治疗效果。在这篇以临床为重点的叙述性综述中,我们对不同类型的AIM进行分类,并讨论它们的神经生物学方面。Further,我们强调使用经过充分验证的测量量表进行准确评估的重要性,并讨论了针对特定AIM表现的可用治疗模式.此外,我们涵盖了全面护理的必要性,以解决AIM的多面性,考虑到他们的身体表现和心理,社会,和患者的功能收费。通过采用多学科方法,健康护理专业人员可以提供以患者为中心的护理,以促进整体福祉并提高患者应对AIM的生活。定期随访评估对于监测治疗反应是必要的,需要时调整药物,并为受AIM影响的个人提供持续支持。
    OBJECTIVE: After participating in this CME activity, the psychiatrist should be better able to:• Categorize and describe different types of abnormal involuntary movements (AIMs).• Identify assessment tools and treatment options for AIMs.
    UNASSIGNED: Abnormal involuntary movements (AIMs) comprise a diverse group of movement disorders characterized by uncontrolled and unintended movements (e.g., tremors, tics, dystonia). AIMs can occur at any stage of life and pose significant challenges for clinicians. It is difficult to determine their underlying causes due to the complex neurobiological mechanisms involved. Therefore, it is crucial to quantify the severity and progression of AIMs using well-validated measurement scales, such as the Abnormal Involuntary Movement Scale (AIMS). By employing reliable assessment approaches, clinicians can objectively evaluate the motoric manifestations of AIMs and track them over time. Treatment of AIMs varies depending on their nature and etiology. While AIMs often respond to treatment, serious side effects can undermine treatment efficacy. In this clinically focused narrative review, we categorize different types of AIMs and discuss their neurobiological aspects. Further, we emphasize the importance of using well-validated measurement scales for accurate assessment and discuss available treatment modalities that target the specific AIMs manifestations. Additionally, we cover the need for comprehensive care to address the multifaceted nature of AIMs, accounting for their physical manifestations as well as their psychological, social, and functional toll on patients. By embracing a multidisciplinary approach, health care professionals can provide patient-centered care that promotes overall well-being and enhances the lives of patients coping with AIMs. Regular follow-up assessments are necessary to monitor treatment response, adjust medications when needed, and provide ongoing support for individuals affected by AIMs.
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  • 文章类型: Meta-Analysis
    目的:该网络荟萃分析评估了疗效,耐受性,和第二代抗精神病药(SGA)对帕金森病精神病(PDP)的可接受性。
    方法:我们搜索了PubMed,Embase,科克伦图书馆,和ClinicalTrials.gov进行随机对照试验,调查截至2023年10月26日的PDPSGA。
    结果:我们纳入了16项研究氯氮平,Melperone,奥氮平,匹马色林,喹硫平,ulotaront,和安慰剂。在SGA和安慰剂之间的比较中,研究结果是:i)标准化的均值差异,95%置信区间(SMD,95CIs),对于精神病症状减少,氯氮平的排名第一(-1.31,-1.73至-0.89),匹马色林的第二级,喹硫平显著劣等(SMD=0.47,0.02至0.92);ii)平均差(MD,95CIs)用于异常运动,根据统一帕金森病评定量表-第三部分评估,表明氯氮平的运动副作用最小(-0.92,-2.75至0.91);iii)风险比(RRs,95%CIs)的不良反应脱落率最低的是melperone(1.02,0.20至5.24);iv)的RRs(95%CIs)的全因脱落率最低的是氯氮平(0.73,0.42至1.25)。
    结论:对于PDP患者,氯氮平可以大大减少精神病症状,运动异常极小,可接受性高,总体耐受性适中。匹马色林,不是喹硫平,可能是另一种选择。
    This network meta-analysis assessed the efficacy, tolerability, and acceptability of second-generation antipsychotics (SGAs) for Parkinson\'s disease psychosis (PDP).
    We searched PubMed, Embase, Cochrane Library, and ClinicalTrials.gov for randomized controlled trials investigating SGAs for PDP up to October 26, 2023.
    We included 16 trials (N = 1252) investigating clozapine, melperone, olanzapine, pimavanserin, quetiapine, ulotaront, and placebo. In comparisons between SGAs and placebo, the findings were: i) Standardized mean differences, 95% confidence intervals (SMDs, 95%CIs), for psychotic-symptom reduction revealed the first rank of clozapine (-1.31, -1.73 to -0.89), the second rank of pimavanserin, with significant inferiority of quetiapine (SMD = 0.47, 0.02 to 0.92); ii) Mean differences (MDs, 95%CIs) for abnormal movement, as assessed by the Unified Parkinson\'s Disease Rating Scale - Part III, indicated that clozapine had the least motor side effects (-0.92, -2.75 to 0.91); iii) Risk ratios (RRs, 95% CIs) for adverse-effect dropout rates were lowest for melperone (1.02, 0.20 to 5.24); and iv) RRs (95% CIs) for all-cause dropout rates were lowest for clozapine (0.73, 0.42 to 1.25).
    For patients with PDP, clozapine may substantially reduce psychotic symptoms with minimal abnormal movement, high acceptability, and moderate overall tolerability. Pimavanserin, not quetiapine, could be an alternative.
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  • 文章类型: Systematic Review
    背景:脑瘫(CP)是一种临床诊断,长期以来被归类为获得性疾病,但是越来越多的遗传病正在被发现。这篇综述旨在确定与遗传CP相关的临床特征,以帮助临床医生选择进行基因检测的候选人。
    方法:系统搜索PubMed数据库以鉴定与CP相关的基因。将伴随这些遗传形式的CP的临床特征与已发表的大型CP群体的数据进行比较,从而鉴定出遗传CP的潜在指标。
    结果:在检索到的1930篇文章中,134人包括在内。在这些中,55个CP基因(在两个或多个案例中描述,n=272)和79个候选基因(仅在一个案例中描述)被报道。最常见的CP相关基因是PLP1(21例),ARG1(17例),CTNNB1(13例)。运动障碍和没有痉挛被认为是遗传CP的强潜在指标。智力残疾的存在,没有早产,没有单侧分布的症状被归类为中度遗传指标。
    结论:CP的遗传原因越来越多。与遗传CP相关的临床特征可以帮助临床医生关于哪个患有CP的个体提供遗传检测。确定的潜在遗传指标需要在大型CP队列中进行验证,但可以为遗传CP的诊断算法提供第一步。
    BACKGROUND: Cerebral palsy (CP) is a clinical diagnosis and was long categorized as an acquired disorder, but more and more genetic etiologies are being identified. This review aims to identify the clinical characteristics that are associated with genetic CP to aid clinicians in selecting candidates for genetic testing.
    METHODS: The PubMed database was systematically searched to identify genes associated with CP. The clinical characteristics accompanying these genetic forms of CP were compared with published data of large CP populations resulting in the identification of potential indicators of genetic CP.
    RESULTS: Of 1930 articles retrieved, 134 were included. In these, 55 CP genes (described in two or more cases, n = 272) and 79 candidate genes (described in only one case) were reported. The most frequently CP-associated genes were PLP1 (21 cases), ARG1 (17 cases), and CTNNB1 (13 cases). Dyskinesia and the absence of spasticity were identified as strong potential indicators of genetic CP. Presence of intellectual disability, no preterm birth, and no unilateral distribution of symptoms were classified as moderate genetic indicators.
    CONCLUSIONS: Genetic causes of CP are increasingly identified. The clinical characteristics associated with genetic CP can aid clinicians regarding to which individual with CP to offer genetic testing. The identified potential genetic indicators need to be validated in large CP cohorts but can provide the first step toward a diagnostic algorithm for genetic CP.
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  • 文章类型: Systematic Review
    背景:MRI引导的聚焦超声(FUS)是一种无切口的热消融程序,可用于治疗药物难治性运动障碍,随着越来越多的潜在解剖靶点和临床应用。从本文的出版开始,美国食品和药物管理局(FDA)批准的FUS用于运动障碍的唯一用途是原发性震颤(ET)和震颤性帕金森病(PD)的丘脑切开术,和针对PD的其他主要症状的苍白球切开术。我们对所有非FDA批准的运动障碍FUS适应症进行了最新的审查,除了最充分描述的ET和PD适应症。我们的目标是总结FUS在这种情况下的安全性和有效性,并为FUS治疗运动障碍的未来方向提供路线图。
    方法:对FUS用于非FDA批准的运动障碍进行了最新的综述。包括所有运动障碍,不包括FDA批准的ET和PD用途。
    结果:共纳入了172例患者的25项研究。在震颤加肌张力障碍综合征患者中(n=6),丘脑腹侧中间核(VIM)-FUS减少50%的震颤,2/6患者的肌张力障碍无改善,肌张力障碍恶化。腹侧-口肌复合体(VO)-FUS对局灶性手部肌张力障碍(n=6)改善了50%,对音乐家肌张力障碍(n=6)恢复了100%的音乐表现。在多发性硬化症(MS)和震颤(n=3)的患者中,在2例颅骨密度比良好的患者中观察到震颤改善;VIM-FUS后未发现MS疾病改变.在患有震颤和共济失调综合征的患者中(n=3),没有发现VIM-FUS后共济失调恶化;所有患者都有临床上显著的震颤改善。丘脑底核(STN)-FUS治疗PD(n=49)可改善PD运动症状约50%,与肌张力障碍和轻度运动障碍可能的不良反应。ET的小脑丘脑(CTT-FUS)(n=42)改善了55-90%的震颤,步态功能障碍是一种罕见的持续不良反应。用于PD(n=50)的手掌丘脑(PTT-FUS)可改善运动症状约50%,轻度言语功能障碍作为可能的不良反应。
    结论:VIM-FUS对异质性震颤病因安全有效,VO-FUS对孤立的节段性肌张力障碍最有效。STN-FUS可有效减轻PD症状;术后肌张力障碍和轻度服药运动障碍需要医疗管理。使用CTT-FUS进行ET和PTT-FUS进行PD的基于Tractography的靶向显示了有希望的早期结果。需要进行长期随访的大型前瞻性试验来评估FUS的安全性和有效性。
    BACKGROUND: MRI-guided focused ultrasound (FUS) is an incisionless thermo-ablative procedure that may be used to treat medication-refractory movement disorders, with a growing number of potential anatomic targets and clinical applications. As of this article\'s publication, the only US Food and Drug Administration (FDA)-approved uses of FUS for movement disorders are thalamotomy for essential tremor (ET) and tremor-dominant Parkinson\'s Disease (PD), and pallidotomy for other cardinal symptoms of PD. We present a state-of-the-art review on all non-FDA approved indications of FUS for movement disorders, beyond the most well-described indications of ET and PD. Our objective was to summarize the safety and efficacy of FUS in this setting and provide a roadmap for future directions of FUS for movement disorders.
    METHODS: A state-of-the-art review was conducted on use of FUS for non-FDA approved movement disorders. All movement disorders excluding FDA-approved uses for ET and PD were included.
    RESULTS: A total of 25 studies on 172 patients were included. In patients with tremor plus dystonia syndromes (n = 6), ventralis intermediate nucleus of the thalamus (VIM)-FUS gave >50% tremor reduction, with no improvement in dystonia and worsened dystonia in 2/6 patients. Ventral-oralis complex (VO)-FUS gave >50% improvement for focal hand dystonia (n = 6) and 100% return to musical performance in musician\'s dystonia (n = 6). In patients with multiple sclerosis (MS) and tremor (n = 3), improvement in tremor was seen in 2 patients with a favorable skull density ratio; no MS disease change was noted after VIM-FUS. In patients with tremor and comorbid ataxia syndromes (n = 3), none were found to have worsened ataxia after VIM-FUS; all had clinically significant tremor improvement. Subthalamic nucleus (STN)-FUS for PD (n = 49) gave approximately 50% improvement in PD motor symptoms, with dystonia and mild dyskinesias as possible adverse effects. Cerebellothalamic tract (CTT-FUS) for ET (n = 42) gave 55-90% tremor improvement, with gait dysfunction as a rare persistent adverse effect. Pallidothalamic tract (PTT-FUS) for PD (n = 50) gave approximately 50% improvement in motor symptoms, with mild speech dysfunction as a possible adverse effect.
    CONCLUSIONS: VIM-FUS appeared safe and effective for heterogenous tremor etiologies, and VO-FUS appeared most effective for isolated segmental dystonia. STN-FUS was effective for PD symptom reduction; postoperative dystonia and mild on-medication dyskinesias required medical management. Tractography-based targeting with CTT-FUS for ET and PTT-FUS for PD demonstrated promising early results. Larger prospective trials with long-term follow-up are needed to the evaluate the safety and efficacy non-FDA approved indications for FUS.
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  • 文章类型: Review
    舞蹈症是一种多动运动障碍,伴有肌张力障碍,肌阵鸣,Tics,刻板印象,和颤抖。它的特点是过度,令人痛苦的无目的运动,不规则定时,随机分布。舞蹈症可以存在于许多疾病中,比如世袭,代谢紊乱,药物诱导,和功能障碍,and,很少,遗传,自身免疫,和传染病。原发性骨髓纤维化(PMF)是导致无效克隆造血的骨髓增殖性肿瘤,骨髓中的纤维组织沉积,髓外造血,脾肿大.在极少数情况下,遵循不确定的病理机制,它可以呈现舞蹈症,特别是影响四肢,头部,和口面舌肌。我们介绍了一名男性患者的情况,该患者多年来一直在发展PMF,他因进行性认知障碍和广泛性不自主运动障碍而入院。我们还对过去40年中发表的所有伴有舞蹈病的骨髓增生性疾病病例进行了综述。
    Chorea is a hyperkinetic movement disorder, accompanied by dystonia, myoclonus, tics, stereotypies, and tremors. It is characterized by excessive, purposeless movements that are distressing, irregularly timed, and randomly distributed. Chorea can be present in many diseases, such as hereditary, metabolic disturbance, drug-induced, and functional disorders, and, rarely, genetic, autoimmune, and infectious diseases. Primary myelofibrosis (PMF) is a myeloproliferative neoplasm that leads to ineffective clonal hematopoiesis, fibrous tissue deposits in the bone marrow, extramedullary hematopoiesis, and splenomegaly. In rare cases, following uncertain pathological mechanisms, it can present with chorea, particularly affecting the limbs, head, and orofaciolingual muscles. We present a case of a male patient with evolving PMF over several years who was admitted for progressive cognitive impairment and generalized involuntary movement disorder. We also present a review of all cases of myeloproliferative disorders presenting with chorea published in the last 40 years.
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