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Tremor and Other Hyperkinetic Movements最新文献

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Subacute Sclerosing Panencephalitis Causing Rapidly Progressive Dementia and Myoclonic Jerks in a Sexagenarian Woman 一名性别歧视妇女的亚急性硬化性全脑炎导致快速进展性痴呆和肌阵挛性抽搐
IF 2.2 Q2 CLINICAL NEUROLOGY Pub Date : 2019-08-27 DOI: 10.7916/tohm.v0.680
A. J. Reyes, K. Ramcharan, Sean Perot, S. Giddings, F. Rampersad, R. Gobin
Background Subacute sclerosing panencephalitis (SSPE) is a disease of childhood and adolescence, but can affect adults. Rapidly progressive cognitive decline, seizures including myoclonic jerks, spasticity, ataxia, visual disturbances, and incontinence are typical manifestations. Case report A 62-year-old woman who presented with rapidly progressive dementia and myoclonus was diagnosed with SSPE. There was resolution of the movement disorder with clonazepam and valproic acid treatment and some amelioration of cognitive decline after 3 months of therapy with interferon alfa and isoprinosine. Discussion With the recent rise in measles cases worldwide, any increased incidence of SSPE would require vigilance for early interventions.
背景亚急性硬化性全脑炎(SSPE)是一种儿童和青少年疾病,但可影响成人。快速进行性认知能力下降、癫痫发作(包括肌阵挛性抽搐、痉挛、共济失调、视觉障碍和失禁)是典型的表现。病例报告一位62岁的女性,表现为快速进行性痴呆和肌阵挛,被诊断为SSPE。氯硝西泮和丙戊酸治疗后运动障碍得到了缓解,干扰素α和异丙素治疗3个月后认知能力下降有所改善。讨论随着最近全球麻疹病例的增加,任何SSPE发病率的增加都需要警惕早期干预。
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引用次数: 4
A Review of the Clinical Evidence for Complementary and Alternative Medicine in Huntington’s Disease 亨廷顿舞蹈症补充和替代医学的临床证据综述
IF 2.2 Q2 CLINICAL NEUROLOGY Pub Date : 2019-08-26 DOI: 10.7916/tohm.v0.678
Margaret Yu, D. Bega
Background There is a lack of published guidelines related to the use of complementary and alternative medicine (CAM) for Huntington’s disease (HD). We conducted a review of the literature to summarize the available evidence for various mind–body practices and nutraceuticals. Methods PubMed and Cochrane Library electronic databases were searched independently from inception to February 2019 by two independent raters. Studies were classified for the level of evidence (Class I, II, III, or IV) according to the American Academy of Neurology (AAN) classification scale. Results Randomized controlled trials in HD were reviewed for mind–body interventions (dance therapy, music therapy, and exercise), alternative systems (traditional Chinese medicine [TCM]), and nutraceuticals/diet (aminooxyacetic acid [AOAA], coenzyme q10, creatine, cannabinoids, alpha-tocopherol, eicosapentaenoic acid, idebenone, levocarnitine, and triheptanoin). Few studies met AAN Class I or II level of evidence for benefits, and these are highlighted. Discussion There is a relative paucity of clinical trials examining CAM modalities in HD when compared to other neurodegenerative disorders. Currently, there is no evidence supporting disease modification or symptom improvement with any specific dietary or nutraceutical supplement for HD. Supervised exercise and contemporary dance are safe for people with HD, but more robust studies are warranted to guide specific recommendations for these and other mind–body interventions.
背景缺乏关于亨廷顿舞蹈症(HD)补充和替代药物(CAM)使用的已发表指南。我们对文献进行了回顾,总结了各种身心实践和营养品的可用证据。方法PubMed和Cochrane图书馆电子数据库由两名独立评分者从成立到2019年2月进行独立检索。根据美国神经病学学会(AAN)的分类量表,对研究的证据水平(I、II、III或IV级)进行了分类。结果回顾了HD的随机对照试验,包括身心干预(舞蹈治疗、音乐治疗和锻炼)、替代系统(中药)和营养品/饮食(氨基氧乙酸[AOAA]、辅酶q10、肌酸、大麻素、α-生育酚、二十碳五烯酸、依地苯酮、左旋肉碱和三庚酸)。很少有研究符合AAN一级或二级的益处证据,这些都得到了强调。讨论与其他神经退行性疾病相比,检查HD CAM模式的临床试验相对较少。目前,没有证据支持通过任何特定的HD饮食或营养补充剂来改善疾病或症状。有监督的锻炼和现代舞蹈对HD患者来说是安全的,但有必要进行更有力的研究来指导这些和其他身心干预的具体建议。
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引用次数: 8
Neurodegeneration with Brain Iron Accumulation: Two Additional Cases with Dystonic Opisthotonus 伴有脑铁积聚的神经退行性变:再发两例强直性Opisthotinus
IF 2.2 Q2 CLINICAL NEUROLOGY Pub Date : 2019-08-21 DOI: 10.7916/tohm.v0.683
S. Mehta, V. Lal
Background Specific phenomenology and pattern of involvement in movement disorders point toward a probable clinical diagnosis. For example, forehead chorea usually suggests Huntington’s disease; feeding dystonia suggests neuroacanthocytosis and risus sardonicus is commonly seen in Wilson’s disease. Dystonic opisthotonus has been described as a characteristic feature of neurodegeneration with brain iron accumulation (NBIA) related to PANK2 and PLA2G6 mutations. Case report We describe two additional patients in their 30s with severe extensor truncal dystonia causing opisthotonic posturing in whom evaluation revealed the diagnosis of NBIA confirmed by genetic testing. Discussion Dystonic opisthotonus may be more common in NBIA than it is reported and its presence especially in a young patient should alert the neurologists to a possibility of probable NBIA.
背景:运动障碍的特殊现象学和参与模式指向可能的临床诊断。例如,前额舞蹈症通常表明患有亨廷顿舞蹈症;进食障碍提示威尔逊氏病常见于神经性棘细胞增多症和软骨炎。张力张力障碍被描述为与PANK2和PLA2G6突变相关的神经变性伴脑铁积累(NBIA)的特征。病例报告我们描述了另外两名30多岁的患者,他们患有严重的伸肌-截肌张力障碍,导致体位紧张性姿势,在他们的评估中,基因检测证实了NBIA的诊断。肌张力障碍在NBIA中可能比报道的更常见,特别是在年轻患者中,应该提醒神经科医生注意NBIA的可能性。
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引用次数: 1
The Top 50 Most-Cited Articles in Orthostatic Tremor: A Bibliometric Review 《直立震颤》50篇被引用最多的文章:文献计量学综述
IF 2.2 Q2 CLINICAL NEUROLOGY Pub Date : 2019-08-05 DOI: 10.7916/tohm.v0.679
M. L. Ruiz, Julián Benito-León
Background Article-level citation count is a hallmark indicating scientific impact. We aimed to pinpoint and evaluate the top 50 most-cited articles in orthostatic tremor (OT). Methods The ISI Web of Knowledge database and 2017 Journal Citation Report Science Edition were used to retrieve the 50 top-cited OT articles published from 1984 to April 2019. Information was collected by the Analyze Tool on the Web of Science, including number of citations, publication title, journal name, publication year, and country and institution of origin. Supplementary analyses were undertaken to clarify authorship, study design, level of evidence, and category. Results Up to 66% of manuscripts were recovered from five journals: Movement Disorders (n = 18), Brain (n = 4), Journal of Clinical Neurophysiology (n = 4), Neurology (n = 4), and Clinical Neurophysiology (n = 3). Articles were published between 1984 and 2018, with expert opinion as the predominant design (n = 22) and review as category (n = 17). Most articles had level 5 evidence (n = 26). According to their countries of origin, 34% of articles belonged to the United States (n = 17) leading the list, followed by United Kingdom (n = 15). University College London yielded the greater number of articles (n = 12), followed by the University of Kiel (n = 9). Most popular authors were G. Deuschl (n = 10), C.D. Marsden (n = 6), J. Jankovic (n = 5), P.D. Thompson (n = 5), J.C. Rothwell (n = 5), L.J. Findley (n = 4), and P. Brown (n = 4), who together accounted for 48% of them. All papers were in English. Discussion Publishing high-cited OT articles could be facilitated by source journal, study design, category, publication language, and country and institution of origin.
文章级引用数是科学影响的标志。我们的目标是确定和评估在直立性震颤(OT)中被引用最多的前50篇文章。方法利用ISI Web of Knowledge数据库和2017 Journal Citation Report Science Edition检索1984年至2019年4月发表的50篇被引频次最高的OT论文。通过Web of Science上的Analyze Tool收集信息,包括引用次数、出版标题、期刊名称、出版年份、原产国家和机构。进行补充分析以澄清作者、研究设计、证据水平和类别。结果高达66%的手稿来自5种期刊:运动障碍(n = 18)、脑(n = 4)、临床神经生理学杂志(n = 4)、神经病学(n = 4)和临床神经生理学(n = 3)。文章发表于1984年至2018年之间,以专家意见为主要设计(n = 22),综述为分类(n = 17)。大多数文章为5级证据(n = 26)。按原产国划分,34%的文章属于美国(n = 17),排名第一,其次是英国(n = 15)。伦敦大学学院发表的文章最多(n = 12),其次是基尔大学(n = 9)。最受欢迎的作者是G. Deuschl (n = 10)、C.D. Marsden (n = 6)、J. Jankovic (n = 5)、P. d . Thompson (n = 5)、J. c . Rothwell (n = 5)、L.J. Findley (n = 4)和P. Brown (n = 4),他们共占论文的48%。所有的论文都是英文的。发表高被引的OT文章可以通过来源期刊、研究设计、类别、出版语言、国家和机构来促进。
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引用次数: 4
Gait Impairment in Myoclonus–Dystonia (DYT-SGCE) 肌阵挛-肌张力障碍的步态障碍(DYT-SGCE)
IF 2.2 Q2 CLINICAL NEUROLOGY Pub Date : 2019-08-02 DOI: 10.7916/tohm.v0.656
G. Haeri, G. Shahidi, A. Fasano, M. Rohani
Background Myoclonus–dystonia usually presents variable combination of myoclonus and dystonia mainly affecting the neck and arms, but leg involvement, especially as the presenting sign, is not common. Case report A 29-year-old lady with a heterozygous mutation in Epsilon-sarcoglycan (SGCE) gene is presented with rapid jerks of the right leg interfering with walking. She has also manifested dystonic posture and jerks of the trunk and proximal upper limbs. Discussion Although it is not typical, leg involvement could be a manifestation of myoclonus–dystonia either at presentation or during disease progression.
背景肌阵挛-肌张力障碍通常表现为肌阵挛和肌张力障碍的可变组合,主要累及颈部和手臂,但累及腿部,尤其是作为首发症状,并不常见。病例报告一名29岁的Epsilon-sarcoglycan (SGCE)基因杂合突变的女性表现为右腿快速抽搐,干扰行走。她还表现出张力障碍姿势和躯干和上肢近端抽搐。虽然不典型,但累及腿部可能是肌阵挛-肌张力障碍的表现,无论是在首发时还是在疾病进展过程中。
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引用次数: 0
Uncommon Movement Disorders in Chronic Hepatic Disease with Response to Rifaximin 慢性肝病中罕见的运动障碍对利法昔明的反应
IF 2.2 Q2 CLINICAL NEUROLOGY Pub Date : 2019-08-02 DOI: 10.7916/tohm.v0.649
A. L. Sousa, P. Salgado, J. Alves, S. Silva, S. Ferreira, M. Magalhães
Background Chronic hepatic disease can present with extrapyramidal symptoms. We describe two cases that presented with highly unusual movement disorders: ballism and gait freezing. Case report Patient 1 is a 42-year-old man with previous episodes of hepatic encephalopathy (HE) who presented with upper limb dystonia and generalized chorea that progressed to ballism. Patient 2 is a 55-year-old woman who presented with pronounced gait freezing. In both patients, features of HE and acquired hepatocerebral degeneration coexisted. They improved markedly, though transiently, with rifaximin. Discussion Ammonia-reducing treatments should be considered in patients presenting with movement disorders due to chronic liver disease.
背景:慢性肝病可表现为锥体外系症状。我们描述了两个案例,提出了高度不寻常的运动障碍:弹道和步态冻结。病例报告:患者1是一名42岁男性,既往有肝性脑病(HE)发作,表现为上肢肌张力障碍和全身性舞蹈病,并发展为肾小球症。患者2为55岁女性,表现为明显的步态冻结。在这两例患者中,HE和获得性肝脑变性特征并存。使用利福昔明后,症状明显改善,尽管是短暂的。慢性肝病所致运动障碍患者应考虑氨还原治疗。
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引用次数: 5
Successful Treatment of Primary Orthostatic Tremor Using Perampanel Perampanel成功治疗原发性直立性震颤
IF 2.2 Q2 CLINICAL NEUROLOGY Pub Date : 2019-08-01 DOI: 10.7916/tohm.v0.681
Anant Wadhwa, S. Schaefer
Background Primary orthostatic tremor (POT) remains a therapeutic conundrum. Various medication classes have been tried, yielding modest results at best. Case Report A 62-year-old female with a 13-year history of POT, refractory to clonazepam up to 20 mg/day, was treated with perampanel 1–2 mg/day. She reported 90% subjective symptomatic improvement. Discussion This case highlights the potential for use of perampanel, a novel AMPA (α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid) receptor antagonist for the treatment of POT. There has been one prior report citing its use for POT with complete resolution of symptoms. We encourage further studies to highlight its efficacy for POT.
原发性直立性震颤(POT)仍然是一个治疗难题。已经尝试了各种各样的药物,最多只能产生一般的效果。病例报告一名62岁女性,有13年的POT病史,氯硝西泮20mg /天难治性,用perampanel 1 - 2mg /天治疗。她报告90%的主观症状改善。本病例强调了使用perampanel(一种新型AMPA (α-氨基-3-羟基-5-甲基-4-异恶唑丙酸)受体拮抗剂治疗POT的潜力。先前有一篇报道引用其用于POT并完全解决症状。我们鼓励进一步研究以突出其对POT的疗效。
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引用次数: 5
Facio-Oculo-Palatal Myoclonus Complicated by a Recurrent Brainstem Stroke 面-眼-腭肌阵挛并发复发性脑干中风
IF 2.2 Q2 CLINICAL NEUROLOGY Pub Date : 2019-07-31 DOI: 10.7916/tohm.v0.658
Y. Pitakpatapee, P. Srivanitchapoom
Background A 54-year-old Thai male who has suffered from multiple episodes of ischemic and hemorrhagic strokes developed facio-oculo-palatal myoclonus (FOPM) 1 month after the latest episode of the brainstem stroke. Phenomenology Shown The patient presented with semirhythmic, involuntary, horizontal jerky, and rotatory ocular oscillation concomitant with asymmetrical palatal and perioral myoclonus consistent with FOPM. Educational value FOPM is a useful clinical clue for diagnosing brainstem lesions, specifically in the Guillain–Mollaret triangle. The commonest etiology is cerebrovascular diseases.
背景:一名54岁的泰国男性,曾多次发生缺血性和出血性中风,在最近一次脑干卒中发作1个月后出现面-眼-腭肌阵挛(FOPM)。现象学显示患者表现为半节律性、非自主性、水平急动和旋转性眼球振荡,伴有不对称腭部和口周肌阵挛,符合FOPM。FOPM具有教育价值,是诊断脑干病变的有用临床线索,特别是在格林-莫拉莱三角区。最常见的病因是脑血管疾病。
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引用次数: 0
Functional Movement Disorders in Elderly 老年人功能性运动障碍
IF 2.2 Q2 CLINICAL NEUROLOGY Pub Date : 2019-07-30 DOI: 10.7916/tohm.v0.691
A. Chouksey, S. Pandey
Background Functional movement disorders (FMDs) have been rarely described in the elderly population. Methods This is a retrospective chart review of elderly patients with FMDs (onset >60 years) attending the movement disorders clinic at a tertiary care teaching institute in India. Results Out of 117 patients diagnosed with FMD at our center, 18 patients had an onset after the age of 60 years. The male-to-female ratio was 10:8 and the duration of symptoms ranged from 1 day to 5 years. Social (10/18) and physical factors (5/18) with an evident temporal relationship with the onset of FMD were identified in 15 out of 18 patients. Six of them had a past history of depression, anxiety, or other psychiatric illnesses. The tremor was the most frequent phenomenology seen in 11 (61.1%) patients, followed by dystonia in seven (38.8%), choreoballism and tics in two each, and hemifacial spasm and functional gait in one each. Seven patients had more than one phenotype. Discussion Tremor was the most frequent movement disorder seen in our patients with FMD. Surprisingly, tics (n = 2) and choreoballistic (n = 2) movements were also found in our patients with FMD, which has not been reported previously in an elderly population. Both physical and social factors were identified preceding the development of FMDs in majority of our patients.
背景功能性运动障碍(FMD)在老年人群中很少被描述。方法这是对印度一所三级护理教学机构运动障碍诊所的老年FMD患者(发病>60岁)的回顾性图表回顾。结果本中心117例FMD患者中,18例60岁以后发病。男女比例为10:8,症状持续时间为1天至5年。在18名患者中的15名患者中发现了与FMD发作具有明显时间关系的社会因素(10/18)和身体因素(5/18)。其中6人有抑郁症、焦虑症或其他精神疾病病史。震颤是11例(61.1%)患者中最常见的现象,其次是肌张力障碍7例(38.8%),舞蹈障碍和抽搐各2例,面肌痉挛和功能性步态各1例。7名患者具有一种以上表型。讨论震颤是我们FMD患者中最常见的运动障碍。令人惊讶的是,在我们的FMD患者身上也发现了抽搐(n=2)和舞蹈性运动(n=2),这在老年人群中以前没有报道过。在我们的大多数患者中,身体和社会因素都是在FMD发展之前确定的。
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引用次数: 10
Treatment with Botulinum Neurotoxin Improves Activities of Daily Living and Quality of Life in Patients with Upper Limb Tremor 肉毒杆菌神经毒素治疗可改善上肢震颤患者的日常生活活动和生活质量
IF 2.2 Q2 CLINICAL NEUROLOGY Pub Date : 2019-07-26 DOI: 10.7916/tohm.v0.640
A. Kreisler, Benoîte Bouchain, L. Defebvre, P. Krystkowiak
Background Botulinum neurotoxin’s degree of effectiveness on upper limb tremor is subject to debate; although this treatment reduces the tremor’s amplitude, a clear functional benefit has not been demonstrated. The objective of this study was to assess the effect of botulinum neurotoxin type A treatment on activities of daily living and quality of life in patients with upper limb tremor. Methods We retrospectively examined the medical records of 50 consecutive patients treated with botulinum neurotoxin for upper limb tremor that was refractory to oral medication. One month after the injection, the patient was evaluated according to the Quality of Life in Essential Tremor Questionnaire, and the Essential Tremor Embarrassment Assessment. Results Full data sets were available for 38 patients suffering variously from essential tremor (n = 21), Holmes tremor secondary to a focal brain lesion (n = 8), idiopathic dystonic tremor (n = 4), primary writing tremor (n = 4), and Parkinson’s disease (n = 1). The Quality of Life Essential Tremor Questionnaire and the Essential Tremor Embarrassment Assessment scores improved significantly (p < 0.001) in the study population as a whole, and in the essential tremor and Holmes tremor subgroups. Discussion Botulinum neurotoxin treatment of patients with upper limb tremor is associated with improved quality of life and activities of daily living, irrespective of the tremor’s etiology. Long-term treatment enables the physician to adjust the injection strategy to the patient’s needs. Our study was limited by its retrospective design. The results must therefore be confirmed in a prospective, double-blind, placebo-controlled, randomized clinical trial.
背景肉毒杆菌神经毒素对上肢震颤的有效程度存在争议;虽然这种治疗减少了震颤的幅度,但尚未证明其在功能上有明显的益处。本研究的目的是评估A型肉毒杆菌神经毒素治疗对上肢震颤患者日常生活活动和生活质量的影响。方法回顾性分析50例连续使用肉毒杆菌神经毒素治疗口服药难治性上肢震颤患者的病历。注射后1个月,根据特发性震颤生活质量问卷和特发性震颤尴尬评估对患者进行评估。结果完整的数据集用于38从特发性震颤患者不同(n = 21),福尔摩斯震颤次要焦大脑病变(n = 8)特发性矛盾的震颤(n = 4),主要写震颤(n = 4)和帕金森病(n = 1)。生活质量问卷特发性震颤和特发性震颤尴尬评估分数明显改善(p < 0.001)在研究人口作为一个整体,在特发性震颤和福尔摩斯地震子组。肉毒杆菌神经毒素治疗上肢震颤患者与改善生活质量和日常生活活动相关,而不考虑震颤的病因。长期治疗使医生能够根据病人的需要调整注射策略。我们的研究受到回顾性设计的限制。因此,这些结果必须在前瞻性、双盲、安慰剂对照、随机临床试验中得到证实。
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引用次数: 10
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Tremor and Other Hyperkinetic Movements
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