帕金森病震颤和原发性震颤的电生理学特征研究

目的探讨帕金森病(Parkinson’s disease,PD)震颤和原发性震颤(essential tremor,ET)患者电生理上的异同,为PD和ET的诊断提供思路。方法选取2015-05—2016-04在浙江大学附属第二医院门诊就诊的50例PD患者(PD组),70例ET患者(ET组),以及45例年龄、性别匹配的健康志愿者(生理性震颤组),完善PD以及ET患者的震颤评分,通过加速度传感器对所有受试者进行3种姿势状态下的震颤分析检査。比较受试者的震颤幅度、震颤频率、震颤频谱宽度以及负重对其影响,总结2组患者的电生理学特点。结果 3组平均震颤幅度静止状态同负重状态比较,生理性震颤组变化不大,而PD和ET患者静止状态震颤幅度较负重状态明显减少,差异有统计学意义(P0.05);震颤峰值频率比较:3组静止状态震颤频率均高于负重状态,健康对照组下降明显,差异有统计学意义(P0.05),PD组和ET组差异无统计学意义(P0.05)。结论 PD震颤和ET震颤参数接近,加速度传感器测定的震颤参数难以区分二者,但对于生理性震颤和病理性震颤的鉴别存在一定的意义。     

生理性震颤与特发性震颤的评分比较

目的　通过震颤幅度评分 ,对生理性震颤 (PT)和特发性震颤 (ET)进行鉴别。方法　采用震颤幅度评分和 6项任务震颤幅度评分法 ,对 63例PT患者 (PT组 )和 38例ET患者 (ET组 )的上肢震颤幅度进行比较。结果　PT组与ET组震颤幅度评分差异无显著性 (P >0 0 5)。 6项任务震颤幅度评分差异有高度显著性 (P <0 0 1 )。PT组两项任务震颤评分接近或 >2分 ,而且非利手评分 >利手 ,差异有显著性 (P <0 0 5)。ET组 4项任务震颤评分 >2分 ,差异有显著性 (P <0 0 5) ,但利手与非利手间差异无显著性 (P >0 0 5)。结论　对上肢震颤幅度进行评分量化分析有助PT与ET的鉴别诊断     

合并静止性震颤的原发性震颤患者临床特点分析

目的了解北京地区社区中老年人原发性震颤(essential tremor,ET)的临床特征及其与预后的关系。方法2000年在北京城区、近郊平原、远郊山区调查了2835名55岁以上中老年人,采用筛查和临床诊断两步法确诊患者。发现ET患者135例,对其中合并静止性震颤的患者进行分析,并于2004年对其进行随访。结果其中合并上肢静止性震颤患者17例,占12.6%,合并静止性震颤组震颤总分、头部震颤的比例、肢体震颤总分均明显高于对照组。两组年龄、性别组成、病史无显著性差异,姿势性震颤和动作性震颤的程度差异不明显。4年后随访到13例(13/17),其中3例死亡,实际调查的10例中有9例仍诊断为ET,另1例诊断为临床很可能ET。结论有相当部分社区ET患者可以合并静止性震颤,合并静止性震颤的患者提示病情较重。     

特发性震颤的非运动症状研究

目的对特发性震颤(ET)患者的非运动症状(NMS)进行研究,以期发现其临床和病理解剖特点。方法把60名ET患者和63名对照组分为4组:中青年对照组、中青年ET组、老年对照组和老年ET组,分别对各组进行听力检测和认知功能评估。结果与相应的对照组比较,中青年ET组和老年ET组均有明确的听力下降(P=0.026;P=0.005);中青年ET组无认知功能下降(P=0.061),老年ET组认知功能下降明显(P=0.003)。老年ET组的视空间和执行能力下降幅度较中青年ET组更明显(P=0.012)。ET患者听力下降和认知功能下降有一定相关性(P=0.005)。结论 ET患者的非运动症状中,听力下降出现较早,认知功能下降出现较晚,老年性ET患者在认知功能的视空间和执行能力方面表现更差;二者发病具有一定正相关性。ET的病理解剖基础可能为听神经﹣脑干﹣小脑系统受损较早,而海马﹣边缘系统﹣皮质的退化变性发生较晚。     

特发性震颤和痉挛性斜颈的头部震颤的临床和电生理研究

目的探讨痉挛性斜颈(CD)和特发性震颤(ET)的头部震颤的临床和电生理特点。方法对17例确诊特发性震颤(ET)和15例确诊痉挛性斜颈(CD)并且具有头部震颤的患者行肌电图震颤分析研究,并探讨两种疾病头部震颤的各自特点。结果 ET组平均年龄(59.58±2.78)岁,平均病程(100.94±31.46)个月,男性4例,女性13例,8例有ET家族史,头部抖动摇头型14例,点头型3例,混合型1例,17例ET患者均有手抖,2例下肢抖动,1例下颌抖动。静止震颤未引出,姿势震颤频率(7.45±0.34)Hz,负重震颤频率(7.52±0.39)Hz,头部震颤评分总分(3.39±0.22)分,平卧分数0分,站立分数(1.29±0.11)分,时间分数(2.41±0.19)分;CD组平均年龄(46.2±3.15)岁,平均病程83.2±21.79月,男性7例,女性8例,2例有ET家族史,头部抖动摇头型9例,点头型4例,混合型2例,4例CD患者有手抖,0例下肢抖动,0例下颌抖动。静止震颤频率(5.55±0.18)Hz,姿势震颤频率(5.58±0.19)Hz,负重震颤频率(5.88±0.23)Hz,头部震颤评分总分(5.67±0.33)分,平卧分数(1.6±0.16)分,站立分数(2.0±0.14)分,时间分数(2.07±0.15)分。CD的头部震颤存在感觉诡计和转颈后特定位置的震颤加重。统计学分析提示ET和CD在发病年龄、姿势性震颤和负重震颤频率、震颤评分总分及平卧分数、站立分数差异均有统计学意义。P值分别为0.033,0.000,0.001,0.000,0.000.0.000。结论痉挛性斜颈比较特发性震颤就诊年龄更年轻,存在静止性震颤,姿势性和负重震颤频率更慢,头部震颤评分的总分和平卧分数及站立分数均较高。并且可以存在感觉诡计及转颈后加重。     

特发性震颤与帕金森病患者嗅觉障碍的定量分析研究

目的检测特发性震颤(ET)与帕金森病(PD)患者的嗅觉功能,并探讨ET与PD的关系。方法应用中国科学院半导体研究所研制的五味嗅觉测试液检测30例ET、30例PD患者及30例健康对照组,从嗅觉察觉阈值和嗅觉识别阈值两个方面比较各组的差异。结果 PD组的嗅觉察觉阈值和嗅觉识别阈值均明显高于ET组和对照组(P<0.01),而ET组与对照组的嗅觉察觉阈值和嗅觉识别阈值差异无统计学意义(P>0.05)。结论帕金森病有明显的嗅觉功能障碍,而特发性震颤无明显的嗅觉功能障碍,ET与PD可能是两种不同性质的疾病。     

原发性震颤与特发性帕金森病嗅觉障碍的研究

目的检测原发性震颤(ET)与特发性帕金森病(PD)患者的嗅觉功能,并探讨ET与PD的关系.方法采用Bain等(国际震颤研究小组)提出的ET诊断标准及国际通用的PD诊断标准,收集80例ET 和96例PD患者,分别进行简易嗅觉检查,并随机选择两组年龄、性别分别与ET、PD患者相匹配的健康人作为对照.结果 PD患者嗅觉障碍远高于对照组,而ET患者与对照组相近.结论 ET与PD可能是两种不同性质的疾病.     

震颤分析在帕金森病和原发性震颤鉴别诊断中的应用

目的 探讨震颤分析在帕金森病(parkinson's disease,PD)和原发性震颤(essential trem-or,ET)鉴别诊断中的应用价值.方法 选取2017年9月至2020年11月在福建省立金山医院门诊和住院确诊的PD患者27例(PD组)和ET患者23例(ET组),所有患者均至少有一侧上肢静止性或姿势性...     

原发性震颤患者的非运动症状

目的 研究原发性震颤(essential tremor,ET)患者嗅觉障碍等非运动症状的发生情况.方法 对62例ET患者应用震颤评分量表(Falm-Tolosa-Matin Tremor Rating Scale,TRS)、帕金森病非运动症状30问卷量表(Parkinson's disease non-motor symptoms questionnaire,NMS Quest)和MMSE进行评分,T&T标准嗅觉测试液检测嗅觉功能,并与60名健康体检者进行对照.结果 ET患者的嗅觉障碍发生率为51.6%(32/62),明显高于健康对照组(30.0%,18/60,x~2=12.371,P<0.05);平均每例ET患者出现5项左右不同的非运动症状,以对近期发生的事情记忆有困难或忘记做一些事情、嗅觉障碍、令人紧张或害怕的梦或生动梦境的发生率较高.嗅觉障碍等非运动症状的发生与ET患者的病程、病情严重程度、治疗与否没有明显的相关性.结论 除姿势性震颤或动作性震颤外,ET患者还会出现嗅觉障碍等非运动症状,需要全面认识和及时干预.     

原发性震颤患者睡眠质量的临床研究

目的评价原发性震颤患者睡眠质量,探讨原发性震颤(essential tremor,ET)患者睡眠障碍的临床特征及相关因素。方法采用匹兹堡睡眠质量指数(Pittsburgh sleep qualityindex,PQS1)、爱泼沃斯思睡量表(Epworth sleepiness scale,ESS)、汉密尔顿抑郁量表(Hamilton depression rating scale for depression,HAMD)对68例原发性震颤患者和70名正常对照者的睡眠状况及抑郁进行评估。结果 ET组睡眠障碍发生率为52.9%,较正常对照组高35.7%(P0.05);ET组抑郁评分(15.82±4.87)高于正常对照组(9.96±5.65)(P0.01);ET患者日间过度思睡发生率(44.1%)较正常对照组(15.7%)高(P0.01)。结论 ET患者总体睡眠质量差,伴发睡眠障碍较对照组常见,其临床主要表现为入睡困难、片断睡眠及日间过度思睡,睡眠障碍与年龄、抑郁有关。     

Arylsulphatase A (ASA) activity in parkinsonism and symptomatic essential tremor

Arylsulphatase A (ASA) activity was evaluated in 47 patients with a diagnosis of parkinsonism or essential tremor. Mean ASA activity was significantly reduced compared with both a healthy control group of 71 individuals (p < 0.01) and with a group of 44 neurological patients without movement disorders (p < 0.02). Using definite clinical criteria the patients were classified as typical or atypical with respect to Parkinson's disease (PD) or essential tremor (ET). A normal ASA level was found in all the cases showing typical clinical features (PD and ET), while ASA activity was significantly lowered (p < 0.01) in 55.6% of the atypical cases (Parkinsonian syndrome or symptomatic ET). Our data support the hypothesis of a non-casual association between low ASA level and the clinical features of parkinsonism or symptomatic ET.     

Intention tremor of the head in patients with essential tremor.

Patients with essential tremor (ET) have kinetic arm tremor; this tremor can also have an intentional component. We are unaware of reports of intention tremor of the head in ET. Our aims were to describe, provide electrophysiological data and video documentation of, and estimate the prevalence of intention tremor of the head in our sample. Ten (9.0%; 95% confidence interval = 4.7%-14.3%) of 111 patients had intention tremor of the head; in 7 it involved the neck and in 3 the chin. These patients trended toward having more severe kinetic arm tremor and they had more severe intention tremor of the arms. These observations provide further support for the evolving view that the cerebellum may be involved in ET.     

微电极导向立体定向手术治疗原发性震颤

目的：探讨丘脑腹外则Vim核中与震颤症状相关的神经细胞电活动的规律,总结丘脑毁损术治疗原性震颤（ET）的临床效果和安全性。方法：对42例ET患者行单侧微电极导向的丘脑毁损术。采用FAHN的临床震颤评分法对其中11例患者术前及术后进行定量评估和分析。结果：Vim核中神经细胞的簇状电活动节律与肢体震颤的节律有肯定的一致性,毁损这些与震颤症状相关的神经细胞后,所有ET患者手术对侧肢体的震颤完全消失;震颤的整体改善率52％,特殊动作和功能改善54％,功能残疾改善率77％,长期随访疗效稳定,其中40例患者的震颤症状密切相关,毁损这些细胞能完全永久性地消除震颤症状。     

Essential tremor: an overdiagnosed condition?

The diagnosis of essential tremor (ET) and its differentiation from other types of tremor is often difficult. In 1994 Bain et al. defined a classical phenotype by studying 20 patients with pure essential tremor and similarly affected family members in at least three generations. We assessed how many of the patients diagnosed by different neurologists at our institution as having ET conformed to this defined phenotype. We randomly selected 50 patients who were diagnosed with ET by any neurologist at the National Hospital for Neurology and Neurosurgery since the publication of the Bain et al. report, and determined the number of patients who had clinical features compatible with the phenotype that it had defined. Only 25 (50%) of these patients had ET so defined. Ten patients clearly had alternative diagnoses: four had clear additional dystonia, two neuropathic tremor, two had unilateral leg tremor, one drud-induced tremor, and one sudden onset after head trauma. The remaining 15 patients also had atypical features including myoclonus (one), onset in a body part other than the arms (six), sudden onset (two), rest tremor (seven), onset after the age of 65 years (four), a family member with an isolated head tremor (one), or reduced armswing (two). The diagnosis of ET is overused even among experienced neurologists, and other types of tremor should be considered in atypical patients before making this diagnosis. Received: 30 November 1999 / Received in revised form: 12 May 2000 / Accepted: 21 June 2000     

Clinical characteristics of essential tremor in Taiwan: an exploratory‐comparative study

Background: There are few large‐scale clinical analyses of essential tremor (ET) in Asia. We studied the detailed clinical profile with emphasizing the age of onset, tremor location, specific tremor patterns, and rate of progression (ROP) to delineate the characteristics of Taiwanese ET patients and found the difference between the Taiwanese and the Caucasians ET patients. Methods: All ET patients fulfilled the Movement Disorders Society diagnosis criteria were investigated with a standardized assessment protocol, which including clinical evaluation, uniform severity scoring, self‐reported questionnaires, accelerometry, surface electromyography, and videotaped tremor examination. Results: Of 219 patients recruited from July 2008 to October 2009, 153 completed the study protocol. Their mean age was 58.9 years and 47% were women, and 33.3% had family history (FH). There was bimodal distribution in age of tremor onset in patients without but not in those with FH. Head tremor (HT) was present in 48 of 153 (31%) patients. Patients with HT showed slower tremor frequency and less ROP than those without HT. Sixty‐seven (44%) patients presented with intention tremor (IT). Male gender and voice tremor were predictive factors of IT occurrence. Conclusions: Comparing with the Caucasians, Taiwanese ET patients have different patterns of onset‐age distribution and lack of female predominance in ET with HT. However, patients with IT and without HT also progressed more rapid as found in the Caucasian.     

Mirtazapine in the treatment of essential tremor: an open-label, observer-blind study

Introduction

Essential tremor (ET) is the most common movement disorder in the adult population. At present ET treatment shows limited efficacy, particularly in patients with severe and disabling symptoms. This study evaluates the clinical efficacy of mirtazapine in an untreated ET patient population.

Materials and methods

30 ET patients (female/male = 19/11; average age = 71.4 ± 8.3 years) were examined by clinical criteria, electromyographic (EMG), and apomorphine tests to study the cortical silent period. The patients were all treated with mirtazapine 30 mg daily.

Results

Mirtazapine proved to be a good control agent for tremor symptomatology in 23/27 patients (85%) who completed 1 month of treatment, with a marked reduction of tremor; the benefit was maintained during the 12-month follow-up. No significant variation in EMG parameters was observed aside from two prevalent and distinct frequencies of tremors (5–6 Hz and 7–8 Hz) and a group of selected patients whose cortical silent period (SP) was markedly reduced. There were no clinical differences between the two subgroups. All apomorphine-tested patients showed an SP with no significant modifications.

Conclusions

Mirtazapine proved to be an efficacious drug treatment for tremor symptoms in patients suffering from ET. It had limited side effects and excellent overall tolerability, could be used as daily monotherapy, and did not interfere with any of the many other medications being taken simultaneously by the patients.     

Subclinical tremor in normal controls with versus without a family history of essential tremor: data from the United States and Turkey

Background and purpose: Mild action tremor is very common in the population. One fundamental question is whether this tremor is related to the neurological disease essential tremor (ET), which occurs in a much smaller segment of the population? ET is often genetic, and variable phenotypic expression is well‐documented in the literature. We determined whether normal controls who report a family history of ET have greater action tremor than normal controls who do not report such a history. Methods: Controls, enrolled in two epidemiological studies (New York and Turkey), were examined in detail and action tremor was rated using a valid and reliable clinical rating scale, resulting in a total tremor score (range 0–36). Results: In New York, the total tremor score was higher in 44/406 (10.8%) controls who reported a family history of ET than in 362/406 controls with no such history (4.25 ± 2.51 vs. 3.78 ± 2.93, P = 0.02). Controls who reported a first‐degree relative with ET had the highest total tremor scores. In Turkey, the total tremor score was higher in 7/89 (7.9%) controls with a family history than in 82/89 controls with no family history (3.43 ± 4.54 vs. 1.13 ± 2.54, P = 0.048). All affected relatives in Turkey were first‐degree. Conclusions: These data suggest that some of the normal tremor exhibited by people in the population is likely to be subclinical, partially expressed ET and that the sphere of ET is wider than is apparent from a consideration of clinically diagnosed cases.