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...     

家族性原发性震颤2例报告

家族性原发性震颤２例报告于代君，陈宁原发性震颤是一种病因未明、具有常染色体显性遗传特征的病症，在临床上常被误诊为帕金森病。其实，这两种疾病在病因、病理、发病机理、临床症状以及治疗方面均有明显不同。目前，药物治疗效果国内尚未有报道。现将两例原发性震颤的...     

原发性震颤

<正> 原发性震颤是一种病因不明,以姿势性震颤为主要临床特征的较常见的神经系统疾病。此病随年龄的增长而患病率增加,而临床症状显缓慢进展,易与帕金森氏病相混淆,为提高对两病的鉴别,现将近期内我科住院收治的2例报告如下。     

阿尔马尔治疗原发性震颤的临床观察

原发性震颤(ｅｓｓｅｎｔｉａｌｔｒｅｍｏｒ,ＥＴ)多发生于中老年人,部分病人为常染色体显性遗传,表现为频率5～8Ｈｚ的肢体和(或)头部震颤,精神紧张时症状加重。病程长,发展慢,严重者影响日常生活。神经系统查体无阳性发现。治疗多采用β受体阻断剂。阿尔马尔(盐酸阿罗洛尔,ａｒｏｔｉｎｏｌｏｌ)是一种新的α、β受体阻断剂,其β受体阻断作用是心得安的5～6倍。我们对13例ＥＴ病人应用阿尔马尔进行了为期6周的临床观察,获得了非常满意的疗效。现报道如下。资料和方法:　13例中,男7例,女6例,年龄47～78岁,平均61岁。平均发病年龄为(47±15…     

原发性震颤与帕金森震颤时手指捏合过程中的动作震颤和捏合震颤的初步…

本文报道应用捏合仪及电子运行分析技术，对ＥＴ和ＰＴ病人手指捏合过程中手震颤时ＴＭＦ，ＴＭＡ，ＧＴＦ及ＧＴＡ四项指标变化的定量研究结果：（１）两组ＴＭＦ和ＧＴＦ各项试验中变化很小；ＴＭＡ及ＧＴＡ值变化与震颤严重度相一致（２）ＥＴ组总ＧＴＡ水平高于ＰＴ组，其变化不如ＴＭＡ显著，尤在快运动时；ＰＴ组ＴＭＡ变化通常与捏俣强度成比例，运动时ＧＴＡ变化比ＴＭＡ更明显。     

原发性震颤运动和非运动性症状的研究进展

原发性震颤(ET)临床表现较为复杂,可出现运动症状和非运动性症状。运动症状包括姿势性震颤或动作性震颤和静止性震颤;非运动性症状包括认知障碍、个性改变、体重指数改变及听力下降等。     

原发性震颤与帕金森震颤时手指捏合过程中的动作震颤和捏合震颤的初步研究

本文报道应用捏合仪及光电子运动分析技术，对ＥＴ和ＰＴ病人手指捏合过程中手震颤时ＴＭＦ、ＴＭＡ、ＧＴＦ及ＧＴＡ四项指标变化的定量研究结果：（１）两组ＴＭＦ和ＧＴＦ各项试验中变化很小；ＴＭＡ及ＧＴＡ值变化与震颤严重度相一致。（２）ＥＴ组总ＧＴＡ水平高于ＰＴ组，其变化不如ＴＭＡ显著，尤在快运动时；ＰＴ组ＴＭＡ变化通常与捏合强度成比例，运动时ＧＴＡ变化比ＴＭＡ更明显。结果提示：（１）此两震颤成分可能源自同一机制。（２）捏合时ＥＴ病人手震颤性质可能主要为手指捏合震颤，而ＰＴ病人主要为手动作震颤。对其可能的临床价值进行了讨论。     

氯氮平治疗原发性震颤的临床和血药浓度研究

目的探讨氯氮平治疗原发性震颤的口服剂量、血药浓度和临床疗效的关系．并确定其有效血药浓度范围。方法符合Bain原发性震颤诊断标准的22例患者均单用氯氮平治疗．于维持量治疗四周后检查其氯氮平血药浓度及临床疗效。结果氯氮平口服剂量范围在8133～37．5mg／d，血药浓度变异很大（20-157ng／L）。口服剂量与血药浓度存在指数曲线关系（R2=0．799．P〈0．01），其方程式为Y=16．4458e^0.0505X。血药浓度与临床疗效显著相关（R^2=0．579，P〈0．01），呈二次模型曲线关系，方程式为Y=31．2902—0．8994X＋0．0212X^2。血药浓度与副反应量表关系不大。结论氯氮平治疗原发性震颤有显著疗效，其有效血药浓度范围为30．9-97．8ng／L，长期使用应做血药浓度监测。     

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。     

同期丘脑和苍白球切开术治疗合并原发性震颤的帕金森病

目的：本文旨在研究合并原发性城颤（ET）的帕金森病手术治疗的最佳术式,评估同期丘脑和苍白球切开术的手术必要性、临床治疗效果。安全性及其适应证。方法：应用微电极导向立体定向技术,对16例合并ET的PD患者实施了同期单侧丘脑和苍白球切开术,用UPDRS方法对所有患者进行术前和术后的病情评分。结果：所有16例患者症状均有明显改善。手术对侧和躯干尤为明显,手术对侧各种形式的震颤在“开、关”两种形态下均完全消失,改善率为100%,对震颤的治疗效果优于单纯苍白球切开术,无严重并发症发生,随访结果表明,患者的自理能力明显提高,UPDRS各项评估指标稳定,反映了持续改善的手术效果。结论：同期丘脑和苍白球切开术治疗合并ET的PD患者是一种安全而有效的治疗方案,适应证的合理选择和微电极导向技术的应用是手术成功的关键。     

Rest tremor suppression may separate essential from parkinsonian rest tremor

Rest tremor at 4–6 Hz is typical for classical rest tremor (PT) of Parkinson's disease (PD). But rest tremor also appears in other tremor syndromes and may therefore cause a misdiagnosis. In this study we evaluated if suppression of tremor during movement onset is a characteristic feature of Parkinsonian Tremor distinguishing PT from Essential tremor (ET) and if this sign can be reliably diagnosed.Clinically diagnosed patients with PT (n = 44) and ET (n = 22) with rest tremor were included. Video sequences were recorded according to a standardized protocol focusing on the change of tremor amplitude during transition from rest to posture (test 1) or to a target-directed movement (test 2). These videos were assessed for rest tremor suppression by 4 reviewers (2 specialists and 2 residents) blinded to the clinical diagnosis and were compared to the personal assessment of an unblinded movement disorder specialist.Rest tremor suppression was found in 39/44 PD patients and in 2/22 patients with ET during the personal assessment. Rest tremor suppression showed a high sensitivity (0.92–1.00) and an acceptable specificity (0.69–0.95) for PD tremor in both tests. The interrater-reliability of the video-sequences was good to very good (κ 0.73–0.91). Less than 3% of the video sequences were misclassified.We conclude that the assessment of the suppression of rest tremor during movement initiation is a simple and reliable tool to separate PT from rest tremor in ET also suggesting that the mechanisms of rest tremor in these two diseases are different.     

特发性震颤的临床特点分析

目的探讨特发性震颤(essential tremor,ET)的临床特点。方法对92例ET患者的临床资料进行回顾性分析。结果92例ET中男58例,女34例,发病年龄12～80岁,平均(50.2±17.3)岁,病程6个月～60年,平均(16.2±8.9)年。49例(53.3%)患者有阳性家族史,多呈常染色体显性遗传,临床主要表现为单症状的姿势性震颤,累及部位依次为手(92.4%)、头(25.0%)、咽喉部(21.7%)、下颏(15.2%)等。16.3%患者因震颤致日常生活困难。42例饮酒患者中,85.7%显示对酒精有反应性。6.5%患者并发帕金森病(Parkinson’s disease,PD)。67.3%患者小剂量普萘洛尔治疗有效。结论本组ET患者男多于女,临床表现为单症状姿势性震颤,部分病例可伴发PD,小剂量普萘洛尔治疗大多有效。     

The response of the central and peripheral tremor component to octanoic acid in patients with essential tremor

Objective

To investigate the effect of octanoic acid (OA) on the peripheral component of tremor, as well as OA’s differential effects on the central and peripheral tremor component in essential tremor (ET) patients.

Primidone and essential tremor

Summary To clarify whether primidone itself, and not only its metabolite phenobarbitone, suppresses essential tremor, the effect of a high single dose of primidone was tested. Of 11 patients, 8 showed a reduction of their tremor by 54%–69% for up to 28h. The serum concentration of primidone was as expected, whereas those of the metabolites phenyl-ethylmalonamide and phenobarbitone were very low. The tremor suppression can thus be considered to be an effect of primidone. Three of the 11 patients did not show a reduction of tremor.     

Topography of essential tremor

IntroductionTopography of tremor manifestations is poorly investigated in essential tremor. The present study explores the prevalence and clinical correlates of head and/or voice tremor in essential tremor.MethodsOut of a prospectively designed registry of 972 patients, 884 patients with definite and probable essential tremor had complete information on tremor localization. Demographic and clinical characteristics were compared among four subgroups: group A (without head or voice tremor, n = 619), B (with head but without voice tremor, n = 155), C (with voice but without head tremor, n = 47), and D (with both head and voice tremor, n = 63).ResultsIn our patients, total prevalence of tremor was 24.7% for head, 12.4% for voice and 7.1% for the combination of head and voice. Logistic regression analyses showed that female gender is strongly associated with head tremor, which was confirmed by an additional meta-analysis. Severe hand tremor was the only factor associated with voice tremor. Both female gender and severe hand tremor increase the odds for having the combination of head and voice tremor. For males, hand tremor severity is significantly increased among those with head and voice tremor alone and in combination, but for females only for the combination. Patients with both head and voice tremor have more frequent involvement of legs and other localizations and are less responsive to β-blockers.ConclusionsFemale gender and severe hand tremor may increase the odds of head and/or voice tremor in essential tremor. The association of hand tremor severity with midline tremor is stronger for males than females.     

Olfaction in essential tremor patients with and without isolated rest tremor.

An olfactory deficit is present in patients with essential tremor (ET), but it is often milder than that in patients with Parkinson's disease (PD). In both, the deficit occurs early in the disease. Isolated rest tremor without other signs of parkinsonism can occur in patients with ET. If the rest tremor in these patients represents a manifestation of ET rather than early PD, we hypothesized that their University of Pennsylvania Smell Identification Test (UPSIT) scores would be similar to those of ET patients without rest tremor. The mean UPSIT score in 13 ET patients with isolated rest tremor did not differ from that of 58 ET patients without rest tremor (29.3 +/- 4.3 vs. 29.4 +/- 6.4; P = 0.69). Several ET patients with rest tremor had UPSIT scores that fell outside of the range that is seen in 95% of patients with PD. These data raise the possibility that some ET patients with isolated rest tremor may not have early PD and that the pathological process that is responsible for their ET is also involving the basal ganglia.     

Triggering essential head tremor with sustained phonation: A clinical phenomenon with potential diagnostic value

Here we report a clinical phenomenon that we have observed repeatedly in clinical research settings; namely, a triggering and/or exacerbation of head tremor during or immediately following sustained phonation. To our knowledge, it has not been reported previously nor has it been the subject of study. Here we: 1) report the phenomenon, 2) provide several visual illustrations, 3) estimate its prevalence, and 4) analyze its clinical correlates. Head tremor was assessed qualitatively, and scores were assigned pre-, during, and post-sustained phonation using the Tremor Research Group Essential Tremor Rating Assessment Scale. Seventy (68.6%) of 102 essential tremor (ET) patients exhibited a qualitative increase in head tremor amplitude during and/or immediately after sustained phonation; in 5 (4.9%), head tremor would not have been detected without the voice activation maneuver (i.e., it was not visible at any other point in the videotape aside from the period during/immediately following sustained phonation). Women were more likely than men to exhibit this phenomenon (p = 0.05), whereas age, age at onset, duration of tremor, and total tremor score did not predict responsiveness of head tremor to sustained phonation. Sustained voice activation is a useful examination maneuver that may elicit or amplify head tremor in ET. Head tremor is not reported to occur in patients with enhanced physiological tremor. Thus, this maneuver, by triggering head tremor, may be a useful diagnostic supplement, particularly in research/clinical settings where arm tremor is mild and the diagnosis (mild ET vs. enhanced physiological tremor) would otherwise be ambiguous.     

特发性震颤研究进展

特发性震颤除表现为震颤外,还可以出现共济失调等运动症状以及认知功能障碍、情感障碍和听力下降等非运动症状。发病机制尚不明确,小脑、脑干、红核、丘脑和基底神经核均受累,致病基因的克隆为发病机制的研究奠定基础。治疗方法主要包括药物治疗和外科手术,其中,表现为头部、手部和声音震颤的患者可肌肉注射A型肉毒毒素,外科手术包括丘脑毁损术和脑深部电刺激术等。