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。     

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...     

吸烟、饮酒对中老年原发性震颤发病的纵向影响

目的 分析吸烟、饮酒等生活习惯对原发性震颤(essential tremor,ET)发病的影响.方法 研究人群为北京老龄纵向研究队列,该队列为1992年在北京市3个社区随即抽取的3257名55岁以上的中老年人群,2000年随访进行横断面调查,采用筛查和临床诊断两步法确诊患者.采用回顾性前瞻设计的方法,分析1992年确认未患病研究对象当时的吸烟、饮酒等暴露情况在2000年随访时诊断为ET患者的相关关系.结果 2000年共诊断出1992年调查时无症状的86例新发ET患者.吸烟人群在ET和非ET组所占比例分别为34.9%、33.3%.不同吸烟情况人群在两组所占比例无统计学差异(X2=0.412,P:0.814).饮酒人群在ET和非ET组所占比例分别为16.3%、24.8%.无统计学差异(X2=2.675,P=0.102).男性ET饮酒比例为23.6%.明显低于对照组(38.2%),差异有统计学意义(X2=3.860,P=0.049,OR=0.50,95%CI:0.23～1.06).结论 吸烟对ET发病没有显著性影响,饮酒对男性ET可能具有一定的保护作用.     

特发性震颤的临床和电生理学特点

目的 探讨特发性震颤(EI)的临床和电生理学特点.方法 回顾性分析并比较33例ET患者(ET组)和30例生理性震颤患者(对照组)的震颤类型、程度、幅度和负重对其的影响,以总结ET的临床和电生理学特点.结果 ET组患者动作性震颤(KT)的震颤程度明显高于对照组,震颤幅度明显大于对照组(均P＜0.01);而姿势性震颤(PT...     

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     

帕金森病和原发性震颤嗅觉障碍研究

目的检测帕金森病（PD ）、原发性震颤（E T ）、原发性震颤并帕金森病（E T-PD ）患者的嗅觉功能,探讨嗅觉障碍在PD诊断及鉴别诊断中的意义,同时探讨 PD与 ET 的关系。方法采用国际通用的英国脑库 PD诊断标准、Bain等（国际震颤研究小组）提出的 ET 诊断标准及Geraghty、Jankovic提出的国际公认的ET-PD诊断标准,收集早期PD 30例,ET 30例,ET-PD 30例,分别进行简易嗅觉检查,并随机选择3组年龄、性别、文化程度分别与PD、ET、ET-PD患者相匹配的各30例健康人作对照。结果 PD、ET-PD患者嗅觉障碍率远高于对照组及ET组,差异有统计学意义（P＜0.05）。ET组与对照组相比差异无统计学意义（P＞0.05）。结论早期原发性帕金森病及E T-PD患者存在明显嗅觉障碍,嗅觉检测可作为 PD早期诊断及鉴别诊断的参考指标,PD与E T 可能是两种不同性质的疾病,合并嗅觉障碍的ET患者更易进展为PD。     

多巴胺转运体显像对特发性震颤与早期帕金森病的鉴别诊断价值

目的探讨多巴胺转运体(DAT)显像对特发性震颤(ET)、早期帕金森病(PD)鉴别诊断的价值.方法以99mTc-2β-[N,N'-双(2-巯乙基)乙撑二胺基]甲基-3β-(4-氯苯基)托烷(99mTc-TRODAT-1)为显像剂,用单光子发射计算机断层扫描(SPECT)对8例姿势性震颤(PT)患者,5例姿势性震颤伴静止性震颤(PT+RT)患者、12例早期PD(H-Y I级)患者进行DAT显像.结果与PT组相比,PT+RT组两侧纹状体(ST)与枕叶(OC)的ST/OC均值略高,但无显著性差异;早期PD组ST/OC均值显著性降低.PT+RT组两侧ST/OC均值明显高于早期PD组患肢对侧,但与其同侧无显著性差异.结论PT伴RT的ET患者存在轻度多巴胺神经元的缺失,99mTc-TRODAT-1 SPECT DAT显像对ET和早期PD有鉴别诊断的价值.     

心率变异性对原发性震颤和早期震颤型帕金森病的诊断价值

目的研究心率变异性(HRV)对原发性震颤(ET)和早期震颤型帕金森病(PD)的诊断价值。方法对26例ET患者(ET组)、30例早期震颤型PD患者(早期震颤型PD组)和23例健康对照者(健康对照组)进行24 h ECG检查,比较HRV指标的差异。结果与健康对照组比较,早期震颤型PD组全程NN间期的标准差(SDNN)、相邻NN之差的均方根(RMSSD)、低频功率(LF)和高频功率(HF)显著降低(均P0.01),LF/HF差异无统计学意义(P0.05);ET组RMSSD显著降低(P0.05),SDNN、LF、HF和LF/HF差异无统计学意义(均P0.05)。与早期震颤型PD组比较,ET组SDNN、LF和HF均显著升高(均P0.01),RMSSD、LF/HF差异无统计学意义(均P0.05)。结论震颤型PD患者存在交感和迷走神经的双重损害,早期几乎所有的HRV参数均显著降低。HRV可能有助于区分震颤型PD及ET。     

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。方法回顾性分析98例ET患者的临床资料。结果98例ET中男56例,女42例。发病年龄6～72岁,平均43．08±18．18岁。病程1～48年,平均14．04±11．39年。48例（48．98％）患者有阳性家族史。临床主要表现为单症状的姿势性震颤,累及部位依次为手98例（100％）、头38例（38．78％）、下肢28例（28．57％）、咽喉部16例（16．33％）、下颏10例（10．20％）等。64例患者做了饮酒试验,其中58例（90．63％）有酒精反应。84．62％的患者服用盐酸阿罗洛尔治疗有效。结论特发性震颤的临床表现以单症状姿势性震颤为主,手及头部受累明显,多数患者对酒精有反应,盐酸阿罗洛尔治疗有效。     

临床不同分期帕金森病患者震颤特点分析

目的研究不同临床分期帕金森病(PD)患者震颤的特点。方法收集2014-11—2015-05首都医科大学附属北京天坛医院神内病房临床确诊或临床诊断PD可能性大的75例患者。患者至少具有一侧上肢静止性或姿势性震颤,按照Hoehn-Yahr分期分为1~1.5期、2~2.5期、3期3组,分别检测各组患者静止、姿势及持物1000g震颤的优势频率、振幅及震颤节律形式。结果 (1)震颤优势频率:静止、姿势状态下为4~7 Hz,1~1.5期与2~2.5期、3期组间比较差异无统计学意义(P0.05)。持物1000g状态下,1~1.5期与2~2.5期、3期组间比较差异有统计学意义(P0.01),1~1.5期患者优势频率除了4~7Hz之外,还有7~10Hz、无规律及无震颤。(2)震颤振幅:随病情进展有下降趋势。(3)震颤节律形式:临床不同分期患者静止、姿势及持物1000g时震颤的节律形式均以交替形式为主,但随着病情进展,非交替节律比例有增加趋势。结论 PD患者震颤属于中枢性震颤,静止、姿势震颤优势频率为4~7Hz,不随病情进展而变化。疾病初期,持物1000g状态震颤优势频率可能受外周调节而表现多样。震颤节律不受病情进展及姿势影响。随病情进展,非交替节律比例有增加趋势,震颤振幅有下降趋势。     

Rest tremor in patients with essential tremor: prevalence,clinical correlates,and electrophysiologic characteristics

BACKGROUND: Isolated rest tremor, which is observed in some patients with essential tremor (ET), poses a diagnostic challenge. The phenomenon has been examined in few studies and is poorly understood. OBJECTIVES: To determine the prevalence and study the clinical correlates of rest tremor in ET and to examine the electrophysiologic features in a subgroup of patients. METHODS: Sixty-four patients with ET cared for at a tertiary referral center underwent neurologic examination. Five of 12 patients with rest tremor also underwent quantitative computerized tremor analysis using accelerometry and handwritten spiral analysis. RESULTS: Twelve of 64 patients with ET had rest tremor (prevalence, 18.8%; 95% confidence interval, 9.2%-28.4%). Compared with the 52 patients with ET without rest tremor, these 12 had disease of longer duration and greater severity. Also, their ET was more widely disseminated, as evidenced by a larger proportion with head tremor. None had clinical signs of bradykinesia or rigidity. The 5 patients with rest tremor who underwent electrophysiologic study had electrophysiologic features consistent with parkinsonism (eg, slow spiral speed and increased decrement of spiral speed with radius). CONCLUSIONS: In our sample, 1 in 5 patients with ET had rest tremor. The tremor was associated with disease that was more severe, more disseminated, and of longer duration. Some of these patients had electrophysiologic features consistent with parkinsonism. The basis for the rest tremor could be basal ganglia involvement, raising the possibility that the pathologic process responsible for ET may extend to these structures.     

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.     

Jaw tremor: prevalence and clinical correlates in three essential tremor case samples.

The spectrum of involuntary movements seen in essential tremor (ET) is limited. Jaw tremor is one such movement. The prevalence and clinical correlates of jaw tremor have not been studied in detail. The objective of this study was to estimate the prevalence and examine the clinical correlates of jaw tremor in ET using ET cases from three distinct settings (population, tertiary-referral center, brain repository). All ET cases underwent a videotaped tremor examination in which tremors (including limb, head, voice, and jaw) were assessed. The prevalence [95% confidence interval (CI)] of jaw tremor was lowest in the population sample (7.5%; 3.9%-14.2%), intermediate in the tertiary-referral center (10.1%; 6.8%-14.7%), and highest in the brain repository (18.0%; 12.3%-25.5%; P = 0.03). Jaw tremor was associated with older age (P < 0.001), more severe action tremor of the arms (P < 0.001), and presence of head and voice tremor (P < 0.001). Jaw tremor was present in 4/14 (28.6%) ET cases with consistent rest tremor vs. 15/193 (7.8%) cases without rest tremor (odds ratio = 4.8; 95% CI = 1.3-7.0; P = 0.009). The prevalence of jaw tremor was 7.5% to 18.0% and was dependent on the mode of ascertainment, being least prevalent in a population-based sample. ET cases with jaw tremor had a more clinically severe and more topographically widespread disorder. The association in our study between jaw tremor and rest tremor, along with the published observation that jaw tremor can occur in Parkinson's disease (PD), raises the question whether jaw tremor in ET is a marker for subsequent conversion to PD.     

Factors associated with increased risk of head tremor in essential tremor: a community-based study in northern Manhattan.

Head tremor is one of the major expressions of essential tremor (ET). It is not well understood why some patients develop head tremor, whereas others do not. A study of the characteristics of patients with head tremor has not been undertaken. Our goal was to estimate the prevalence of head tremor and to identify demographic and clinical characteristics associated with an increased risk of head tremor in ET. Cases were ascertained from a community-based study of ET in northern Manhattan, New York. Arm tremor severity was rated with a total tremor score. Logistic regression analyses resulted in odds ratios (OR). Head tremor was present in 37 (34.9%) of 106 ET cases. Female gender was associated with a fourfold increased risk of head tremor (OR = 3.73; P = 0.005). Total tremor score was divided into quartiles; individuals in the lowest or highest quartile were four times more likely to have head tremor (OR = 4.16; P = 0.001). Individuals with both risk factors (female gender and lowest or highest total tremor score quartile) were 16 times more likely to have head tremor (OR = 15.88; P = 0.0006). Being related to a proband with head tremor marginally increased the risk of head tremor (OR = 11.30; P = 0.08). Age and tremor duration did not influence the risk of head tremor. We identified several factors that were associated with an increased risk of head tremor in ET; female gender, coexisting arm tremor that was either very mild or extremely severe, and relation to an ET case with head tremor. These disease associations require further exploration, and might provide insight into the mechanisms underlying head tremor.     

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.     

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.     

Increased risk of head tremor in women with essential tremor: longitudinal data from the Rochester Epidemiology Project.

In one cross-sectional study of a community in northern Manhattan, women with essential tremor (ET) were more likely to have head tremor than were men. In that study, patients were seen at one point in time, rather than followed longitudinally. Head tremor often develops after arm tremor, and its appearance in patients with ET may therefore be a function of duration of follow-up. In a second epidemiological study utilizing the Rochester Epidemiology Project, in which ET subjects were followed from disease diagnosis to death, we determined whether there was an association between female gender and head tremor. We utilized the records-linkage system of the Rochester Epidemiology Project to identify ET cases. Records were reviewed and clinical data abstracted by a neurologist specializing in movement disorders. A second neurologist reviewed a subsample of records. There were 107 ET cases (69 women, 38 men) followed for 10.1 +/- 9.1 years from ET diagnosis to death. Head tremor was present in 37 (53.6%) women and 5 (13.2%) men (odds ratio [OR] = 7.6, 95% confidence interval [CI] = 2.7-21.9, P < 0.001). In a multivariate linear regression analysis, women remained at high risk for head tremor (OR = 6.5, 95% CI = 2.2-19.0, P = 0.001) independent of disease duration. We found in this longitudinal epidemiological study that women with ET were six times more likely to develop head tremor over the course of their illness than were men. The reason for the association between gender and head tremor, which has now been demonstrated in several studies, is not known, but it could reflect gender differences in the distribution of disease pathology within the brain.     

Effects of zonisamide on isolated head tremor

Background and purpose: The medical treatment available for patients with essential tremor (ET) is often inadequate. Furthermore, the efficacy of the medical treatments currently available for patients with ET of cranial nerve areas is less satisfactory than that of the medical treatments available for patients with ET involving the upper extremities. This pilot study was performed to evaluate whether zonisamide (ZNS) is effective in the treatment of patients with isolated head tremor. Methods: All subjects with isolated head tremor were randomly treated with either ZNS or propranolol. After a washout period, the subjects were switched to the alternative drug. Results: ZNS was found to be more effective in the treatment of patients with isolated head tremor than propranolol. No severe adverse effects were reported with either ZNS or propranolol. Conclusion: ZNS may be more useful than propranolol for the treatment of ET patients with head tremor.     

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