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。     

特发性震颤伴发帕金森病17例

特发性震颤 (essentialtremor,ET)与帕金森病(Parkinsondisease ,PD)是两种常见的锥外系统疾病 ,但ET合并PD (ET PD)很少见 ,我们用A型肉毒毒素(BTX A)局部注射治疗难治性ET PD取得了较好的疗效 ,国内尚未见类似报道 ,目前 ,对于ET与PD的相互关系仍有争论 ,本文报道我科诊治的 17例患者。临床资料本组 17例患者中 ,男 11例 ,女 6例 ,ET发病平均年龄 (4 5 5± 4 3)岁 ,PD发病平均年龄为 (5 0 3± 3 7)岁 ,两者相比无差异 (P >0 0 5 )。另有 3例患者 ,就诊时已有ET及PD的症状 ,不能确定其发病时间 ,被排除在本组外。ET患者…     

原发性震颤与特发性帕金森病言语功能障碍的研究

原发性震颤 (ET)与特发性帕金森病 (PD)均为常见的锥体外系疾病。二者均存在高比率的言语功能障碍 ,目前国内外尚未见二者比较研究的报道。现就我院近 4年来神经内科所见 80例 ET与 91例 PD患者的言语功能进行了分析 ,报道如下。1　临床资料1.1　一般资料　本组均为本院 1997年 1月～ 2 0 0 0年 4月神经内科门诊和少数住院的 ET与 PD患者。ET组 80例 ,男5 0例 ,女 30例 ,年龄 11～ 79岁 ,平均 47.7± 17.7岁。诊断采用国际震颤研究小组提出的标准 [1 ] 。 PD组 91例 ,男 5 3例 ,女 38例 ,年龄 31～ 77岁 ,平均 5 6 .7± 11.4岁。 PD…     

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...     

多系统萎缩与帕金森病的早期鉴别诊断

目的　为多系统萎缩 (m ultiple system atrophy,MSA)与帕金森病 (Parkinson disease,PD)的鉴别诊断提供依据。方法　对 12名首发锥体外系症状的 MSA患者和 40名 PD患者的临床症状及主要辅助检查行比较分析。MSA诊断依据 Quinn(1994)提出的临床诊断标准。PD诊断参考英国帕金森病协会 1997年提出的诊断标准。结果　与 PD患者比较 ,MSA患者发病年龄较小 (5 4.3 2± 9.5 0 vs 61.5 6± 9.5 5 ,P<0 .0 5 ) ,主要以行动困难或僵硬为首发症状 (66.7% vs 2 5 % ,P<0 .0 5 ) ;少数以震颤为首发症状 (3 3 .3 % vs 75 % ,P<0 .0 5 ) ,也可以单侧症状发病(5 8.3 % vs 80 % ,P>0 .0 5 ) ,多数对多巴胺反应不良 (66.7% vs 2 0 % ,P<0 .0 5 ) ,植物神经症状、语言障碍比 PD患者显著多见 (分别为 83 .3 % vs 2 5 % ;5 0 % vs12 .5 % ;P<0 .0 5 )。辅助检查 :MRI:大部分 MSA患者有阳性表现 ,橄榄体、脑桥和小脑部位有萎缩 ,PD患者未发现特征性病变。 PET:3例 MSA患者均呈阳性表现。神经心理 :MSA患者未出现智能障碍的情况 (0 % ) ,少数 PD(10 % )患者出现智能障碍 ,但两者比较无统计学意义 (P>0 .0 5 )。结论　依据详细的病史和全面的神经系统查体 ,并结合临床辅助检查 ,可提高 MSA和 PD早期鉴别诊断的准确性     

特发性震颤的临床特点分析

目的 分析特发性震颤(essential tremor,ET)的临床特点。方法 对1997～2000年收治的42例特发性震颤患者发病年龄、性别、病程、震颤部位、震颤扩展方式、家族史、饮酒及药物反应等方面进行临床分析。结果 男女患者之比为28:1,平均发病年龄为(48.7±7.4)岁,呈单峰模式,病程(8.6±6.5)年。61.90％(26/42)的患者有家族史。42例患者中单纯姿势性震颤者30例(71.43％),运动性震颤者4例(9.52％),姿势及运动混合性震颤者2例(4.76％)。累及部位依次为双上肢者26例(61.90％),头部12例(28.57％),单侧上肢10例(23.81％),下颌2例(4.76％),舌2例(4.76％),发声肌1例(2.38％),躯干1例(2.38％)。36例参加饮酒试验者,29例(80.56％)有酒精反应;32例接受普萘洛尔治疗,完成治疗试验者28例(87.50％),服药后患者临床症状明显改善,与用药前比较差异有显著性意义(P<0.01)。结论 特发性震颤的临床表现以单症状姿势性震颤为主,上肢及头部受累明显,多数患者对酒精有反应,普萘洛尔治疗有效。     

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.     

氯氮平治疗帕金森病患者中左旋多巴诱导的异动症

目的 :探讨氯氮平治疗帕金森病 (PD)患者中左旋多巴诱导的异动症 (LID)的疗效、合适剂量、不良反应。方法 :对 12例出现LID的PD患者添加氯氮平治疗 ,治疗前后进行UPDRS的异动症评分、运动功能评分和血白细胞的计数。结果 :氯氮平 (5 0± 5 1)mg显著改善了 91 7% (11/12 )PD患者的异动症状 ,治疗前异动症评分为 3 4± 0 7,治疗后为 1 6± 1 3 ,差异显著 (P <0 0 1)。治疗过程中无患者出现运动功能的恶化和血白细胞的减少。结论 :小剂量 (5 0mg)氯氮平能有效改善PD患者的LID ,安全性好     

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。     

原发性震颤parkin基因缺失突变的初步研究

目的　探讨原发性震颤(ET)患者parkin基因第3～7外显子是否存在缺失突变,及其与该病临 床特点的关系。方法　采集56例发病年龄<50岁的ET患者外周血液,提取基因组DNA,通过聚合酶链反应 (PCR)扩增、琼脂糖凝胶电泳鉴定是否有parkin基因第3～7外显子缺失突变,并结合ET患者临床资料进行 分析。结果　本组ET患者临床表现为单一症状的姿势性震颤,但均未发现parkin基因第3～7外显子缺失。 结论　ET的发生及姿势性震颤可能与parkin基因第3～7外显子缺失突变无关。     

Is essential tremor predominantly a kinetic or a postural tremor? A clinical and electrophysiological study

Both postural and kinetic tremors may occur in essential tremor (ET), however the relative contribution of each is not clear. ET has been variably defined with respect to kinetic and postural tremors. To examine the relative severity of postural and kinetic tremors in ET, 50 ET cases from a clinic and 55 from a community underwent a videotaped tremor examination. Kinetic and postural tremors were rated using a validated clinical rating scale (score range, 0-3). Thirty-one cases also underwent accelerometry to precisely quantify tremor amplitude. In clinic cases, the mean postural tremor rating was 1.25 (S.D., 0.89). The mean kinetic tremor rating was 52% higher (1.90; S.D., 0.57; P < 0.001). The community cases had similar characteristics. Sixty percent of the 105 cases had postural tremor ratings scoring 0 or 1 (no tremor or low amplitude, intermittent tremor). In clinic cases, the mean amplitude of postural tremor during tremor analysis was 0.51 mm (S.D., 0.66 mm), and the mean amplitude of kinetic tremor was 2.91 mm (S.D., 2.11 mm; P < 0.01). Similar values were obtained for community cases. These quantitative data suggest that kinetic tremor is more severe than postural tremor in ET. The majority of cases had mild or absent postural tremor. Despite this, ET is defined only as a postural tremor in many studies. Our data argue for a more consistent inclusion of kinetic tremor in diagnostic criteria for ET.     

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。     

Effect of alcohol on tremors: comparison with propranolol

Intravenous administration of alcohol decreased postural essential tremor but not parkinsonian resting or cerebellar intention tremor. The response to alcohol infusion occurred in all 15 patients studied, whereas only 11 of the 15 had a response to propranolol therapy. Furthermore, the response to ethanol was greater than that due to propranolol.     

Long duration asymmetrical postural tremor is likely to predict development of Parkinson's disease and not essential tremor: clinical follow up study of 13 cases

BACKGROUND: Patients presenting with asymmetrical postural tremor with or without mild rest tremor may be diagnosed as having essential tremor (ET), although there is considerable diagnostic uncertainty as to the long term outcome of these patients. OBJECTIVE: In this study, retrospective observations were made on 13 patients presenting originally with asymmetrical postural tremor, initially thought to have ET based on tremor characteristics, alcohol responsiveness, and family history but who subsequently met the criteria of Parkinson's disease (PD). METHODS: The patients were observed and followed up clinically with ancillary imaging using dopamine transporter SPECT scan or levodopa challenge tests in some cases. The diagnosis at original presentation with postural tremor was made with retrospective case note review. RESULTS: After a variable and long latent period all patients developed additional signs suggesting a clinical diagnosis of PD although picking up an initial label of ET. CONCLUSIONS: We suggest exercising caution regarding a diagnosis of ET in patients presenting with late onset asymmetrical postural tremor even if there is no rest tremor. Alcohol sensitivity of tremor, family history of tremor, or responsiveness to beta blockers may not be helpful in diagnosing ET in these cases and some may develop PD in the long term.     

Clinical response to long action propranolol in 40 patients diagnosed with essential tremor with no previous treatment: an open, non-controlled study

Essential tremor (ET) is the most common movement disorder and betablockers are still considered the first line of treatment. The aim of our study is to report the clinical response to long action propranolol (LAP) of 40 patients diagnosed with essential tremor with no previous treatment. METHOD: 40 patients with ET were evaluated with rating scales for severity of tremor and clinical classification of ET. All patients were evaluated at least twice, at enrollment and one month after starting treatment. RESULTS: thirty-six patients (90%) had type 2 ET and types 3 and 4 occurred in two patients each (10%). Familiar history was positive in 25 patients (62.5%). Mean age at first evaluation was 43.1 years and mean age at onset was 27.4 years. Of all patients, 33 (82.5%) had some degree of benefit and in 52.5 % this benefit was either good or excellent. CONCLUSION: LAP seems to be a good treatment option for ET in our series of 40 patients.     

Essential tremor in childhood: a series of nineteen cases.

One in 20 essential tremor (ET) cases arises during childhood. We report 19 pediatric ET cases (mean age = 12.7 years). The majority (68.4%) were male, and only one had head tremor. Childhood and adult forms of ET may differ in several important respects, providing information about the underlying biology of ET. A possible male preponderance in childhood ET cases could reflect a modification of disease expression by gender, such that males manifest the disease at an earlier age than females. A paucity of childhood cases with head tremor suggests that the neuropathological changes in ET may evolve somatotopically. Head tremor may require midline or more extensive bilateral pathology which may only occur later in the disease.     

Different clinical features of essential tremor: a 200-patient study

Two hundred consecutive patients affected by essential tremor (ET), referred to the Movement Disorders Center of the University of Bologna between January 1977 and December 1981, were studied to identify the different clinical features of this disorder, and its associated neurological signs. In this series, 169 patients suffered from typical essential tremor (TET); in the other 31 cases uncommon clinical features were detected: 25 presented postural, action and rest tremor (continuous tremor: CT); 5 had orthostatic tremor (OT) and 1 was affected by writing tremor (WT). Buccolinguofacial dyskinesias (BLFD) were associated with ET in 19 cases; 2 presented dystonia, and in 6 mild extrapyramidal signs were detected. Uncommon types of tremor: ET plus BLFD and ET associated with extrapyramidal signs had an higher incidence in older people. No patient developed Parkinson's disease (PD) during the follow-up period (2-5 years).     

Isolated tremor and disruption of the nigrostriatal dopaminergic system: an 18F-dopa PET study.

We measured striatal 18F-dopa influx constants (Ki) for 20 patients with isolated, predominantly postural, tremor (eight familial, 12 sporadic) and 11 with predominantly rest tremor. Results were compared with 30 controls and 16 Parkinson's disease (PD) patients. The eight familial essential tremor (ET) patients had normal striatal 18F-dopa uptake. Two of the 12 sporadic postural tremor patients had subnormal putamen 18F-dopa Ki, one (who later became akinetic) falling in the PD range. The mean putamen 18F-dopa uptake of the 11 rest tremor patients was reduced to PD levels (51% of normal). Our findings argue against an association between ET and PD, but support the existence of a "benign" tremulous variant of PD. The presence of low-amplitude rest tremor, cogwheel rigidity, reduced arm swing, and short tremor duration was not a useful predictor of nigral dysfunction in patients with postural tremor. In contrast, patients with predominantly rest tremor, particularly with onset in the leg, consistently showed reduced putamen 18F-dopa uptake.     

Routine neurophysiologic tremor analysis as a diagnostic tool for essential tremor: a prospective study.

The objective of the current study was to investigate the diagnostic potential of the routine tremor neurophysiologic analysis for the diagnosis of essential tremor (ET) in patients with postural tremor syndrome. Three hundred consecutive outpatients attending for postural tremor were included. Accelerometry and surface electromyography was performed between 1 and 4 weeks after the first clinical visit. A final clinical diagnosis (mean follow-up period, 53 months) of the postural tremor syndrome was made by two neurologists blind to the neurophysiologic results. Six neurophysiologic criteria for the diagnosis of ET were applied to define the neurophysiologic examination as compatible or not compatible with ET (all criteria must be present): (1) rhythmic burst of postural tremor on EMG; (2) tremor frequency greater than or equal to 4 Hz; (3) absence of rest tremor or, if present, frequency 1.5 Hz lower than the postural tremor; (4) absence of tremor latency from rest to postural position; (5) changes of the dominant frequency peak less or equal to 1 Hz after the weight load test; and (6) no changes in tremor amplitude after mental concentration. The neurophysiologic criteria for ET showed a sensitivity of 97.7%, a specificity of 82.3%, a positive predictive value of 95.1%, a negative predictive value of 91.1%, and positive probability ratio of 5.5. The odds ratio was 198.43 (95% CI, 66.03 to 596.35). For the diagnosis of ET in patients with postural tremor, routine neurophysiologic tests have high diagnostic and predictive value that justifies its practice in movement disorders clinics.     

Relative efficacy of alcohol and propranolol in action tremor.

Thirty-nine patients with a variety of diseases, including essential tremor, Parkinson's Disease, olivopontocerebellar degeneration, ataxia telangiectasia, and cervical cord injury with action tremor, were evaluated for the effect of one ounce of absolute alcohol ingestion. Tremor significantly subsided in 61.9% of E.T.; 46.6% of P.D.; one patient with A.T.; and one patient with C6 lesion. The tremor became worse in one patient with O.P.C.D. Twenty of these patients were treated with propranolol, an average dose of 92 mgm. per day, and re-evaluated three to six months later. All those who improved on alcohol improved on propranolol and the one whose tremor accentuated with alcohol had a similar response to propranolol. It is concluded that the tremorilytic effect of alcohol is neither specific for, nor limited to, essential tremor and is of no value in differentiating various neurological disorders which manifest as action tremor. It is recommended that one ounce of absolute alcohol by mouth be used as an office procedure to predict the response of patients' tremor to propranolol.