特发性震颤非运动症状研究进展

特发性震颤是以姿势性或动作性震颤为主要表现的运动障碍性疾病,也是临床最为常见的神经系统疾病之一。传统观点认为,特发性震颤是一种单一症状的良性运动障碍性疾病,近年来,相关研究表明其亦可表现出非运动症状。对特发性震颤非运动症状的研究和探索,为临床诊断与治疗以及疾病机制的研究提供了新视角。     

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。     

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。     

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。     

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.     

特发性震颤非运动症状的相关病理学研究进展

特发性震颤(ET)是一种临床表型多样、病因复杂的慢性神经系统综合征,以姿势性或动作性震颤为主要表现.传统的观念认为ET是一种单一症状的良性运动障碍性疾病,无特异性病理学改变.近年的研究提示小脑-丘脑-皮质环路可能参与ET的发病.除典型的震颤外,特发性震颤亦可出现非运动症状,这些非运动症状给患者生活质量造成不良影响,但常...     

原发性震颤患者伴发抑郁的发生率及相关因素

目的 研究原发性震颤(ET)患者抑郁的发生率及相关的因素.方法 应用汉密尔顿抑郁量表(HAMD)对62例ET患者和60名健康体检者进行抑郁评定,以及对ET患者进行震颤评定量表(Fahn-Tolosa-Matin Tremor Rating Scale,TBS)评分、匹兹堡睡眠质量指数(PSQI)量表评分.结果 62例ET患者中,有33例(53.2%)伴发抑郁,其中轻度抑郁22例(35.5%),中度抑郁11例(17.7%);60名对照组中7名(11.7%)伴发抑郁,其中轻度抑郁5名(8.3%),中度抑郁2名(3.3%),两组相比较差异具有统计学意义(X2=23.898、13.043、6.649,均P<0.01).ET患者抑郁组与非抑郁组HAMD各因子比较显示两组焦虑/躯体化、认知障碍、阻滞、睡眠障碍及绝望感差异均有统计学意义;抑郁组与非抑郁组PSQI总分及各因子比较显示PSQI总分、主观睡眠质量(F1)、入睡时间(F2)、睡眠干扰(F5)差异均有统计学意义.抑郁与ET症状的严重程度、睡眠质量、性别相关(β=0.589,P=0.000;β=0.469,P=0.000;β=0.256,P=0.027).结论 ET患者中抑郁有较高发生率,主要表现为情绪低落、工作和兴趣减退、忧虑、睡眠障碍、迟缓、自卑感等,抑郁程度与ET症状的严重程度、睡眠质量、性别相关.     

Characteristics of depressive symptoms in essential tremor

Depressive symptoms are common in essential tremor (ET) and may be a primary feature of the underlying disease. However, it is still unclear whether depression in ET and depression in primary affective disorders share common clinical manifestations. Sixty-one depressed ET patients and 112 depressed patients without ET were assessed using the Montgomery-Asberg Depression Rating Scale (MADRS). We compared the individual depressive symptoms of the two groups by comparing MADRS subitem scores. Although there was no significant difference between the level of cognitive function and the severity of depression, patients with ET had a lower score on items “reported sadness”, “inability to feel” and “pessimistic thoughts”, and a higher score on items “concentration difficulties” and “lassitude” than those of patients without ET. These results show that depressive symptoms in patients with ET possess distinct characteristics compared to those in depressed patients without ET.     

Essential tremor: Is the word “essential” really essential?

Essential tremor (ET) is among the common movement disorders. A surge in research in recent years has considerably improved our understanding of disease etiology and pathogenesis, and its associated clinical phenomenology and natural history. With this progress have emerged a multitude of new questions and conundrums and newly proposed terminologies. Amidst these various related discussions, it is worth revisiting the essence of the nomenclature, “essential tremor”, to assess how well it continues to fit the growing understanding of this entity.Here we revisit the historical underpinnings of the nomenclature, its accuracy, pitfalls of eliminating the word, and advantages of removing the word. There are two primary historical bases for using the word “essential”: (i) idiopathic or unclear etiology, (ii) a unitary (single-featured) trait perceived as a constitutional feature. Numerous studies indicate that ET is neither truly idiopathic nor is fully isolated, making the use of “essential” technically incorrect. There are pitfalls and advantages of eliminating the term “essential” and both are succinctly described in this article. Yet in the absence of any flawless alternatives at present, we conclude that it is preferable for now to persist with “essential” tremor, thereby respecting the historical continuity of this one-and-a-half-century old nomenclature.     

Population-based case-control study of essential tremor

Current epidemiologic data on the association between environmental factors and essential tremor (ET) are scanty. In a population-based case-control study we investigated the relationship between some putative risk factors and ET. In the present study, we included all subjects identified during a door-to-door prevalence survey in a Sicilian community, affected by ET, and alive on 1 November 1987 (n=31). Of the 31 prevalent cases of ET, we were able to contact 28 subjects. Twenty-eight controls were randomly selected from the general population and matched to each case for age and sex. Exposure variables were investigated by interviewing the patients and their controls or close relatives, using a structured questionnaire. The odds for habitual alcohol consumption were 0.3, indicating a protective effect. The odds for exposure to agricultural chemicals and domestic animals were 2.5 and 2.7, respectively, suggesting a role as potential risk factors. However, the associations found by us were not statistically significant.

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Sommario Un argomento poco considerato dalle indagini di epidemiologia analitica riguarda la possibile influenza di fattori di rischio ambientali sull'eziologia del tremore essenziale. Scopo del presente lavoro è quello di verificare l'esistenza di un'associazione tra possibile fattori di rischio e il tremore essenziale, attraverso uno studio caso controllo su popolazione. In un precedente studio di prevalenza di tipo porta a porta, abbiamo identificato tutti i pazienti affetti da tremore essenziale residenti net comune di Terrasini net giorno di prevalenza, 1 Novembre 1987 (n=31). A 28 di tale pazienti e a un gruppo di controloi di uguale numerosità, selezionati dalla popolazione generate e appaiati per età e sesso net rapporto di 1:1, è stato possibile somministrare un questionario strutturato, per valutare l'esposizione a diverse variabili ambientali. Il rischio per it consumo abituale di alcool è risultato di 0,3, suggerendo un effetto protettivo per tale assunzione; al contrario l'esposizione ad anticrittogamici e il contatto abituale con animali domestici hanno mostrato rispettivamente un rischio di 2,5 e 2,7, valori questi the candiderebbero tali esposizioni come fattori the aumentano il rischio di sviluppare it tremore essenziale. Nessuna di tale associazioni è comunque risultata statisticamente significativa.

     

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。     

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.     

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。     

Different effects of essential tremor and Parkinsonian tremor on multiscale dynamics of hand tremor

ObjectiveEssential tremor (ET) and Parkinsonian tremor (PT) are often clinically misdiagnosed due to the overlapping characteristics of their hand tremor. We aim to examine if ET and PT influence the multiscale dynamics of hand tremor, as quantified using complexity, differently, and if such complexity metric is of promise to help identify ET from PT.MethodsForty-eight participants with PT and 48 with ET performed two 30-second tests within each of the following conditions: sitting while resting arms or outstretching arms horizontally. The hand tremor was captured by accelerometers secured to the dorsum of each hand. The complexity was quantified using multiscale entropy.ResultsCompared to PT group, ET group had lower complexity of both hands across conditions (F > 34.2, p < 0.001). Lower complexity was associated with longer disease duration (r² > 0.15, p < 0.009) in both PT and ET, and within PT, greater Unified Parkinson's Disease Rating Scale-III UPDRS-III scores (r² > 0.18, p < 0.009). Receiver-operating-characteristic curves revealed that the complexity metric can distinguish ET from PT (area-under-the-curve > 0.77, cut-off value = 48 (postural), 49 (resting)), which was confirmed in a separate dataset with ET and PT that were clearly diagnosed in prior work.ConclusionsThe PT and ET have different effects on hand tremor complexity, and this metric is promising to help the identification of ET and PT, which still needs to be confirmed in future studies.SignificanceThe characteristics of multiscale dynamics of the hand tremor, as quantified by complexity, provides novel insights into the different pathophysiology between ET and PT.