原发性震颤患者的临床特征
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摘要
第一部分原发性震颤患者的临床特征
     目的:研究原发性震颤(Essention tremor,ET)患者临床特征,探讨ET患者常见临床表现、出现率、影响因素及不同年龄、起病年龄、不同病程间患者临床表现差异。
     方法:选取ET患者62例。分别采用匹兹堡睡眠质量指数(Pittsburgh Sleep QualityIndex PSQI)量表评定患者近1个月的睡眠质量;爱泼沃斯思睡量表(Epworth SleepinessScale ESS)评估患者在日常生活中不同情况下白天的思睡程度;疲劳严重度量表(Fatigue Severity Scale FSS)评估疲劳对患者日常生活的影响;Hamilton抑郁量表(Hamilton Depression Rating Scale for Depression HAMD)(24项)评价患者近1月的抑郁程度;认知功能障碍研究选择简易智能状态检查量表(mini—mental stateexamination,MMSE)。并对患者进行震颤评分(Fahn-Tolosa-Marin Tremor RatingScale,FTMTRS)评分。
     结果:本组ET患者中,家族史比例为48.3%,震颤以肢体震颤(100%)最为常见,其次为头部震颤(50%),女性总的震颤分数小于男性(30.41±13.87,34.17±17.33),女性头部受累的几率较男性高(20/33,11/29)。睡眠障碍发生率为51.6%(32/62);白天过度思睡发生率为11.3%(7/62);显著疲劳发生率为19.4%(12/62);抑郁发生率为53.2%(33/62);MMSE平均得分为26.29±3.75;FTMTRS得分和ET患者非运动症状相关;FTMTRS得分在不同年龄组和不同病程分组中均存在统计学差异。
     结论:上肢和头部是原发性震颤患者最常见的震颤部位。ET患者常最见的运动症状为肢体的动作性震颤,常见的非运动症状为情感障碍、睡眠障碍,其次为认知障碍、白天过度思睡、显著疲劳等。ET相关运动症状及非运动症状量表的使用及推广,有助于全面客观评价患者病情,对患者临床特征的关注,有助于优化治疗方案,最大限度减轻疾病所致残疾,提高患者生活质量,减轻家庭社会负担。
     第二部分原发性震颤患者伴发睡眠障碍
     目的:评价ET患者睡眠质量,探讨ET患者睡眠结构异常的表现、睡眠障碍发生率及睡眠障碍相关因素。
     方法:选取ET患者63例,所有患者均签署知情同意书。记录患者年龄、性别、家族史、既往史、病程、起病部位、目前震颤部位、震颤评分。采用PSQI评定ET患者最近1个月的睡眠质量。整个量表由19个自评和5个他评条目组成,而其中参与计分的自评条目可以组成7个因子:主观睡眠质量、入睡时间、睡眠时间、睡眠效率、睡眠干扰、催眠药物、日间功能,每个因子按0~3分等级计分,累积各因子成分得分为PSQI的总分。得分越高提示睡眠质量越差。同时对患者进行震颤评分法(Fahn-Tolosa-Marin Tremor Rating Scale,FTMTRS),HAMD评分,ESS评分。
     结果:本组ET患者有36例(52.9%)总体睡眠质量差,睡眠障碍的主要类型有入睡困难,片断睡眠和日间过度思睡。而对照组25例(35.7%)睡眠质量差,两组比较,差异有统计学意义(χ~2=4.150,P=0.042)。ET患者中睡眠障碍组与非睡眠障碍组两组年龄、抑郁评分比较有统计学意义(t=2.381,P=0.021;t=4.058,P=0.000);两组PSQI各因子评分比较结果:两组主观睡眠质量(F1)、入睡时间(F2)、睡眠时间(F3)、睡眠效率(F4)、睡眠干扰(F5)、日间功能(F7),均有统计学意义,而催眠药物(F7)无统计学意义。68例ET患者中,有30例(44.1%)伴发日间过度思睡(EDS),其中可疑EDS13例(19.1%),肯定EDS17例(25.0%);70名对照组中11名(15.7%)伴发EDS,其中可疑EDS5例(7.1%),肯定EDS 6名(8.6%),两组相比较差异具有统计学意义(χ~2=4.361,P=0.037;χ~2=6.703,P=0.010)。
     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。
     第三部分帕金森病及原发性震颤抑郁障碍比较研究
     目的:研究帕金森病患者和原发性震颤患者抑郁的发生情况及其相关因素。
     方法:收集患者病史资料,对PD患者进行H-Y分期和统一PD评定量表(UPDRS)运动评分(UPDRS-Ⅲ)评分。对ET患者进行震颤评定量表(TRS)-PartA、PartB评分。汉密尔顿抑郁量表(HAMD,24项版本)评定患者的抑郁程度。
     结果:121例帕金森病患者中抑郁发生率为56.2%,62例原发性震颤患者中抑郁发生率为53.2%,两组抑郁发生率和轻度抑郁、中度抑郁、重度抑郁发生率均无统计学意义(P>0.05)。PD组与ET组HAMD各因子比较显示两组认知障碍及绝望感均有统计学意义(P<0.05),而两组焦虑/躯体化、体重减轻、日夜变化、阻滞及睡眠障碍无统计学意义(P>0.05)。PD患者中,抑郁与UPDRS-Ⅲ显著相关(r=0.511,P<0.01);ET患者中,抑郁与TRS(PartA+PartB)评分显著相关(r=0.828,P<0.01)。
     结论:运动障碍疾病最常见的两个疾病帕金森病和原发性震颤中抑郁有较高发生率,两者的抑郁程度相似,抑郁症状主要表现为焦虑、心境恶劣、工作和兴趣减退、迟缓、失眠等。抑郁与运动障碍呈正相关。
PartⅠClinical characteristics in patients with Essential Tremor
     Objective:To study the Clinical characteristics and the common clinical manifestation,incidence and influencing factors of non-motor symptoms in patients with essential tremor(ET).We also observed the diversity of clinical symptoms among groups with different age,age onset and course of disease.
     Method:62 ET patients were enrolled in this study.Pittsburgh Sleep Quality Index (PSQI),Epworth Sleepiness Scale(ESS),Fatigue Severity Scale(FSS),Hamilton Depression Rating Scale for Depression(HAMD),Mini-Mental State Examination (MMSE) were used to evaluate sleep quality in recent a month,somnolence under different conditions in daily life,the impact of fatigue on daily life,depression in recent a month and cognitive impairment,as well as the Fahn-Tolosa-Marin Tremor Rating Scale (FTMTRS) was performed in patients with ET.
     Result:There were 48.3%patients had family history with ET.Upper limbs(100 %) and head(50%) were the most commonly affected,the total score of tremor was lower in females than in males(30.41±13.87,34.17±17.33),the probability of head tremor was higher in females than in male(20/33,11/29).The incidence of sleep disorders, somnolence,fatigue and depression were 51.6%(32/62),11.3%(7/62),19.4%(12/62) and 53.2%(33/62)separately.The mean score of MMSE is 26.29±3.75 in patients with ET. The FTMTRS total score is associated with non-motor symptoms.There are statistically differences on scores of FTMTRS in groups of different age and onset age.
     Conclusion:ET is characterized by postural and kinetic tremor involving the arms and,less commonly the head,the common non-motor symptoms as affective disorder, sleep disorders,followed by cognitive impairment,excessive daytime sleepiness,severe fatigue.Using motor and non-motor-related symptoms scales about ET contribute to evaluating the severity of ET patients o To concern the clinical features of ET patients can help optimizing the treatment plan,minimizing disability of the disease,improving quality of life,reducing the burden of the family and the society.
     PartⅡSleep disorders Associated With Essential Tremor
     Objective:To evaluate the quality of sleep and investigate the abnormal appearance of sleep structure,the incidence of sleep disorder,factors associated with sleep disorder in ET patients.
     Method:63 ET patients were enrolled in this study.We recorded patients' age,gender, age onset,family history,history of treatments,course of disease,beginning symptom, tremor position,and the score of tremor.PSQI was used to evaluate sleep quality in recent a month.The PSQI consists of 19 self-rated questions and five questions rated by the bedpartner or roommate.Nineteen individual items generate seven "component" scores: subjective sleep quality,sleep latency,sleep duration,habitual sleep efficiency,sleep disturbances,use of sleeping medication,and daytime dysfunction.Each component weighted equally on a 0-3 scale.The seven component scores are summed to yield a global PSQI score,which has a range of 0-21,higher scores indicate worse sleep quality.
     The Hamilton Depression Rating Scale(HAMD)、Fahn-Tolosa-Marin Tremor Rating Scale(FTMTRS)、Epworth sleepiness scale(ESS) were as well as performed in patients with ET.
     Result:The poor quality of sleep was observed in 36 of 68(52.9%) ET patients compared with 25 of 70(35.7%) in controls(x~2=4.150,P=0.042).The manifestation of sleep disorders in 68 ET patients included:difficulties in the initiation of sleep, fragmented sleep and excessive daytime sleepiness(EDS).There were marked differences in the score of Hamilton Depression Rating Scale(HAMD) and age(t=2.381,P=0.021; t=4.058,P=0.000) between sleep disorder group and non-sleep disorder group.Meanwhile, there were statistical differences in subjective sleep(F1),quality sleep latency(F2),sleep duration hatitual(F3),sleep efficiency(F4),sleep disturbances(F5) and daytime dystunction(F7) between sleep disorder group and non-sleep disorder group.44.1%of ET patients and 15.7%of heathy subjects were found to be excessive daytime sleepiness (EDS score of 7or higher),19.1%of ET patients and 15.7%of heathy subjects fell into the mildly excessive daytime sleepiness range(EDS score between 7 and 9),25.0%of ET patients and 8.6%of heathy subjects fell into severely excessive daytime sleepiness range (EDS score of 10 or higher).There were statistical differences in ET group and heathy subjects group(x~2=4.361,P=0.037;x~2=6.703,P=0.010)
     Conclusion:Sleep disorders were more common in ET patients than in controls.The manifestation of sleep disorders in patients with ET include difficulties in the initiation of sleep,fragmented sleep,and excessive daytime sleepiness.Sleep disorders relate to depression and age.
     PartⅢComparision of depression between Parkinson's disease and essential tremor
     Objeetve:To study the incidence and associated factors of depression in Parkinson's disease and essential tremor.
     Methods.Unified Parkinson's Diseas Rating Scale(UPDRS)-motor examination (UPDRS-Ⅲ) and Hoehn and Yahr scale were used in 121 PD patients to evaluate the severity of the disease.Tremor Rating Scale for Tremor-motor examination(items 1-15 of the rating scale) was prforrned in ET patients.Hamilton Depression Rating Scale(24 items) was tested in all participants to measure the depression.
     Results:56.2%of PD patients and 53.2%%of ET patients were found to be depressed. There were no differences on the incidence of depression,mild depression,moderate depression and severe depression between the two groups.Anxiety/somatization,lose weight,changes day and night,block and sleep disorders were different between PD group and ET group.Score of HAMD was positively correlated with the score of UPDRS -Ⅲin PD patients(r=0.511,P<0.01),as well as in ET patients,it was positively correlated with score of Tremor Rating Scale for Tremor-motor subscale(r=0.828,P<0.01P).
     Conclusions:The incidence of depression is higher in PD and ET patients.The severity of depression was similar between the two groups.The mainly manifestations of depression were anxiety/somatization,dysthymia,anhedonia,retardation and insomnia. Depression was positivly correlated with motor disturbance.
引文
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