Wilson病患者冻结步态的临床特征、客观化评估及中西医结合驱铜治疗疗效研究

发布时间：2018-05-27 19:38

本文选题：Wilson病 + 冻结步态　；参考：《安徽中医药大学》2017年硕士论文

【摘要】：Wilson病(Wilson disease,WD)是一种以铜代谢异常为主的神经系统遗传性疾病,主要由于体内铜元素的异常蓄积导致肝脏或/及脑损害,步态异常亦为WD锥体外系常见表现,有不少WD患者可发生不同程度的冻结步态,目前对WD患者冻结步态的临床特征及发生机制研究的文献较少,WD并发冻结步态的相关机制有待深入研究。目的:分析WD患者冻结步态的临床特征,探讨冻结步态与WD患者的临床分型、发病年龄、首发症状、病程以及认知、情感的关系,分析WD患者冻结步态可能的发病机制;探讨利用U型电子步道系统评估WD患者冻结步态严重程度的客观化指标;对WD患者中西结合驱铜治疗前后冻结步态的改善情况进行研究。方法:1.研究对象选取2016年1月至2016年11月我院住院的WD脑型和或脑内脏型患者,实验组为WD合并冻结步态患者20例;对照组为WD未合并冻结步态患者20例。2.收集入组的WD患者的一般临床资料,记录年龄、性别、病程、分型、分级、多巴胺类等药物服用情况。3.对入组的WD患者,在治疗前进行蒙特利尔认知评估量表(MoCA量表)、HANMILTON焦虑量表、冻结足问卷(freezing of gait questionnaire FOGQ)量表的问卷调查。4.应用中科院与我院合作研发的运动障碍疾病数字化客观分析系统,检测患者步态的时空参数包括步长、步速及双支撑时间占周期比(DST/GC)。5.驱铜治疗方法为肝豆片口服1.35g/日3次;二巯基丙磺钠(Sodium Dimercaptopropane Sulfonate,DMPS),静脉注射,20mg/kg/日。6.对入组的患者(对照组及实验组)行中西医结合驱铜综合治疗10疗程后,复行上述数字化客观分析系统、多种量表检查。结果:1.本研究统计了2016年1月至2016年11月我院住院患者中入组患者含实验组20例,其中男性12例,女性8例;对照组20例,其中男性9例,女性11例;实验组年龄为23.5±7岁,病程为6.47±4.32年,分级为3.05±0.605级;对照组年龄24.7±8岁,病程为6.35±5.66年,分级为2.95±0.51级,20例WD合并冻结患者中18例存在不同程度的肌张力障碍,16例表现有肌僵直,12例表现有不同程度构音障碍;20例WD合并冻结步态的患者中用改良Goldstein分级时分为Ⅲ、Ⅳ级占85%,有12例(60%)患者病程超过3年。2.实验组WD患者年龄与疗前FOGQ评分相关性分析得出r=-0.329,p0.05,WD患者年龄与疗前FOGQ评分无相关性;实验组WD患者病程与疗前FOGQ评分相关性分析得出r=0.070,p0.05,WD患者病程与疗前FOGQ评分间无相关性;冻结步态的病程为(3.4±4.5年)与疗前FOGQ评分相关性分析得出,r=-0.034,p=0.8880.05,两者无相关性。表明WD患者冻结步态的严重程度与患者年龄、病程、冻结步态的病程无相关性。3.疗前实验组MoCA总分为27.35±1.23,对照组MoCA总分为28.15±1.27,二者比较,p0.05,两者MoCA总分之间差异性无统计学意义;两组MoCA分项中的视空间与执行功能得分比较,实验组得分为3.85±1.04,对照组得分为4.65±0.67,两者比较p0.05,实验组视空间与执行功能得分较对照组低,二者差异具有统计学意义,其余分项(命名、注意、语言、抽象、延迟记忆、定向)评分比较p0.05,无统计学意义,表明视空间与执行功能障碍可能与WD患者发生冻结步态症状有关。4.实验组患者HANMILTON焦虑量表评分无焦虑(7分)7例占35%,可能焦虑(≥7分)3例占15%,肯定焦虑(≥14分)9例占45%,明显焦虑(≥21分)1例占5%,严重焦虑(≥29分)的0例,肯定焦虑以上患者10例占50%;20例对照组中肯定焦虑以上的患者3例占15%。疗前实验组与对照组患者在HANMILTON焦虑量表评分分值间比较,实验组评分为11.95±5.54,对照组评分为7.25±5.04,实验组与对照组患者评分经两独立样本均数t检验得出t=2.805,p=0.000.05,WD合并冻结步态患者多存在焦虑症状,焦虑症状可能与WD患者冻结步态的发生存在一定关系。5.通过对实验组与对照组步态参数比较,两组步长(m)比较得出,实验组为0.336±0.097m,对照组0.546±0.029m,实验组步长低于对照组,两者存在显著统计学意义(p0.05);两组步速(m/s)比较得出,实验组为0.517±0.161 m/s,对照组为1.189±0.128 m/s,实验组较对照组步速减低,其差异性存在统计学意义(p0.05);DST/GC比较得出,实验组为0.406±0.083,对照组为0.241±0.042,实验组较对照组增高,两者差异存在统计学意义(p0.05),表明WD合并冻结步态患者的步长缩短、步速减慢、DST/GC增高。6.通过对实验组FOGQ评分与步长、步速以及DST/GC的相关性分析,得出实验组步长与FOGQ评分相关性分析,p0.05,两者呈线性相关,r=-0.8820,说明两者呈负相关,FOGQ评分越高,患者步长越短;实验组步速与FOGQ评分相关性分析,p0.05,两者为线性相关,r=-0.6510,两者呈负相关,FOGQ评分越高,患者步速越慢。实验组DST/GC值与FOGQ评分相关性分析,p0.05,r=0.8760,说明两者呈正相关,FOGQ评分越高DST/GC值也越高,表明基于柔性力敏压力传感器搭建的U型步态通道系统提取的步态参数(步长、步速及DST/GC)可反应WD患者冻结步态的严重程度,患者冻结步态症状越严重步长越短,步速越慢,双支撑时间占周期比值越高。7.实验组疗前步长为0.336±0.097m,经10疗程中西医结合驱铜治疗后为0.366±0.104m,疗后较疗前步长增加,两者比较p0.05,两者差异具有统计学意义;实验组疗前步速为0.517±0.161 m/s,疗后为0.928±0.138 m/s,疗后患者步速较疗前增加,两者比较p0.05,两者差异具有统计学意义;治疗前后DST/GC比较,治疗前DST/GC值为0.406±0.083,治疗后为0.361±0.080,较疗前DST/GC值减小,两者比较p0.05,两者差异具有统计学意义;治疗前后FOGQ评分比较,治疗前FOGQ评分为9.30±3.21,治疗后FOGQ评分为7.50±2.03,疗后较疗前FOGQ评分减低,两者比较p0.05,两者差异具有统计学意义。表明经中西医结合驱铜治疗可改善WD患者的冻结步态症状。8.20例实验组患者疗前HANMILTON焦虑量表评分肯定焦虑以上患者10例占50%;经中西医结合驱铜治疗疗后,肯定焦虑以上患者(≥14分)3例占15%,疗前实验组HANMILTON焦虑量表评分为11.95±5.54,疗后为9.45±4.49,经配对样本均数的t检验得出,t=5.008,p0.05,表明WD合并冻结步态患者经DMPS联合肝豆片中西医结合驱铜治疗10个疗程后,冻结步态改善的同时,焦虑症状也有所改善。结论:1.本组WD患者出现冻结步态症状多为青年,且病程较长病情较严重;往往以脑型症状起病,但以冻结步态表现为首发症状较少;WD合并冻结步态患者病程中较常合并不同程度的肌张力障碍、肌僵直及构音障碍。2.WD患者冻结步态的发生与患者焦虑情绪及认知功能中视空间障碍存在一定相关性。3.基于柔性力敏压力传感器搭建的U型步态通道系统提取WD患者的步长、步速及DST/GC参数可用于评估WD患者冻结步态的严重程度,能为WD患者冻结步态的评估提供客观化依据。4.肝豆片联合DMPS中西医结合驱铜治疗可以很好的改善WD患者冻结步态症状。
[Abstract]:Wilson disease (Wilson disease, WD) is a kind of hereditary disease of nervous system, mainly due to abnormal copper metabolism. It is mainly caused by the abnormal accumulation of copper elements in the body and causes liver or / and brain damage. The abnormal gait is also common in the extrapyramidal system of WD. There are many WD patients with different degrees of freezing gait, and the frozen gait of WD patients is present. There are few literatures on the bed characteristics and mechanism of occurrence. The mechanism of WD concurrent frozen gait needs to be studied. Objective: to analyze the clinical characteristics of frozen gait in WD patients and to explore the clinical classification of frozen gait and WD patients, the age of onset, the first symptoms, the course of disease, the relationship of cognition and emotion, and analyze the possible pathogenesis of the frozen gait of WD patients. The objective index of evaluating the severity of the frozen gait of WD patients was evaluated by U type electronic trail system, and the improvement of the frozen gait in the combination of Chinese and Western copper flooding in WD patients was studied. Methods: 1. the subjects selected the WD brain and or visceral type patients hospitalized in our hospital from January 2016 to November 2016, and the experimental group was combined with WD. 20 cases of frozen gait patients were frozen, and the control group was the general clinical data of the WD patients who were collected in the group of 20 patients with WD without frozen gait. The age, sex, course of disease, classification, classification, dopamine and other drugs were recorded in the group of WD patients with.3., and the Montreal cognitive assessment scale (MoCA scale) and HANMILTON anxiety scale were carried out in the treatment. A questionnaire (freezing of gait questionnaire FOGQ) questionnaire survey.4. application in the digital objective analysis system of dyskinesia developed by the Academy of Sciences and our institute. The space-time parameters of the gait of patients include step length, step speed and double support time ratio (DST/GC).5. flooding method for oral 1.35g/ day of liver bean tablets. 3 times; two Sodium Dimercaptopropane Sulfonate, DMPS), intravenous injection, 20mg/kg/ day.6. to the group of patients (control group and experimental group) with the combination of Western medicine and the 10 course of treatment, the above digital objective analysis system, multiple scale examination. Results: 1. studies statistics from January 2016 to November 2016 our hospital. In the hospitalized patients, 20 cases were included in the experimental group, including 12 males and 8 females, and 20 cases in the control group, including 9 males and 11 females. The age of the experimental group was 23.5 + 7 years, the course of disease was 6.47 + 4.32 years, and the control group was 9 years old. There were different degree of dystonia, 16 cases showed muscle stiffness, 12 cases had different degree of dysarthria, 20 cases of WD combined with frozen gait with modified Goldstein grade III, grade IV, 85%, and 12 (60%) patients for more than 3 years.2. experimental group and FOGQ score of r=-0.329, P0 .05, WD patients were not related to the FOGQ score before treatment, and the correlation analysis between the course of the patients in the experimental group and the pre therapy FOGQ score showed that there was no correlation between the course of r=0.070, P0.05, WD patients and the preoperative FOGQ score; the course of the frozen gait was (3.4 + 4.5 years) and the correlation analysis of the FOGQ score before the treatment, r=-0.034, p=0.8880.05, there was no correlation between them. The severity of frozen gait in the patients with WD was not related to the patient's age, course of disease, and the course of the frozen gait. The total score of MoCA in the experimental group before.3. was 27.35 + 1.23, the total score of MoCA in the control group was 28.15 + 1.27, and the two was compared, P0.05, and the difference of the total MoCA score between the two groups was not statistically significant; the visual space and the executive function scores in the two group MoCA sub items were compared, The score of the experimental group was 3.85 + 1.04, the score of the control group was 4.65 + 0.67. The scores of the two groups were P0.05. The scores of visual space and executive function in the experimental group were lower than those of the control group. The difference between the two groups was statistically significant. The other sub items (naming, attention, language, abstract, delayed memory, orientation) were compared with P0.05, which showed that the visual space and executive dysfunction were not statistically significant. The symptoms of frozen gait in WD patients might be related to the symptoms of HANMILTON anxiety in the.4. experimental group. The anxiety scale of the HANMILTON was 35%, the anxiety (> 7) was 15%, the anxiety (> 14) 9 cases was 45%, the anxiety (> 21) was 1 and the serious anxiety (> 29). 3 patients with positive anxiety were compared with the scores of the HANMILTON anxiety scale in the experimental group before 15%. and the control group. The score of the experimental group was 11.95 + 5.54 and the control group was 7.25 + 5.04. The patients in the experimental group and the control group were evaluated by the two independent samples by T test to obtain t= 2.805, p=0.000.05, WD combined with frozen gait patients. In anxiety symptoms, anxiety symptoms may have a certain relationship with the occurrence of frozen gait in WD patients.5. through the comparison of the gait parameters of the experimental group and the control group, the two groups of step length (m) compared, the experimental group was 0.336 + 0.097m, the control group was 0.546 + 0.029m, the experimental group was lower than the control group, the two groups had significant statistical significance (P0.05); m /s) compared with the experimental group, the experimental group was 0.517 + 0.161 m/s, the control group was 1.189 + 0.128 m/s, the experimental group was lower than the control group, and the difference was statistically significant (P0.05). Compared with DST/GC, the experimental group was 0.406 + 0.083, the control group was 0.241 + 0.042, the experimental group was higher than the control group, and the difference was statistically significant (P0.05), indicating WD combination. And the step length of the frozen gait patient was shortened, the pace slowed, and the DST/GC increased.6. through the correlation analysis of the FOGQ score of the experimental group and the pace, the pace and the DST/GC, and obtained the correlation analysis between the step length and the FOGQ score of the experimental group, and P0.05, the two showed linear correlation, r=-0.8820, the higher the FOGQ score, the shorter the pace of the patients, the shorter the pace of the patients; the experimental group. The correlation analysis between the speed and the FOGQ score, P0.05, the linear correlation between the two, r=-0.6510, the negative correlation between the two, the higher the FOGQ score, the slower the pace of the patients. The DST/GC value of the experimental group is correlated with the FOGQ score, P0.05, r=0.8760, indicating that the higher the FOGQ score is, the higher the DST/ GC is, indicating the U based on the flexible force sensitive pressure sensor. The gait parameters (step length, step speed and DST/GC) extracted from the gait channel system can reflect the severity of the frozen gait of the patients with WD. The more serious the severity of the gait of the patients is, the more serious the step of the gait symptoms of the patients, the slower the pace, the higher the ratio of the double support time to the period of the period of the.7. experimental group, which is 0.336 + 0.097m before the treatment of the 10 course of combination of Chinese and Western medicine and 0.366 + 0 after the treatment. .104m, after treatment, compared with P0.05, compared with the two, the difference has statistical significance; the experimental group was 0.517 + 0.161 m/s before treatment and 0.928 + 0.138 m/s after treatment. After treatment, the pace of the patients was increased compared to that before treatment, and the two were P0.05, the difference was statistically significant. The DST/GC value before and after treatment was 0.406 + 0.083, before and after treatment. After treatment, 0.361 + 0.080, compared with pre treatment DST/GC value decreased, the two were compared P0.05, the difference was statistically significant. Before and after treatment, FOGQ score was compared, before treatment, FOGQ score was 9.30 + 3.21, after treatment, FOGQ score was 7.50 + 2.03, after treatment compared with pre treatment FOGQ score decreased, the two were compared to P0.05, the difference was statistically significant. Indicated through traditional Chinese and Western medicine. The combined treatment of copper flooding can improve the frozen gait symptoms of WD patients in.8.20 experimental group. The HANMILTON anxiety scale of the patients in the experimental group was 50%. After the treatment by the combination of Chinese and Western medicine, 3 cases (14) were 15%, the HANMILTON anxiety scale was 11.95 + 5.54 in the pre treatment group and 9.4 after the treatment. 5 + 4.49, according to the t test of the average number of paired samples, t=5.008, P0.05, indicating that the WD combined with the frozen gait after 10 courses of treatment with DMPS combined with the combination of Western medicine in the liver, the frozen gait improved and the anxiety symptoms improved. Conclusion: 1. groups of WD patients with frozen gait symptoms are mostly young, and the longer course of the disease is stricter. Heavy; often with brain type symptoms, but with frozen gait performance as the first symptom, WD combined with frozen gait in the course of patients with different degrees of dystonia, muscle stiffness and dysarthria.2.WD patients' frozen gait is related to the patient's anxiety and cognitive ability in the visual spatial disorder,.3. based on softness The U gait channel system built by the sex sensitive pressure sensor can extract the step length of the WD patients, the step speed and the DST/GC parameters can be used to evaluate the severity of the frozen gait of the WD patients. It can provide an objective basis for the assessment of the frozen gait of the patients with WD, which can improve the frozen gait of the WD patients with the combination of the combination of the.4. and the combination of Chinese and Western medicine and the combination of Chinese and Western medicine in the treatment of the frozen gait of the patients with WD. Symptom.
【学位授予单位】：安徽中医药大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R742.4

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