本文選題：Wilson病 + 凍結(jié)步態(tài)�。� 參考：《安徽中醫(yī)藥大學(xué)》2017年碩士論文

【摘要】：Wilson病(Wilson disease,WD)是一種以銅代謝異常為主的神經(jīng)系統(tǒng)遺傳性疾病,主要由于體內(nèi)銅元素的異常蓄積導(dǎo)致肝臟或/及腦損害,步態(tài)異常亦為WD錐體外系常見(jiàn)表現(xiàn),有不少WD患者可發(fā)生不同程度的凍結(jié)步態(tài),目前對(duì)WD患者凍結(jié)步態(tài)的臨床特征及發(fā)生機(jī)制研究的文獻(xiàn)較少,WD并發(fā)凍結(jié)步態(tài)的相關(guān)機(jī)制有待深入研究。目的:分析WD患者凍結(jié)步態(tài)的臨床特征,探討凍結(jié)步態(tài)與WD患者的臨床分型、發(fā)病年齡、首發(fā)癥狀、病程以及認(rèn)知、情感的關(guān)系,分析WD患者凍結(jié)步態(tài)可能的發(fā)病機(jī)制;探討利用U型電子步道系統(tǒng)評(píng)估WD患者凍結(jié)步態(tài)嚴(yán)重程度的客觀(guān)化指標(biāo);對(duì)WD患者中西結(jié)合驅(qū)銅治療前后凍結(jié)步態(tài)的改善情況進(jìn)行研究。方法:1.研究對(duì)象選取2016年1月至2016年11月我院住院的WD腦型和或腦內(nèi)臟型患者,實(shí)驗(yàn)組為WD合并凍結(jié)步態(tài)患者20例;對(duì)照組為WD未合并凍結(jié)步態(tài)患者20例。2.收集入組的WD患者的一般臨床資料,記錄年齡、性別、病程、分型、分級(jí)、多巴胺類(lèi)等藥物服用情況。3.對(duì)入組的WD患者,在治療前進(jìn)行蒙特利爾認(rèn)知評(píng)估量表(MoCA量表)、HANMILTON焦慮量表、凍結(jié)足問(wèn)卷(freezing of gait questionnaire FOGQ)量表的問(wèn)卷調(diào)查。4.應(yīng)用中科院與我院合作研發(fā)的運(yùn)動(dòng)障礙疾病數(shù)字化客觀(guān)分析系統(tǒng),檢測(cè)患者步態(tài)的時(shí)空參數(shù)包括步長(zhǎng)、步速及雙支撐時(shí)間占周期比(DST/GC)。5.驅(qū)銅治療方法為肝豆片口服1.35g/日3次;二巰基丙磺鈉(Sodium Dimercaptopropane Sulfonate,DMPS),靜脈注射,20mg/kg/日。6.對(duì)入組的患者(對(duì)照組及實(shí)驗(yàn)組)行中西醫(yī)結(jié)合驅(qū)銅綜合治療10療程后,復(fù)行上述數(shù)字化客觀(guān)分析系統(tǒng)、多種量表檢查。結(jié)果:1.本研究統(tǒng)計(jì)了2016年1月至2016年11月我院住院患者中入組患者含實(shí)驗(yàn)組20例,其中男性12例,女性8例;對(duì)照組20例,其中男性9例,女性11例;實(shí)驗(yàn)組年齡為23.5±7歲,病程為6.47±4.32年,分級(jí)為3.05±0.605級(jí);對(duì)照組年齡24.7±8歲,病程為6.35±5.66年,分級(jí)為2.95±0.51級(jí),20例WD合并凍結(jié)患者中18例存在不同程度的肌張力障礙,16例表現(xiàn)有肌僵直,12例表現(xiàn)有不同程度構(gòu)音障礙;20例WD合并凍結(jié)步態(tài)的患者中用改良Goldstein分級(jí)時(shí)分為Ⅲ、Ⅳ級(jí)占85%,有12例(60%)患者病程超過(guò)3年。2.實(shí)驗(yàn)組WD患者年齡與療前FOGQ評(píng)分相關(guān)性分析得出r=-0.329,p0.05,WD患者年齡與療前FOGQ評(píng)分無(wú)相關(guān)性;實(shí)驗(yàn)組WD患者病程與療前FOGQ評(píng)分相關(guān)性分析得出r=0.070,p0.05,WD患者病程與療前FOGQ評(píng)分間無(wú)相關(guān)性;凍結(jié)步態(tài)的病程為(3.4±4.5年)與療前FOGQ評(píng)分相關(guān)性分析得出,r=-0.034,p=0.8880.05,兩者無(wú)相關(guān)性。表明WD患者凍結(jié)步態(tài)的嚴(yán)重程度與患者年齡、病程、凍結(jié)步態(tài)的病程無(wú)相關(guān)性。3.療前實(shí)驗(yàn)組MoCA總分為27.35±1.23,對(duì)照組MoCA總分為28.15±1.27,二者比較,p0.05,兩者M(jìn)oCA總分之間差異性無(wú)統(tǒng)計(jì)學(xué)意義;兩組MoCA分項(xiàng)中的視空間與執(zhí)行功能得分比較,實(shí)驗(yàn)組得分為3.85±1.04,對(duì)照組得分為4.65±0.67,兩者比較p0.05,實(shí)驗(yàn)組視空間與執(zhí)行功能得分較對(duì)照組低,二者差異具有統(tǒng)計(jì)學(xué)意義,其余分項(xiàng)(命名、注意、語(yǔ)言、抽象、延遲記憶、定向)評(píng)分比較p0.05,無(wú)統(tǒng)計(jì)學(xué)意義,表明視空間與執(zhí)行功能障礙可能與WD患者發(fā)生凍結(jié)步態(tài)癥狀有關(guān)。4.實(shí)驗(yàn)組患者HANMILTON焦慮量表評(píng)分無(wú)焦慮(7分)7例占35%,可能焦慮(≥7分)3例占15%,肯定焦慮(≥14分)9例占45%,明顯焦慮(≥21分)1例占5%,嚴(yán)重焦慮(≥29分)的0例,肯定焦慮以上患者10例占50%;20例對(duì)照組中肯定焦慮以上的患者3例占15%。療前實(shí)驗(yàn)組與對(duì)照組患者在HANMILTON焦慮量表評(píng)分分值間比較,實(shí)驗(yàn)組評(píng)分為11.95±5.54,對(duì)照組評(píng)分為7.25±5.04,實(shí)驗(yàn)組與對(duì)照組患者評(píng)分經(jīng)兩獨(dú)立樣本均數(shù)t檢驗(yàn)得出t=2.805,p=0.000.05,WD合并凍結(jié)步態(tài)患者多存在焦慮癥狀,焦慮癥狀可能與WD患者凍結(jié)步態(tài)的發(fā)生存在一定關(guān)系。5.通過(guò)對(duì)實(shí)驗(yàn)組與對(duì)照組步態(tài)參數(shù)比較,兩組步長(zhǎng)(m)比較得出,實(shí)驗(yàn)組為0.336±0.097m,對(duì)照組0.546±0.029m,實(shí)驗(yàn)組步長(zhǎng)低于對(duì)照組,兩者存在顯著統(tǒng)計(jì)學(xué)意義(p0.05);兩組步速(m/s)比較得出,實(shí)驗(yàn)組為0.517±0.161 m/s,對(duì)照組為1.189±0.128 m/s,實(shí)驗(yàn)組較對(duì)照組步速減低,其差異性存在統(tǒng)計(jì)學(xué)意義(p0.05);DST/GC比較得出,實(shí)驗(yàn)組為0.406±0.083,對(duì)照組為0.241±0.042,實(shí)驗(yàn)組較對(duì)照組增高,兩者差異存在統(tǒng)計(jì)學(xué)意義(p0.05),表明WD合并凍結(jié)步態(tài)患者的步長(zhǎng)縮短、步速減慢、DST/GC增高。6.通過(guò)對(duì)實(shí)驗(yàn)組FOGQ評(píng)分與步長(zhǎng)、步速以及DST/GC的相關(guān)性分析,得出實(shí)驗(yàn)組步長(zhǎng)與FOGQ評(píng)分相關(guān)性分析,p0.05,兩者呈線(xiàn)性相關(guān),r=-0.8820,說(shuō)明兩者呈負(fù)相關(guān),FOGQ評(píng)分越高,患者步長(zhǎng)越短;實(shí)驗(yàn)組步速與FOGQ評(píng)分相關(guān)性分析,p0.05,兩者為線(xiàn)性相關(guān),r=-0.6510,兩者呈負(fù)相關(guān),FOGQ評(píng)分越高,患者步速越慢。實(shí)驗(yàn)組DST/GC值與FOGQ評(píng)分相關(guān)性分析,p0.05,r=0.8760,說(shuō)明兩者呈正相關(guān),FOGQ評(píng)分越高DST/GC值也越高,表明基于柔性力敏壓力傳感器搭建的U型步態(tài)通道系統(tǒng)提取的步態(tài)參數(shù)(步長(zhǎng)、步速及DST/GC)可反應(yīng)WD患者凍結(jié)步態(tài)的嚴(yán)重程度,患者凍結(jié)步態(tài)癥狀越嚴(yán)重步長(zhǎng)越短,步速越慢,雙支撐時(shí)間占周期比值越高。7.實(shí)驗(yàn)組療前步長(zhǎng)為0.336±0.097m,經(jīng)10療程中西醫(yī)結(jié)合驅(qū)銅治療后為0.366±0.104m,療后較療前步長(zhǎng)增加,兩者比較p0.05,兩者差異具有統(tǒng)計(jì)學(xué)意義;實(shí)驗(yàn)組療前步速為0.517±0.161 m/s,療后為0.928±0.138 m/s,療后患者步速較療前增加,兩者比較p0.05,兩者差異具有統(tǒng)計(jì)學(xué)意義;治療前后DST/GC比較,治療前DST/GC值為0.406±0.083,治療后為0.361±0.080,較療前DST/GC值減小,兩者比較p0.05,兩者差異具有統(tǒng)計(jì)學(xué)意義;治療前后FOGQ評(píng)分比較,治療前FOGQ評(píng)分為9.30±3.21,治療后FOGQ評(píng)分為7.50±2.03,療后較療前FOGQ評(píng)分減低,兩者比較p0.05,兩者差異具有統(tǒng)計(jì)學(xué)意義。表明經(jīng)中西醫(yī)結(jié)合驅(qū)銅治療可改善WD患者的凍結(jié)步態(tài)癥狀。8.20例實(shí)驗(yàn)組患者療前HANMILTON焦慮量表評(píng)分肯定焦慮以上患者10例占50%;經(jīng)中西醫(yī)結(jié)合驅(qū)銅治療療后,肯定焦慮以上患者(≥14分)3例占15%,療前實(shí)驗(yàn)組HANMILTON焦慮量表評(píng)分為11.95±5.54,療后為9.45±4.49,經(jīng)配對(duì)樣本均數(shù)的t檢驗(yàn)得出,t=5.008,p0.05,表明WD合并凍結(jié)步態(tài)患者經(jīng)DMPS聯(lián)合肝豆片中西醫(yī)結(jié)合驅(qū)銅治療10個(gè)療程后,凍結(jié)步態(tài)改善的同時(shí),焦慮癥狀也有所改善。結(jié)論:1.本組WD患者出現(xiàn)凍結(jié)步態(tài)癥狀多為青年,且病程較長(zhǎng)病情較嚴(yán)重;往往以腦型癥狀起病,但以?xún)鼋Y(jié)步態(tài)表現(xiàn)為首發(fā)癥狀較少;WD合并凍結(jié)步態(tài)患者病程中較常合并不同程度的肌張力障礙、肌僵直及構(gòu)音障礙。2.WD患者凍結(jié)步態(tài)的發(fā)生與患者焦慮情緒及認(rèn)知功能中視空間障礙存在一定相關(guān)性。3.基于柔性力敏壓力傳感器搭建的U型步態(tài)通道系統(tǒng)提取WD患者的步長(zhǎng)、步速及DST/GC參數(shù)可用于評(píng)估WD患者凍結(jié)步態(tài)的嚴(yán)重程度,能為WD患者凍結(jié)步態(tài)的評(píng)估提供客觀(guān)化依據(jù)。4.肝豆片聯(lián)合DMPS中西醫(yī)結(jié)合驅(qū)銅治療可以很好的改善WD患者凍結(jié)步態(tài)癥狀。
[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.
【學(xué)位授予單位】：安徽中醫(yī)藥大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2017
【分類(lèi)號(hào)】：R742.4

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