| Wilson disease is a genetic disease of the nervous system,which is dominated by copper metabolism.The disease is mainly caused by the abnormal accumulation of copper in the body,which causes liver and brain damage.Gait disorder is one of clinical manifestations.Many WD patients may have varying degrees of freezing of gait.At present there is less research on freezing of gait of WD patients and the mechanism of freezing of gait remains to be studied.Objective:To analyze the clinical features of WD patient’s freezing of gait and explore the correlation between the freezing of gait and WD patient’s clinical classification,age of onset,initial symptoms,course of illness as well as cognition and emotion,to analyze the possible mechanism of freezing of gait of WD patient;To explore using the U-type electronic footpath system to find out the objectified indexes that can evaluate the severity of freezing of gait of WD patient;Meanwhile to study the improvement of freezing of gait by anti-copper treatment that combines tradition Chinese and western medicine.Method:1.Object of study:The WD cerebral or/and cerebral visceral patients hospitalized in our hospital from Jan,2016 to Nov,2016 were selected,experimental group: 20 cases of WD patient complicated with freezing of gait were selected;control group: 20 cases of WD patient not complicated with freezing of gait were selected.2.The general clinical data of patients who met the inclusion criteria was collected,including the age,gender,course of illness,classification,grading,medication of dopaminergic agents and other drugs.3.The included WD patients were investigated through Montreal Cognitive Assessment(MoCA scale),HANMILTON anxiety scale and freezing of gait questionnaire(FOGQ)before treatment;4.The time and space parameters(gait length,gait speed and double bracing time-period ratio(DST/GC))of patients’ gait were detected by the use of movement disorder digitalized objective analytic system developed jointly by the Chinese Academy of Science and our hospital.5.The anti-copper treatment DMPS IVqtt,20mg/kg/qd,the Gandou tablets 1.35 g pro 3 times a day.6.After the included patients(control and experiment group)were treated by 10 courses of comprehensive anti-copper treatment that combines tradition Chinese and western medicine,the abovementioned digitized objective analytical system and multiple scales were again performed to them.Result:1.This experiment has carried out a statistics of 20 cases of WD patients complicated with freezing of gait who were hospitalized in our hospital from Jan,2016 to Nov,2016,their mean age was 23.5±7 years-old,which indicated that,12 man cases and 8 female cases while the control group included 20 cases of WD patients which mean age was 24.7±8 years-old,this group included 9 cases male and 11 cases female.The course of disease of experiment group was 6.47±4.32 years-old and the control group was 6.35±5.66 years-old.Among the 20 cases of WD patients,there were18 cases of patients expressing dystonia symptom,16 cases expressing rigidity and 12 cases expressing dysarthria of varying degree.60% of experiment group patients’ disease course was more than 3 years.2.It has been concluded by an analysis on the correlation between WD patient age and pre-treatment FOGQ score that,r=-0.329,p > 0.05,the WD patient age was not correlated with the pre-treatment FOGQ score;It has been concluded by an analysis on the correlation between course of illness of WD patients in experiment group and pre-treatment FOGQ score that,r=0.070,p>0.05,the course of illness of WD patients was not correlated with the pre-treatment FOGQ score;It has been concluded by ananalysis on the correlation between the course of illness of freezing of gait(3.4±4.5years)and pre-treatment FOGQ score that,r=-0.034,p>0.05,there is no correlation between the both,indicating that,the severity of freezing of gait of WD patients was not correlated with the patient age,course of illness and course of freezing of gait.3.The total MoCA score of experiment before treatment was 27.35±1.23,the total MoCA score of control group was 28.15±1.27,both were compared with each other,p=0.086>0.05,the difference in MoCA total score between the both was not statistically significant;in terms of the comparison of visual space and executive function score in the subitem of MoCA,the experiment group scored 3.85±1.04,the control group scored4.65±0.67,both were compared with each other,p=0.019<0.05,the experiment group scored less than the control group did in terms of visual space and executive function,the difference between both was statistically significant,the scores of the rest subitems(naming,attention,language,abstract,delayed memory and orientation)of both were compared,which demonstrated that all the p were more than 0.05,which was not statistically significant,indicating that,the disorder of visual space and executive function may be associated with the WD patient’s development of freezing of gait.4.The score of HANMILTON anxiety scale of 20 cases of patient in experiment group showed that there were 7 cases who didn’t have anxiety(less than 7 points),accounting for 35%,3 cases who might have anxiety(≥ 7 points),accounting for 15%,9cases who had definite anxiety(≥14 points),accounting for 45%,1 case who had evident anxiety(≥21 points),accounting for 5%,and 0 case who had severe anxiety(≥29 points);there were 10 cases of patient who had definite anxiety or above,accounting for 50%;3 out of 20 cases of patient in control group had definite anxiety or above,accounting for 15%.the grading of HANMILTON anxiety scale of experiment group was compared with that of control group before treatment,the experiment group was graded as 11.95±5.54,the control group was graded as 7.25±5.04,it was worked out by a t-test of two independent sample means of grading of patients in experiment and control group that,t=2.805,p < 0.05,anxiety symptom existed in most of the WDpatients with freezing of gait,the anxiety symptom may be associated with the occurrence of freezing of gait of WD patients to some degree.5.Through a comparison of gait parameters between experiment and control group,it was worked out by a comparison of gait length(m)between the two groups that,the experiment group was 0.336±0.097 m,the control group was 0.546±0.029 m,the gait length of experiment group was less than that of control group,the difference was statistically significant p<0.05;It was worked out by a comparison of gait speed(m/s)between the both that,the experiment group was 0.517±0.161 m/s,the control group was(1.189±0.128 m/s),the experiment group had a lower gait speed than the control group did,,the difference was statistically significant(p<0.05);A comparison of DST/GC between both groups demonstrated that,the experiment group was 0.406±0.083,the control group was 0.241±0.042,the experiment group was higher than the control group,the difference was statistically significant(p<0.05),indicating that,WD patients complicated with freezing of gait had a shortened gait length,decreased gait speed and DST/GC.6.An analysis on the correlation between gait length of experiment and FOGQ score was done by an analysis on the correlation between the experiment group FOGQ and gait length,gait speed and DST/GC,p<0.05,the both were correlated to each other linearly,r=-0.882<0,indicating that the both were correlated to each other negatively,the higher the FOGQ score is,the shorter the gait length is;It was worked out by an analysis on the correlation between gait speed of experiment group and FOGQ score that,p<0.05,the both were linearly correlated to each other,r=-0.651<0,the both were correlated to each other negatively,the higher the FOGQ score is,the slower the patient’s gait speed is.It was worked out by an analysis on the correlation between DST/GC value of experiment group and FOGQ score that,p<0.05,r=0.876>0,the both were positively correlated to each other.The higher the FOGQ score is,the greater the DST/GC value is,indicating that,the gait parameters(gait length,speed and DST/GC)extracted by the U-type gait passage system that is based on flexible stress-sensitivepressure sensor can reflect the severity of freezing of gait of WD patients,the more severe the symptoms of freezing of gait of patient is,the longer the gait length is,the slower the gait speed is,and the higher the DST/GC value is.7.The pre-treatment gait length of experiment group was 0.336±0.097 m,which was turned into 0.366±0.104 m by 10 courses of anti-copper treatment combining traditional Chinese and western medicine,the post-treatment gait length was longer than the pre-treatment one,p<0.05,the difference between both was statistically significant;compared with the pre-treatment situation,the pre-treatment gait speed of experiment group was 0.517±0.161 m/s,which was turned into 0.928±0.138 m/s after treatment,the post-treatment gait speed was faster than the pre-treatment one,p<0.05,the difference between both was statistically significant;As to the comparison between pre-treatment DST/GC value and post-treatment one,the former was 0.406±0.083,the latter was0.361±0.080,the post-treatment DST/GC value was less than the pre-treatment one,p<0.05,the difference between both was statistically significant;As to the comparison between pre-treatment FOGQ score and post-treatment one,the former was9.30±3.21,the latter was 7.50±2.03,the post-treatment FOGQ was less than the pre-treatment one,p<0.05,the difference between both was statistically significant;it was indicated that,the therapy of DMPS combined with Gandou tablets and anti-copper treatment combining traditional chinese and western medicine can improve the symptom of freezing of gait of WD patients.8.10 out of 20 cases of patient in experiment group were evaluated as definite anxiety or above by a pre-treatment HANMILTON anxiety scale,accounting for 50%;having been treated by a therapy of DMPS combined with Gandou tablets and anti-copper treatment combining traditional chinese and western medicine,there were 3cases of patient who were evaluated as definite anxiety or above,accounting for 15%(≥14 points);as for the experiment group,the pre-treatment HANMILTON anxiety scale score was 11.95±5.54,the post-treatment one was 9.45±4.49,by a t-test of paired sample mean,it was worked out that,t=5.008,p<0.05,which indicates that the anxietyof WD patients complicated with freezing of gait had been improved by 10 courses of DMPS combined with Gandou tablets and anti-copper treatment combining traditional chinese and western medicine at the same time their symptom of freezing of gait was improved.Conclusion:1.WD patients with freezing of gait were mostly young people and had a long course of illness and severe condition;the disease usually starts with a cerebral-type symptom,but few patients were found to have a freezing of gait as the initial symptom;in the course of illness,the WD patients complicated with freezing of gait are usually complicated with dystonia,muscular rigidity and dysarthria of varying degree.2.The occurrence of freezing of gait in WD patients is to some extent correlated with the patient’s antient emotion and visual space disorder in cognitive function.3.The gait parameters(gait length,speed and DST/GC)extracted by the U-type gait passage system that is based on flexible stress-sensitive pressure sensor can be used to evaluate the severity of freezing of gait of WD patients,and can provide an objectified basis for the evaluation of freezing of gait of WD patients.4.Therapy of DMPS combined with Gandou tablets and anti-copper treatment combining traditional chinese and western medicine can well improve the symptom of freezing of gait of WD patients. |
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