The Research On The Cognitive And Emotional State Of Adult Moyamoya Disease And Its Influence Factors.

Moyamoya disease(MMD)is a cerebrovascular disease characterized by progressive occlusion of at the terminal portion of bilateral internal carotid arteries and the initiating portion of anterior cerebral artery and middle cerebral artery,and an abnormal vascular network at the base of the brain.MMD not only cause strokes in relatively adult people,but also cause the severe neuropsychological disorders,including cognitive decline,depression and anxiety that cause the quality of life decline.However,the neuropsychological disorders of MMD patients in China haven't been paid enough attention.The pathogenesis of the neuropsychological disorders of MMD is unkown.Whether the polymorphism of susceptibility gene RNF213 is one of the influencing factors? The effect of revascularization on the neuropsychological impairment is uncertain.This research would investigate the neuropsychological status of the adult MMD patients and the effect of revascularization surgery on the neuropsychological disorders.Part I The changes of cognitive and emotional state in adult moyamoya disease.Objective: To investigate the cognitive and emotional state of adult MMD and its influencing factors.Methods: We collected the clinical and imaging data of continuous adult MMD patients hospitalized in the CHANGHAI Hospital from January 2015 to June 2016.Used several scales(MoCA,TMT-A,TMT-B,PHQ-9,GAD-7,SF-36)to assess the MMD patients' neuropsychological function and qualities of life.According to the MMD patients' baseline,selected the matched physical examination patients as the contrast.Compared the neuropsychological function between the groups and analyzed the relatedinfluencing factors.Used the SPSS17.0 software for statistical analysis,and P<0.05 was significant statistically.Results:The MMD and the control group had significantly difference in the MoCA score,the TMT-A and the TMT-B' time-consuming,the PHQ-9 and GAD-7ratio.(19±8.14 vs 25±6.27,P=0.006;114±32 vs 58±25,p=0.000;233±45 vs 143±48,p=0.000;46.88% vs 14.581%,p=0.005;23.44% vs 6.25%,p=0.008).The ratio of depression and anxity were higher in the cognitive impairment MMD group compared with the non-cognitive impairment MMD group(59.52% vs 22.73%,p=0.000).The MMD patients with stoke had higher ratie in the cognitive disorder and anxiety(70% vs50 %,p=0.023;23.44 % vs 6.25%,p=0.000)than the MMD patients without stoke.The MMD patients with stoke' CBF value was less than MMD patients without stoke.The MMD patients with stoke' TTP values was longer less than MMD patients without stoke(43.58±8.87 vs 48.65±5.69,p=0.015;17.65±0.78 vs 13.53±0.64,p=0.002).The physiological function,role physical,social function and role emotional scores were less than the contrast.Multiariable analysis found that the PHQ-9 score and the educated years were the independent risk factors for SF-36 of the adult MMD patients.Conclusion: Adult MMD patients had more ratio of cognitive and emotional disorders than the contrast population.Brain CTP is applicable to assess cerebral perfusion in MMD patients.Cognitive impairment and depression related to frontal low perfusion,and anxiety related to the temporal lobe hypoperfusion.PHQ-9 scores and the educated years were the independent risk factors of the patients' quality of life.Adult MMD patients in China had severe psychological disorder.These patients need more attention.Part II The effect on the cognitive and emotional function of revascularization surgery for adult moyamoya diseaseObjective: To investigate the effect on the cognitive and emotional function of revascularization surgeon for adult moyamoya disease.Compare the advantage between combined revascularization surgery and indirect revascularization surgery in improving the MMD neuropsychological disorders.Methods: We selected the adult MMD patients who got the revascularization surgery hospitalized in the CHANGHAI Hospital from January 2015 to June 2016,and selected the adult MMD patients who refused surgery.Used several scales(MoCA,TMT-A,TMT-B,PHQ-9,GAD-7,SF-36)to assess the patients' neuropsychological function and qualities of life.Follow up all patients six months,and assess the patients' neuropsychological function once more.Compared the two groups' clinical characteristics and neuropsychological function.Then analyzed the related influencing factors.Used the SPSS17.0 software for statistical analysis,and P<0.05 was significant statistically.Results : The surgery group had 42 patients.16 patients got the combined revascularization surgery(STA-MCA+EDAS).26 patients got the indirect revascularization surgery(EDAS or MBH).22 patients who didn't get surgery were the contrast group.The surgery and contrast groups' baseline were no difference.The MoCAscore improved 6 months after operation?p<0.05?.There was no difference in the degree of improvement between the combined reconstruction group and the indirect revascularization group.After 6 months,the non-operate group ‘ MoCA score,PHQ-9and GAD-7 had no statistically difference compared with the beginning.Six months after operation,the score of emotion was decreased compared with that before operation?p<0.05?.The improvement of depression in MBH subgroup was better than the combined reconstruction group and the EDA subgroup.The SF-36 score of the operation group was higher than before.The combined operation group was more obvious in the improvement of vitality,and the improvement of physical function and mental health was more obvious in the indirect operation group,but there was no statistical difference.The physiological function,vitality and mental health of the non operation group were decreased(p < 0.05).Multivariate analysis showed that the improvement of CTP in brain CBF was the influencing factor of SF-36.Conclusion: Reconstruction surgery can improve the cognitive and emotional disorders of adult MMD patients after six months,and improve the quality of life of patients.There was no difference between STA-MCA combined with EDAS and indirect revascularization in these three aspects.Improvement of depression in MBH subgroup was better than the combined reconstruction group and the pure EDAS group.Surgery may improve MMD patients' neuropsychological function by improve cerebral perfusion,especially TTP and CBF.MBH was more significant in improving frontal lobe CBF.STA-MCA combined with MBH may improve cerebral perfusion better.Adult MMD patients with neuropsychological impairment but no stroke events are advised to perform MBH early.Part III The relationship between RNF213 gene polymorphism and cognitive and emotional state in patients with moyamoya disease.Objective: To investigate the relationship between rs12735431,rs138130613 and rs14873719 gene polymorphism in RNF213 and cognitive and emotional in adults with MMD.Methods: Choose the adult MMD patients.To evaluate the cognitive function of by using MoCA,TMT-A,TMT-B,PHQ-9 and GAD-7 to evaluate the emotion,and SF-36 to evaluate the quality of life.Got all the patients' peripheral blood,and extraction blood DNA.Three RNF213 ‘common mutations rs12735431,rs138130613,rs14873719 gene polymorphism was measured using Abi3730 instrument.To compare the difference ofneuropsychological disorders between different genotypes.Used the SPSS17.0 software for statistical analysis,and P<0.05 was significant statistically.Results:There were 42 adult MMD patients,including 22 male and 20 female,with a mean age of 40±10 years old.The mutation rate of RNF213 rsl12735431 was 21.42%(type GA9 cases,type GG 33 cases).The mutation rate of RNF213 rsl38130613 was9.52%(type CT 4 cases,type CC 38 cases).There was a difference in the distribution of GA genotypes between cognitive impairment group and non cognitive impairment group(9/30 vs 0/12,p=0.041).Patients with rsl12735431 GA genotype had earlier onset age and lower MoCA score(P=0.045;P=0.049)than those with GG genotype.Conclusion: The GA mutation of RNF213 gene in Chinese adult MMD patients was 21.42%,and the mutation rate of rsl38130613 was CT.The mutation rate was,and no rsl4873719 mutation was found in rsl12735431.Rsl12735431 GA genotype patients had the characteristic with early onset,lower MoCA score,more prone to vascular dementia.We need pay more attention to these rsl12735431 GA genotype MMD patients.