Natural History Of Moyamoya Disease In Chinese Adult Patients

Objective The study aims to clarify the incidence and clinical features of disease progression in adult moyamoya disease,and realize the predictor of disease progression.Method 113 adult patients who were diagnosed with moyamoya disease at 307 Hospital(between 2002.12.25 and 2010.1.31)were enrolled in this study. The symptoms at onset of all the case experiences transient ischemic attack (TIA). The patients with and without disease progression were categorized into the progression group and stable group. Categorical variables as onset age,gender,symptoms type at onset,TIA frequency change,TIA time change, angiographical stage, spontaneously developed artery anastomosis were compared between two group.Results 1.Demographic Features: 113 patients were enrolled in this study,there were 41 male and 72 female patients,a male-to-female ratio is 1:1.76.Disease progression occurred in 56 of 113 patients (49.6%, 21 male and 35 female) during the follow-up period。With the age distribution of moyamoya disease, there was a major peak among patients aged 30 to 39 years(47.5%),and there was no significant difference in onset age distribution between the 2 groups.2.natural factor:①patients'gender:there were 21 male and 35 female patients in the progression group,and 20 male and 37 female in the stable group;②Onset age:the mean age at onset was36.09±6.95 years in the progression group,and the stable group was32.88±8.24years;③Symptoms type at onset: There were 33 patients perform limb dysfunction,4 patients about visual impairment and 19 patients about headache in the progression group.In the stable group,There were 30 patients perform limb dysfunction and 27 patients about headache.④TIA frequency: during the follow-up period,There were 39 patients perform increase about the TIA frequency,and 17 patients perform stable in the progression group.And the TIA frequency perform increase or stable in the stable group was 18:39;⑤TIA time:during the follow-up period,There were 10 patients perform extension about the TIA time,and 46 patients perform stable in the progression group.And the TIA frequency perform extension or stable in the stable group was 6:51. As the results of univariate analysis, there was a significant difference in onset age and TIA frequency change between the stable group and the progression group.(p<0.05).3.Angiographical stage:Suzuki's Angiographical stage of the progression group were graded according to the progression side,and the stable group were according to the severe side.As the result,the progression group:Ⅱ-Ⅵstage respectively is 8.9%,17.9%,35.7%, 23.2%, 14.3%;and the stable group:Ⅰ-Ⅵstage respectively is 3.5%,10.5%,12.3%, 36.8%,24.6%,12.3%.There was no difference between two group.4.Spontaneously developed artery anastomosis: there were 18 patients with spontaneously developed ECA anastomosis and 25 patients with spontaneously developed posterior circulations anastomosis in the progression group;and the result with the stable group respectively is 17 and 26. There was no difference between two group.5.Disease progression:56 patients experienced cerebrovascular events.Of these,44 patients had ischemic stroke, and 12 had intracranial bleeding during follow-up periods.In the patients of ischemic stroke, there were 37 patients perform limb dysfunction,6 patients about visual impairment and 1 patients about both. In the patients of intracranial bleeding, 9 patients perform cerebral hemorrhage,2 patients about cerebral ventricle hemorrhage,1 patients about subarachnoid hemorrhage.6.Multivariate analysis: Multivariate logistic regression analysis showed that patients'TIA frequency change was an independent predictor of disease progression during follow-up periods (P=0.0035).The odds ratio conferred by a frequency change patient was 4.971 for disease progression.Conclusion: 1.This study is the first to focus on natural factor of the progression in the major intracranial arteries in a large population of patients with adult China moyamoya disease.The result could be provide some help for understanding the Natural history of moyamoya disease.2.The clinical features of the patients who's symptoms at onset experiences TIA is similar to the result about the Chinese adult Patients before.3.there was no difference in gender,symptoms type at onset,TIA time change between the stable group and the progression group,and although there was a difference in onset age between two group,it had no influence on disease progression.4.patients'TIA frequency change was an independent predictor of disease progression.The odds ratio conferred by a frequency change patient was 4.971 for disease progression.5.Several factors, including the aggravation of the stage,out of the balance of artery compensatory mechanism,cerebral hemodynamic changes were associated with the progression of the disease.Technical refinement and standard of the evaluation for the disease progression should be found.6.he incidence of disease progression in adult moyamoya disease is much higher than recognized before, and that who has a higher TIA frequency may be at higher risk for it.Careful and long-term neurological and radiological follow-up would be essential in adult patients with moyamoya disease to prevent additional stroke events and to improve their outcome.