73 Patients With Adult Moyamoya Disease: A Retrospective Case Series Study

Objective: To investigate the clinical characteristics of adult moyamoya disease, localizations, compensatory models,vascular stage and influencing factors of recurrence and rebleeding.Methods: From 2009 to 2014 admission in the second affilliated hospital of soochow university hospital, 73 cases of adult patients with moyamoya disease were enrolled and the clinical baseline data were collected such as the age of onset, the initial mode of onset and neuroimaging data,etc. The patients were devided into cerebral hemorrhage group and cerebral infarction group according to the initial onset of clinical manifestations, with comparing to the risk factors and imaging characteristics between the both groups. clinical inducing factors of rebleeding were also analyzed, and to reveal the probable factors affecting their prognosis.Results: Adult moyamoya diseases start at the age of 40-45 years, the initial onset of intracranial hemorrhage was 60.3%, which was more common in women. Age of onset and the initial mode of onset were not associated with gender. In cerebral hemorrhage group, the proportion of intraventricular and basal ganglia hemorrhage(68.2%) was higher than in infarction group(24.1%)(P<0.001), while the rate of patients with cortex infarction(41.4%) was higher than in hemorrhage group( 11.4%)(P = 0.003). 146 branches of lesion vessels were found by DSA, of which ICA was 112 branches(76.7%), MCA 25 branches(17.1%), and ACA branches artery(6.2%), for the lesion vessels, there were no significant differences between the both groups. 137 branches of cerebral vessels(93.8%) were compensated by deep penetrating artery, 90 branches(65.7%) compensated by Willis’ Ring, 101 branches of meningeal vessels compensation were 69.2%, 89 branches by posterior pericallosal artery(61.0%), ophthalmic artery compensation were 36 branches(24.7 %), and 16 branches of vessels(11.0 %) via middle meningeal artery; However, compensatory model via deep penetrating branches was the highest, and the lowest rate of compensation by middle meningeal artery. Ophthalmic artery compensatory manner was much higher in cerebral infarction group(37.9%) than in cerebral hemorrhage group(15.9%)(P = 0.003).There were no significant difference in the other models of compensation. The compensatory vessels of Suzuki gradeⅤ(34.5%) and Ⅵ(17.2%) in cerebral infarction group were significantly higher than in cerebral hemorrhage group(7.9%, 2.3%), while the compensatory vessels of Suzuki grade Ⅲ(39.8%) in hemorrhage group were much higher than the in patients with cerebral infarction(17.2%). By analyzing a number of clinical risk factors in the study, it was indicated that rebleeding was closely related to the morbidity of aneurysm(P = 0.014).Conclusion: Most onset of adult moyamoya diseases were cerebral hemorrhage.Cerebral hemorrhage located mainly in of the ventricles and adjacent area, which the compensatory vessels of Suzuki grade Ⅲ-Ⅳ were common; However, the locations of cerebral infarction in the cortex with chief compensatory vessels of Suzuki grade Ⅴ-Ⅵ.Collateral circulation played an important role in the compensation and regulation of cerebral blood flow, and rebleeding was closely related to the morbidity of aneurysm.