RESUMO
Objective:To investigate the effect of aspirin on the outcomes in adult patients with ischemic moyamoya disease treated with encephaloduroarteriosynangiosis (EDAS).Methods:Adult patients with ischemic moyamoya disease treated EDAS in the Department of Neurosurgery, the Fifth Medical Center, PLA General Hospital from January 2015 to September 2018 were enrolled retrospectively. The control group only received EDAS treatment, and the aspirin group received EDAS and aspirin antiplatelet treatment. The data of the both groups were analyzed retrospectively and the effective rate of operation, the incidence of perioperative intracerebral hemorrhage, the incidence of recurrent cerebrovascular events at 6 months after operation and the improvement rate of the modified Rankin Scale (mRS) score were compared.Results:A total of 120 adult patients with ischemic moyamoya disease were enrolled, including 60 in the aspirin group and 60 in the control group. EDAS was performed on 107 cerebral hemispheres in both groups. The operative effective rate in the aspirin group was significantly higher than that in the control group (82.24% vs. 65.42%; χ2=7.836, P=0.005). There was no perioperative cerebral hemorrhage event in the aspirin group and the control group. There was no significant difference in the incidence of cerebral infarction within 6 months after operation, but the incidence of transient ischemic attack in the aspirin group was significantly lower than that in the control group (15% vs. 40%; χ2=9.404, P=0.002). In addition, the improvement rate of mRS score in the aspirin group at 6 months after operation was significantly higher than that in the control group (85% vs. 63.33%; χ2=7.350, P=0.007). Conclusions:The combination of EDAS and aspirin can effectively improve the outcomes of adult patients with ischemic moyamoya disease without increasing the risk of perioperative intracerebral hemorrhage.
RESUMO
Objective:To investigate the correlation between RNF213 gene p. R4810K polymorphism and posterior cerebral artery involvement in Chinese children with familial moyamoya disease.Methods:Children with familial moyamoya disease admitted to the Department of Neurosurgery, the Fifth Medical Center of PLA General Hospital from August 2004 to June 2018 were enrolled, and they were divided into posterior cerebral artery involved group and posterior cerebral artery uninvolved group. RNF213 gene p. R4810K single nucleotide polymorphism was detected. Multivariate logistic regression analysis was used to determine the independent risk factors for posterior cerebral artery involvement. Results:A total of 65 children with familial moyamoya disease were enrolled. Their age was 6.98±4.46 years and 37 (56.9%) were male. The first symptom of 55 children (84.6%) was cerebral ischemia, and 37 (56.9%) involved posterior cerebral artery. There were 3 (4.6%) children with p. R4810K AA genotype, 26 (40.0%) with GA genotype, and 36 (55.4%) with GG genotype. The p. R4810K genotype distribution in the posterior cerebral artery involved group was statistically different from that in the uninvolved group (GA+ AA genotype: 56.8% vs. 28.6%; χ2=5.124, P=0.024), and there were no statistical difference in gender, age, first symptom, and genetic pattern. Multivariate logistic regression analysis showed that after adjusting the first onset age and gender, p. R4810K G>A mutation was the only independent risk factor for posterior cerebral artery involvement (odds ratio 3.240, 95% confidence interval 1.082-9.705; P=0.020). Conclusion:The p. R4810K polymorphism of RNF213 gene is associated with posterior cerebral artery involvement in Chinese children with familial moyamoya disease.
RESUMO
Objective To preliminarily explore the clinical features,treatment,and outcomes of moyamoya disease in the elderly.Methods The clinical data of the elderly patients with moyamoya disease (aged ＞ 60 years) admitted to the Department of Neurosurgery,the 307th Hospital of PLA from May 2007 to July 2016 were collected retrospectively.Their clinical features,imaging features,and surgical outcomes were analyzed.Results A total of 68 patients were enrolled,including 35 females (51.47％) and 33 males (48.53％).The ratio of male to female was 1:1.06.The age at the time of diagnosis of moyamoya disease was 62.82 ±3.08 years.Fifty-two patients (76.5％) had vascular risk factors.The most common clinical manifestation was cerebral ischemia (n =61,89.7％).Thirty of them (44.1％) presented as transient ischemic attack.The Suzuki staging of most patients was 4-6 (71.6％),12 patients (17.6％) complicated with posterior cerebral artery stenosis or occlusion.Thirty-one patients were treated with encephalo-duroarterio-synangiosis (EDAS).Among them,17 patients underwent bilateral surgery and 14 underwent unilateral surgery.The incidence of perioperative infarction or hemorrhage was 5.6％ (2 patients developed cerebral infarction and 1 patient developed cerebral hemorrhage);37 patients received conservative treatment.During the follow-up period,5 patients developed cerebral infarction (1 in the surgical treatment group and 4 in the conservative treatment group);there was no significant difference between the 2 groups.There were no significant differences in age,sex,vascular risk factor,clinical symptoms,and preoperative modified Rankin Scale (mRS) scores between the 2 groups.Cerebral angiography was performed 6-9 months after operation in the surgical treatment group.A total of 24 cerebral hemispheres were evaluated by Matsushima typing,of which 17 (70.8％) were excellent.During the follow-up period,the proportion of patients with clinical outcome excellent (the mRS score was 0) (Z =-5.268,P ＜ 0.00l) and clinical improvement (the mRS score was improved ≥ 1 compared to the baseline) (Z =-3.780,P ＜ 0.001) were significantly higher than the conservative treatment group.Conclusions The clinical symptoms of old patients with moyamoya disease were mainly cerebral ischemia.Most of them had vascular risk factors,and the imaging manifestations showed higher Suzuki staging.The perioperative risk of EDAS in the old patients with moyamoya disease was lower.It might be an effective method to prevent clinical symptoms progress and improve the outcomes.
RESUMO
Objective To detect the serum levels of angiogenic factors and inflammatory cytokines in patients w ith moyamoya disease and explore their roles in the pathogenesis of the disease. Methods The serum levels of vascular endothelial grow th factor (VEGF), angiopoietin -1 (Ang-1), interleukin-8 (IL-8), granulocyte colony stimulating factor (G -CSF), granulocyte-macrophage colony stimulating factor ( GM-CSF) and monocyte chemotactic protein 1 (MCP -1) in 56 patients w ith moyamoya disease and 26 healthy controls w ere measured by cytometric bead array. Results The serum levels of VEGF (2.81 ± 1.77 pg/ml vs.1.98 ±0.66 pg/ml; t = 3.081, P = 0.003 ) and IL-8 (0.89 ±0.69 pg/ml vs.0.63 ± 0.45 pg/ml; t'=2.0371, P < 0.05) in the moyamoya disease group w ere significantly higher than those in the healthy control group, and the serum level of Ang -1 in the moyamoya disease group w as significantly low er than that in the healthy control group (830.01 ±289.29 pg/ml vs.961.65 ±232.87 pg/ml; t =-2.032, P =0.045). Conclusions There are significant difference in serum levels of VEGF, Ang -1 and IL-8 betw een patients w ith moyamoya disease and healthy controls. The results indicate that angiogenic factors and inflammatory cytokines play some roles in the pathogenesis of moyamoya disease.
RESUMO
Objective To preliminarily investigate the clinical features and efficacy of surgical treatment of moyamoya syndrome in patients w ith hyperthyroidism. Methods From December 2002 to April 2013, 41 patients w ith moyamoya syndrome based on the disease of hyperthyroidism admitted to the Department of Neurosurgery, the 307th Hospital of PLA w ere analyzed retrospectively. The clinical data w ere colected, including sex, age of onset, initial symptoms, progress symptoms, imaging features, Suzuki staging, and surgical efficacy, and they w ere compared w ith the clinical data of the patients w ith moyamoya disease treated at the same time. Results The ratio of male to female w as about 1∶4 in moyamoya syndrome patients w ith hyperthyroidism. The peak age of onset w as 25 to 34 years old. Compared w ith the patients w ith moyamoya disease at the same period, the proportion of patients w ith cerebral infarction as initial symptom w as higher in the moyamoya syndrome group ( 39.0% vs.24.2%; χ2 = 4.796, P =0.029), more patients had symptomatic progression (46.3% vs.25.4%; χ2 =9.207, P =0.002), and the proportion of of patients w ith cerebral hemorrhage as initial symptom w as low er ( 2.4% vs.14.6%; χ2 = 4.829, P =0.028). Thirty-seven patients w ho received encephalo -duro-arterio-synangiosis (EDAS) w ere folow ed up for 43 ± 19 months. The results show ed that the clinical symptoms of 31 patients had different degrees of improvement. Conclusions Moyamoya syndrome of hyperthyroidism is more common in w omen. The risk of cerebral infarction is higher and more prone to have disease progression. The efficacy of EDAS is better for controling the progression of moyamoya syndrome in patients w ith hyperthyroidism.
RESUMO
Objective To investigate the clinical and angiographic features in hemorrhagic moyamoya disease patients with the posterior circulation involvement (PCI). Methods The clinical and imaging data of 224 patients with hemorrhagic moyamoya disease from December 2002 to December 2011 were analyzed retrospectively. The patients were divided into either a PCI group (n=57)or a non-PCI group (n=167) according to whether they had PCI or not. Results (1)Suzuki staging concentrated in stageⅤandⅥin the PCI group,accounting for 42. 1%(24/57)and 40. 4%(23/57)respectively;Suzuki staging concentrated in stage Ⅲ and Ⅳ in the non-PCI group,accounting for 31. 1%(52/167)and 41. 9%(70/167)respectively. There was significant difference in the distribution of Suzuki staging between the patients of both groups (P <0. 01). (2)In the PCI group,the thalamic hemorrhage,intraventricular hemorrhage,ganglia hemorrhage,subarachnoid hemorrhage accounted for 43. 9%(26/57),38. 6%(22/57), 3. 5%(2/57),14. 0%(8/57),respectively;There was no cerebral lobe hemorrhage. In the non-PCI group, the thalamic hemorrhage,intraventricular hemorrhage,basal ganglia hemorrhage,subarachnoid hemorrhage and cerebral lobe hemorrhage accounted for 0,37. 7%(63/167),25. 1%(42/167),15. 6%(26/167),21.6 (36/167),respectively. The proportion of hemorrhage types between the PCI group and non-PCI group was significantly different(P<0. 01). (3)The dilation of anterior,posterior choroidal artery ,and patency of posterior communicating artery,posterior pericallosal artery in bleeding side accounting for 19. 3%(11/57),82. 5%(47/57),36. 8%(21/57),78. 9%(45/57)in PCI group and 36. 5%(61/167),3. 0%(5/167),68. 9%(115/167),80. 2%(134/167)in non-PCI group. The ratio of of abnormal vessels between PCI group and non-PCI group had significantly statistical difference (P<0. 01). Conclusion Hemorrhagic moyamoya with PCI is common. Suzuki staging is usually in the advanced stage of vascular staging of moyamoya disease. The thalamic hemorrhage is the main type of bleeding. Posterior choroidal artery rupture may be the main reason of hemorrhage.
RESUMO
Objective To investigate the clinical features of moyamoya disease in children and the prognosis of encephaloduroarteriosynangiosis ( EDAS) . Methods According to the age of first operated patients,317 children with moyamoya disease who received EDAS from January 2004 to December 2010 were divided into 3 groups:infant group (n=16,<3 years of age),preschool group (n=42,3 to 6 years of age),and adolescent group (n=259,6 to 17 years of age). The clinical data and the efficacy of operation of the patients were analyzed retrospectively. Results (1) Among the 3 groups of patients,the incidences of cerebral infarction in the infant group (81. 2%,13/16) or the preschool group (69. 0%,29/42) before procedure were significantly higher than the adolescent group (48. 3%,125/259). There were significant differences (χ2 =11. 741,P<0. 01). (2) Before surgical intervention,the infarct volume enlargement or the recurrence of infarction rate at different parts of brain in the infant group (62. 5%,10/16) was higher than that of the preschool group (31. 0%,13/42) and adolescent group (3. 9%,10/259). There was significant difference (χ2 =77. 437,P <0. 01). (3) The overall rate of favourable prognosis was 86. 4% (274/317). There were significant differences between the 3 groups (χ2 =9. 026,P<0.02). Conclusion The conditions of children with moyamoya disease progresses rapidly and their clinical prognosis is poor. It is safe and effective to perform EDAS early moyamoya disease in children.