Effect of aspirin on the outcomes in adult patients with ischemic moyamoya disease treated with encephaloduroarteriosynangiosis / 国际脑血管病杂志

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.

Correlation between RNF213 gene p. R4810K polymorphism and posterior cerebral artery involvement in Chinese children with familial moyamoya disease / 国际脑血管病杂志

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.

Thinking about the diagnosis and treatment of moyamoya disease under the epidemic of coronavirus disease 2019 / 国际脑血管病杂志

Coronavirus disease 2019 (COVID-19), caused by the 2019 novel coronavirus (2019-nCoV), is spreading on a large scale in China. COVID-19 mainly affects the respiratory system and cause symptoms such as severe hypoxemia and high fever. At present, there is no specific treatment drug, and patients' autoimmunity is closely related to disease prognosis. The brain tissue of patients with moyamoya disease is in a state of ischemia and hypoxia for a long time. Hypoxemia and high fever will aggravate the cerebral ischemia and hypoxia in patients with moyamoya disease, and patients with moyamoya disease may also have autoimmune abnormalities. Because people with moyamoya disease are also likely to have COVID-19 and COVID-19 can cause many conditions that may aggravate the symptoms of patients with moyamoya disease, which brings new problems and challenges to the clinical diagnosis and treatment of moyamoya disease. In this article, the diagnosis and treatment process and prevention and control measures of patients with moyamoya disease under the current epidemic situation are thought, and the key issues in the treatment of patients with moyamoya disease after suffering from COVID-19 are sorted out. It is hoped to provide reference for the diagnosis and treatment of moyamoya disease under the COVID-19 epidemic situation.

Clinical features, treatment, and outcomes of moyamoya disease in the elderly / 国际脑血管病杂志

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.

Clinical features of familial moyamoya disease in China / 国际脑血管病杂志

Objective To investigate the clinical features of familial moyamoya disease in China. Methods The patients w ith familial moyamoya disease admitted to the department of Neurosurgery, the 307th Hospital of PLA from March 2009 to June 2012 w ere analyzed retrospectively. Results Of 1 108 patients w ith moyamoya disease admitted to the department of Neurosurgery, the 307th Hospital of PLA, 87 patients (7.8%) w ith familial moyamoya disease w ere identified. Familial moyamoya disease w as observed both in the Han nationality and the minority nationalities. The male to female ratio w as 1∶1.02. The age at first onset ranged from 8 months to 59 years. There w ere tw o peak ages -of-onset, 5-9 years and 30-34 years, respectively). The most common initial symptom w as cerebral ischemia (74 .7%). The first degree relatives w ere the most affected in patients w ith familial moyamoya disease (78/87, 89 .66%), in w hich the siblings accounted for most of the disease ( 38/78, 48.72%), and the proportions of mother-to-child inheritance (21/78, 26.92%) and father-to-child inheritance ( 19/78, 24.36%) w ere similar. Conclusions There are tw o peak ages-of-onset, cerebral ischemia is the most common initial symptom, the first degree relatives are the most affected in patients w ith familial moyamoya disease in China.

Headache in children w ith moyamoya disease / 国际脑血管病杂志

Headache is one of the most common symptoms in children w ith moyamoya disease. Its related pathogenesis, clinical manifestations, and operation effect remain unclear. This article review s the advances in headache in children w ith moyamoya disease by review ing the related literatures on the study of headache in children w ith moyamoya disease.

Clinical features and prognosis of patients with moyamoya disease and renal artery stenosis / 中国脑血管病杂志

Objective To investigate the clinical features and prognosis of patients with moyamoya disease and renal artery stenosis. Methods The clinical data such as the first symptom,intracranial vascular lesions,renal artery lesions,treatment and follow-up results of the 15 patients with moyamoya disease and complicated with renal artery stenosis (RAS group)and the baseline-matched 30 moyamoya diseased patients without renal artery stenosis (MMD group)treated at the Department of Neurosurgery,the 307 th Hospital of People′s Liberation Army from March 2009 to June 2012 were analyzed retrospectively. Results (1)The moyamoya diseased patients with renal artery stenosis accounted for 1. 6%(15 / 927)of the total number of patients with moyamoya disease admitted in the same period. In the RAS group,the left renal artery stenosis accounted for 5 cases,the right renal artery stenosis accounted for 4 cases,and the bilateral renal artery stenosis accounted for 6 cases. Mild stenosis accounted for 71. 4%(15 / 21 sides), proximal stenosis accounted for 85. 7% (18 / 21 sides). The incidence of hypertension (80. 0%,n = 12) of the RAS group was significantly higher than that of the MMD group (30. 0%,n =9). There was significant difference (χ2 = 10. 045,P Conclusion The incidence of hypertension in patients with RAS is significantly higher than that without RAS,and there is difference between the degree of intracranial vascular lesions and the patients without RAS. Renal artery stenosis is mainly the proximal mild stenosis. Interventional therapy is an effective method for the treatment of moyamoya disease with severe renal artery stenosis;however,the long-term prognosis needs to be further followed up.

Moyamoya disease associated w ith intracranial aneurysm：clinical features, risk factors of hemorrhage and treatment outcomes / 国际脑血管病杂志

ObjectiveToinvestigatetheclinicalfeatures,riskfactorsforbleedingandtreatment outcomes in moyamoya disease patients w ith intracranial aneurysms. Methods The clinical symptoms, location and size of aneurysm, treatment and the long-term folow-up results of the moyamoya disease patients w ith intracranial aneurysms w ere analyzed retrospectively. Results A total of 34 moyamoya disease patients w ith intracranial aneurysms (35 aneurysms) w ere enrol ed, including 22 (64.7%) in the intracranial hemorrhage group and 12 ( 35.3%) in the non-intracranial hemorrhage group. Of the 35 intracranial aneurysms, 23 (main artery type 11, peripheral artery type 12) w ere in the intracranial hemorrhage group and 12 (main artery type 11, peripheral artery type 1) w ere in the non-intracranial hemorrhage group. There w ere 29 smal aneurysms and 6 medium aneurysms (al w ere patients w ith hemorrhagic moyamoya disease). The aneurysms w ere mainly peripheral arterial type in the intracranial hemorrhage group, and the aneurysms w ere mainly artery type in the non-intracranial hemorrhage group. There w as significant difference in aneurysm typing betw een the tw o groups ( P= 0.013 ). Tw o patients did not perform encephalo-duro-arterio-synangiosis (EDAS) in the intracranial hemorrhage group, other patients and those of the non-intracranial hemorrhage group performed EDAS. Angiographical reexamination revealed that 3 patients w ith peripheral aneurysm disappeared, and 1 aneurysm recurred after aneurysm embolization, and the remaining aneurysms did not have any change. Long-term fol ow-up show ed that 1 patient died of sudden cerebral hemorrhage at 1 year after procedure in the intracranial hemorrhage group, and the others did not have ischemic or hemorrhagic stroke. The modified Rankin scale scores w ere improved in 21 patients. Conclusions There are differences in moyamoya disease patients w ith intracranial aneurysm typing w ith different clinical manifestations. Moyamoya disease patients w ith intracranial aneurysms are mostly smal aneurysms and they can not temporarily be treated directly and can perform EDAS directly. Intracranial aneurysms after procedure may remain long-term stability, and some peripheral aneurysms may disappear.