Hemorrhagic Moyamoya Disease Patients Research Articles

Bypass Surgery for Adult-Onset Hemorrhagic Moyamoya Disease: Analysis Classified by Site of Initial Bleeding

Prevention of rebleeding events is crucial for patients with hemorrhagic moyamoya disease (MMD), as these increase the risk of mortality. Bypass surgery is effective in preventing subsequent hemorrhage, particularly in patients with posterior hemorrhage, but its efficacy in those with anterior hemorrhage remains unclear. We analyzed the effects of surgical intervention, stroke risk factors, and radiological features on rebleeding events. Patients with hemorrhagic-onset MMD who were followed at our institution between 2000 and 2022 were included (41 adult patients, 45 hemispheres). Baseline characteristics and radiological features (anterior or posterior hemorrhagic site, Suzuki grade, posterior cerebral artery involvement, and periventricular anastomosis) were thoroughly reviewed. Of the 45 hemispheres, hemorrhage developed in 9 (20%) hemispheres, with a median duration until rebleeding of 38 (range: 1-44) months. Rebleeding rates were significantly lower in the surgical group than in the nonsurgical group (odds ratio: 0.09; 95% confidence interval [CI]: 0.01-0.79; P= 0.011), and Kaplan-Meier analysis revealed a significantly longer interval between bleeding events in the surgical group (1.3%/y vs. 5.3%/y; P= 0.002), especially in the anterior hemorrhage group (1.3%/y vs. 5.1%/y; P= 0.019). The hazard ratio of surgical intervention for rebleeding with initial anterior hemorrhage was 0.11 (95% CI: 0.01-0.98; P= 0.048). In the nonsurgical group, the presence of hypertension shortened the time to subsequent hemorrhage (P= 0.004). Surgical intervention may decrease the risk of rebleeding in hemorrhagic onset MMD patients, even in those presenting with anterior hemorrhage. Hypertension was a significant risk factor for rebleeding in nonsurgical patients.

Transdural Collateral Circulation Indicates Cerebral Ischemia in Moyamoya Disease

Moyamoya disease (MMD) is an idiopathic occlusive cerebrovascular disorder. The development of collateral circulation originates from the dural and pial collaterals. Currently, the clinical significance of transdural collateral in MMD has not been established. We sought to study the relationship between transdural collateral circulation and the side of relative cerebral ischemia in MMD. Data from MMD patients were collected at Xiangya Hospital from January 2016 to April 2022. A collateral circulation grading system with scores was established, the dominant side of transdural collateral with a higher point. Cerebral perfusion was used to identify the side of relative cerebral ischemia. A total of 102 patients were recruited. Results of digital subtraction angiography showed that 74 (72.5%) patients had transdural collaterals. The transdural collaterals were more common in patients with infarctions than in those with headaches or transient ischemic attacks (P= 0.0074). The dominant side for the formation of transdural collateral circulation was more easily found at the side of relative cerebral ischemia (P < 0.0001). Additionally, the side of the brain with a higher score of transdural collaterals was more likely to experience relative cerebral ischemia (P < 0.0001). There was no significant difference in the formation of transdural collateral circulation between ischemic and hemorrhagic MMD patients. Transdural collateral circulation was common in MMD patients. The transdural collaterals were associated with the occurrence of infarction. Transdural collaterals were well established on the cerebral ischemic side, which indicated higher ischemic levels in the ipsilateral than contralateral side.

Classify patients with Moyamoya disease according to their cognitive performance might be helpful in clinical and practical with support vector machine based on hypergraph.

Moyamoya disease (MMD) patients were now classified according to their cerebrovascular manifestations, with cognition and emotion ignored, which attenuated the therapy. The present study tried to classify them based on their cognitive and emotional performance and explored the neural basis underlying this classification using resting-state fMRI (rs-fMRI). Thirty-nine MMD patients were recruited, assessed mental function and MRI scanned. We adopted hierarchical analysis of their mental performance for new subtypes. Next, a three-step analysis, with each step consisting of 10 random cross validation, was conducted for robust brain regions in classifying the three subtypes of patients in a support vector machine (SVM) model with hypergraph of rs-fMRI. We found three new subtypes including high depression-high anxiety-low cognition (HE-LC, 50%), low depression-low anxiety-high cognition (LE-HC, 14%), and low depression-low anxiety-low cognition (LE-LC, 36%), and no hemorrhagic MMD patients fell into the LE-HC group. The temporal and the bilateral superior frontal cortex, and so forth were included in all 10 randomized SVM modeling. The classification accuracy of the final three-way classification model was 67.5% in average of 10 random cross validation. In addition, the S value between the frontal cortex and the angular cortex was positively correlated with the anxiety score and backward digit span (p<.05). Our results might provide a new perspective for MMD classification concerning patients' mental status, guide timely surgery and suggest angular cortex, and so forth should be protected in surgery for cognitive consideration.

Data-Independent Acquisition-Based Serum Proteomic Profiling of Adult Moyamoya Disease Patients Reveals the Potential Pathogenesis of Vascular Changes.

Moyamoya disease (MMD) is a chronic cerebrovascular disease with unknown etiology. The pathogenesis of vascular changes remains unclear. Ischemic and hemorrhagic adult MMD patients and healthy volunteers were enrolled to collect serum for data-independent acquisition (DIA)-based proteomic analysis and ELISA validation. DIA serum proteomic revealed that apolipoprotein C-I (APOC1), apolipoprotein D (APOD), and apolipoprotein A-IV (APOA4) were decreased. The reductases glutathione S-transferase omega-1 (GSTO1) and peptidyl-prolyl cis-trans isomerase A (PPIA) were upregulated, and ADAMTS-like protein 4 (ADAMTSL4) was downregulated in both ischemic and hemorrhagic MMD. Afamin (AFM) and transforming growth factor-beta-induced protein ig-h3 (TGFBI) increased in ischemic patients but decreased in hemorrhagic patients. Serum ELISA results confirmed that APOA4, APOC1, and APOD were decreased compared to controls. Then, we retrospectively analyzed biochemical indexes of 200 MMD patients. A total of 54 enrolled MMD patients showed decreased total cholesterol (TC) and high-density lipoprotein cholesterol (HDL-c). APOA4, APOC1, and APOD were vital factors in the HDL decrease in MMD patients. Lipoprotein dysfunction in MMD patients is involved in MMD. Intimal thickening by enhanced adhesion, middle layer vascular smooth muscle cell migration, and decreased lipid antioxidant function represented by HDL are potential pathogeneses of vascular changes in MMD.

MMP-9 as a Biomarker for Predicting Hemorrhagic Strokes in Moyamoya Disease.

Objective: This study was conducted in order to investigate the association of matrix metalloproteinase (MMP)-9 levels with phenotypes of moyamoya disease (MMD).Methods: This study included plasma samples from 84 MMD patients. The clinical variables of these patients were reviewed from the medical record. The serum concentration of tight junction, adherens junction proteins, and MMPs (MMP-2 and MMP-9) was determined using the ELISA method. Patients with hemorrhagic-onset MMD were compared with those with ischemic-onset MMD.Results: Compared with pediatric patients, the expression of MMP-9 was significantly higher, while the MMP-2 and vascular endothelial-cadherin were lower in adult patients. In adult subgroup analysis, hemorrhagic MMD patients exhibited significantly higher serum concentrations of MMP-9 compared with ischemic MMD patients. The ROC curve identified that a baseline serum MMP-9 level >1,011 ng/ml may be associated with spontaneous hemorrhage in adult MMD patients with 70.37% sensitivity and 71.88% specificity [area under curve (AUC), 0.73; 95% CI 0.597–0.864; P = 0.003]. A late Suzuki stage (>4) (OR 4.565, 95% CI 1.028–20.280, P = 0.046) and serum concentrations of MMP-9 >1,011 ng/ml (OR 7.218, 95% CI 1.826–28.533, P = 0.005) are risk predictors of hemorrhages in MMD patients. Hemorrhagic-type MMD patients had higher serum levels of MMP-9 and BBB permeability compared with ischemic-type MMD patients. Adult MMD patients had higher serum levels of MMP-9 and BBB permeability compared with pediatric patients.Conclusions: MMP-9 might serve as a biomarker for hemorrhage prediction in MMD. Serum MMP-9 level >1,011 ng/ml is an independent risk factor of MMD hemorrhagic strokes.

Proteomic Profiling of Exosomes From Hemorrhagic Moyamoya Disease and Dysfunction of Mitochondria in Endothelial Cells.

Moyamoya disease (MMD) is a rare steno-occlusive and slowly progressing cerebrovascular disorder. The detailed mechanism of the underlying pathogenesis is still blurry. Tandem Mass Tag-labeled quantitative proteomics was performed on serum-derived exosomes (SDEs) extracted from adult patients diagnosed with pure ischemic MMD or hemorrhagic MMD and healthy controls. Then mouse brain vascular endothelial cell (EC), human umbilical vein EC, neuroblastoma cell, and human hepatocyte cell were treated with exosomes, and changes of the protein expression in mouse brain vascular EC cells were identified. Proteomics analysis results showed that 859 shared proteins were detected in SDEs from ischemic and hemorrhagic MMD patients with 231 differently expressed compared with healthy controls. Bioinformatic analysis revealed dysregulated cell growth and maintenance and indicated disturbed actin dynamics in MMD, with CFL1 (Cofilin-1) and ACTR2/3 (actin-related protein 2/3; also known as ARP2/3) downregulated in ischemic and hemorrhagic patients’ SDEs. We also found immunity dysfunction in hemorrhagic MMD. Following treatment with MMD SDEs, mouse brain vascular EC cells showed significantly higher levels of proliferation and more ethynyl-2-deoxyuridine-positive cells compared with the healthy control group, while there were no obvious changes in the human umbilical vein EC and human hepatocyte cell. Interestingly, we also found that SDEs from ischemic MMD promoted neuroblastoma cell proliferation. Proteomic analysis of mouse brain vascular EC cells suggested that SDEs from hemorrhagic MMD patients induced dysfunction of the mitochondria in cerebrovascular ECs. This study highlighted potential molecular mechanisms underlying the pathogenesis of MMD patients, thereby providing new therapeutic strategies for MMD.

Delayed Anastomotic Occlusion after Direct Revascularization in Adult Hemorrhagic Moyamoya Disease.

Delayed anastomotic occlusion occurred in a considerable proportion of hemorrhagic moyamoya disease (MMD) patients undergoing direct revascularization. This study aimed to investigate the predictors and outcomes of delayed anastomotic occlusion in adult hemorrhagic MMD. The authors retrospectively reviewed 87 adult hemorrhagic MMD patients. Univariate and multivariate logistic regression analyses were performed. After an average of 9.1 ± 6.9 months of angiographic follow-up, the long-term graft patency rates were 79.8%. The occluded group had significantly worse angiogenesis than the non-occluded group (p < 0.001). However, the improvement of dilated anterior choroidal artery–posterior communicating artery was similar (p = 0.090). After an average of 4.0 ± 2.5 years of clinical follow-up, the neurological statues and postoperative annualized rupture risk were similar between the occluded and non-occluded groups (p = 0.750; p = 0.679; respectively). In the multivariate logistic regression analysis, collateral circulation Grade III (OR, 4.772; 95% CI, 1.184–19.230; p = 0.028) and preoperative computed tomography perfusion (CTP) Grade I–II (OR, 4.129; 95% CI, 1.294–13.175; p = 0.017) were independent predictors of delayed anastomotic occlusion. Delayed anastomotic occlusion in adult hemorrhagic MMD might be a benign phenomenon. Good collateral circulation (Grade III) and compensable preoperative intracranial perfusion (CTP Grade I–II) are independent predictors for this phenomenon. Moreover, the delayed anastomotic occlusion has no significant correlations with the long-term angiographic and neurological outcomes, except neoangiogenesis.

Flow-augmentation bypass for moyamoya disease

Moyamoya disease (MMD) is a chronic, occlusive cerebrovascular disease with unknown etiology, which is characterized by progressive steno-occlusive changes at the terminal portion of the internal carotid artery and an abnormal vascular network formation at the base of the brain. MMD has an intrinsic temporal nature to attempt a gradual conversion of the vascular supply for the brain from intracranial/internal carotid (IC) system to extracranial/external carotid (EC) system, so called "IC-EC conversion." Compatible cerebrovascular angio-architecture could be found in association with a variety of conditions such as neuro-fibromatosis type-1, Down's syndrome and cranial irradiation, which is called as moyamoya syndrome, akin/quasi MMD, or secondary MMD. Diagnosis of moyamoya vasculopathy, either idiopathic or secondary, is clinically important because flow-augmentation bypass is markedly beneficial for this entity to prevent cerebral ischemic attack by improving cerebral blood flow. Moreover, recent evidence indicated that flow-augmentation bypass could prevent re-bleeding in hemorrhagic MMD patients. Based on these backgrounds, there is a worldwide increase in the number of MMD patients undergoing bypass surgery. We sought to demonstrate our standard surgical procedure of superficial temporal artery-middle cerebral artery bypass with indirect pial synangiosis for MMD and its technical pitfall. We also discuss the intrinsic peri-operative hemodynamics of MMD after bypass surgery, including local cerebral hyper-perfusion and characteristic hemodynamic ischemia caused by watershed shift phenomenon. The aim of this review article is to understand the basic pathology of MMD, which is essential for complication avoidance while conducting flow-augmentation bypass for MMD.

Cerebral Hemodynamic Changes After Revascularization in Patients With Hemorrhagic Moyamoya Disease.

Objective: To explore the cerebral hemodynamic changes after revascularization in patients with hemorrhagic moyamoya disease (MMD).Materials and Methods: We retrospectively included 57 hemorrhagic MMD patients in a high-volume stroke center from January 2016 to December 2018. All subjects were evaluated with whole-brain CT perfusion (CTP) before and after surgical revascularization. Absolute and relative CTP values in the regions of cortical middle cerebral artery territory (CMT) and deep brain area (DBA) of hemorrhagic hemispheres were measured. Differences between pre- and post-operative CTP values were assessed comprehensively. The patients were categorized into subgroups based on revascularization subtypes and postoperative CTP intervals.Results: The relative cerebral blood volume (rCBV) in DBA and CMT significantly reduced in postoperative CTP (P < 0.05). The median and interquartile range of the proportion of rCBV decrease (rCBVc%) were 7.2% (2.3–13.2%). The rCBV reduction retained statistical significant in patients who received subtypes of revascularization, and in patients with variable intervals of follow-up (P < 0.05). There was no significant difference of rCBVc% between patients who received different revascularization and among patients with different postoperative CTP intervals (P > 0.05). The relative mean transit time (rMTT) and relative time to peak (rTTP) also showed downward trends, but without retainable statistical significance in stratified analysis. There was no significant change in relative cerebral blood flow (rCBF) (P > 0.05).Conclusion: In patients with hemorrhagic MMD, the CBV appeared to decrease and be relatively stable in the chronic phase after revascularization, with varying degrees of MTT and TTP shortening. However, there was no significant change in CBF.

Functional Outcomes After Revascularization Procedures in Patients With Hemorrhagic Moyamoya Disease.

Poor natural history of hemorrhagic Moyamoya disease (MMD) is related to high rehemorrhage rates between 32% and 61%. Postrevascularization, rehemorrhage rates reportedly decrease to 12% to 17%. To evaluate long-term functional outcomes and rehemorrhage rates of hemorrhagic MMD patients treated with surgical revascularization and examine these in relation to clinical and radiological factors. Patients treated surgically for hemorrhagic MMD over a 26-yr period were identified. Modified Rankin scale (mRS) was used to assess clinical status at presentation and functional outcomes. Multivariable regression analyses were performed to evaluate the risk factors associated with rehemorrhage rates and functional outcomes. A total of 104 patients (mean age: 38.04 yr) were identified. The mean mRS score at baseline was 1.3. Of 172 revascularized hemispheres, 157 (91.3%) were direct superficial temporal artery (STA)-middle cerebral artery (MCA) bypasses and the rest indirect. Over the mean follow-up of 61.4 mo, 8 of 104 patients (7.7%) experienced rehemorrhage with rehemorrhage rate per person-years of 1.9%. A total of 4 patients died with 1 related to rehemorrhage. At the last follow-up, mean mRS score improved to 1.1. No significant risk factors were identified in relation to the rehemorrhage rates (P<.05). The patients' initial mRS score was positively associated with mRS scores at the final follow-up (P<.001). STA-MCA direct bypass was associated with better performance status (P=.033). Rehemorrhage rate following surgical revascularization of the hemorrhagic MMD patients at 7.7% is lower compared with much higher natural history rates. Surgical revascularization improved patients' performance status. These outcomes support performing revascularization procedure with a preference for direct STA-MCA bypasses.

Neuro-endoscopic management of hemorrhagic moyamoya disease in the acute stage: single institute experience

ABSTRACTBackground: Management of hemorrhagic moyamoya disease (MMD) is one of the most challenging issues in neurosurgical practice. Recently, surgical revascularization by superficial temporal artery-middle cerebral artery anastomosis was reported to significantly reduce the risk of re-bleeding in hemorrhagic-onset MMD patients in the chronic state, but the optimal treatment strategy in the acute phase of hemorrhagic MMD is undetermined. Thus, we retrospectively analyzed our surgical results for hemorrhagic MMD in the acute stage, focusing on the efficacy of neuro-endoscopic management for hematoma removal.Materials and methods: Among 26 consecutive hemorrhagic MMD patients who were managed at our institution in the acute stage, 8 patients with intracerebral hemorrhage underwent surgical evacuation of the hematoma. All patients were diagnosed with MMD before surgery by magnetic resonance angiography and/or catheter angiography.Results: The initial surgical procedure was neuro-endoscopic hematoma removal in seven patients and microsurgical hematoma removal by craniotomy in one patient. One patient treated by the neuro-endoscopic procedure required subsequent small craniotomy due to difficulty in hemostasis. Satisfactory hematoma evacuation was achieved in all eight patients, as demonstrated by post-operative computed tomography, although one patient who was deeply comatose before surgery died in the early post-operative period.Conclusion: Neuro-endoscopic hematoma removal may be the optimal management method for hemorrhagic MMD in the acute stage in terms of the maximum preservation of pre-existing collateral anastomosis without affecting the potential donor vessels used for future revascularization surgery.Abbreviations: MMD: Moyamoya disease; STA: Superficial temporal artery; MCA: Middle cerebral artery; JAM: Japan Adult Moyamoya; ICH: Intracerebral hemorrhage; IVH: Intraventricular hemorrhage; SAH: Subarachnoid hemorrhage; CT: Computed tomography; TE: Total evacuation; PE: Partial evacuation; SMR: Light mass reduction; MR: Magnetic resonance; mRS: Modified Rankin scale; GCS: Glasgow Coma Scale; ICP: Intracranial pressure.

Prognostic factors for adult patients with hemorrhagic moyamoya disease in the acute stage

ObjectivesHemorrhagic moyamoya disease (MMD) is one common subtype in adult patients. However, the study about outcome of hemorrhagic MMD patients in the acute stage is still lacking. This study is aimed to explore the short-term prognostic factors for adult patients with hemorrhagic MMD in the acute stage. Patients and MethodsAdult hemorrhagic MMD patients in the acute stage awere retrospectively analyzed. Both clinical and imaging data were collected. Unfavorable functional outcome at discharge was considered when modified Rankin Scale score ≥3. Multivariate logistic regression was used to investigate the prognostic factors in patients with hemorrhagic MMD in the acute stage. ResultsA total of 107 patients were included in this study. Among these patients, 17 died and 59 had unfavorable functional outcome at 9.6 ± 7.8 days. In multivariate logistic regression, admission blood glucose (odds ratio (OR) = 1.457, 95% confidence interval (CI) 1.156–1.836, P = 0.001), midline shift >5 mm (OR = 24.268, 95%CI 4.324–136.191, P < 0.001), and subarachnoid hemorrhage (OR = 13.067, 95%CI 2.020–84.512, P = 0.007) were independently associated with death at discharge. Moreover, admission Glasgow Coma Scale (GCS) score (OR = 0.420, 95%CI 0.296–0.598, P < 0.001), midline shift >5 mm (OR = 6.685, 95%CI 1.226–36.455, P = 0.028), and intraparenchymal hemorrhage (OR = 4.790, 95%CI 1.184–19.381, P = 0.028) were independently associated with unfavorable functional outcome at discharge. ConclusionThis study shows that admission blood glucose, midline shift >5 mm, and subarachnoid hemorrhage are independent predictors of short-term mortality in hemorrhagic MMD in the acute stage. In addition, admission GCS score, midline shift >5 mm, and intraparenchymal hemorrhage are independent predictors of short-term unfavorable functional outcome in hemorrhagic MMD in the acute stage.

Significance of cyclooxygenase-2 elevation in middle cerebral artery for patients with hemorrhagic moyamoya disease.

The etiology and pathogenesis of moyamoya disease (MMD) remain elusive. Some inflammatory proteins, such as cyclooxygenase (COX)-2, are believed to be implicated in the development of MMD. So far, the relationship between COX-2 and MMD is poorly understood and reports on the intracranial vessels of MMD patients are scanty. In this study, tiny pieces of middle cerebral artery (MCA) and superficial temporal artery (STA) from 13 MMD patients were surgically harvested. The MCA and STA samples from 5 control patients were also collected by using the same technique. The expression of COX-2 was immunohistochemically detected and the average absorbance (A) of positively-stained areas was measured. High-level COX-2 expression was found in all layers of the MCA samples from all 5 hemorrhagic MMD patients, while positive but weak expression of COX-2 was observed only in the endothelial layer of the MCA samples from most ischemic MMD patients (6/8, 75%). The average A values of COX-2 in the hemorrhagic MMD patients were substantially higher than those in their ischemic counterparts (t=4.632, P=0.001). There was no significant difference in the COX-2 expression among the "gender" groups, or "radiographic grade" groups, or "lesion location" groups (P>0.05 for all). The COX-2 expression was detected neither in the MCA samples from the controls nor in all STA specimens. Our results suggested that COX-2 was up-regulated in the MCA of MMD patients, especially in hemorrhagic MMD patients. We are led to speculate that COX-2 may be involved in the pathogenesis of MMD and even contribute to the hemorrhagic stroke of MMD patients.

Moyamoya disease presenting with subarachnoid hemorrhage: Clinical features and neuroimaging of a case series

Background. Hemorrhage is a major category of the clinical symptoms of moyamoya disease (MMD). Intracerebral and intraventricular hemorrhages are the most common hemorrhages in MMD, and subarachnoid hemorrhages (SAHs) are notably rare. There have only been a few previous case reports of this imaging manifestation. In the present study, we investigated the clinical and neuroimaging features of MMD patients who presented with SAH and sought to identify the possible causes of hemorrhage. Methods. The right and left cerebral hemispheres of each patient were identified as hemorrhagic or non-hemorrhagic. Each hemisphere was assessed for Suzuki stage, and dilatation and branch extension of the anterior choroidal artery (AChA) and posterior communicating artery (P-CoM) and for the degree of transdural anastomosis and posterior circulation compensation. Next, these data were statistically analyzed for correlations with the SAH events. Results. In 34 of 349 hemorrhagic MMD patients, the first episode of hemorrhage was a SAH, among which, 26 (76.5%) patients were women and there was only one pediatric patient. The Suzuki stage was not statistically correlated with the SAH events. There were also no correlations between SAH and dilatation or abnormal branching of the AChA and P-CoM. In contrast, SAH and transdural anastomosis were correlated. 25 of the 34 hemorrhagic hemispheres (73.5%) were positive for transdural anastomosis, and this number was 15 (44.1%) in the non-hemorrhagic hemispheres; this difference was statistically significant (P<0.05). Conclusions. SAH is an important type of hemorrhage in MMD patients and ranks as the fourth most common type after intracerebral hemorrhage (ICH), intraventricular hemorrhage (IVH), and ICH + IVH. It mainly occurs in adult women, and the rupture of the transdural anastomosis might be the main cause of this condition.

Outcome in adult patients with hemorrhagic moyamoya disease after combined extracranial-intracranial bypass.

The outcome of patients with hemorrhagic moyamoya disease (MMD) after cerebral revascularization is uncertain. The purpose of this study was to delineate the efficacy of this surgical method in the treatment of hemorrhagic MMD. Between January 2007 and August 2011, a consecutive cohort of 113 patients with hemorrhagic MMD was enrolled into this prospective single-center cohort study. The surgical method was combined direct and indirect bypass. The cumulative probability of the primary end point (all stroke and deaths from surgery through 30 days after surgery and ipsilateral recurrent hemorrhage afterward) was analyzed. The angiographic outcome was measured by the following parameters: bypass patency, reduction of basal MMD vessels, improved degree of dilation, and branch extension of the anterior choroidal and posterior communicating arteries (AChA-PCoA). Of the 113 enrolled cases, CT scans revealed pure intraventricular hemorrhage (IVH) in 63 cases (55.7%), pure intracranial hemorrhage (ICH) in 14 cases (12.4%), and ICH with IVH in 36 cases (31.9%). In 74 of 113 hemorrhagic hemispheres (65.5%), the AChA-PCoA was extremely dilated with extensive branches beyond the choroidal fissure. A total of 114 surgeries were performed. No patient suffered ischemic or hemorrhagic stroke through 30 days after surgery. Ipsilateral rebleeding occurred in 5 patients, 4 of whom died of the rebleeding event. The cumulative probability of the primary end point was 0% at 1 year and 1.9% at 2 years. The annual rebleeding rate was 1.87%/person/year. The improvement in AChA-PCoA extension was observed in 75 of 107 operated hemispheres (70.1%), which was higher than that in 7 of 105 unoperated hemispheres (35.2%). Revascularization may provide a benefit over conservative therapy for hemorrhagic MMD patients. The improvement of dilation and branch extension of AChA-PCoA might be correlated with the low rebleeding rate.

Assessment of cognitive function in adult patients with hemorrhagic moyamoya disease who received no surgical revascularization

Due to controversial surgical treatment for hemorrhagic moyamoya disease (MMD), a large proportion of these patients chose conservative treatment. The aim of this study was to assess cognitive function in adult patients with hemorrhagic MMD who received no surgical revascularization. Twenty-six adult hemorrhagic MMD patients with only intraventricular hemorrhage (IVH) confirmed by positive computed tomography and magnetic resonance imaging scan, 20 patients with spontaneous IVH whose digital subtraction angiography results were negative, and 30 healthy controls were identified and matched for age, gender, education background and living area. Cognitive function was evaluated by Montreal Cognitive Assessment (MoCA). The non-parametric test was used for comparisons among the three groups. No patient was confirmed cognitive dysfunction at the initial screening. Twenty-four (92%) cases presented mild cognitive impairment (MCI) after 1 year. All the cases demonstrated MCI after 2 years. The difference between cases and healthy controls was statistically significant at the second screening (P = 0.000) and the third screening (P = 0.000), as was that between cases and patients with spontaneous IVH at the second screening (P = 0.000) and the third screening (P = 0.000). In addition, there were significant decreases in all MoCA subscores (P = 0.000) with special regards to delayed recall, visual space and executive function in cases compared with the other two groups. Moreover, significant differences were found in the subgroups of smoking (P = 0.021) and Suzuki angiographic classification of MMD (P = 0.030). Cognitive impairment is a long-term complication for adult hemorrhagic MMD patients who underwent conservative treatment.

Quality of life and psychological impact in adult patients with hemorrhagic moyamoya disease who received no surgical revascularization

ObjectivesSurgical treatment for adult hemorrhagic moyamoya disease (MMD) remains controversial. A large proportion of Chinese adult patients with hemorrhagic MMD still choose conservative treatment. In this study, we investigated to assess psychological function and quality of life (QoL) in adult patients with hemorrhagic MMD who received no surgical revascularization. Methods26 adult patients with hemorrhagic MMD who presented with only intraventricular hemorrhage (IVH), 20 patients with spontaneous IVH whose DSA results were negative and 30 healthy controls were identified and matched for age, gender, living area, etc. Psychological function and QoL were evaluated by Short Form-36 (SF-36), Symptom Check List 90 (SCL-90), Self-rating Depression Scale (SDS), Self-rating Anxiety Scale (SAS) and daily life questionnaire respectively one year after the initial stroke. Multiple logistic regression model was built up to screen out the independent risk factors related to depression and reduced QoL. ResultsHeavier social and mental burden was observed in adult patients with hemorrhagic MMD compared with the patients with spontaneous IVH. The QoL of cases was particularly reduced in the psychological domains. 19 (73%) cases developed depression, indicating the probable higher incidence of psychological disorder in Asian patients. Multiple logistic regression analysis suggested the independent risks of reduced QoL and depression involved in personality types and education background. ConclusionsOur data revealed that poor education background or introverted personality type may be attributed to the development of depression in Chinese adult hemorrhagic MMD patients who received no surgical revascularization associated with QoL impairment. The treatment decisions for these patients should consider the possible improvement of QoL.