Posterior Cerebral Artery Stenosis Research Articles

Derivation and Validation of a Scoring System for Predicting Poor Outcome After Posterior Circulation Ischemic Stroke in China.

Guidelines for posterior circulation ischemic stroke (PCIS) treatment are lacking and outcome prediction is crucial for patients and clinicians. We aimed to develop and validate a prognostic score to predict the poor outcome for patients with PCIS. The score was developed from a prospective derivation cohort named the Third China National Stroke Registry (August 2015-March 2018) and validated in a spatiotemporal independent validation cohort (December 2017-March 2023) in China. Patients with PCIS with acute infarctions defined as hyperintense lesions on diffusion-weighted imaging were included in this study. The poor outcome was measured as modified Rankin scale (mRS) score 3-6 at 3 months after PCIS. Multivariable logistic regression analysis was used to identify predictors for poor outcome. The prognostic score, namely PCIS Outcome Score (PCISOS), was developed by assigning points to variables based on their relative β-coefficients in the logistic model. The PCISOS was derived from 3,294 patients (median age 62 [interquartile range (IQR) 55-70] years; 2,250 [68.3%] men) and validated in 501 patients (median age 61 [IQR 53-68] years; 404 [80.6%] men). Among them, 384 (11.7%) and 64 (12.8%) had poor outcome 3 months after stroke in respective cohorts. Age, mRS before admission, NIH Stroke Scale on admission, ischemic stroke history, infarction distribution, basilar artery, and posterior cerebral artery stenosis or occlusion were identified as independent predictors for poor outcome and included in PCISOS. This easy-to-use integer scoring system identified a marked risk gradient between 4 risk groups. PCISOS performed better than previous scores, with an excellent discrimination (C statistic) of 0.80 (95% CI 0.77-0.83) in the derivation cohort and 0.81 (95% CI 0.77-0.84) in the validation cohort. Calibration test showed high agreement between the predicted and observed outcomes in both cohorts. PCISOS can be applied for patients with PCIS with acute infarctions to predict functional outcome at 3 months post-PCIS. This simple tool helps clinicians to identify patients with PCIS with higher risk of poor outcome and provides reliable outcome expectations for patients. This information might be used for personalized rehabilitation plan and patient selection for future clinical trials to reduce disability and mortality.

Abstract WP226: Perioperative Stroke and Cardiac Anomalies Associated With Posterior Cerebral Artery Stenosis in Children With Moyamoya

Introduction: Moyamoya is a progressive steno-occlusive arteriopathy of the intracranial circulation affecting the anterior circulation and less often the posterior circulation. Risk factors and severity of disease progression in children with moyamoya and posterior cerebral artery (PCA) stenosis have not been well described in the literature. Previous research has shown an association between PCA stenosis and increased risk of post-operative stroke. We wanted to further explore this relationship in children with moyamoya and delineate the associated risk factors. Methods: Children with moyamoya who underwent surgical revascularization at our institution between December 2015 and December 2022 were reviewed for presence of PCA stenosis. Clinical and radiographic features were compared between children with moyamoya with and without PCA involvement. Categorical variables were compared using Fisher's exact test and continuous variables using Wilcoxon rank-sum. Associations between perioperative stroke and PCA stenosis were analyzes using a multivariate logistic regression. Results: In our cohort of 160 children with moyamoya, 45 (28%) had PCA stenosis; 26 of these 45 (57%) had PCA stenosis at time of diagnosis. Of the 19 children who did not have PCA stenosis at time of diagnosis but developed it during follow up, the median time to development of PCA stenosis was 1.0 years. Children with moyamoya and PCA stenosis had significant more cardiac anomalies than those without (p=0.02). Other associated disorders were found in 13 (28%) including trisomy 21 (6, 13%), cranial radiation (2,4%) and NF1 (3,7%). Interestingly, we found no significant difference in age, sex or prevalence of bilateral disease between the groups. Children with moyamoya and PCA stenosis had a 3.5 higher odds of having a stroke within 2 weeks of surgery (p=0.049). Conclusion: Children with moyamoya and PCA stenosis had a 3 fold higher odds of perioperative stroke compared to those without PCA stenosis. Genetic evaluations may be warranted given association of PCA stenosis and cardiac anomalies.

Abstract WP222: Basilar Artery Stenosis in Children With Moyamoya

Introduction: Moyamoya is a progressive cerebrovascular disease characterized by stenosis of the cerebral arteries in the anterior circulation and less often the posterior circulation. To our knowledge, studies examining the prevalence of basilar artery stenosis do not exist outside of case reports. Methods: We retrospectively examined children with moyamoya followed at a single institution from November 2011 through August 2023. Children with moyamoya and basilar stenosis mentioned in their chart were initially included. A pediatric neurologist reviewed all records and neuroimaging to verify diagnosis of basilar artery stenosis. Results: Out of our cohort of 331 children with moyamoya, 24 (7.2%) children had basilar artery stenosis. In the group of 24 children with moyamoya and basilar artery stenosis 63% were females. The median age at moyamoya diagnosis was 4.8 years (interquartile range [IQR] 2.2,8.3). At time of diagnosis 16 children (67%) had basilar artery stenosis. The median time from diagnosis was 0 years (IQR 0, 1.3). At time of diagnosis all children had anterior circulation stenosis (internal carotid artery, middle cerebral artery and/or anterior cerebral artery), 13 (54%) had posterior cerebral artery stenosis and 6 (25%) had vertebral artery stenosis. Post-operative strokes following revascularization surgery occurred in 8 (33%) out of the 24 children. An associated disorder was found in 11 (46%) including ACTA2, PHACE syndrome, cranial radiation, trisomy 21, sickle cell disease, neurofibromatosis type 1 and morning glory disc. Conclusion: To our knowledge we present the first study to examine prevalence of basilar artery stenosis in a large single center cohort of children with moyamoya. In our cohort 7.2% of children with moyamoya had basilar artery stenosis. A large proportion of children with basilar artery stenosis developed stroke post-operatively. Genetic evaluation may be warranted in children with moyamoya and basilar artery stenosis given that 46% of the children had an associated disorder identified.

A long-term study of posterior circulation changes after revascularization in patients with moyamoya disease.

This study aimed to explore the long-term course of posterior circulation changes and predictors in patients with moyamoya disease (MMD). The authors retrospectively enrolled patients who were diagnosed with MMD and underwent encephaloduroarteriosynangiosis (EDAS) surgery at the authors' department from December 2002 to September 2011. A comparative study between short-term (6-12 months) and long-term (≥ 9 years) follow-up angiography was conducted. The progression of lesions was defined from lower to higher stages of the posterior cerebral artery (PCA). Eighty-eight patients who received indirect EDAS were enrolled in the study. The mean age at first surgery was 28.1 ± 15.0 years. Among these 88 patients with MMD, 39 (44.3%) exhibited transient ischemic attack and 27 (30.7%) exhibited infarction, comprising 5 with occipital lobe infarction, 14 (15.9%) with hemorrhagic symptoms, and 8 (9.1%) with atypical symptoms as the initial symptoms. Heterozygous mutations occurred significantly more frequently in the cases that presented with PCA involvement. During follow-up, stage progression of PCA was observed in 21 patients (28 hemispheres). At short-term follow-up, 21/176 (11.9%) hemispheres had progression of steno-occlusive lesions in the PCA. At long-term follow-up, 7 (4.0%) hemispheres had progression of steno-occlusive lesions in the PCA. At short-term follow-up, the progression of steno-occlusive lesions in the PCA was associated with progression of the internal carotid artery. Stage progression of PCA occurred significantly more frequently in the cases with PCA involvement on preoperative angiography. Nine strokes (10.2%) occurred in 88 patients during long-term follow-up. Four patients (4.5%) presented with ischemic stroke, including 2 with occipital lobe infarctions. Progression of PCA stenosis is common in patients with MMD, even if the PCA is normal initially. Mutations of RNF213 p.R4810K may predict PCA involvement or progression. Follow-up of the PCA in MMD patients should be conducted, and timely surgical revascularization is needed.

Monitoring Posterior Cerebral Perfusion Changes With Dynamic Susceptibility Contrast-Enhanced Perfusion MRI After Anterior Revascularization Surgery in Pediatric Moyamoya Disease.

To determine whether dynamic susceptibility contrast-enhanced (DSC) perfusion magnetic resonance imaging (MRI) can be used to evaluate posterior cerebral circulation in pediatric patients with moyamoya disease (MMD) who underwent anterior revascularization. This study retrospectively included 73 patients with MMD who underwent DSC perfusion MRI (age, 12.2 ± 6.1 years) between January 2016 and December 2020, owing to recent-onset clinical symptoms during the follow-up period after completion of anterior revascularization. DSC perfusion images were analyzed using a dedicated software package (NordicICE; Nordic NeuroLab) for the middle cerebral artery (MCA), posterior cerebral artery (PCA), and posterior border zone between the two regions (PCA-MCA). Patients were divided into two groups; the PCA stenosis group included 30 patients with newly confirmed PCA involvement, while the no PCA stenosis group included 43 patients without PCA involvement. The relationship between DSC perfusion parameters and PCA stenosis, as well as the performance of the parameters in discriminating between groups, were analyzed. In the PCA stenosis group, the mean follow-up duration was 5.3 years after anterior revascularization, and visual disturbances were a common symptom. Normalized cerebral blood volume was increased, and both the normalized time-to-peak (nTTP) and mean transit time values were significantly delayed in the PCA stenosis group compared with those in the no PCA stenosis group in the PCA and PCA-MCA border zones. TTPPCA (odds ratio [OR] = 6.745; 95% confidence interval [CI] = 2.665-17.074; P < 0.001) and CBVPCA-MCA (OR = 1.567; 95% CI = 1.021-2.406; P = 0.040) were independently associated with PCA stenosis. TTPPCA showed the highest receiver operating characteristic curve area in discriminating for PCA stenosis (0.895; 95% CI = 0.803-0.986). nTTP can be used to effectively diagnose PCA stenosis. Therefore, DSC perfusion MRI may be a valuable tool for monitoring PCA stenosis in patients with MMD.

Childhood Primary Angiitis of the Central Nervous System Presenting as Isolated Middle Cerebral Artery Dissection

Isolated middle cerebral artery dissection and primary angiitis of the central nervous system are two rare but serious causes of ischemic stroke in children. We report a case of a patient who presented with headache, left-sided hemiparesis, and left hemi-hypoesthesia with acute cerebral infarction in the right middle cerebral artery territory. Brain magnetic resonance imaging showed isolated right middle cerebral artery dissection with arterial wall contrast enhancement extending beyond the length of the dissection and diffuse narrowing with stenosis in the right posterior cerebral artery suggesting angiitis. After exclusion of other diagnoses the patient was treated with corticosteroids and dual antiplatelet therapy. Follow up magnetic resonance imaging performed 4 months later showed partially improved contrast enhancement in the right middle cerebral artery and persistent narrowing and focal stenosis of the right posterior cerebral artery. Although rare, primary angiitis of the central nervous system should be considered in the differential diagnosis of acute stroke in children. High-resolution vessel wall imaging magnetic resonance studies can provide important information in determining the etiology of stroke.

The Potential Mechanism Behind Native and Therapeutic Collaterals in Moyamoya

Background and PurposeTo explore the genetic basis and molecular mechanism of native arteriogenesis and therapeutic synangiosis in moyamoya disease (MMD).MethodsAn angiography-based study using patients from a prospective trial of encephaloduroarteriosynangiosis (EDAS) surgery was performed. The spontaneous collaterals grades were evaluated according to the system described by a new grading system. Blood samples were collected from all the recruited patients before EDAS and during the second hospitalization 3 months post-EDAS. We performed Boolean analysis using a combination of specific cell surface markers of CD34briCD133+CD45dimKDR+. Genotyping of p.R4810K was also performed. The correlation of age, sex, initial symptoms at diagnosis, collateral grade, Suzuki stages, the RNF213 genotype, time to peak (TTP), and endothelial progenitor cell (EPC) count with good collateral circulation was evaluated.ResultsEighty-five patients with MMD were included in this study. The mutation rate of RNF213 p.R4810K in our study was 25.9% (22/85). The heterozygous mutations were occurred significantly more frequently in the cases that were presented with infarction, worse neurological status, severe posterior cerebral artery (PCA) stenosis, and longer TTP delay. Further, the heterozygous mutations occurred significantly more frequently in the poor collateral stage group. Lower grades were significantly correlated with severe ischemia symptoms, worse neurological status, and a longer TTP delay. The post-operative angiographic findings showed that a good Matsushima grade was correlated with heterozygous mutations, a lower collateral stage, and a longer TTP delay. The CD34briCD133+CD45dimKDR+ cell count in patients 3 months post-EDAS was significantly higher as compared to the count before EDAS in the good Matsushima grade group. However, this change was not observed in the poor Matsushima grade group.ConclusionsThese data imply that mutations of RNF213 p.R4810K affect the establishment of spontaneous collateral circulation, and EPCs are involved in the process of formation of new EDAS collaterals.

Posterior Cerebral Artery Stenosis Presenting With Amaurosis Fugax.

Posterior Cerebral Artery Stenosis Presenting With Amaurosis Fugax.

Spatially separate cerebral infarction in the posterior cerebral artery territory after combined revascularization of the middle cerebral artery territory in an adult patient with moyamoya disease and fetal-type posterior communicating artery: illustrative case.

BACKGROUNDRemote cerebral infarction after combined revascularization of the middle cerebral artery (MCA) territory is rare in patients with moyamoya disease (MMD) with a fetal-type posterior communicating artery (PCoA).OBSERVATIONSA 57-year-old woman developed numbness in her right upper limb and transient motor weakness and was diagnosed with MMD. She also had a headache attack and a scintillating scotoma in the right visual field. Preoperative magnetic resonance angiography (MRA) showed stenosis of the left posterior cerebral artery (PCA). Combined revascularization was performed for the left MCA territory. No new neurological deficits were observed for 2 days after the operation, but right hemianopia, alexia, and agraphia appeared on postoperative day (POD) 4. Magnetic resonance imaging showed a new left occipitoparietal lobe infarction, and MRA showed occlusion of the distal left PCA. After that point, the alexia and agraphia gradually improved, but right hemianopia remained at the time of discharge on POD 18.LESSONSCerebral ischemia in the PCA territory may occur after combined revascularization of the MCA territory in patients with fetal-type PCoA. For these cases, a double-barrel bypass or indirect revascularization to induce a slow conversion could be considered on its own as a treatment option.

Occipital artery to middle cerebral artery bypass in pediatric moyamoya disease: rescue therapy after failed revascularization.

Patients with pediatric moyamoya disease (PMMD) showing recurrent symptoms or decreased cerebral blood flow after initial revascularization therapy may require additional revascularization to improve their clinical condition. The authors evaluated the clinical and hemodynamic benefits of an occipital artery (OA)-middle cerebral artery (MCA) bypass for patients with PMMD who have undergone an initial revascularization procedure. The authors retrospectively identified 9 patients with PMMD who had undergone OA-MCA bypass between March 2013 and December 2017, and who had received a previous superficial temporal artery-MCA bypass. The following clinical data were collected: initial revascularization procedure, symptoms (presence or recurrence), pre- and postoperative cerebral blood flow and cerebrovascular reactivity (CVR) changes, posterior cerebral artery (PCA) stenosis, PCA-related and nonrelated symptoms, and latest follow-up. Preoperatively, all patients (n = 9) suffered non-PCA-related recurrent symptoms, and 4 had PCA-related symptoms. At 1-year follow-up, all patients with PCA-related symptoms showed complete recovery. Additionally, 8 (89%) patients with non-PCA symptoms experienced improvement. Only 1 (11%) patient showed no improvement after the surgical procedure. The mean pre- and postoperative CVR values of the MCA territory were 14.8% and 31.3%, respectively, whereas the respective mean CVR values of the PCA territory were 22.8% and 40.0%. The OA-MCA bypass is an effective rescue therapy to improve the clinical condition and hemodynamic changes caused by PMMD in patients who experience recurrent symptoms after initial revascularization.

Visual cortex changes in children with sickle cell disease and normal visual acuity: a multimodal magnetic resonance imaging study.

The visual system is primarily affected in sickle cell disease (SCD), and eye examination is recommended starting in late childhood. So far, to our knowledge, all studies have focused on the retina, neglecting the changes that might be present in the cortical portion of the visual system. We performed a multimodal magnetic resonance imaging (MRI) evaluation of the visual cortex in 25 children with SCD (mean age: 12·3±1·9 years) and 31 controls (mean age: 12·7±1·6 years). At ophthalmologic examination, 3/25 SCD children had mild visual acuity deficits and 2/25 had mild tortuosity of the retinal vessels. None showed optic pathway infarcts at MRI or Transcranial Doppler abnormal blood velocities, and 6/25 disclosed posterior cerebral artery stenosis (five mild and one severe) at MR-angiography. Compared to controls, SCD children had increased posterior pericalcarine cortical thickness, with a different trajectory of cortical maturation and decreased connectivity within medial and ventral visual neural networks. Our findings suggest that SCD affects the development and the tuning of the visual cortex, leading to anatomical and functional changes in childhood even in the absence of retinopathy, and set the basis for future studies to determine if these changes can represent useful predictors of visual impairment in adulthood, biomarkers of disease progression or treatment response.

An Analysis of Clinical Characteristics of Rare Bilateral Cerebral Peduncular Infarction.

Objective: To investigate the anatomical characteristics, clinical manifestations, and imaging features of bilateral cerebral peduncular infarction.Methods: A retrospective analysis was performed on 11 patients diagnosed with bilateral cerebral peduncular infarction in the Affiliated Hospital of Xuzhou Medical University from December 2014 to December 2018. Their clinical and imaging features were analyzed and summarized in combination with the relevant national and international literature.Results: Among all the patients, there were eight cases with a history of hypertension, four cases with a history of diabetes mellitus, and four cases with a history of smoking. Conscious disturbance was observed in nine cases, quadriplegia in seven cases, pseudobulbar paralysis in three cases, and ataxia in one case. Brain magnetic resonance (MR) scans of bilateral cerebral peduncles showed patchy abnormal shadows with a hypointense signal on T1-weighted imaging (T1WI) and apparent diffusion coefficient (ADC) and hyperintense signal on T2-weighted imaging (T2WI), fluid-attenuated inversion recovery (FLAIR), and diffusion-weighted imaging (DWI). Computed tomography angiography (CTA) scans of head and neck showed severe stenosis or occlusion of vertebral artery, basilar artery, or posterior cerebral artery. All the patients received standardized treatment for cerebral infarction. Six patients died while five were left disabled.Conclusion: Bilateral cerebral peduncle infarction may be related to cerebral perfusion insufficiency caused by the stenosis or occlusion of vertebrobasilar artery and its branches. The main clinical manifestations are locked-in syndrome and persistent vegetative state. The specific imaging feature of “Mickey Mouse ear”-like infarction is associated with a poor prognosis.

Analysis of the risk factors for poor prognosis of progressive cerebral infarction

Objective To analyze the risk factors affecting the poor prognosis of progressive cerebral infarction. Methods The clinical data of 326 patients with cerebral infarction admitted to Dermatology and Venereal Disease Prevention and Cure Hospital of Zaozhuang from January 2013 to March 2017 were retrospectively analyzed.The patients were divided into poor prognosis group(n=148) and good prognosis group(n=178) according to their prognosis during hospitalization.Univariate analysis and multivariate logistic regression analysis were used to explore the risk factors affecting prognosis. Results Statistical analysis showed that there were no statistically significant differences in sex composition, average age, diastolic blood pressure, LDL, anterior cerebral artery stenosis, posterior cerebral artery stenosis and vertebral basilar artery stenosis between the two groups (all P>0.05). The incidence rates of hypertension history (60.81% vs. 48.88%, χ2=1.683, P=0.031), diabetes history (41.89% vs. 30.90%, χ2=4.244, P=0.039), hyperlipidemia history (62.84% vs. 49.44%, χ2=5.875, P=0.015) in the poor prognosis group were higher than those in the good prognosis group.The systolic blood pressure in poor prognosis group was lower than that in the good prognosis group [(144±17)mmHg vs. (150±22)mmHg, t=2.928, P=0.004]. The body temperature[(38.10±0.22)℃ vs. (38.03±0.34)℃, t=2.240, P=0.026], FPG[(7.85±1.95)mmol/L vs. (6.91±1.77)mmol/L, t=4.558, P=0.000], TC[(5.21±1.66)mmol/L vs. (4.84±1.55)mmol/L, t=2.708, P=0.039], TG[(2.49±0.79 mmol/L vs. (2.30±0.54)mmol/L, t=2.483, P=0.014], HDL [(1.30±0.51)mmol/L vs. (1.17±0.44)mmol/L, t=2.470, P=0.014] and FIB[(4.37±1.67)g/L vs. (3.79±1.42)g/L, t=3.339, P=0.001], stenosis of internal carotid artery (31.08% vs. 20.79%, χ2=4.636, P=0.031), stenosis of middle cerebral artery(39.86% vs. 29.21%, χ2=4.083, P=0.043), and plaque of internal carotid artery (48.65% vs. 36.52%, χ2=4.882, P=0.027) in the poor prognosis group were higher than those in the good prognosis group.Multivariate regression analysis showed that history of hypertension, diabetes, hyperlipidemia, fever, decreased blood pressure, elevated TC, elevated TG, elevated HDL and elevated FIB were the risk factors for the prognosis of progressive cerebral infarction. Conclusion There are many risk factors affecting the poor prognosis of progressive cerebral infarction, and the physiological mechanism is complex.In clinical treatment, attention should be paid to patients' past diseases, prevention and treatment should be carried out according to different mechanisms. Key words: Infarction, middle cerebral artery; Prognosis; Risk factors; Factor analysis, statistical; Blood glucose; Cholesterol esters

Study on the relationship between post-circulatory lesions and cerebral infarction in children with ischemic moyamoya disease

Objective To explore the relationship between post-circulatory lesions and cerebral infarction in children with ischemic moyamoya disease (MMD). Methods A total of 44 children with ischemic MMD who were admitted to Department of Neurosurgery, Tianjin Medical University General Hospital from January 2010 to December 2018 were retrospectively enrolled into this study. All of them were divided into cerebral infarction group (15 cases) and non-cerebral infarction group (29 cases) according to whether they had cerebral infarction or not. Three cases were treated with encephalo-myosynangiosis (EMS), 5 cases with superficial temporal artery-middle cerebral artery anastomosis bypass (STA-MCA bypass) and 36 cases with EMS+ STA-MCA. All children were followed up by outpatient visits or telephone and were asked about the improvement of clinical symptoms and whether there were any cerebral infarction events. The outcome was assessed by the modified Rankin scale score (mRS). The clinical efficacy was compared between the 2 groups of children. The clinical factors affecting the children with cerebral infarction were further analyzed by single factor and multiple factor logistic regression. Results All 44 patients underwent successful operation. Postoperative complications occurred in 12 cases (27.3%, 12/44). There was no statistically significant difference in the incidence of complications between 2 groups [40.0% (6/15) vs. 28.7% (6/29), P=0.615]. The follow-up duration of 44 cases was 7.2±1.5 months (6-12 months). During the follow-up period, none of the children had cerebral infarction. Among the 44 cases, 35 (79.5%) had good outcomes and 9 (20.5%) had poor outcomes. There was no statistically significant difference in the favorable prognosis rate between the 2 groups [66.7% (10/15) vs. 86.3% (25/29), P=0.130]. Single factor analysis showed that MCA stenosis, PCA (posterior cerebral artery) stenosis and transient ischemic attack before operation were the influencing factors of cerebral infarction in children (all P < 0.05). Further multiple factor analysis showed that PCA stenosis was an independent risk factor for cerebral infarction in children (OR=5.465, 95% CI: 1.224-24.400, P=0.026). Conclusion In children with ischemic MMD, PCA stenosis is an independent risk factors for cerebral infarction, and whether there is cerebral infarction has no significant effect on the prognosis of those children. Key words: Moyamoya disease; Child; Factor analysis, statistical; Post-circulatory lesions; Cerebral infarction

Revascularization Surgery in Patients with Ischemic-Type Moyamoya Disease: Predictors for Postoperative Stroke and Long-Term Outcomes

Recurrent stroke after surgical revascularization is still a big issue for moyamoya disease (MMD). This study aims to identify predictors for postoperative stroke and unfavorable outcome in ischemic-type MMD. We identified a consecutive series of patients with ischemic-type MMD who underwent revascularization between January 2005 and December 2012. Predictors for postoperative stroke and functional outcomes were assessed with logistic and Cox regression analysis. A total of 346 patients underwent 437 revascularization procedures and the mean follow-up period was 4.0 years. The incidence of perioperative stroke was 6.9%. Being adult at onset (odds ratio [OR], 5.033; 95% confidence interval [CI], 1.447-17.506; P= 0.011) and posterior cerebral artery (PCA) stenosis (OR, 3.364; 95% CI, 1.588-7.265; P= 0.002) before surgery were predictors of perioperative stroke. The annual subsequent stroke rate beyond 30 days after surgery was 1.2%. Subsequent stroke events tended to occur throughout the first 5 years after surgery in adults, whereas in children they mainly occurred within the first 2 years after surgery. Age at onset (OR, 1.025; 95% CI, 1.003-1.048; P=0.023), ischemic stroke or transient ischemic attack at presentation (OR, 2.703; 95% CI, 1.062-6.875; P= 0.037), and PCA involvement (OR, 2.664; 95% CI, 1.462-4.854; P= 0.001) were associated with higher risk of overall postoperative stroke. PCA involvement (OR, 2.62; 95% CI, 1.33-5.15; P=0.005), internal carotid artery supraclinoid segment occlusion (OR, 2.76; 95% CI, 1.27-6.03; P= 0.011), and older age at onset (OR, 1.03; 95% CI, 1.01-1.05; P= 0.033) were predictive of unfavorable outcome. Patients with ischemic-type MMD at an older age and more severe angiopathy might be at higher risk of recurrent stroke and unfavorable outcome after revascularization.

Regression of White Matter Hyperintensity after Indirect Bypass Surgery in a Patient with Moyamoya Disease.

Radiologically, white matter hyperintensities (WMHs) are defined as confluent hyperintensities on T2-weighted and fluid attenuated inversion recovery (FLAIR) sequences, and generally thought to be the result of irreversible small-vessel disease secondary to arteriosclerosis. However, though rare and infrequent, the reversibility of WMHs has been reported.1 The transient disruption of the blood–brain barrier, small vessel ischemia, endothelial dysfunction and venous pressure has also been proposed to be the possible mechanism, but none of these hypotheses have been verified. A 41-year-old female without significant past medical history suddenly developed visual impairment of the left lower part of the visual field. MRI in a local clinic revealed acute cerebral infarction of the right occipital cortex (Fig. 1, arrowhead) and WMH of the right occipital lobe (Fig. 1, arrow) adjacent to the infarction. MRA showed multiple intracranial arterial stenosis consistent with Moyamoya disease (Fig. 1a). After a detailed workup in our university hospital, she was diagnosed as Moyamoya disease with severe stenosis of the right posterior cerebral artery (Suzuki stage 4/3), and her right hemisphere suffered misery perfusion (Fig. 1e). Neurite orientation dispersion and density imaging (NODDI)2 revealed increased isotropic volume fraction (Viso), which reflects water components of the brain parenchyma, in the most affected right occipital lobe where the infarction and WMH existed (Fig. 1f). Three months later, she underwent the indirect bypass surgery on the right side to improve her hemodynamic condition, which resulted in a new infarction (Fig. 1h–1j, white arrowhead). Two months after the operation, dramatic improvement of the perfusion of the right hemisphere was observed; at the same time, the WMH of the right occipital lobe had disappeared. The increase in Viso observed before the operation had also been subsided (Fig. 1l, arrow). Open in a separate window Fig. 1 Comparison of the MR images obtained before (a–f) and 1 year after the indirect bypass surgery (g–l). After the restoration of the hemodynamic impairment (e and k), the white matter hyperintensity (WMH) (arrow) adjacent to the occipital infarction (arrowhead) has completely disappeared, as well as the increase of Viso (f and I). ADC, apparent diffusion coefficient; ASL, arterial spin labeling; DWI, diffusion weighted image; FLAIR, fluid attenuated inversion recovery; Viso, isotropic volume fraction.

Correlation of Brain Perfusion with White Matter Hyperintensity, Brain Atrophy, and Cognition in Patients with Posterior Cerebral Artery Stenosis and Subjective Cognitive Decline.

BackgroundThis study aimed to investigate the correlation of brain perfusion with white matter hyperintensity (WMH), brain atrophy, and cognition in patients with moderate to severe posterior cerebral artery stenosis (PCAS).Material/Methods65 patients with memory decline as the main complaint and no history of brain infarction were recruited from the Department of Neurology of Tongji Hospital. Patients with moderate to severe PCAS were included in case group, and subjects with normal intracranial blood vessels served as controls. The demographics and vascular risk factors were recorded. Montreal Cognitive Assessment (MoCA) was used to evaluate the cognition. CT perfusion imaging was performed, and WASID was employed for the assessment of intracranial artery stenosis. The region of interest (ROI) was analyzed based on the whole brain perfusion. Cranial MRI was performed, and Scheltens scoring system was used for the assessment of WMH on FLAIR. T1 weighed images were obtained, and global cortical atrophy (GCA) scale was employed for the assessment of brain atrophy. The detections of brain perfusion, WMH and brain atrophy were done at centrum ovale, parietal lateral ventricle and basal ganglia layers.ResultsIn PCAS patients we found low perfusion in the antecornu and postcornu blood supply areas at the lateral ventricle, the blood supply area of the anterior cerebral artery, the blood supply area of the posterior cerebral artery, and the blood supply area at the hippocampus as compared with control subjects (p<0.05). As compared with control subjects, the incidence of WMH in the blood supply areas at the deep brain and lateral ventricle was significantly higher in PCAS patients (p<0.05). When compared with controls, the incidence of brain atrophy increased significantly in PCAS patients (p<0.01). Correlation analysis showed the brain perfusion at the blood supply area of the posterior cerebral artery was positively correlated to the total MoCA score and negatively correlated to the severity of WMH at the blood supply area of the posterior cerebral artery (p<0.05). Further analysis showed the brain perfusion at the blood supply area of the posterior cerebral artery was negatively associated with cortex supplied by the posterior cerebral artery, posterior cingulate, and hippocampus (p<0.01).ConclusionsPCAS patients have a higher incidence of brain atrophy, and the perfusion at the area supplied by the posterior cerebral artery is correlated to the severity of brain atrophy and of WMH, as well as to cognition decline.

Posterior cerebral artery stenosis and posterior circulation revascularization surgery in pediatric patients with moyamoya disease

OBJECTIVE Some pediatric patients with moyamoya disease (MMD) present with posterior cerebral artery (PCA) stenosis before and after anterior circulation revascularization surgery and require posterior circulation revascularization surgery. This study evaluated the factors associated with PCA stenosis and assessed the efficacy of posterior circulation revascularization surgery, including occipital artery (OA)-PCA bypass, in pediatric patients with MMD. METHODS The presence of PCA stenosis before and after anterior circulation revascularization surgery and its clinical characteristics were investigated in 62 pediatric patients (< 16 years of age) with MMD. RESULTS Twenty-three pediatric patients (37%) with MMD presented with PCA stenosis at the time of the initial diagnosis. A strong correlation between the presence of infarction and PCA stenosis before anterior revascularization was observed (p < 0.001). In addition, progressive PCA stenosis was observed in 12 patients (19.4%) after anterior revascularization. The presence of infarction and a younger age at the time of initial diagnosis were risk factors for progressive PCA stenosis after anterior revascularization (p < 0.001 and p = 0.002, respectively). Posterior circulation revascularization surgery, including OA-PCA bypass, was performed in 9 of the 12 patients with progressive PCA stenosis, all of whom showed symptomatic and/or radiological improvement. CONCLUSIONS PCA stenosis is an important clinical factor related to poor prognosis in pediatric MMD. One should be aware of the possibility of progressive PCA stenosis during the postoperative follow-up period and consider performing posterior circulation revascularization surgery.

Causes, clinical and imaging characteristics of moyamoya disease in children

Introduction: Moyamoya disease is a progressive, chronic occlusive vascular disease of the circle of Willis arteries leading to the development of collateral circulation to compensate the occlusion. Purpose: This study aims to investigate the potential causes and clinical and imaging characteristics of Moyamoya Disease (MMD) in children. Methods: A retrospective analysis was performed on the clinical data of 14 MMD children, in which the potential causes were analysed, and the clinical and imaging characteristics were observed. Results: The average patient age was 6.15 y (range 21 months-13 y), and the female/male ratio was 1.3:1.0. There were 332 cases of ischemia and 196 hemorrhages. Eight cases had inducing factors including five cases of upper respiratory tract infection. Twelve cases exhibited limb weakness or hemiplegia. Among these patients, eight cases developed these manifestations as initial symptoms. Furthermore, seven cases presented language barriers, two cases had twitch, and two cases had headaches with unclear vision. All 14 patients underwent cranial Magnetic Resonance Imaging (MRI) and the results revealed that twelve cases had cerebral infarction, and two cases had cerebral hemorrhage. Five of 14 patients experienced stenosis or occlusion at the siphon part of the bilateral internal carotid arteries, seven patients had bilateral anterior cerebral artery stenosis or occlusion, 8 were bilateral middle cerebral artery stenosis or occlusion, and 2 were bilateral posterior cerebral artery stenosis or occlusion. All patients smoke-like vessels in the basal ganglia region. Conclusion: High-risk age of child MMD ranged between 4-10 y old, which mostly manifested as transient cerebral ischemic insults; and inducing factors were mostly upper respiratory tract infections.

Distribution of atherosclerotic stenosis determining early neurologic deterioration in acute ischemic stroke.

Background and purposeEarly neurologic deterioration (END) during the acute stage of stroke is clinically important because of its association with poor outcomes. The purpose of this study was (1) to investigate variables associated with END, (2) to determine the distribution of atherosclerotic stenosis associated with END, and (3) to clarify the relationship between END and clinical outcomes.Methods516 patients with acute ischemic stroke were included. The median follow-up period was 31.7 months. END was defined as a ≥2 point increase in the National Institutes of Health Stroke Scale (NIHSS), ≥1 point increase in level of consciousness or motor item of the NIHSS, or the development of any new neurological deficits during the first 72 hours of hospitalization. A signal loss on 1.5-T magnetic resonance angiography exceeding 50% was considered to be significant for the categorization of stenosis pattern.ResultsThe prevalence of END was 19.0%. END was associated with intracranial atherosclerotic stenosis (IAS) together with large artery atherosclerosis (LAA) subtype. In particular, stenosis of basilar artery or posterior cerebral artery was independently associated with END. Lesion growth or hypoperfusion was more accountable for END in patients with IAS, whereas intracerebral hemorrhage or edema/herniation was more frequently observed in END patients without IAS. Patients with END had a higher rate of mortality, but a similar rate of further vascular events compared to patients without END.ConclusionPre-stroke IAS and LAA subtype could determine the development of END during the acute stage of ischemic stroke.