Correlation between collateral circulation and cerebral hemodynamics in moyamoya disease and moyamoya syndrome based on 4-dimensional CT angiography-CT perfusion / 中华放射学杂志

Objective:To explore the significance of four-dimensional CT angiography(4D CTA) and CT perfusion (CTP) imaging in evaluating collateral circulation grades in patients with moyamoya disease and moyamoya syndrome and their relationship with cerebral hemodynamics.Methods:The clinical and imaging data of 32 patients with moyamoya disease and moyamoya syndrome in Beijing Hospital from January 2017 to January 2022 were retrospectively analyzed. All patients underwent 4D CTA-CTP imaging. Collateral circulation was scored on CTA images by using Alberta stroke program early CT score system, and on digital subtraction angiography (DSA) images by using American society of interventional and therapeutic neuroradiology/Society of interventional radiology score system, respectively. The patients were divided into Ⅰ-Ⅲ circulation compensation grades based on collateral circulation score. Regions of interest were delineated at basal ganglia on perfusion maps and the perfusion parameters were obtained including cerebral blood volume (CBV), cerebral blood flow (CBF), mean transit time (MTT), mean transit time (TTP) and delay time (DLY). The Kruskal-Wallis test was used to compare the perfusion parameters in different collateral circulation grades, and pairwise comparison was performed with Bonferroni correction. Kappa and Spearman tests were used to analyze the consistency and correlation of 4D CTA and DSA in the classification of collateral circulation.Results:4D CTA and DSA had a moderate consistency (Kappa=0.693, P<0.001) and a strong correlation ( r=0.805, P<0.001) in evaluating collateral grades. There were statistically significant differences in CBF, MTT and TTP among collateral compensation grade Ⅰ, grade Ⅱ and grade Ⅲ ( H values were 7.91, 11.69, 8.93; P values were 0.019, 0.003 and 0.012, respectively). Further pairwise comparison showed that the CBF of collateral compensation grade Ⅰ was lower than that of grade Ⅲ ( P=0.015), MTT of grade Ⅱ was higher than that of grade Ⅲ ( P=0.005), and TTP of grade Ⅰ was higher than that of grade Ⅲ ( P=0.015). There was no statistical significance of other indicators in pairwise comparison. There were no significant differences in CBV and DLY among collateral compensation grade Ⅰ, grade Ⅱ and grade Ⅲ ( P>0.05). Conclusions:4D CTA-CTP is equivalent to DSA in evaluating collateral circulation in patients with moyamoya disease and moyamoya syndrome. It can also evaluate the cerebral hemodynamics comprehensively, which has high clinical significance for disease monitoring.

Hemodynamic evaluation of the efficacy for superficial temporal artery-middle cerebral artery bypass with vascular ultrasonography / 中华超声影像学杂志

Objective:To evaluate the hemodynamic changes and short-term (one year) outcomes after superficial temporal artery(STA)-middle cerebral artery (MCA) bypass by vascular ultrasonography.Methods:Operation group included a total of 41 hemispheres of 38 patients who underwent STA-MCA bypass for severe stenosis or occlusion of MCA or severe stenosis or occlusion of internal carotid artery(ICA), or Moyamoya disease at Xuanwu Hospital between August 2017 and June 2020. The following examinations were performed in all patients: cranial MRI, CT perfusion imaging, and cerebral angiography. All of the patients were retrospectively followed up for one year postsurgery. Normal group included a total of 40 hemispheres of 20 healthy people who had been examined by vascular ultrasonography of carotid artery and intracranial artery. Inner diameter, peak systolic velocity (PSV), end-diastolic velocity (EDV), mean velocity (MV), resistance index (RI), pulsatility index (PI) and flow of STA, PSV, EDV, MV, PI, RI of external carotid artery (ECA) and PSV, EDV, MV, PI, RI of MCA at 1 week, 6 months and 12 months after STA-MCA bypass of operation group were compared with normal group.Results:①Inner diameter, PSV, MV and flow of STA were increased significantly in operation group at 1 week, 6 and 12 months than normal group (all P<0.05). The flow of STA was decreased significantly from 1 week to 12 months after operation (all P<0.05), but Inner diameter, PSV and MV were only decreased gradually from 1 week to 12 months after operation (all P>0.05). ②STA/ECA PI and RI decreased significantly after operation compared with normal group (all P<0.01). Conclusions:Vascular ultrasonography is a non-invasive examination which can objectively evaluate the extracranial and intracranial hemodynamic changes after STA-MCA bypass, and provide reference effectiveness of the operation. The flow of STA has decreased gradually from 1 week to 12 months after operation to achieve the homeostasis, but it is still higher than normal.STA/ECA PI and RI can be a steady indirect pointer to show the fluency of bypass.

Rare cerebral small vessel disease and retinal vascular abnormalities / 中国医师杂志

Cerebral small vessel disease (CSVD) is a series of clinical, imaging, and pathological syndromes resulting from various etiologies affecting small arteries (microarteries, capillaries, microvenules, and small veins in the brain). The diagnosis of CSVD is based on imaging presentations, but the high cost and bleeding risk of cranial imaging methods make the diagnosis of rare CSVD more difficult. Retinal vessels are the only vasculature visible in vivo and share anatomical and embryological features with small brain vessels. Retinal vascular abnormalities have been shown to exist in rare CSVD such as cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), cerebral amyloid angiopathy (CAA) and moyamoya disease (MMD). Retinal vascular examination may provide new ideas for the study of rare CSVD.

Repair of scalp defect with anterolateral thigh perforator flap after revascularisation of moyamoya disease: a report of 7 cases / 中华显微外科杂志

Objective:To investigate the clinical effect of free anterolateral thigh perforator flap(ALTPF) in reconstruction of temporal scalp defect after blood circulation reconstruction surgery for moyamoya disease.Methods:From May 2020 to July 2022, 7 patients with scalp defect after revascularisation of moyamoya disease were treated in Department of Hand and Foot Microsurgery, Henan Provincial People’s Hospital (People’s Hospital of Zhengzhou University). The patients were 4 males and 3 females, aged 33-59 years old, at 43 years old in average. There were 5 defects in left tempus and 2 in right tempus. The sizes of scalp defect were 4.5 cm × 5.5 cm-7.5 cm × 9.5 cm. Debridement and VSD management were primarily performed. After wounds were stabilised, ultrasound location of perforator vessels of ALTPFs was performed. Having confirmed that the perforator vessels were suitable for the surgical requirements, flap transfers were then performed. The descending branch of the lateral femoral circumflex artery was end-to-side anastomosed with the superficial temporal artery, and the descending branch of the lateral femoral circumflex vein was end-to-end anastomosed with the superficial temporal vein. Postoperative follow-up was conducted through outpatient clinic visits, telephone and WeChat reviews. Appearance, texture of ALTPFs and the flap donor sites were observed in follow-ups. Comparisons of the changes of nervous system before and after surgery were made. Cognitive function of the patients was assessed with the Mini Mental State Examination (MMSE), together with the Activities of Daily Living (ADL) .Results:All 7 flaps survived. One flap had vascular compromise 6 hours after surgery, and was rectified after surgical intervention. All the patients were included in the postoperative follow-up for 7-33 (average 19) months. All flaps had good appearance with soft texture. There was no obvious difference in colour comparing with the skin around the recipient region. The donor sites healed well without hypertrophic scar. Examinations of nervous system of the patients were found the same as that before surgery. Using Manual Muscle Testing (MMT), the average limb muscle strength of the patients was 4 before surgery and 4 after surgery, without change; Using the Ashworth assessment scale, the average preoperative and postoperative limb muscle tension in this group of patients was 1, without change; The Berg balance scale was used to evaluate the patient's balance function, with an average score of 42 before surgery and 42 after surgery, without any changes; There was no change in limb sensation before and after surgery; Using the MMSE, the average preoperative score and postoperative score of this group of patients were 25 points, without any change. Using the modified Barthel index scoring standard, the average preoperative score for this group of patients was 75 points, and the average postoperative score was 79 points, and the ADL of the patient had improved to various levels.Conclusion:Reconstruction of scalp defect with free ALTPF after revascularisation of moyamoya disease has obvious advantages, such as it closes the wound quickly, prevents infection and achieves a good appearance. This surgical procedure can produce a good clinical effect.

Neural epidermal growth factor-like 1 protein-related membranous nephropathy complicated with moyamoya syndrome: a case report and literature review / 中华肾脏病杂志

Except for IgA nephropathy, membranous nephropathy (MN) is the most common primary glomerulonephritis in China. Neural epidermal growth factor-like 1 protein (NELL-1) has been currently considered as one of the target antigens for diagnosis of primary MN. Moyamoya syndrome is a syndrome with clinical and imaging manifestations of moyamoya disease and possible vascular stenosis. By far, primary MN complicated with moyamoya syndrome has not been reported internationally. The paper reports a case of NELL-1-related MN complicated with moyamoya syndrome and reviews relevant literature to provide evidence for clinical diagnosis and treatment.

Moyamoya syndrome complicated with bilateral posterior cerebral artery embolism: a case report and literature review / 中华神经科杂志

No case of moyamoya syndrome with bilateral posterior cerebral artery (PCA) occlusion has been reported in China so far as this disease is extremely rare. The case shown in this article is a middle-aged women who has a history of atrial fibrillation, hypertension and type 2 diabetes acutely attacked by this syndrome. The main clinical manifestations included binocular blindness, right limb weakness. Imaging findings showed bilateral acute cerebral infarction in the parietal occipital lobe, bilateral anterior cerebral artery and middle cerebral artery smoke angiogenesis, bilateral PCA occlusion with distal smoke angiogenesis. Considering the medical history of the patient, the cause of the disease was diagnosed as embolic stroke of undetermined source. The patient′s consciousness has been recovered and the limb weakness has been improved after active symptomatic treatment. However, the blindness did not see any improvements. This case report aims to improve clinicians′ understanding of bilateral PCA embolization in patients with moyamoya syndrome so the occurrence of cerebral infarction can be effectively prevented.

A case report of moyamoya disease in a pedigree with glucocorticoid-remediable aldosteronism / 中华内分泌代谢杂志

We report a family of glucocorticoid-remediable aldosteronism (GRA). A 20-year-old man presented with early-onset hypertension accompanied by hypokalemia was admitted to our hospital. Clinical data and family history were collected. Following genetic analyses with PCR and Sanger sequencing to document the chimeric gene and crossover site, respectively, we identified CYP11B1/CYP11B2 and determined the breakpoint of unequal crossover to be located in 264-380 nucletide, which was considered as GRA. There were 4 cases of GRA in the family, the average age of onset was 28 years, and all had different degrees of hypertension. Among them, the proband′s uncle suffered from moyamoya disease and died 6 months later due to sudden cerebral hemorrhage. In order to improve the understanding of this rare disease, the pathogenesis, biochemical profiles, diagnosis and treatment of GRA were summarized and analyzed.

Endovascular treatment of ischemic moyamoya disease / 国际脑血管病杂志

Moyamoya disease is a relatively rare cerebrovascular disease. Extracranial and intracranial vascular bypass is the first choice for moyamoya disease. However, due to the risk of complications and symptoms recurrence after surgery, there is still some controversy about surgical treatment. In recent years, with the development of minimally invasive interventional technology, the endovascular treatment of atherosclerotic ischemic cerebrovascular disease has been widely carried out in the world. Some doctors are also beginning to try endovascular treatment of ischemic moyamoya disease, but its efficacy and safety are still unclear. This article reviews the endovascular treatment of ischemic moyamoya disease.

Plasma exosomal microRNAs are non-invasive biomarkers of moyamoya disease: A pilot study

Abstract Background As a progressive cerebrovascular disease, Moyamoya Disease (MMD) is a common cause of stroke in children and adults. However, the early biomarkers and pathogenesis of MMD remain poorly understood. Methods and material This study was conducted using plasma exosome samples from MMD patients. Next-generation high-throughput sequencing, real-time quantitative PCR, gene ontology analysis, and Kyoto Encyclopaedia of Genes and Genomes pathway analysis of ideal exosomal miRNAs that could be used as potential biomarkers of MMD were performed. The area under the Receiver Operating Characteristic (ROC) curve was used to evaluate the sensitivity and specificity of biomarkers for predicting events. Results Exosomes were successfully isolated and miRNA-sequence analysis yielded 1,002 differentially expressed miRNAs. Functional analysis revealed that they were mainly enriched in axon guidance, regulation of the actin cytoskeleton and the MAPK signaling pathway. Furthermore, 10 miRNAs (miR-1306-5p, miR-196b-5p, miR-19a-3p, miR-22-3p, miR-320b, miR-34a-5p, miR-485-3p, miR-489-3p, miR-501-3p, and miR-487-3p) were found to be associated with the most sensitive and specific pathways for MMD prediction. Conclusions Several plasma secretory miRNAs closely related to the development of MMD have been identified, which can be used as biomarkers of MMD and contribute to differentiating MMD from non-MMD patients before digital subtraction angiography.

Study on the serum level of CoQ10B in patients with Moyamoya disease and its mechanism of affecting disease progression / Estudo sobre o nível sérico de CoQ10B em pacientes com doença de Moyamoya e seu mecanismo de afetar a progressão da doença

ABSTRACT Background: At present, the etiology and pathogenesis of Moyamoya disease (MMD) are not completely clear. Patients are usually diagnosed after cerebrovascular events. Therefore, it is of great clinical significance to explore the predictive factors of MMD. Objective: This study aimed to investigate the serum level of CoQ10B, the amount of endothelial progenitor cells (EPCs), and mitochondrial function of EPCs in MMD patients. Methods: Forty-one MMD patients and 20 healthy controls were recruited in this study. Patients with MMD were divided into two groups: Ischemic type (n=23) and hemorrhagic type (n=18). Blood samples were collected from the antecubital vein and analyzed by CoQ10B ELISA and flow cytometry. Measures of mitochondrial function of EPCs include oxygen consumption rate (OCR), mitochondrial membrane potential, Ca2+ concentration, adenosine triphosphatases activity and ROS level. Results: The serum CoQ10B level in MMD patients was significantly lower than that in healthy controls (p<0.001). The relative number of EPCs in MMD patients was significantly higher than that in healthy controls (p<0.001). Moreover, the OCR, mitochondrial membrane potential and ATPase activity were decreased and the Ca2+ and reactive oxygen species levels were increased in MMD patients (p<0.001). Conclusions: Our results showed obviously decreased serum CoQ10B level and increased EPCs number in patients with MMD compared with healthy patients, and the mitochondria function of EPCs in MMD patients was abnormal.

RESUMO Antecedentes: No momento, a etiologia e a patogênese da doença de Moyamoya (DMM) não são completamente claras. Os pacientes geralmente são diagnosticados após eventos cerebrovasculares. Sendo assim, é de grande importância clínica explorar os fatores preditivos de DMM. Objetivo: Este estudo teve como objetivo investigar o nível sérico de CoQ10B, a quantidade de células progenitoras endoteliais (CPE) e a função mitocondrial de CPE em pacientes com DMM. Métodos: Quarenta e um pacientes com DMM e 20 controles saudáveis foram recrutados neste estudo. Aqueles com DMM foram divididos em dois grupos: tipo isquêmico (n=23) e tipo hemorrágico (n=18). Amostras de sangue foram coletadas da veia antecubital e analisadas por CoQ10B Ensaio de Imunoadsorção Enzimática (ELISA) e citometria de fluxo. As medidas da função mitocondrial de CPE incluem taxa de consumo de oxigênio (TCO), potencial de membrana mitocondrial, concentração de Ca2+, atividade de adenosina trifosfatases (ATPase) e nível de espécies reativas de oxigênio (ROS). Resultados: O nível sérico de CoQ10B em pacientes com DMM foi significativamente menor do que em controles saudáveis (p<0,001). O número relativo de CPE em pacientes com MMD foi significativamente maior do que em controles saudáveis (p<0,001). Além disso, a TCO, o potencial de membrana mitocondrial e a atividade ATPase diminuíram e os níveis de Ca2+e ROS aumentaram em pacientes com MMD (p<0,001). Conclusões: Nossos resultados mostraram obviamente diminuição do nível sérico de CoQ10B e aumento do número de CPE em pacientes com DMM em comparação com pacientes saudáveis, e a função mitocondrial de CPE em pacientes com DMM estava anormal.

Moyamoya disease and syndrome: a review / Doença e síndrome de moyamoya: uma revisão

Abstract Moyamoya disease is a chronic occlusive cerebrovascular disease that is non-inflammatory and non-atherosclerotic. It is characterized by endothelial hyperplasia and fibrosis of the intracranial portion of the carotid artery and its proximal branches, leading to progressive stenosis and occlusion, often clinically manifesting as ischemic or hemorrhagic stroke with high rates of morbidity and mortality. On cerebral angiography, the formation of collateral vessels has the appearance of a puff of smoke (moyamoya in Japanese), which became more conspicuous with the refinement of modern imaging techniques. When there is associated disease, it is known as moyamoya syndrome. Treatments are currently limited, although surgical revascularization may prevent ischemic events and preserve quality of life. In this review, we summarize recent advances in moyamoya disease, covering aspects of epidemiology, etiology, presentation, imaging, and treatment strategies.

RESUMO A doença de moyamoya, ou doença cerebrovascular oclusiva crônica, é uma afecção não inflamatória e não aterosclerótica, caracterizada por hiperplasia endotelial e fibrose dos segmentos intracranianos das artérias carótidas internas e da porção proximal de seus ramos. Isso provoca estenose progressiva e oclusão, frequentemente manifestada clinicamente como isquemia cerebral ou hemorragia intracraniana, com alta morbimortalidade. A formação compensatória de vasos colaterais produz, na angiografia encefálica, um aspecto de nuvem de fumaça (moyamoya, em japonês). Quando existe doença subjacente que possa estar relacionada, a doença recebe o nome de síndrome de moyamoya. Embora a incidência esteja aumentando graças aos novos métodos diagnósticos, as estratégias terapêuticas ainda são limitadas. O diagnóstico precoce permite cirurgias de revascularização cerebral que podem evitar novos acidentes vasculares e melhorar a qualidade de vida. Nesta revisão são apresentados os avanços recentes sobre a doença de moyamoya, citando aspectos de epidemiologia, etiologia, apresentação, exames diagnósticos e tratamento.

Optimization strategy for anesthesia in patients with moyamoya disease undergoing revascularization: scalp nerve block combined with general anesthesia / 中华麻醉学杂志

Objective:To evaluate the efficacy of scalp nerve block combined with general anesthesia in optimizing anesthesia in the patients with moyamoya disease undergoing revascularization.Methods:A total of 154 patients with moyamoya disease, aged 18-64 yr, of American Society of Anesthesiologists Physical Status classification Ⅱ or Ⅲ, undergoing elective revascularization, were divided into 2 groups ( n=77 each) using a random nunber table method: scalp nerve block combined with general anesthesia group (GN group) and general anaesthesia group (G group). Anesthesia was induced with intravenous midazolam 0.05 mg/kg, sufentanil 0.5-1.0 μg/kg, rocuronium 0.6 mg/kg and etomidate 0.2-0.3 mg/kg.After the patients were tracheally intubated after anesthesia induction, ipsilateral scalp nerve block (2 ml for supraorbital nerve block, 2 ml for supratrochlear nerve block; 3 ml for auriculotemporal nerve block, 3 ml for greater occipital nerve block, 3 ml for less occipital nerve block) was performed with 0.5% ropivacaine in GN group.The equal volume of normal saline was locally injected in G group.Anesthesia was maintained by inhalation of sevoflurane and intravenous infusion of remifentanil 0.05-0.10 μg·kg -1·min -1 and cisatracurium 0.1 mg·kg -1·h -1.The consumption of intraoperative remifentanil, requirement for postoperative rescue analgesia, nausea and vomiting, length of postoperative hospital stay, and early neurological complications were recorded.The modified Rankin Scale scores were evaluated before operation, at discharge and at 6 months after operation. Results:Compared with G group, the consumption of intraoperative remifentanil and requirement for postoperative rescue analgesia were significantly decreased ( P<0.05), and no significant change was found in the incidence of postoperative nausea and vomiting, incidence of neurological complications, length of postoperative hospital stay, and modified Rankin Scale scores at each time point in GN group ( P>0.05). Conclusions:Scalp nerve block combined with general anesthesia can increase the perioperative analgesic efficacy and is helpful in achieving a low-opioid anesthesia mode when used in the patients with moyamoya disease undergoing revascularization.

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.

Magnetic resonance vessel wall imaging of moyamoya disease and atherosclerotic moyamoya syndrome / 国际脑血管病杂志

Compared with routine imaging examination, magnetic resonance vascular wall imaging (MR-VWI) has the advantages of non-invasive and accurate in the diagnosis and differentiation of moyamoya disease and atherosclerotic moyamoya syndrome, and is of great significance in guiding clinical treatment and predicting outcomes. This article reviews the features of MR-VWI in moyamoya disease and atherosclerotic moyamoya syndrome.

Analysis of clinical outcomes and effectiveness of combined revascularization in the treatment of elderly patients with moyamoya disease / 中华老年医学杂志

Objective:To analyze the characteristics of elderly patients with moyamoya disease and clinical outcomes after combined revascularization, and to evaluate the safety and efficacy of this method in elderly patients.Methods:Moyamoya disease patients aged 60 years or older who had undergone combined revascularization at the Neurosurgery Center of Aeronautical General Hospital from January 2016 to September 2021 were selected as study subjects.Then patients' clinical data and postoperative complications within two weeks of surgery were collected and a followed-up was conducted.The results were compared with those of previous reports.Results:A total of 46 combined revascularization procedures were carried out in 30 patients.The ages at surgery ranged between 60-75 years, with a mean age of(66.1±4.4)years.The rate of complications within two weeks after surgery was 37%, and the most common complication was speech dysfunction, followed by epilepsy and hyperperfusion.No serious complications such as cerebral hemorrhage and death occurred.The follow-up varied between 3-67 months, with an average follow-up time of(26.8±18.4)months.The MRS score was <3 at 3 months after surgery, and there was no death or severe disability.Conclusions:Perioperative complications in elderly patients with moyamoya disease have a higher incidence rate than in other populations, but patients can usually recover after close perioperative management for 2 weeks.The follow-up has demonstrated that outcomes at 3 months and in longer terms after surgery are satisfactory and clinical symptoms will improve significantly.

Arteriopathy in pediatric stroke: an underestimated clinical entity / Arteriopatia em crianças com acidente vascular cerebral: uma entidade clínica subestimada

ABSTRACT Background: Pediatric arterial ischemic stroke (AIS), which was thought to be a rare disorder, is being increasingly recognized as an important cause of neurological morbidity, thanks to new advances in neuroimaging. Objective: The aim of this study was to review the main etiologies of stroke due to arteriopathy in children. Methods: Using a series of cases from our institution, we addressed its epidemiological aspects, physiopathology, imaging findings from CT, MR angiography, MR conventional sequences and MR DWI, and nuclear medicine findings. Results: Through discussion of the most recent classification for childhood AIS (Childhood AIS Standardized Classification and Diagnostic Evaluation, CASCADE), we propose a modified classification based on the anatomical site of disease, which includes vasculitis, varicella, arterial dissection, moyamoya, fibromuscular dysplasia, Takayasu's arteritis and genetic causes (such as ACTA-2 mutation, PHACE syndrome and ADA-2 deficiency). We have detailed each of these separately. Conclusions: Prompt recognition of AIS and thorough investigation for potential risk factors are crucial for a better outcome. In this scenario, neurovascular imaging plays an important role in diagnosing AIS and identifying children at high risk of recurrent stroke.

RESUMO Introdução: O acidente vascular cerebral (AVC) pediátrico, considerado um distúrbio raro, está sendo cada vez mais reconhecido como importante causa de morbidade neurológica, graças aos novos avanços na neuroimagem. Objetivo: Revisar as principais etiologias do AVC por arteriopatia em crianças. Métodos: Utilizando-se de uma série de casos de nossa instituição, abordamos seus aspectos epidemiológicos, fisiopatológicos e de imagem na angiotomografia computadorizada e angiorressonância magnética, sequências convencionais e avançadas de ressonância magnética e medicina nuclear. Resultados: Com base na classificação mais recente de AVC na infância (Classificação Padronizada e Avaliação Diagnóstica do AVC na Infância - CASCADE) propusemos uma classificação modificada com base no local anatômico da doença, que inclui vasculite, varicela, dissecção arterial, Moyamoya, displasia fibromuscular, arterite de Takayasu e causas genéticas (como mutação ACTA-2, síndrome PHACE e deficiência de ADA-2), detalhando cada uma separadamente. Conclusões: O reconhecimento imediato do AVC na infância e a investigação minuciosa de possíveis fatores de risco são cruciais para um melhor resultado. Nesse cenário, a imagem neurovascular desempenha papel importante no diagnóstico de AVC e na identificação de crianças com alto risco de recorrência.

Risk factors for early neurological complications after revascularization in adult patients with moyamoya disease / 中华麻醉学杂志

Objective:To identify the risk factors for early neurological complications after revascularization in adult patients with moyamoya disease.Methods:The medical records of patients of both sexes with moyamoya disease, aged 18-65 yr, of American Society of Anesthesiologists physical status Ⅱ or Ⅲ, who underwent revascularization in our hospital from January 2017 to June 2019, were retrospectively collected.According to the occurrence of early postoperative neurological complications, patients were divided into early postoperative neurological complication group and non-early postoperative neurological complication group.The factors such as patient′s age, gender, preoperative clinical symptoms, previous history of hypertension, history of diabetes, history of coronary heart disease, American Society of Anesthesiologists physical status, methods of anesthesia, type of operation, anesthesia time, time for start of operation, operation time, intraoperative urine volume, times of intraoperative vasoactive drugs used, and time of the post-anaesthesia observation room (PACU) stay were collected.Logistic regression analysis was used to identify the risk factors for postoperative early neurological complications.Results:A total of 510 adult patients with moyamoya disease underwent revascularization were enrolled in this study, and the incidence of early postoperative neurological complications was 9.0%.The results of logistic regression analysis showed that preoperative ischemia, intraoperative use of vasoactive drugs more than 3 times and PACU stay time>90 min were risk factors for postoperative neurological complications ( P<0.05). Conclusion:Preoperative ischemia, intraoperative use of vasoactive drugs >3 times and PACU stay time>90 min are risk factors for early neurological complications after revascularization in the patients with moyamoya disease.

Unusual presentation of moyamoya disease with popliteal involvement: case report and review of the literature / Apresentação incomum de doença de moyamoya com envolvimento poplíteo: relato de caso e revisão da literatura

Abstract Moyamoya disease is a rare disorder that involves the cerebrovascular system. Usually, it leads to occlusion of the arteries of the cerebral system and causes cerebral circulatory complaints. A 48-year-old female patient was admitted to our clinic with intermittent claudication in both legs. Biphasic and monophasic waveform patterns were detected bilaterally in distal (trifurcation arteries) lower extremities with Doppler sonography. The patient therefore underwent systemic vascular examination. Computed tomography angiography revealed bilateral carotid occlusion at the level of supraclinoid segments, and opacifications were detected at the distal segments of the bilateral anterior cerebellar and middle cerebellar arteries. The patient was diagnosed with moyamoya disease, and anticoagulant treatment was started. In conclusion, most previous reports have presented the cerebrovascular involvement of moyamoya disease. However, this disease can involve different peripheral vascular systems and careful and systemic vascular examination is necessary for an exact diagnosis.

Resumo A doença de moyamoya é um distúrbio raro que envolve o sistema cerebrovascular. Normalmente, leva à oclusão das artérias do sistema cerebral e causa problemas circulatórios no cérebro. Uma mulher de 48 anos foi admitida em nossa clínica com claudicação intermitente em ambas as pernas. Na ultrassonografia com Doppler, foram detectados padrões de formato de onda bifásico e monofásico nas extremidades inferiores distais (artérias da trifurcação) de forma bilateral. Portanto, realizou-se o exame vascular sistêmico na paciente. A angiografia por tomografia computadorizada revelou oclusão carotídea bilateral no nível dos segmentos supraclinoides, e opacificações foram detectadas nos segmentos distais das artérias cerebelares anteriores e médias de forma bilateral. A paciente foi diagnosticada com doença de moyamoya, e o tratamento anticoagulante foi iniciado. Em conclusão, a maioria dos relatos anteriores apresentou o envolvimento cerebrovascular da doença de moyamoya. No entanto, essa doença pode envolver diferentes sistemas vasculares periféricos, e um exame vascular sistêmico minucioso é necessário para um diagnóstico exato.

Bypass temporosilviano: enfermedad Moyamoya / Temporosilvian Bypass: Moyamoya disease

Introducción: En 1957, Takeuchi y Shimizu describen una vasculopatía oclusiva que involucra la arteria carótida interna bilateral, con la formación de vasos colaterales. En 1969, Suzuki y Takaku denominan a la conexión vascular colateral en las imágenes de angiografía "moyamoya" que significa nube de humo.2,3 Objetivos: El propósito del siguiente video es la descripción detallada de una cirugía de revascularización directa a través de un bypass temporosilviano en paciente con enfermedad Moyamoya. Materiales y Métodos: Se describe el caso de un paciente masculino de 27 años de edad que presentó de accidente vascular cerebral hemorrágico derecho. En la angiografía se diagnosticó estenosis del 70% de la arteria carótida interna supraclinoidea derecha, acompañado de estenosis de la arteria cerebral media y cerebral anterior homolateral. Se realizó cirugía de revascularización cerebral directa con bypass temporosilviano derecho.4,5 Resultados: Luego de realizado el bypass se confirmó adecuada permeabilidad del mismo y en la angiografía postoperatoria se observó el desarrollo de circulación colateral a través de la anastomosis. El paciente no presentó déficit en el periodo postoperatorio. Conclusión: Aunque la incidencia de enfermedad de Moyamoya no es elevada, es una causa probable de stroke isquémico o hemorrágico en niños y adultos. El manejo adecuado es fundamental para mejorar el pronostico a largo plazo de los pacientes con esta rara patología.

Introduction: In 1957, Takeuchi and Shimizu describes an occlusive vasculopathy involving the bilateral internal carotid arteries, with the formation of collateral vessels. In 1969, Suzuki and Takaku designate the collateral vascular connections in the angiographical images "moyamoya" which means puff of smoke.2,3 Objectives: The purpose of the following video is the detailed description of a direct revascularization surgery through a temporosilvian bypass in a patient with Moyamoya disease. Materials and methods: We present a case of a 27-year-old male patient with a history of right hemorrhagic cerebral vascular accident. In the angiography, 70% stenosis of the right supraclinoid internal carotid artery was diagnosed, accompanied by stenosis of the middle and anterior homolateral cerebral artery. Direct cerebral revascularization surgery was performed with right temporosilvian bypass.4,5 Results: After performing the bypass, adequate permeability is confirmed and in the postoperative angiography the development of collateral circulation through the anastomosis was observed. The patient did not present a deficit in the postoperative period. Conclusion: Although the incidence of Moyamoya disease is not high, it is a probable cause of ischemic or hemorrhagic stroke in children and adults. Proper management is essential to improve the long-term prognosis of patients with this rare pathology.

Endothelial progenitor cells in the peripheral blood of patients with moyamoya disease labeled with superparamagnetic iron oxide in vitro for MRI detection

Moyamoya disease (MMD) is currently thought to involve endothelial progenitor cells (EPCs). We investigated whether superparamagnetic iron oxide (SPIO) can be used to label EPCs. Mononuclear cells from 10 moyamoya disease patients were isolated, and cluster of differentiation 133 (CD133) positive cells sorted by magnetic-activated cell sorting were cultured in vitro. The positive rates of CD133, vascular endothelial growth factor receptor (VEGFR)-2, and cluster of differentiation 34 (CD34) were detected by flow cytometry. The cells were co-cultured with fluorescence labeled Dil-acetylated-low-density lipoprotein (Dil-ac-LDL) and Ulex europaeus agglutinin-1 (UEA-1) to observe the endocytosis of Dil-ac-LDL and binding to UEA-1. Prussian blue staining and transmission electron microscopy were used to observe the endocytosis of different SPIO concentrations in EPCs, and CCK-8 was used to detect proliferation of cells transfected with different concentrations of SPIO. T2 weighted imaging (T2WI) signals from magnetic resonance imaging after SPIO endocytosis were compared. Positive rates of CD133, VEGFR-2, and CD34 on sorted mononuclear cells were 68.2±3.8, 57.5±4.2, and 36.8±6.5%, respectively. The double-positive expression rate of CD34 and VEGFR-2 was 19.6±4.7%, and 83.1±10.4% of cells, which showed the uptake of Dil-ac-LDL and binding with UEA-1. The labeling efficiencies of SPIO at concentrations of 25 and 50 μg/mL were higher than for 12.5 μg/mL. The proliferation of cells was not influenced by SPIO concentrations of 12.5 and 25 μg/mL. After labeling, the T2WI of EPCs was reduced. The concentration of 25 μg/mL SPIO had high labeling efficiency detected by magnetic resonance imaging (MRI) without decreased EPCs viability.