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Moyamoya disease

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https://www.readbyqxmd.com/read/29220356/color-doppler-ultrasonography-as-an-alternative-tool-for-postoperative-evaluation-of-collaterals-after-indirect-revascularization-surgery-in-moyamoya-disease
#1
Shin-Joe Yeh, Sung-Chun Tang, Li-Kai Tsai, Chung-Wei Lee, Ya-Fang Chen, Hon-Man Liu, Shih-Hung Yang, Meng-Fai Kuo, Jiann-Shing Jeng
The cerebral hypoperfusion caused by chronic progressive stenosis or occlusion of intracranial arteries in moyamoya disease can be treated by direct bypass or indirect revascularization procedures. The extent of collaterals from the external carotid artery (ECA) after indirect revascularization surgery is the key point of angiographic follow-up, and the invasiveness of angiography impelled us to investigate the role of ultrasonography in the evaluation of collaterals. We hypothesized that the collaterals shown on angiography might produce corresponding hemodynamic changes in color Doppler ultrasonography...
2017: PloS One
https://www.readbyqxmd.com/read/29219755/mitochondrial-abnormalities-related-to-the-dysfunction-of-circulating-endothelial-colony-forming-cells-in-moyamoya-disease
#2
Jung Won Choi, Sung Min Son, Inhee Mook-Jung, Youn Joo Moon, Ji Yeoun Lee, Kyu-Chang Wang, Hyun-Seung Kang, Ji Hoon Phi, Seung Ah Choi, Sangjoon Chong, Jayoung Byun, Seung-Ki Kim
OBJECTIVE Moyamoya disease (MMD) is a unique cerebrovascular disorder characterized by the progressive occlusion of the bilateral internal carotid arteries. Endothelial colony-forming cells (ECFCs), previously termed "endothelial progenitor cells," play an important role in the pathogenesis of MMD. In this study, the authors performed morphological and functional studies of the mitochondria of ECFCs from patients with MMD to present new insights into the pathogenesis of the disease. METHODS The morphology of ECFCs from 5 MMD patients and 5 healthy controls was examined under both a transmission electron microscope and a confocal laser scanning microscope...
December 8, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/29202781/acta2-mutation-and-postpartum-hemorrhage-a-case-report
#3
Kylie Cooper, Stephen Brown
BACKGROUND: ACTA2 encodes smooth muscle specific ╬▒-actin, a critical component or the contractile complex of vascular smooth muscle. Mutations in ACTA2 are the most common genetic cause of thoracic aortic aneurysm, and are also the cause of other disorders, including Moyamoya disease, coronary artery disease and stroke as well as Multisystemic Smooth Muscle Dysfunction Syndrome. We note that ACTA2 is also expressed in uterine smooth muscle, and this raises the possibility that women harboring ACTA2 mutations might exhibit uterine smooth muscle dysfunction...
December 4, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/29202378/lacunar-infarction-in-adult-patients-with-moyamoya-disease
#4
Meng Zhao, Xiaofeng Deng, Shuo Wang, Dong Zhang, Yan Zhang, Jizong Zhao
OBJECTIVES: Lacunar infarctions in moyamoya disease have rarely been described. We aimed to evaluate the features and prognosis of adult moyamoya patients with lacunar infarction. PATIENTS AND METHODS: We reviewed 696 consecutive moyamoya patients admitted to our hospital from 2009 to 2015 to identify patients with lacunar infarctions. We compared the clinical features and prognosis of adult moyamoya patients with lacunar infarction and those with non-lacunar infarction...
October 31, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/29196251/effectiveness-of-ipsilateral-stroke-prevention-between-conservative-management-and-indirect-revascularization-for-moyamoya-disease-mmd-in-a-north-american-cohort
#5
Jose L Porras, Wuyang Yang, Risheng Xu, Tomas Garzon-Muvdi, Justin M Caplan, Geoffrey P Colby, Alexander L Coon, Edward S Ahn, Rafael J Tamargo, Judy Huang
BACKGROUND: Few reports compare surgical intervention with conservative treatment for moyamoya disease (MMD) in non-Asian cohorts. This study describes the effectiveness of follow-up stroke prevention by indirect revascularization relative to conservative management in a Northeast United States study population. METHOD: We retrospectively reviewed records of patients with MMD at our institution from 1990-2014. Baseline characteristics and follow-up results including subsequent ipsilateral strokes were collected, and compared between an indirect revascularization group and a conservatively managed group on a per-hemisphere basis...
November 28, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29180090/neurological-deterioration-in-patients-with-moyamoya-disease-during-pregnancy-delivery-and-puerperium
#6
Wonhyoung Park, Jae Sung Ahn, Jaewoo Chung, Yeongu Chung, Seungjoo Lee, Jung Cheol Park, Byung Duk Kwun
BACKGROUND: We reviewed our clinical experience of patients with moyamoya disease (MMD) who gave birth and assessed characteristics of those experiencing neurological deterioration. METHODS: The patients were classified into patients diagnosed with MMD during pregnancy and puerperium (Group 1) and those diagnosed before pregnancy (Group 2). We retrospectively reviewed patient characteristics, MMD treatment, neurological symptoms before and during pregnancy and/after puerperium, obstetrical history, and delivery type in Groups 1 and 2...
November 24, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29174692/increased-serum-production-of-soluble-cd163-and-cxcl5-in-patients-with-moyamoya-disease-involvement-of-intrinsic-immune-reaction-in-its-pathogenesis
#7
Miki Fujimura, Taku Fujimura, Aya Kakizaki, Mika Sato-Maeda, Kuniyasu Niizuma, Yasutake Tomata, Setsuya Aiba, Teiji Tominaga
Moyamoya disease (MMD) is a rare cerebrovascular disease characterized by a progressive stenosis at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain. Although its etiology is still unknown, intrinsic immune reactions such as autoimmune response has been implicated in the pathogenesis of MMD. Recently, the RING finger protein 213 (RNF213) was found to be an important risk gene for MMD, and is predominantly expressed in blood cells and the spleen. Thus, we hypothesized that patients with MMD represent an intrinsic autoimmune status mediated by M2-polarized macrophages, which play an important role in tissue remodeling and angiogenesis...
November 22, 2017: Brain Research
https://www.readbyqxmd.com/read/29165161/rare-variants-of-rnf213-and-moyamoya-non-moyamoya-intracranial-artery-stenosis-occlusion-disease-risk-a-meta-analysis-and-systematic-review
#8
Xin Liao, Jing Deng, Wenjie Dai, Tong Zhang, Junxia Yan
BACKGROUND: The p.R4810K and other rare variants of ring finger protein 213 gene (RNF213) were illustrated as susceptibility variants for moyamoya (MMD) and non-moyamoya intracranial artery stenosis/occlusion disease (ICASO) recently. However, the effect sizes of p.R4810K were in great discrepancy even in studies of the same ethnic population and firm conclusions of other rare variants have been elusive given the small sample sizes and lack of replication. Thus, we performed this study to quantitatively evaluate whether or to what extent the rare variants of RNF213 contribute to MMD and ICASO in different populations...
November 2, 2017: Environmental Health and Preventive Medicine
https://www.readbyqxmd.com/read/29165136/genetic-analysis-of-rnf213-p-r4810k-variant-in-non-moyamoya-intracranial-artery-stenosis-occlusion-disease-in-a-chinese-population
#9
Tong Zhang, Congrong Guo, Xin Liao, Jian Xia, XiaoXiao Wang, Jing Deng, Junxia Yan
OBJECTIVES: RNF213 p.R4810K was identified as a susceptibility variant for moyamoya disease in Asia and non-moyamoya intracranial artery stenosis/occlusion disease in Japan and Korea recently. The occurrence of this variant was evaluated in patients with non-moyamoya intracranial artery stenosis/occlusion disease in China. METHODS: Two study populations were used in this study. One was recruited from the Second Hospital of Hebei Medical University from April 2015 to May 2016...
April 24, 2017: Environmental Health and Preventive Medicine
https://www.readbyqxmd.com/read/29160859/the-role-of-rnf213-4810g-a-and-4950g-a-variants-in-patients-with-moyamoya-disease-in-korea
#10
Young Seok Park, Hui Jeong An, Jung Oh Kim, Won Seop Kim, In Bo Han, Ok Joon Kim, Nam Keun Kim, Dong-Seok Kim
Although a founder variant of RNF213 4810G>A is a major genetic risk factor for moyamoya disease (MMD) in East Asians, the frequency and disease susceptibility of RNF213 variants remain largely unknown. This study investigated the mutation analysis of RNF213 (4448, 4810, 4863, and 4950) between Korean MMD and healthy controls. We performed a polymerase chain reaction-restriction fragment length polymorphism analysis. To identify the association between RNF213 gene polymorphisms and MMD disease, we performed statistical analyses such as multivariable logistic regression and Fisher's exact test...
November 21, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29158056/factors-associated-with-moyamoya-syndrome-in-a-kentucky-regional-population
#11
Catherine Y Wang, Stephen L Grupke, Jill Roberts, Jessica Lee, Justin F Fraser
OBJECTIVES: Our study aimed to report both new and previously identified conditions associated with moyamoya syndrome in a Western population and to present our outcomes after surgical treatment with indirect bypass. METHODS: We performed a retrospective chart review of patients evaluated at our institution from June 2011 to June 2015 who were diagnosed with moyamoya. Data collected include patient demographics, presenting manifestations, vessels involved, comorbid conditions, abnormal laboratory values, treatments administered, and clinical outcomes...
November 17, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/29143295/posterior-circulation-involvement-in-pediatric-and-adult-patients-with-moyamoya-disease-a-single-center-experience-in-574-patients
#12
Meng Zhao, Dong Zhang, Shuo Wang, Yan Zhang, Rong Wang, Xiaofeng Deng, Jizong Zhao
The importance of the posterior cerebral artery (PCA) involvement in moyamoya disease has been highlighted in recent years. However, few studies compared the impact of PCA lesions in moyamoya disease between pediatric and adult patients. We conducted this study to summarize the clinical features of moyamoya patients with PCA lesions and describe the difference between pediatric and adult patients. We reviewed the records of 696 consecutive moyamoya vasculopathy patients from 2009 to 2015. The Suzuki and the Miyamoto stages were used to evaluate the steno-occlusive lesions of the anterior and posterior arteries...
November 15, 2017: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/29138020/moyamoya-syndrome-in-a-pediatric-patient-with-congenital-human-immunodeficiency-virus-type-1-infection-resulting-in-intracranial-hemorrhage
#13
Junko Yamanaka, Ikuma Nozaki, Mizue Tanaka, Hideko Uryuu, Noriko Sato, Takeji Matsushita, Hiroyuki Shichino
In the era of Antiretroviral Therapy (ART) in which human immunodeficiency virus type 1 (HIV-1) infection affected children can expect a better prognosis, the importance of careful follow up of pediatric HIV-1 cases for neurological complications has been growing. We present a case of hemorrhagic Moyamoya syndrome in a child with congenital HIV-1 infection. A 10-year-old girl was referred to our hospital for the treatment of Pneumocystis Jirovecii Pneumonia (PCP: Pneumocystis pneumonia). Her HIV-1 control was poor and Moyamoya syndrome was found during the opportunistic infection screening at admission...
November 11, 2017: Journal of Infection and Chemotherapy: Official Journal of the Japan Society of Chemotherapy
https://www.readbyqxmd.com/read/29128331/risk-factors-for-epilepsy-recurrence-after-revascularization-in-pediatric-patients-with-moyamoya-disease
#14
Yonggang Ma, Meng Zhao, Qian Zhang, Xingju Liu, Dong Zhang, Shuo Wang, Yan Zhang, Maogui Li, Jizong Zhao
OBJECTIVE: We aimed to explore the risk factors of seizure recurrence and the optimal surgical procedure for epileptic pediatric patients with moyamoya disease (MMD). METHODS: We reviewed 696 consecutive patients with MMD admitted to our hospital from 2009 to 2015. Pediatric patients with MMD who had seizures before revascularization were identified. We summarized the characteristics of seizures in pediatric patients with MMD and analyzed the predictive factors of recurrent seizure events...
November 8, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/29112977/initial-clinical-experience-with-dual-layer-detector-spectral-ct-in-patients-with-acute-intracerebral-haemorrhage-a-single-centre-pilot-study
#15
Soo Buem Cho, Hye Jin Baek, Kyeong Hwa Ryu, Jin Il Moon, Bo Hwa Choi, Sung Eun Park, Kyungsoo Bae, Kyung Nyeo Jeon, Dong Wook Kim
PURPOSE: The purpose of this study was to investigate the clinical feasibility of spectral analyses using dual-layer detector spectral computed tomography (CT) in acute intracerebral haemorrhage (ICH). MATERIAL AND METHODS: We retrospectively reviewed patients with acute ICH who underwent CT angiography on a dual-layer detector spectral CT scanner. A spectral data analysis was performed to detect contrast enhancement in or adjacent to acute ICH by using spectral image reconstructions including monoenergetic (MonoE), virtual noncontrast (VNC), and iodine overlay fusion images...
2017: PloS One
https://www.readbyqxmd.com/read/29107436/the-collateral-circulation-in-moyamoya-disease-a-single-center-experience-in-140-pediatric-patients
#16
Meng Zhao, Dong Zhang, Shuo Wang, Yan Zhang, Xiaofeng Deng, Jizong Zhao
BACKGROUND: The patterns and clinical significance of the presence of collaterals in moyamoya disease has not been elucidated thoroughly. We aimed to summarize the clinical features of collaterals in pediatric moyamoya disease. BASIC PROCEDURES: We reviewed consecutive pediatric patients with moyamoya vasculopathy who were admitted to our hospital from 2009 to 2015. Only pediatric moyamoya patients with digital subtraction angiography examinations were included in this study...
September 14, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/29102749/surgical-revascularization-for-children-with-moyamoya-disease-a-new-modification-to-the-pial-synangiosis
#17
Chuan Chen, Hui Wang, Bo Hou, Lun Luo, Ying Guo
OBJECTIVE: To summarize the therapeutic efficacy of modified pial synangiosis in children with moyamoya disease and our experience with this method. METHOD: A retrospective study was conducted to analyze the clinical efficacy of modified pial synangiosis in children with moyamoya disease who were treated between October 2002 and August 2015 in our center. The clinical characteristics of these rare cases were summarized, and the surgical efficacy was assessed based on long-term follow-up results...
November 1, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29102581/variant-angina-leading-to-cardiac-arrest%C3%A2-in-a-patient-with-moyamoya-disease
#18
Masahiko Noguchi, Kotaro Obunai, Kuniyasu Saigusa, Hiroyuki Watanabe
No abstract text is available yet for this article.
October 25, 2017: JACC. Cardiovascular Interventions
https://www.readbyqxmd.com/read/29077743/proposing-a-validated-clinical-app-predicting-hospitalization-cost-for-extracranial-intracranial-bypass-surgery
#19
Hai Sun, Piyush Kalakoti, Kanika Sharma, Jai Deep Thakur, Rimal H Dossani, Devi Prasad Patra, Kevin Phan, Hesam Akbarian-Tefaghi, Frank Farokhi, Christina Notarianni, Bharat Guthikonda, Anil Nanda
OBJECT: United States healthcare reforms are focused on curtailing rising expenditures. In neurosurgical domain, limited or no data exists identifying potential modifiable targets associated with high-hospitalization cost for cerebrovascular procedures such as extracranial-intracranial (ECIC) bypass. Our study objective was to develop a predictive model of initial cost for patients undergoing bypass surgery. METHODS: In an observational cohort study, we analyzed patients registered in the Nationwide Inpatient Sample (2002-2011) that underwent ECIC bypass...
2017: PloS One
https://www.readbyqxmd.com/read/29076784/direct-versus-indirect-revascularization-in-the-treatment-of-moyamoya-disease
#20
Seong-Eun Park, Ju-Seong Kim, Eun Kyung Park, Kyu-Won Shim, Dong-Seok Kim
OBJECTIVE For patients with moyamoya disease (MMD), surgical intervention is usually required because of progressive occlusion of the internal carotid artery. The indirect bypass method has been widely accepted as the treatment of choice in pediatric patients. However, in adult patients with MMD, the most effective treatment method remains a matter of debate. Here, the authors compared the clinical outcomes from MMD patients treated with either extracranial-intracranial arterial bypass (EIAB; 43 hemispheres) or modified encephaloduroarteriosynangiosis (mEDAS; 75 hemispheres) to investigate whether mEDAS is an effective surgical method for treating adults with symptomatic MMD...
October 27, 2017: Journal of Neurosurgery
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