Moyamoya Disease (Moya-Moya Disease)

A noninflammatory, progressive occlusion of the intracranial CAROTID ARTERIES and the formation of netlike collateral arteries arising from the CIRCLE OF WILLIS. Cerebral angiogram shows the puff-of-smoke (moyamoya) collaterals at the base of the brain. It is characterized by endothelial HYPERPLASIA and FIBROSIS with thickening of arterial walls. This disease primarily affects children but can also occur in adults.

Also Known As:

Moya-Moya Disease; Moyamoya Disease, Classic; Moyamoya Disease, Primary; Moyamoya Disease, Secondary; Moyamoya Syndrome; Classic Moyamoya Disease; Disease, Classic Moyamoya; Disease, Moya-Moya; Disease, Primary Moyamoya; Moya Moya Disease; Moyamoya Diseases, Primary; Primary Moyamoya Disease; Primary Moyamoya Diseases; Secondary Moyamoya Disease; Cerebrovascular Moyamoya Disease; Progressive Intracranial Occlusive Arteropathy (Moyamoya)

Networked: 1027 relevant articles (52 outcomes, 108 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Stroke (Strokes)
2.	Ischemia
3.	Hypertension (High Blood Pressure)
4.	Cerebrovascular Disorders (Cerebrovascular Occlusion)
5.	Ischemic Stroke

Experts

1.	Zhang, Dong: 26 articles (01/2022 - 10/2009)
2.	Fujimura, Miki: 24 articles (08/2022 - 11/2009)
3.	Tominaga, Teiji: 21 articles (08/2021 - 08/2006)
4.	Kuroda, Satoshi: 21 articles (02/2021 - 12/2004)
5.	Wang, Rong: 18 articles (12/2021 - 10/2009)
6.	Houkin, Kiyohiro: 18 articles (02/2021 - 12/2004)
7.	Zhao, Jizong: 18 articles (01/2021 - 02/2011)
8.	Araki, Yoshio: 16 articles (06/2022 - 11/2010)
9.	Steinberg, Gary K: 16 articles (01/2022 - 05/2009)
10.	Okamoto, Sho: 14 articles (06/2022 - 11/2010)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Moyamoya Disease:

1.	Cytidine Triphosphate (CTP)IBA 11/01/2021 - "To explore the value of computed tomography perfusion (CTP) imaging for evaluating the efficacy of encephaloduroarteriosynangiosis (EDAS) treatment of moyamoya syndrome (MMS). " 04/01/2019 - "CTP-Sis could help identify in a noninvasive manner the presence and intensity of collateral flow in patients with unilateral Moyamoya disease using DSA as a gold standard. " 02/01/2010 - "Our purpose in this study is to evaluate the use of computed tomographic perfusion (CTP) as an imaging modality to assess revascularization after superficial temporal artery (STA) to middle cerebral artery (MCA) bypass in patients with moyamoya disease. " 01/01/2022 - "To build CT perfusion (CTP)-based delta-radiomics models to identify collateral vessel formation after revascularization in patients with moyamoya disease (MMD). " 11/01/2019 - "To explore the feasibility of computed tomography perfusion imaging (CTP) for evaluating hemodynamics in hemorrhagic moyamoya disease (MMD). "
2.	Aspirin (Acetylsalicylic Acid)FDA LinkGeneric 09/01/2017 - "Effect of Aspirin in Postoperative Management of Adult Ischemic Moyamoya Disease." 01/01/2023 - "To analyze the safety and efficacy of regular aspirin use after combined cerebral revascularization in patients with ischemic moyamoya disease. " 01/01/2023 - "Safety and efficacy of aspirin after combined cerebral revascularization for ischemic moyamoya disease: A prospective study." 09/01/2017 - "In this study, we retrospectively reviewed data of 184 adult patients (197 hemispheres) presented with ischemic-onset MMD who had undergone direct or combined revascularization in our hospital, to clarify the effect of postoperative aspirin therapy in the management of moyamoya disease. " 10/01/2021 - "Genetic testing evidenced a novel, unique pathogenic variant on the NF-1 gene. The patient underwent combined bypass surgery first and then was placed on aspirin and a blood pressure control regimen. Our case illustrates the need for clinicians to include moyamoya disease in the list of differential diagnoses when encountering a young patient, without major risk factors, presenting with ischemic stroke. "
3.	FlunarizineIBA 01/01/1996 - "This report documents the efficacy of flunarizine to improve regional cerebral perfusion in Moyamoya disease." 05/01/1988 - "[The efficacy of the flunarizine in the treatment of ischemic attack of moyamoya disease]." 01/01/1996 - "Recently, it has been suggested that flunarizine could be used to treat Moyamoya disease. "
4.	Hemoglobins (Hemoglobin)IBA 03/01/1994 - "The magnitude of variance in oxygenated hemoglobin concentration (C-HV) was significantly greater in moyamoya disease patients than in healthy controls (P < .003, Wilcoxon test). " 01/01/2022 - "Near-infrared spectroscopy (NIRS) was used to study the signals of the cerebral tissue oxygen saturation index (TOI) and the changes in oxygenated and deoxygenated hemoglobin concentrations (HbO and Hb) in 64 patients with moyamoya disease and 64 healthy volunteers. " 10/01/2006 - "This study was designed to measure the brain blood flow velocity, brain oxygenation, hemoglobin concentrations, hematocrit, and cell free hemoglobin at pre- and post-exchange red cell transfusion in an 18 year old male patient with sickle cell disease and moyamoya syndrome (MMS). " 02/01/2008 - "Moyamoya disease associated with hemoglobin Fairfax and beta-thalassemia." 05/01/2000 - "Near-infrared spectroscopy was used to measure the relative concentration changes of oxygenated hemoglobin (HbO(2)), deoxygenated hemoglobin (HbR), and total hemoglobin (t-Hb) before, during, and after hyperventilation in eight patients with childhood moyamoya disease who underwent surgery. "
5.	Cilostazol (Pletal)FDA LinkGeneric 10/01/2023 - "Cilostazol for the management of moyamoya disease: a systematic review of the early evidence, efficacy, safety, and future directions." 11/01/2022 - "Vessel Wall Changes on Serial High-Resolution MRI and the Use of Cilostazol in Patients With Adult-Onset Moyamoya Disease." 01/01/2021 - "Conclusions Antiplatelet therapy is associated with substantial improvement in survival in patients with moyamoya disease, and cilostazol is associated with greater survival benefit compared with other antiplatelet regimens. " 01/01/2021 - "Association of Antiplatelet Therapy, Including Cilostazol, With Improved Survival in Patients With Moyamoya Disease in a Nationwide Study." 05/01/2019 - "Cilostazol may improve cognition better than clopidogrel in non-surgical adult patients with ischemic moyamoya disease: subanalysis of a prospective cohort."
6.	Simvastatin (Zocor)FDA LinkGeneric 07/06/2015 - "Treating moyamoya disease by multipoint skull drilling for indirect revascularization combined with mobilization of autologous bone marrow stem cells and simvastatin is a safe and effective method as it can promote recovery of neurological functions and improve patients' daily living abilities and quality of life." 07/06/2015 - "This study discusses the clinical efficacy of multipoint skull drilling for indirect revascularization combined with mobilization of autologous bone marrow stem cells and use of simvastatin in the treatment of moyamoya disease. "
7.	Indicators and Reagents (Reagents)IBA 09/01/2017 - "Acceleration of blood flow as an indicator of improved hemodynamics after indirect bypass surgery in Moyamoya disease." 08/25/2023 - "Choroidal anastomosis (ChA) has been implicated as the main indicator of an increased hemorrhagic risk in adult moyamoya disease. " 11/01/2013 - "Although optimal management is still under debate, ivy sign may be an indicator of misery perfusion, and patients with asymptomatic moyamoya disease and ivy sign on FLAIR imaging will benefit from more careful follow-up." 01/01/2010 - "Therefore, posterior circulation system in moyamoya disease is important as a collateral circulation in addition to the revascularization procedures, and we believe that the progression of the steno-occlusive lesion of PCA is an important indicator, predicting recurrence." 04/01/1998 - "Cerebral blood flow measurement as an indicator for an indirect revascularization procedure for adult patients with moyamoya disease."
8.	CollagenIBA 01/01/2021 - "From October 2012 to July 2014, 35 patients (male: 19, female: 16 average age: 10.5 ± 3.9) with ischemic onset-type pediatric moyamoya disease and who were admitted or commuted to hospital and had surgical treatment were evaluated for background, moyamoya staging (Suzuki), presence/absence of TIA, and platelet aggregation activity by adenosine diphosphate (ADP)/collagen turbidity test. " 01/01/2021 - "In patients with ischemic onset-type pediatric moyamoya disease who experience headaches, the platelet aggregation activity is accelerated, and aspirin administration is effective in alleviating headaches by inhibiting platelet activation, detected by the collagen PAT." 01/01/2021 - "Connections from the APA to the vertebrobasilar system are hypothesized to result from a lack of regression of an embryological anastomosis, in line with the more common persistent trigeminal and persistent hypoglossal arteries.1 Patients with moyamoya disease have a significantly higher rate of persistent carotid-vertebrobasilar anastomoses than the general population,3 and are also 26 times more likely to have Down syndrome.4 Correspondingly, patients with Down syndrome have significantly higher levels of moyamoya disease, and are more than 10 times as likely as the general population to have abnormalities of the Circle of Willis5 and vertebral arteries.6 Several genes on chromosome 21 are known to affect angiogenesis, namely collagen XIII/endostatin (COL18A1), DYRK1A, and Down syndrome candidate region 1 (DSCR1), possibly through inhibition of VEGF activity.7 Whether additional copies of these genes are responsible for the anomalous vascular development seen in Down syndrome, in turn predisposing to the development of moyamoya disease, could benefit from further exploration."
9.	TitaniumIBA 05/01/2014 - "The titanium mesh appears to be effective and useful for prevention of ischemic insult in the treatment of moyamoya disease. " 05/01/2014 - "We used various types of titanium mesh to cover the bony defect area in 8 patients (10 operations) with moyamoya disease. "
10.	Remifentanil (Ultiva)FDA Link 10/01/2016 - "Remifentanil was effective for treating perioperative hypertension undergoing caesarean delivery in a patient with moyamoya disease." 10/01/2016 - "[General Anesthesia with Remifentanil for Cesarean Section in a Patient with Moyamoya Disease]."

Therapies and Procedures

1.	Cerebral Revascularization 12/01/2021 - "Superficial temporal artery to middle cerebral artery (STA-MCA) bypass is the most effective treatment for Moyamoya disease (MMD). " 01/01/2020 - "For several variants of quasi-moyamoya disease, cerebral revascularization treatment is as effective as it is for the more typical cases of moyamoya disease. " 01/01/2023 - "Cerebral revascularization surgery is the mainstay of treatment for moyamoya syndrome (MMS) today, and intraoperative determination of the patency of the revascularized vessel is a critical factor in the success of the procedure. " 04/01/2022 - "Cerebral revascularization for moyamoya disease (MMD) is an effective treatment for improving cerebral ischaemia and preventing rebleeding. " 06/12/2020 - "The only effective treatment for ischemic moyamoya disease (iMMD) is cerebral revascularization by an extracranial to intracranial bypass. "
2.	Therapeutics 10/01/2017 - "Safety and efficacy of remote ischemic conditioning in pediatric moyamoya disease patients treated with revascularization therapy." 01/01/2021 - "These results provisionally support the use of antiplatelet therapy in patients with moyamoya disease and the conduct of confirmatory randomized controlled trials." 12/01/2018 - "This study reports results of a nationwide survey conducted in 2016 on the trends of antiplatelet therapy for moyamoya disease in Japan. " 02/01/2016 - "Effects of Surgery and Antiplatelet Therapy in Ten-Year Follow-Up from the Registry Study of Research Committee on Moyamoya Disease in Japan." 08/01/2023 - "Antiplatelet therapy may improve the prognosis of patients with moyamoya disease: a 12-year retrospective study."
3.	Operative Surgical Procedures 11/15/1998 - "Based on analysis of these findings, the authors recommend the MCI procedure as the appropriate surgical procedure in the treatment of children with moyamoya disease, although the best treatment is the STA-MCA anastomosis with EMS when feasible." 07/01/2007 - "The EC-IC bypass surgical procedure is also beneficial in moya-moya disease." 01/01/1986 - "From these results, it is concluded that the EDAS surgical procedure is superior to that of EMS for moyamoya disease." 06/01/2010 - "These findings strongly suggest that STA-MCA anastomosis and EDMAPS using a frontal pericranial flap is a safe and effective surgical procedure to further improve the long-term prognosis in moyamoya disease by improving cerebral hemodynamics in both the MCA and anterior cerebral artery territories." 01/01/2022 - "In this study, the authors aimed to evaluate the clinical and angiographic outcomes of a new surgical procedure: superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis combined with multiple burr hole (MBH) surgery and dural inversion synangiosis for the treatment of moyamoya disease in adults. "
4.	Craniotomy 06/01/2008 - "The "target bypass" method might be effective for cases with moyamoya disease or for cases requiring surgery through a small craniotomy." 10/01/2006 - "The "target bypass" method might be effective for cases with moyamoya disease or for cases requiring surgery through a small craniotomy." 01/01/2020 - "In this study perioperative complications in revascularization surgery for moyamoya disease are evaluated, focusing on craniotomy size. " 10/01/2023 - "Comparison of Bifrontal Craniotomy and Multiple Burr Hole Encephalogaleoperiosteal-Synangiosis for Pediatric Moyamoya Disease: An Experience of 346 Patients." 06/01/2023 - "To investigate factors associated with improvements in cerebral blood flow (CBF) and cerebrovascular reactivity (CVR) after combined revascularization surgery for moyamoya disease, with special attention to the size of craniotomy. "
5.	Conservative Treatment 01/01/2023 - "The optimal treatment approach for hemorrhagic moyamoya disease (HMMD) remains a topic of debate, particularly regarding the comparative efficacy of revascularization versus conservative treatment. " 07/01/2019 - "The purpose of this study was to evaluate the efficacy of surgical revascularization versus conservative treatment and different surgical modalities, in order to provide evidence for the patient with moyamoya disease (MMD) to choose the appropriate treatment. " 01/01/2023 - "Comparison of revascularization and conservative treatment for hemorrhagic moyamoya disease in East Asian Countries: a single-center case series and a systematic review with meta-analysis." 04/01/2022 - "Surgical revascularization vs. conservative treatment for adult hemorrhagic moyamoya disease: analysis of rebleeding in 322 consecutive patients." 01/01/2021 - "Comparison of Operative and Conservative Treatment for Asymptomatic Moyamoya Disease: Preliminary Experience in Small Retrospective Series."