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Zhongguo Dang Dai Er Ke Za Zhi 2019 Apr;21(4):354-358

Department of Radiology, Shengjing Hospital of China Medical University, Shenyang 110004, China.

Objective: To summarize and analyze the etiology, clinical manifestations and imaging features of children with cerebral infarction.

Methods: A retrospective analysis was performed for the clinical data of 54 children with cerebral infarction, including etiology, clinical manifestations, distribution of infarcts, type of infarcts and clinical outcome.

Results: Of the 54 children, 93% had a clear cause, among whom 46% had the coexistence of multiple factors, and the top three causes were infection (54%), vascular disease (40%) and trauma (26%). Read More

Brain Circ 2019 Jan-Mar;5(1):32-35. Epub 2019 Mar 27.

Department of Neurology, UT Health McGovern School of Medicine, Houston, Texas, USA.

Objective: The objective of this study was to investigate patterns of utilization and safety of extracranial-intracranial (EC-IC) bypass in patients with symptomatic cerebrovascular steno-occlusive disorders.

Methods: Patients with one of the steno-occlusive conditions (defined as symptomatic intracranial stenosis, extracranial stenosis, and moyamoya disease) were identified using all nonfederal hospitalizations in New York (2005-2014) and Florida (2005-2015). EC-IC bypass surgery was defined using the corresponding procedure codes. Read More

World Neurosurg 2019 Apr 14. Epub 2019 Apr 14.

Department of Neurosurgery, Kyoto University Graduate School of Medicine, Kyoto, Japan.

Background: While superficial temporal artery (STA) to middle cerebral artery (MCA) anastomosis is a beneficial treatment for hemorrhagic moyamoya disease, indeterminate mechanisms can cause rebleeding even after successful bypass surgery. The authors describe a case with a prominent collateral from the lenticulostriate artery (LSA) causing multiple recurrent hemorrhages after successful STA-MCA anastomosis.

Case: Description: A 49-year-old Japanese woman with moyamoya disease was referred to our institution after suffering intracranial hemorrhage on two occasions. Read More

World Neurosurg 2019 Apr 14. Epub 2019 Apr 14.

Department of Neurosurgery, Tianjin Medical University General Hospital, 154 Anshan Road, Tianjin 300052, China; Key Laboratory of Post-Neurotrauma Neurorepair and Regeneration in Central Nervous System, Ministry of Education in China and Tianjin, Tianjin Neurological Institute, Tianjin, 30052China. Electronic address:

Objective: 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 MMD to choose the appropriate treatment.

Methods: We comprehensively searched PubMed, Embase, Web of Science and the Cochrane Library for the articles published regarding MMD treatment. If the I value, which evaluated the heterogeneity, was less than 50%, a fixed effect model was used; if not, a random effect model was applied. Read More

Behav Neurol 2019 12;2019:7908392. Epub 2019 Mar 12.

Beijing Tsinghua Changgung Hospital, School of Clinical Medicine, Tsinghua University, Beijing, China.

Background And Purpose: Recently, several studies indicated the c.14576G>A variant on the ring finger protein 213 (RNF213), a founder variant of moyamoya diseases (MMD), was associated with non-MMD intracranial major artery stenosis/occlusion (non-MMD ICASO). We proposed that RNF213 variant-related ICASO including MMD might be a special entity with its own characteristics based on a genetic background. Read More

Neurosurgery 2019 Apr 15. Epub 2019 Apr 15.

Department of Neurosurgery and Stan-ford Stroke Center, Stanford University School of Medicine, Stanford, California.

Background: Poor natural history of hemorrhagic Moyamoya disease (MMD) is related to high rehemorrhage rates between 32% and 61%. Postrevascularization, rehemorrhage rates reportedly decrease to 12% to 17%.

Objective: To evaluate long-term functional outcomes and rehemorrhage rates of hemorrhagic MMD patients treated with surgical revascularization and examine these in relation to clinical and radiological factors. Read More

World Neurosurg 2019 Apr 10. Epub 2019 Apr 10.

Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, Sichuan, P.R.China. Electronic address:

Objectives: The neutrophil-to-lymphocyte ratio (NLR) reflects the balance between innate and adaptive inflammatory responses. This study intended to evaluate parameters associated with admission high NLR and its impact on clinical outcome in patients with primary intraventricular hemorrhage (PIVH).

Methods: This study retrospectively analyzed consecutive PIVH patients without a history of head trauma or parenchymal/subarachnoid hemorrhage on computed tomography scan between 2010 and 2016 in a single center. Read More

J Neurosurg Pediatr 2019 Apr 12:1-5. Epub 2019 Apr 12.

Departments of1Neurosurgery and.

Moyamoya syndrome predisposes patients to ischemic or hemorrhagic stroke due to progressive narrowing of intracranial vessels with subsequent small-vessel collateralization. Dural arteriovenous fistulae (DAVFs) are most commonly noted after venous sinus or cortical vein thrombosis and are believed to be primarily due to venous hypertension and elevated sinus pressures, although there is no known association with moyamoya syndrome, or with surgical treatment for moyamoya disease (MMD). The authors present the case of a 14-year-old girl with Down syndrome treated using pial synangiosis for MMD who subsequently was noted to have bilateral DAVFs. Read More

Zhonghua Yi Xue Za Zhi 2019 Apr;99(13):998-1002

Department of Neurosurgery, the Second Hospital of Shandong University, Jinan 250033, China.

To investigate the clinical efficacy of superficial temporal artery -middle cerebral artery combined with encephalo-duro-arterio-myo-synangiosis (STA-MCA+EDAMS) and encephalo-duro-arterio-myo-synangiosis (EDAMS) in the treatment of adult moyamoya disease. The clinical data of 47 adult patients with moyamoya disease who received vascular reconstruction in the Department of Neurosurgery of Taizhou Hospital of Zhejiang Province from January 2014 to January 2018 were retrospectively analyzed. Among them, 21 patients received EDAMS alone (EDAMS group, 14 patients with hemorrhagic moyamoya disease, 7 patients with ischemic moyamoya disease), 26 patients received STA-MCA combined with EDAMS (STA-MCA+EDAMS group, 17 patients with hemorrhagic moyamoya disease, 9 patients with ischemic moyamoya disease). Read More

World Neurosurg 2019 Apr 4. Epub 2019 Apr 4.

Department of Pediatric Neurosurgery Santobono-Pausilipon Children's Hospital, Naples, Italy.

Objective: Moyamoya disease (MMD) is characterized by a progressive spontaneous occlusion of distal internal carotid arteries (ICAs) and its main branches . It's necessary to promptly diagnose and treat this condition, especially in children, because of the high risk of stroke and consequent severe disability. In our paper, we examine the role of multiple burr holes surgery (MBHS) in the treatment of pediatric patients with MMD and quasi MMD and the role of perfusion MRI for the surgical indication and during the follow-up. Read More

Brain Behav 2019 Apr 5:e01241. Epub 2019 Apr 5.

Neuroimaging Laboratory, IRCCS Centro Neurolesi "Bonino Pulejo", Messina, Italy.

Introduction: Moyamoya disease (MMD) meaning "hazy puff of smoke" in Japanese is a rare chronic cerebrovascular syndrome characterized by progressive stenosis and occlusion of the internal carotid arteries (ICAs) anterior cerebral arteries (ACAs), and middle cerebral arteries (MCAs).

Methods: Our moyamoya patient with severely impaired cognitive and motor functions underwent a specific motor and neuropsychological rehabilitative treatments to assess the recovery of consciousness.

Results: Results obtained showed an improvement of clinical and neuropsychological examination. Read More

Cerebrovasc Dis 2019 Apr 4:1-7. Epub 2019 Apr 4.

Department of Neurosurgery, Tokyo Women's Medical University, Tokyo, Japan.

Background: Homozygosity of this p.R4810K founder variant of RNF213 moyamoya disease (MMD) susceptibility gene is known to influence the severity of the clinical disease phenotype at disease onset. However, the association between this genotype and long-term clinical manifestations has remained unclear. Read More

J Pediatr Neurosci 2018 Oct-Dec;13(4):514-516

Department of Neurology, Institute of Human Behaviour and Allied Sciences, New Delhi, India.

Case: Moyamoya disease (MMD) is a neurological disease involving internal carotid artery (ICA) leading to its occlusion. Among the children, the disease presents as ischemic strokes, whereas in adults, it presents as hemorrhagic strokes. Movement disorder among the MMD is very rare with varied presentation. Read More

Stroke 2019 May;50(5):1130-1135

From the Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea (C.J., J.Y.Y., S.-C.H., J.-S.K.).

Background and Purpose- Hemodynamic compromise has been implicated in moyamoya disease (MMD) with transient ischemic attacks or ischemic stroke. However, increasing evidence supports the notion that artery-to-artery embolism may also contribute to ischemic events based on microembolic signal (MES) monitoring. Methods- A total of 48 patients aged between 20 and 60 years with newly diagnosed MMD were enrolled and angiographically classified according to the Suzuki staging system. Read More

Ugeskr Laeger 2019 Mar;181(12)

The indication for cerebral bypass has changed drastically within the latest two decades due to advances in medical therapy and endovascular treatment. In this review, we discuss the various types of cerebral bypass and their application in modern neurosurgery, from cerebral hypoperfusion including cerebral atherosclerosis and moyamoya disease, to the treatment of complex cerebral aneurysms and skull-base tumours. The number of Danish patients treated abroad with cerebral bypass over the latest decade warrants establishment of cerebral bypass surgery in Denmark. Read More

Am J Psychiatry 2019 Apr;176(4):269-274

The Department of Psychiatry, Semel Institute for Neuroscience and Human Behavior, University of California, Los Angeles.

Neuroradiol J 2019 Mar 29:1971400919840843. Epub 2019 Mar 29.

1 Department of Neurosurgery, The First Hospital of Jilin University, China.

The posterior meningeal artery, which arises from the vertebral artery, is a critical artery in neurological lesions. However, a comprehensive review of the importance of the posterior meningeal artery is currently lacking. In this study, we used the PubMed database to perform a review of the literature on the posterior meningeal artery to increase our understanding of its role in vascular lesions. Read More

World Neurosurg 2019 Mar 25. Epub 2019 Mar 25.

Department of Neurosurgery, Tokyo Women's Medical University, Tokyo, Japan.

Background: Moyamoya syndrome (MMS), distinguished from definite moyamoya disease (MMD), is characterized by moyamoya vasculopathy thought to develop secondary to underlying conditions (e.g., hyperthyroidism). Read More

J Neuroimaging 2019 Mar 28. Epub 2019 Mar 28.

Department of Radiology, SUNY Upstate Medical University, Syracuse, NY.

Pediatric arteriopathies are increasingly recognized in school-aged children with a variety of presenting symptoms ranging from headache, seizures, encephalopathy, and neuropsychiatric symptoms as well as focal neurologic deficits due to acute ischemic strokes. However, unlike the adult stroke population, there are differences in the clinical manifestations, the stroke mechanism, and risk factors in pediatric ischemic stroke. There has been increasing awareness and recognition of pediatric cerebral arteriopathies as a predominant stroke etiology. Read More

Stroke 2019 May;50(5):1060-1066

From the Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, China (S.K., X.L., D.Z., R.W., Y.Z., Q.Z., J.-z.Z.).

Background and Purpose- Evidence on the natural history of hemorrhagic moyamoya disease is still insufficient. We investigated the incidence of recurrent intracranial bleeding, mortality, and risk factors for rebleeding in patients with moyamoya disease. Methods- A total of 128 conservatively managed patients with hemorrhagic presentation and complete follow-up data were included. Read More

Stroke 2019 Apr;50(4):789-796

Department of Neurology, Alfried Krupp Hospital Essen, Germany (J.C.S., M.K.).

Background and Purpose Moyamoya angiopathy (MMA) is a rare cerebral vasculopathy outside of Asia. In Japanese patients, a vast majority of patients carry the founder p.R4810K variant in the RNF213 gene, and familial cases are around 10%. Read More

Cureus 2019 Jan 10;11(1):e3863. Epub 2019 Jan 10.

Anesthesiology, Stanford University Medical Center, Stanford, USA.

Background Postoperative management in patients undergoing craniotomy is unique and challenging. We utilized a population of patients who underwent bilateral extracranial-to-intracranial (EC-IC bypass) revascularization procedures for moyamoya disease and hypothesized that 1 gram (gm) of intravenous (IV) acetaminophen given immediately after intubation and again 45 minutes prior to the end of craniotomy may be more effective than saline in minimizing opiate consumption and decreasing pain scores. Methods In a double-blind, randomized, placebo-controlled crossover pilot study, 40 craniotomies in 20 patients were studied. Read More

BMJ Open 2019 Mar 20;9(3):e025566. Epub 2019 Mar 20.

Department of Neurosurgery, Beijing Tiantan Hospital , Capital Medical University, Beijing, China.

Introduction: Revascularisation surgery is an important treatment of moyamoya disease (MMD). Several general methods of revascularisation had been used: direct, indirect and combined techniques. However, there had been no reports about the criteria of recipient arteries selection in bypass surgery for MMD. Read More

Mol Med Rep 2019 May 14;19(5):3564-3574. Epub 2019 Mar 14.

Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing 100050, P.R. China.

Moyamoya disease (MMD) is a rare chronic cerebrovascular disease mainly found in individuals of East Asian ethnicity, and its pathogenesis is largely unknown. Transfer RNA‑derived fragments (tRFs) are novel biological entities involved in many biological processes; however, whether tRFs contribute towards MMD pathogenesis remains unexplored. In the present study, deep sequencing technology was used to identify alterations in tRF expression profiles between patients with MMD and healthy controls. Read More

J Craniofac Surg 2019 Mar 19. Epub 2019 Mar 19.

Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University.

Background And Objective: Angiographic staging progression in the operated hemisphere of patient with moyamoya disease (MMD) is a common phenomenon that neurosurgeons may encounter. However, few studies have been carried out to demonstrate the correlation between the operation and angiographic staging progression. This study aimed to reveal whether cerebral revascularization would affect the stage progression in patient with MMD. Read More

J Pediatr Rehabil Med 2019 ;12(1):71-74

Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India.

We report the case of a six-year-old girl with Moyamoya disease who presented with bilateral internal carotid artery malignant infarct following encephaloduroarteriosynangiosis (EDAS). During her neurorehabilitation, she developed gradually worsening dystonic spasms with opisthotonic posturing, tachycardia, tachypnea and desaturation. This rare life threatening movement disorder was diagnosed as status dystonicus based on the history and clinical presentation. Read More

J Neurosurg Anesthesiol 2019 Mar 13. Epub 2019 Mar 13.

Departments of Anesthesiology.

Background: Moyamoya disease is a cerebrovascular disease characterized by bilateral stenosis of the intracranial internal carotid arteries and an abnormal collateral vascular network at the base of the brain. Transient neurological events (TNEs), which are episodes of neurological dysfunction lasting <24 hours, are associated with stroke in pediatric patients with Moyamoya disease. Perioperative agitation often occurs in pediatric patients. Read More

Acta Neurochir (Wien) 2019 Mar 18. Epub 2019 Mar 18.

Department of Neurosurgery, Kyoto University Graduate School of Medicine, 54 Kawahara-cho, Shogoin, Sakyo-ku, Kyoto, 606-8507, Japan.

Background: While periventricular anastomosis, a unique abnormal vasculature in moyamoya disease, has been studied in relation to intracranial hemorrhage, no study has addressed its change after bypass surgery. The authors sought to test whether direct bypass surgery could restore normal periventricular vasculature.

Methods: Patients who had undergone direct bypass surgery for moyamoya disease at a single institution were eligible for the study. Read More

World Neurosurg 2019 Mar 12. Epub 2019 Mar 12.

Department of Neurosurgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.

Background: Peripheral aneurysms are known to be a source of intracranial hemorrhage in patients with moyamoya disease. However, the natural history of ruptured peripheral aneurysms remains unclear. Some regress spontaneously, while others may show rebleeding. Read More

Exp Ther Med 2019 Mar 25;17(3):1977-1984. Epub 2019 Jan 25.

Department of Neurosurgery, The First People's Hospital of Jining, Jining, Shandong 272011, P.R. China.

Moyamoya disease (MMD) is a type of chronic cerebrovascular occlusion disease, which frequently occurs in East Asian populations, including pediatric and adult patients, and may lead to ischemic or hemorrhagic stroke, headache, epilepsy or transient ischemic attack. To date, the underlying mechanisms of MMD have remained to be fully elucidated, but certain studies have indicated that genetic factors may be an important component of its development. Cerebral angiography is the best approach for diagnosing MMD. Read More

Neurosurgery 2019 Mar 13. Epub 2019 Mar 13.

Department of Neurosurgery and Stan-ford Stroke Center, Stanford University School of Medicine and Stanford Health Care, Stanford, California.

Background: Traditional moyamoya disease (MMD) classification relies on morphological digital subtraction angiography (DSA) assessment, which do not reflect hemodynamic status, clinical symptoms, or surgical treatment outcome.

Objective: To (1) validate the new Berlin MMD preoperative symptomatology grading system and (2) determine the clinical application of the grading system in predicting radiological and clinical outcomes after surgical revascularization.

Methods: Ninety-six MMD patients (192 hemispheres) with all 3 investigations (DSA, magnetic resonance imaging [MRI], Xenon-CT) performed preoperatively at our institution (2007-2013) were included. Read More

Case Rep Cardiol 2019 4;2019:7124072. Epub 2019 Feb 4.

Department of Medicine, University of Tennessee Graduate School of Medicine, Knoxville, TN, USA.

Moyamoya disease is a rare condition that is primarily reported in Asian populations, characterized by stenoocclusive intracranial angiopathy with small, fragile, and multiple collateral vessel formation. Extracranial complications, mainly abnormalities within the renal vasculature, have been described; however, there are very few case reports of cardiovascular complications in patients with Moyamoya disease. We report a 26-year-old Caucasian female with known Moyamoya disease who presented with both typical and atypical chest pain, mimicking symptoms of a previous non-ST-elevation myocardial infarction. Read More

World Neurosurg 2019 Mar 9. Epub 2019 Mar 9.

Department of Neurosurgery, West China Hospital of Sichuan University, Sichuan, P.R. China.

Backgound: Asymmetric cortical vessel sign (ACVS) on susceptibility-weighted imaging (SWI) indicates elevated concentration of deoxyhemoglobin and elevated oxygen extraction fraction in patients with cerebral ischemia. This study aimed to clarify whether ACVS is associated with impaired hemodynamics and hyperperfusion syndrome in patients with moyamoya disease (MMD).

Methods: Consecutive adult patients with MMD were enrolled. Read More

Catheter Cardiovasc Interv 2019 Mar 12. Epub 2019 Mar 12.

Cardiovascular Division, Department of Medicine, University of Virginia Health System, Charlottesville, Virginia.

Tyrosine kinase inhibitors (TKIs) have assumed an increasingly vital role in treating various hematologic and oncologic malignancies. However, adverse effects with respect to vascular disease have been reported following administration of this class of medications. Here, we present a case report of TKI toxicity, manifesting as comorbid Moyamoya syndrome and obstructive coronary artery disease leading to a type 1 non-ST-elevation myocardial infarction. Read More

Magn Reson Imaging 2019 Jun 5;59:46-52. Epub 2019 Mar 5.

The Russell H. Morgan Department of Radiology & Radiological Science, Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA. Electronic address:

Purpose: To demonstrate the feasibility of mapping cerebrovascular reactivity (CVR) using resting-state functional MRI (fMRI) data without gas or other challenges in patients with cerebrovascular diseases and to show that brain regions affected by the diseases have diminished vascular reactivity.

Materials And Methods: Two sub-studies were performed on patients with stroke and Moyamoya disease. In Study 1, 20 stroke patients (56. Read More

Mol Cell 2019 Apr 4;74(1):32-44.e8. Epub 2019 Mar 4.

Department of Genetics and Complex Diseases, Harvard T.H. Chan School of Public Health, Boston, MA 02115, USA; Department of Cell Biology, Harvard Medical School, Boston, MA 02115, USA; Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA. Electronic address:

Excessive levels of saturated fatty acids are toxic to cells, although the basis for this lipotoxicity remains incompletely understood. Here, we analyzed the transcriptome, lipidome, and genetic interactions of human leukemia cells exposed to palmitate. Palmitate treatment increased saturated glycerolipids, accompanied by a transcriptional stress response, including upregulation of the endoplasmic reticulum (ER) stress response. Read More

World Neurosurg 2019 Mar 4. Epub 2019 Mar 4.

Department of Neurosurgery, Graduate School of Medicine and Pharmaceutical Science, University of Toyama, Toyama, Japan.

Objective: The involved carotid forks in moyamoya disease (MMD) will show decreases in both luminal caliber and outer diameter. The present study aimed to clarify the changes in the outer diameter associated with luminal stenosis/occlusion of the posterior cerebral artery (PCA) in patients with MMD.

Methods: The present study included 24 pediatric and 48 adult patients with MMD and 17 healthy adult controls. Read More

J Neurosurg Pediatr 2019 Mar 1:1-7. Epub 2019 Mar 1.

OBJECTIVEThere are limited data on the long-term outcomes for children undergoing surgical revascularization for moyamoya disease (MMD) in North America. The authors present a series of pediatric MMD patients who underwent a standard revascularization procedure, pial synangiosis, more than 20 years previously at a single institution by a single surgical team.METHODSThis study is a retrospective review of all patients aged 21 years or younger treated for MMD at Boston Children's Hospital who were operated on more than 20 years previously by the senior author (R. Read More

World Neurosurg 2019 Mar 1. Epub 2019 Mar 1.

Department of neurosurgery, the First affiliated hospital of soochow university. Electronic address:

Background: Extracranial internal carotid aneurysm (EICA) is regarded as one kind of rare lesion. What is more, patients suffer EICAs and hemorrhagic moyamoya disease(MMD) simultaneously, which make neurosurgeons feel overwhelmed and helpless.

Case Description: A 38-year-old male was admitted to our hospital and underwent emergent tracheal intubation for hoarseness and breathing difficulty for one day. Read More

BMC Neurol 2019 Mar 2;19(1):34. Epub 2019 Mar 2.

Department of Neurology, National Hospital Organization Nagoya Medical Center, 4-1-1 Sannomaru, Naka-ku, Nagoya, Aichi, Japan.

Background: Moyamoya vasculopathy (MMV) associated with Graves' disease (GD) is a rare condition resulting in ischemic stroke accompanied by thyrotoxicity. Radiological findings of vasculitis have been reported in the walls of distal internal carotid arteries (ICAs) in these patients; however, no reports have described in detail the processes of progression of the lesions in the proximal ICA. Moreover, treatments to prevent recurrence of ischemic stroke and progression of MMV have not yet been sufficiently elucidated. Read More

Curr Neurol Neurosci Rep 2019 Feb 28;19(4):17. Epub 2019 Feb 28.

Department of Neurology, University of Illinois at Chicago College of Medicine, 912 S Wood Street, Suite 164C, Chicago, IL, USA.

Purpose Of Review: Sickle cell anemia is a multiorgan disease with acute and chronic complications. Involvement of the central nervous system (CNS) is associated with increased mortality and morbidity. This review highlights the broad spectrum of neurological complications seen in patients with sickle cell disease. Read More

J Neurol Surg A Cent Eur Neurosurg 2019 May 28;80(3):143-148. Epub 2019 Feb 28.

Department of Anesthesiology, University of Texas John P. and Katherine G. McGovern Medical School, Houston, Texas, United States.

Background:  Moyamoya disease (MMD) is a rare cerebrovascular condition, often presenting as a headache or stroke in adults. Anesthetic management of this illness may challenge providers because it can affect the long-term neurologic outcome and hospital length of stay (LOS) in patients with MMD.

Materials And Methods:  A literature search was conducted to assess etiology and epidemiology, as well as existing reports of intraoperative management of MMD. Read More

Childs Nerv Syst 2019 May 27;35(5):823-831. Epub 2019 Feb 27.

Division of Pediatric Neurosurgery, Seoul Pediatric Clinical Neuroscience Center, Seoul National University Children's Hospital, 101 Daehak-ro, Jongno-gu, Seoul, 110-744, Republic of Korea.

Purpose: Moyamoya disease (MMD) is one of the most common causes of pediatric stroke. We found defective angiogenic function and downregulation of retinaldehyde dehydrogenase 2 (RALDH2) in MMD endothelial colony-forming cells (ECFCs). Downregulation of RALDH2 mRNA was caused by decreased binding of acetyl-histone H3 (Ac-H3) to the RALDH2 promoter. Read More

Radiol Case Rep 2019 Apr 11;14(4):476-479. Epub 2019 Feb 11.

Department of Cerebrovascular Surgery, International Medical Center, Saitama Medical University, Hidaka, Japan.

Moyamoya disease is a chronic cerebrovascular disease characterized by spontaneous and progressive stenosis or occlusion of the internal carotid artery and its branches. Revascularization procedures have been shown to improve cerebral hemodynamics and decrease the risk of strokes, but several postoperative complications are known to occur.A 14-year-old girl with moyamoya disease with a history of left-sided revascularization surgery underwent right-sided revascularization. Read More

Neurol Res 2019 May 18;41(5):480-487. Epub 2019 Feb 18.

a Department of Neurosurgery , School of Medicine, Iwate Medical University , Morioka , Japan.

Objective: Adult patients with ischemic moyamoya disease (MMD) who receive treatment with antiplatelet drugs reportedly show improvements in neuropsychological test scores after around 2 years. The purpose of the present study subanalyzing the same patient cohort used in a previous study was to determine which antiplatelet drug, clopidogrel or cilostazol, results in better improvement of cognitive function among non-surgical adult patients showing ischemic MMD without severe hemodynamic compromise.

Methods: Sixty-six patients without cerebral misery perfusion on O gas positron emission tomography were treated with pharmacotherapy alone. Read More

J Stroke Cerebrovasc Dis 2019 May 13;28(5):e46-e50. Epub 2019 Feb 13.

Department of Neurosurgery, Tohoku University, Sendai, Japan.

Moyamoya syndrome (MMS) is an uncommon late complication after cranial irradiation. Its hemorrhagic presentation from the associated pseudo-aneurysm is extremely rare, and the optimal management strategy is undetermined. We herein report a 36-year-old man who developed intraventricular hemorrhage from a pseudo-aneurysm at the extended left anterior choroidal artery as an abnormal collateral of MMS 30 years after surgical removal and cranial irradiation for childhood craniopharyngioma. Read More

J Neurosurg 2019 Feb 15:1-13. Epub 2019 Feb 15.

1Department of Neurosurgery, Peking University International Hospital.

OBJECTIVEPostoperative neurological deficits impair the overall outcome of revascularization surgery for patients with moyamoya disease (MMD). dl-3-n-butylphthalide (NBP) is approved for the treatment of ischemic stroke in China. This pilot study evaluated the effect of NBP on perioperative stroke and neurological deficits in patients with MMD. Read More

Neurosurg Rev 2019 Feb 14. Epub 2019 Feb 14.

Department of Neurosurgery, Sapporo Medical University, South 1 West 16, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan.

Extracranial-to-intracranial (EC-IC) bypass surgery is an effective treatment for patients with moyamoya disease and other conditions. Some patients with moyamoya disease have a risk of acute thrombogenesis at the anastomotic site just after bypass surgery. The purpose of this study was to study risk factors of acute thrombogenesis and determine effective countermeasures. Read More

Clin Neurol Neurosurg 2019 Mar 4;178:86-92. Epub 2019 Feb 4.

Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, No. 119 South Fourth Ring Road, Beijing, 100070, China. Electronic address:

Objectives: Anterior cerebral artery (ACA) territory, a crucial area of intellectual development in children, is frequently involved in the progress of moyamoya disease (MMD). However, revascularization surgeries for this area are not as established as surgeries for middle cerebral artery (MCA) territory. This study aimed to describe our experience and study the effect of revascularizing ACA territory with periocranium and dural leaflets, which is referred to as 'encephalo-duro-periosteal-synangiosis (EDPS)'. Read More

Neuroimage Clin 2019 Feb 4;22:101713. Epub 2019 Feb 4.

Department of Neurosurgery, Eberhard Karls University Tübingen, Germany.

Background And Purpose: Patients with Moyamoya Disease (MMD) need hemodynamic evaluation of vascular territories at risk of stroke. Today's investigative standards include HO PET/CT with pharmacological challenges with acetazolamide (ACZ). Recent developments suggest that CO-triggered blood‑oxygen-level-dependent (BOLD) functional MRI might provide comparable results to current standard methods for evaluation of territorial hemodynamics, while being a more widely available and easily implementable method. Read More