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Circ Genom Precis Med 2018 Dec;11(12):e002317

Center for Medical Genetics, Keio University School of Medicine, Tokyo, Japan (H.S., K. Kosaki).

J Neurosurg 2018 Dec 14:1-7. Epub 2018 Dec 14.

OBJECTIVERevascularization surgery is a safe and effective surgical treatment for symptomatic moyamoya disease (MMD) and has been shown to reduce the frequency of future ischemic events and improve quality of life in affected patients. The authors sought to investigate the occurrence of acute perioperative occlusion of the contralateral internal carotid artery (ICA) with contralateral stroke following revascularization surgery, a rare complication that has not been previously reported.METHODSThis study is a retrospective review of a prospective database of a single surgeon's series of revascularization operations in patients with MMD. Read More

J Neurosurg 2018 Dec 14:1-9. Epub 2018 Dec 14.

OBJECTIVESome adult patients with moyamoya disease (MMD) undergoing revascularization surgery show an improvement or decline in cognition postoperatively. Revascularization surgery for ischemic MMD augments cerebral blood flow (CBF) and improves cerebral oxygen metabolism. However, cerebral hyperperfusion, which is a short-term, major increase in ipsilateral CBF that is much greater than the metabolic needs of the brain, sometimes occurs as a complication. Read More

Case Rep Crit Care 2018 13;2018:2591494. Epub 2018 Nov 13.

Lincoln Medical and Mental Health Center, 234 E 149Th St., Bronx, NY 10451, USA.

Moyamoya disease is a rare condition affecting the circle of Willis and its branching arteries. While the pathogenesis is unclear, it causes progressive occlusion of multiple cerebral vessels leading to severe strokes. We report a case of a 47-year-old Hispanic woman with HTN presented with altered mental status and bilateral upper and lower extremity weakness with dystonic-like upper extremity movement. Read More

Surg Neurol Int 2018 2;9:224. Epub 2018 Nov 2.

Department of Neurosurgery, Division of Pediatric Neurosurgery, Baylor College of Medicine/Texas Children's Hospital, Houston, TX 77030, USA.

World Neurosurg 2018 Dec 5. Epub 2018 Dec 5.

Department of Neurosurgery, Kyoto University Graduate School of Medicine, 54 Kawaramachi, Shogoin, Sakyo-ku, Kyoto City, Kyoto 606-8507, JAPAN.

Background: The surgical intervention in a case of internal carotid artery stenosis with Moyamoya vessels has not been well described. We present such a case with detailed description about its procedure and perioperative management.

Case Description: A 58-year-old man with symptomatic internal carotid artery stenosis had concurrent moyamoya vessels intracranially. Read More

Magn Reson Med Sci 2018 Dec 6. Epub 2018 Dec 6.

Department of Neurosurgery, Tokyo Medical and Dental University, 1-5-45.

Turk J Pediatr 2018 ;60(3):331-334

Departments of Vascular Surgery, Xuanwu Hospital, Capital Medical University; Institute of Vascular Surgery, Capital Medical University, Beijing, China.

Guo J, Guo L, Zeng G, Tong Z, Gao X, Gu Y. An infant case of renovascular hypertension in moyamoya disease treated by angioplasty. Turk J Pediatr 2018; 60: 331-334. Read More

Neurosurg Rev 2018 Dec 3. Epub 2018 Dec 3.

Department of Neurosurgery, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo, 162-8666, Japan.

The effects of superficial temporal artery to middle cerebral artery (STA-MCA) double bypass on recurrent hemorrhage in the operated hemisphere in hemorrhagic moyamoya disease (HMD) have not been clearly demonstrated. This study evaluated the effectiveness of STA-MCA double bypass in the prevention of further hemorrhagic or ischemic events in the operated hemispheric sides in comparison to the conservatively treated non-operated sides. We retrospectively analyzed 52 hemispheres of 36 patients with adult-onset HMD treated with STA-MCA double bypass. Read More

Clin Neuroradiol 2018 Dec 3. Epub 2018 Dec 3.

Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, 6th Tiantanxili, 100050, Beijing, China.

Objective: Indirect bypass surgery, which induces spontaneous neoangiogenesis in ischemic brain tissue and improves cerebral blood flow, is an effective treatment for moyamoya disease (MMD). The time course of neoangiogenesis development has not yet been investigated. This study aimed to determine the critical period of neovascularization after indirect bypass in MMD patients. Read More

Brain Imaging Behav 2018 Dec 3. Epub 2018 Dec 3.

Department of Neurosurgery, Huashan Hospital, Fudan University, Wulumuqi Zhong Road 12, Shanghai, 200040, China.

Treatment of vascular cognitive impairment (VCI) in adult moyamoya disease (MMD) is still unclear because of its unveiled neural synchronization. This study introduced a dynamic measurement of connectivity number entropy (CNE) to characterize both spatial and temporal dimensions of network interactions. Fifty-one patients with MMD were recruited (27 with VCI and 24 with intact cognition), as well as 26 normal controls (NCs). Read More

J Neurosurg Pediatr 2018 Nov 1:1-6. Epub 2018 Nov 1.

Analysis of direct STA-MCA bypass procedures in young children to achieve flow augmentation via direct revascularization. Read More

J Neurosurg 2018 Nov 1:1-7. Epub 2018 Nov 1.

Moyamoya disease (MMD) is a progressive, idiopathic cerebrovascular occlusive disease. Various revascularization techniques including direct, indirect, and combined microvascular bypasses have been described. This article presents a modified revascularization technique for MMD utilizing a pedicled temporoparietal fascial flap (TPFF) for combined revascularization. Read More

World Neurosurg 2018 Nov 26. Epub 2018 Nov 26.

Division of Pediatric Neurosurgery, University of Florida Health Jacksonville, Jacksonville, FL. Electronic address:

Background: The present systematic review aims to identify and analyze the available evidence on the safety and efficacy of surgical revascularization for pediatric patients with sickle cell disease (SCD) and moyamoya disease (MMD).

Methods: A systematic review was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The following databases were searched: PubMed, Ovid MEDLINE, and Scopus. Read More

Acta Neurochir (Wien) 2018 Nov 24. Epub 2018 Nov 24.

Department of Neurosurgery, Antwerp University Hospital, Wilrijkstraat 10, 2650, Edegem, Belgium.

Background: Burr hole surgery for moyamoya disease and moyamoya syndrome is known to be an effective, versatile, and relatively simple revascularization technique. We will focus on the technical operative aspects of multiple burr hole surgery as we perform it in our center.

Methods: Periosteal flaps are prepared and placed in a burr hole with beveled edge, after opening the dura and arachnoid membrane, in order to facilitate neovascularization into the ischemic cortex. Read More

Int J Mol Sci 2018 Nov 20;19(11). Epub 2018 Nov 20.

Cerebrovascular Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy.

Moyamoya angiopathy (MA) is a cerebrovascular disease determining a progressive stenosis of the terminal part of the internal carotid arteries (ICAs) and their proximal branches and the compensatory development of abnormal "moyamoya" vessels. MA occurs as an isolated cerebral angiopathy (so-called moyamoya disease) or in association with various conditions (moyamoya syndromes) including several heritable conditions such as Down syndrome, neurofibromatosis type 1 and other genomic defects. Although the mechanism that links MA to these genetic syndromes is still unclear, it is believed that the involved genes may contribute to the disease susceptibility. Read More

Medicine (Baltimore) 2018 Nov;97(47):e13497

Asian J Neurosurg 2018 Oct-Dec;13(4):1236-1238

Department of Neurosurgery, Konyang University Hospital, Daejeon, South Korea.

Collateral vessel formation in moyamoya disease is a well-described phenomenon. However, the occurrence of unusual anastomosis pattern (moyamoya-like) associated with isolated middle cerebral artery (MCA) stenosis or occlusion has been reported very rarely and is not well known the relationship with aneurysm. We report a case of ruptured aneurysm treated with N-butyl cyanoacrylate (NBCA) located in moyamoya like collateral network with isolated MCA occlusion. Read More

Asian J Neurosurg 2018 Oct-Dec;13(4):1008-1010

Department of Internal Medicine, Seth Gordhandas Sunderdas Medical College and King Edward Memorial Hospital, Mumbai, Maharashtra, India.

Background: Craniotomy and surgical clipping is the standard modality of treatment in patients of cerebral aneurysms. However, the surgical clipping of aneurysm may pose serious difficulties in the case of a bilateral carotid artery occlusion. The endovascular treatment has shown promising results in this disease. Read More

Brain Behav 2018 Nov 19:e01154. Epub 2018 Nov 19.

Department of Biomedical Engineering, Center for Biomedical Imaging Research, Tsinghua University School of Medicine, Beijing, China.

Background: It has been shown that intracranial artery stenosis (ICAS) plays a key role in Chinese ischemic stroke or transient ischemic attack (TIA) patients. Many vascular diseases can lead to ICAS, such as atherosclerosis, dissection, vasculitis, moyamoya disease, and reversible cerebral vasoconstriction syndrome (RCVS). In addition, progression of intracranial atherosclerotic disease (ICAD) will increase the risk of ischemic cerebrovascular events. Read More

CMAJ 2018 Nov;190(46):E1364

Department of General Medicine, Chiba University Hospital, Chiba, Japan

Kyobu Geka 2018 Nov;71(12):1031-1034

Department of Cardiovascular Surgery, Japanese Red Cross Kochi Hospital, Kochi, Japan.

A 62-year-old female with Moyamoya disease (MMD) had cardiomegaly pointed out by chest X-ray and was admitted to our hospital. Chest computed tomography (CT) scan and echocardiography revealed a large dissecting ascending aortic aneurysm 78 mm in diameter combined with severe aortic regurgitation and mild mitral regurgitation. She had a history of intracranial hemorrhage related with MMD twice. Read More

Rev Neurol (Paris) 2018 Nov 14. Epub 2018 Nov 14.

Service de neurochirurgie, université Bourgogne-Franche-Comté, CHRU de Besançon, 25000 Besançon, France.

Introduction: Moyamoya angiopathy (MMA) is a progressive steno-occlusive disease of the distal internal carotid arteries mainly described in Asia. It induces the development of collateral vascular networks to reduce chronic cerebral hypoperfusion. Symptoms depend on the patient's age in Asia: children are at greater risk of transient or constituted ischemic events, whereas adults are more exposed to hemorrhagic stroke. Read More

World Neurosurg 2018 Nov 14. Epub 2018 Nov 14.

Radiology Department, Beijing Tiantan Hospital, Beijing Neurosurgical Institute, Capital Medical University, Beijing, China. Electronic address:

Objective: To use territory arterial spin labeling (T-ASL) in the early postoperative period to evaluate the revascularization area (RA) obtained by superficial temporal artery-to-middle cerebral artery bypass and to evaluate subsequent perfusion territory changes of the major cerebral arteries.

Methods: Thirty patients with moyamoya disease treated via unilateral superficial temporal artery-to-middle cerebral artery bypass were included. T-ASL was performed preoperatively and within 1 week postoperatively. Read More

Magn Reson Med 2018 Nov 15. Epub 2018 Nov 15.

Philips Healthcare BIU MR, Asia Pacific, Tokyo, Japan.

Purpose: A new sequence for intracranial MRA is developed, named enhanced acceleration-selective arterial spin labeling (eAccASL), to improve main artery visualization at middle cerebral artery (MCA). The aim of this study is to assess the visualization improvement using eAccASL, compared with the previously developed AccASL.

Methods: eAccASL and AccASL were performed in 8 healthy volunteers and images were compared between the 2 sequences. Read More

J Korean Neurosurg Soc 2018 Nov 15. Epub 2018 Nov 15.

Department of Neurosurgery, Tohoku University, Sendai, Japan.

Moyamoya disease (MMD) is a progressive cerebrovascular disease with unknown etiology, characterized by bilateral stenoocclusive changes at the terminal portion of the internal carotid artery and an abnormal vascular network formation at the base of the brain. MMD has an intrinsic nature to convert the vascular supply for the brain from internal carotid (IC) system to the external carotid (EC) system, as indicated by Suzuki's angiographic staging. Insufficiency of this 'IC-EC conversion system' could result not only in cerebral ischemia, but also in intracranial hemorrhage from inadequate collateral anastomosis, both of which represent the clinical manifestation of MMD. Read More

Pediatr Neurol 2018 Oct 15. Epub 2018 Oct 15.

Department of Neurology, University of Washington, Seattle, Washington.

Moyamoya is a chronic, progressive steno-occlusive arteriopathy that typically affects the anterior circulation arteries of the circle of Willis. A network of deep thalamoperforating and lenticulostriate collaterals develop to by-pass the occlusion giving rise to the characteristic angiographic "puff of smoke" appearance. Moyamoya confers a lifelong risk of stroke and neurological demise, with peak age of presentation in childhood ranging between five and 10 years. Read More

Interv Neurol 2018 Oct 16;7(6):323-326. Epub 2018 May 16.

Interventional Neurology, Mercy Health St. Vincent Medical Center, Toledo, Ohio, USA.

The anterior cerebral artery (ACA) is a unique artery with many important variations with substantial clinical significance. Tortuous intracranial arteries usually occur in basilar, communicating, anterior, posterior cerebral arteries and in the white matter arterioles. This could happen for many reasons including but not limited to ageing, hypertension, patients with Moyamoya disease, congenital malformation, or increased flow associated with elastin degradation. Read More

J Stroke Cerebrovasc Dis 2018 Nov 5. Epub 2018 Nov 5.

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

Background: There are a variety of collateral routes to compensate persistent cerebral ischemia in moyamoya disease. However, there is no report presenting the persistent primitive olfactory artery (POA) as a spontaneous collateral route to the anterior cerebral artery (ACA) in moyamoya disease.

Methods: We precisely examined cerebral angiography in 84 patients with moyamoya disease to identify the collateral channel through the persistent POA. Read More

World Neurosurg 2018 Nov 4. Epub 2018 Nov 4.

Department of Neurosurgery, The Center for Cerebral Vascular Disease, Academy of Military Medical Science, Beijing, China. Electronic address:

Background: To our knowledge, no previous study has described the nationwide epidemiologic features of moyamoya disease (MMD) in China. We describe the epidemiologic features including the relative prevalence, age distribution, gender distribution, and initial clinical manifestations of patients with MMD treated at a single institution in China.

Methods: Our cohort included 4128 patients with MMD. Read More

Neuroradiology 2018 Nov 6. Epub 2018 Nov 6.

Center for Biomedical Imaging Research, Department of Biomedical Engineering, Tsinghua University School of Medicine, Beijing, China.

Purpose: To investigate the association between proximal internal carotid artery (ICA) luminal narrowing and diffuse wall thickening (DWT) in ipsilateral petrous ICA in moyamoya disease (MMD) patients.

Methods: Forty-one MMD (mean age 42.8 ± 11. Read More

J Stroke Cerebrovasc Dis 2018 Nov 1. Epub 2018 Nov 1.

Department of Neurosurgery, Gonzalez Neurovascular Laboratory, Cedars-Sinai Medical Center, Los Angeles, California; Department of Neurology, Cedars-Sinai Medical Center, Los Angeles, California. Electronic address:

Background: Vascular endothelial growth factor-A (VEGF-A) has been identified as a combination of 2 alternative splice variants: proangiogenic VEGF-Aa and antiangiogenic VEGF-Ab. Intracranial atherosclerotic disease (ICAD) and moyamoya disease (MMD) are 2 main types of intracranial arterial steno-occlusive disorders with distinct capacities for collateral formation. Recent studies indicate that VEGF-A regulates collateral growth in ischemia. Read More

J Clin Neurosci 2018 Oct 30. Epub 2018 Oct 30.

Department of Neurosurgery, Stanford University School of Medicine, Stanford, CA, USA. Electronic address:

Patients with moyamoya disease develop intracranial aneurysms at a higher rate than the general population. The authors aimed to test the hypothesis for development of aneurysms on large arteries in such patients using quantitative vessel imaging. Twenty-six patients representing 3. Read More

Sichuan Da Xue Xue Bao Yi Xue Ban 2018 Sep;49(5):749-753

Department of Radiology, West China Hospital, Sichuan University, Chengdu 610041, China.

Objective: To determine consistencies between MR perfusion weighted imaging (PW-MRI) and CT perfusion imaging (CTP) in assessing hemodynamics of patients with moyamoya disease (MMD).

Methods: Images of PWI and CTP scan [including the map of cerebral blood flow (CBF), cerebral blood volume (CBV), time to peak (TTP), and mean transmit time (MTT)] of 24 MMD patients diagnosed by digital subtraction angiography(DSA) or time of flight-MR angiography (TOF-MRA) were obtained in the week before vascular reconstruction operations. Relative perfusion parameters (rCBF, rCBV, rMTT, rTTP) at the operative side within the middle cerebral artery territory and cerebellar hemisphere were compared with those of 17 patients without hypoperfusion and cerebrovascular disease using independent sample test. Read More

Neurol Med Chir (Tokyo) 2018 Nov 25;58(11):481-484. Epub 2018 Oct 25.

Department of Neurosurgery, Iwate Medical University School of Medicine.

Cerebral hyperperfusion syndrome following arterial bypass surgery is known as a surgical complication of moyamoya disease (MMD). How cerebral hyperperfusion affects neural function and causes neurological deficits remains unknown. We report here a case with cerebral hyperperfusion syndrome after arterial bypass surgery for ischemic MMD. Read More

Indian J Ophthalmol 2018 Nov;66(11):1644-1646

Department of Ophthalmology, Little Flower Hospital and Research Centre, Angamaly, Kerala, India.

Moyamoya disease (MMD) is a chronic progressive, occlusive cerebrovascular disease in the circle of Willis and the feeding arteries. Morning glory disc anomaly (MGDA) is characterized by an abnormal excavated optic disc with radial emergence of blood vessels from the rim of the disc. We describe a case of moyamoya syndrome (MMS), a rare entity among Indian ethnicity, associated with MGDA and regressed facial capillary hemangiomas, which are relatively less reported presentations of MMD. Read More

Stroke 2018 Nov;49(11):2597-2604

From the Translational and Stem Cell Research Laboratory on Stroke (J.-W.C., D.H.K., M.J.O.,Y.H.C., E.H.K., O.Y.B.), Samsung Medical Center, Seoul, Republic of Korea.

Background and Purpose- Moyamoya disease (MMD) is a unique cerebrovascular occlusive disease characterized by progressive stenosis and negative remodeling of the distal internal carotid artery (ICA). We hypothesized that cav-1 (caveolin-1)-a protein that controls the regulation of endothelial vesicular trafficking and signal transduction-is associated with negative remodeling in MMD. Methods- We prospectively recruited 77 consecutive patients with MMD diagnosed via conventional angiography. Read More

Stroke 2018 Oct;49(10):2504-2507

From the Department of Neurosurgery, Tokyo Medical and Dental University, Japan (S.H., Y.T, M.I., T.M., and T.N.).

Background and Purpose -Microstructural damage in the brain induced by chronic ischemia is suggested to play a pivotal role in the neurocognitive dysfunction of adults with Moyamoya disease (MMD). We investigated specific changes in the brain microstructure and their correlations with neurocognitive dysfunction in patients with MMD using a multishell diffusion magnetic resonance imaging technique called neurite orientation dispersion and density imaging. Methods- We evaluated 26 patients with MMD (16-63 years old, 20 females) and 20 age- and sex-matched normal volunteers using neurite orientation dispersion and density imaging and neuropsychological batteries. Read More

World Neurosurg 2018 Oct 19. Epub 2018 Oct 19.

Department of Gynecology and Obstetrics, Kyoto University Graduate School of Medicine, Kyoto, Japan.

Background: Pregnancy is a potential risk factor for stroke in women with Moyamoya disease. However, the rarity of the disease has limited clinical expertise to ensure a healthy pregnancy. The aim of the present study was to explore the possible risk factors for hemorrhagic and ischemic stroke in pregnant women with Moyamoya disease. Read More

Med Sci Monit 2018 Oct 19;24:7469-7474. Epub 2018 Oct 19.

Department of Neurosurgery, The Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou, Jiangsu, China (mainland).

BACKGROUND Moyamoya disease (MMD) is an idiopathic disease caused by progressive steno-occlusion of the distal internal carotid artery. Ideal surgical treatment for adult patients with ischemic-type MMD has not been achieved. The aim of this study was to evaluate the efficacy of single-barrel superficial temporal artery-middle cerebral artery (STA-MCA) bypass in treatment for adult patients with ischemic-type MMD by analyzing clinical and radiological data retrospectively. Read More

NMC Case Rep J 2018 Oct 13;5(4):105-109. Epub 2018 Sep 13.

Department of Neurosurgery, Hiroshima University Graduate School of Biomedical and Health Sciences, Hiroshima, Hiroshima, Japan.

Concurrent multiple tumors developing in the spinal cord are rare, except for in genetic disorders, such as neurofibromatosis and von Hippel-Lindau disease. Furthermore, concurrent tumors arising in the same spinal level with discrete histopathology are much rarer. We report two such cases. Read More

World Neurosurg 2018 Oct 11. Epub 2018 Oct 11.

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

Objective: Recent three-dimensional constructive interference in steady state (3D-CISS) studies have shown that the involved arteries decrease not only their own luminal caliber but also outer diameter in moyamoya disease (MMD). This study was aimed to clarify how the outer diameter of the involved arteries serially change during disease progression in MMD using qunatitaive 3D-CISS imaging.

Methods: This study included 8 hemispheres of 7 patients with MMD whose Suzuki angiographic stage spontaneously progressed during follow-up. Read More

Medicine (Baltimore) 2018 Oct;97(41):e12768

Department of Radiology.

The aim of this study was to explore the correlation between multi-inversion time arterial spin labeling (mTI-ASL) and dynamic susceptibility contrast-enhanced perfusion weighted imaging (DSC-PWI) in assessment of hemodynamics of patients with Moyamoya disease (MMD).In this study, 24 MMD patients and 21 healthy subjects were enrolled between June 2017 and December 2017. The images of mTI-ASL and DSC-PWI in the week before revascularization surgery were retrospectively analyzed. Read More

J Stroke 2018 Sep 30;20(3):350-361. Epub 2018 Sep 30.

Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.

Background And Purpose: The pathogenesis of moyamoya disease (MMD) remains poorly understood, and no reliable molecular biomarkers for MMD have been identified to date. The present study aimed to identify epigenetic biomarkers for use in the diagnosis of MMD.

Methods: We performed integrated analyses of gene expression profiles and DNA methylation profiles in endothelial colony forming cells (ECFCs) from three patients with MMD and two healthy individuals. Read More

J Stroke 2018 Sep 30;20(3):332-341. Epub 2018 Sep 30.

Department of Neurology and Neurosurgery, Brain Center Rudolf Magnus, University Medical Center (UMC) Utrecht, Utrecht, The Netherlands.

Background And Purpose: Patients with moyamoya vasculopathy (MMV) may experience cognitive impairment, but its reported frequency, severity, and nature vary. In a systematic review and metaanalysis, we aimed to assess the presence, severity, and nature of cognitive impairments in children and adults with MMV.

Methods: We followed the MOOSE guidelines for meta-analysis and systematic reviews of observational studies. Read More

Cerebrovasc Dis 2018 9;46(3-4):161-171. Epub 2018 Oct 9.

Carilion Clinic, Section of Neurosurgery, Roanoke, Virginia, USA.

Background: Missense mutations in the gene that codes for smooth muscle actin, ACTA2, cause diffuse smooth muscle dysfunction and a distinct cerebral arteriopathy collectively known as multisystemic smooth muscle dysfunction syndrome (MSMDS). Until recently, ACTA2 cerebral arteriopathy was considered to be a variant of moyamoya disease. However, recent basic science and clinical data have demonstrated that the cerebral arteriopathy caused by mutant ACTA2 exhibits genetic loci, histopathology, neurological sequelae, and radiographic findings unique from moyamoya disease. Read More

Eur J Intern Med 2018 Oct 4. Epub 2018 Oct 4.

Department of Gastroenterology, Fuyoukai Murakami Hospital, 3-3-14 Hamada, Aomori 030-0843, Japan. Electronic address:

Childs Nerv Syst 2018 Oct 3. Epub 2018 Oct 3.

Department of Neurosurgery, Korea University Medical Center, Ansan Hospital, 123 Jeokgeum-ro, Danwon-gu, Ansan, Gyeonggi-do, 15355, South Korea.

Purpose: Moyamoya disease is a chronic cerebrovascular disorder characterized by progressive stenosis of the circle of Willis with a compensatory collateral vessel network. Recent studies have identified the ring finger protein 213 gene (RNF213) as the unique susceptibility gene for moyamoya disease. The purpose of this study was to compare clinical features of moyamoya disease, especially angiographic findings, between patients with and without the RNF213 mutation. Read More

Asian J Neurosurg 2018 Jul-Sep;13(3):769-773

Department of Neurosurgery, Istituto Giannina Gaslini Children's Hospital, Genova, Italy.

Introduction: Moyamoya disease is a steno-occlusive cerebrovascular disease of unknown etiology involving the terminal portion of the internal carotid artery and the proximal portions of the anterior and middle cerebral arteries with associated collateral vascular network. When the vascular pattern is associated with a particular condition (e.g. Read More

Brain Circ 2018 Jan-Mar;4(1):33-39. Epub 2018 Apr 18.

Department of Neurology, Xuanwu Hospital of Capital Medical University, Beijing, China.

Background And Purpose: The ring finger protein 213 (RNF213) gene R4810K variant, a susceptibility locus for moyamoya disease (MMD), has recently been identified to be associated with intracranial major artery stenosis/occlusion (ICASO) without satisfying the diagnostic criteria of MMD in the Japanese population. However, further studies are needed to determine whether this variant is associated with ICASO in other populations and whether R4810K variant-related ICASO could be categorized as MMD. The aim of this study is to elucidate whether the R4810K variant was associated with ICASO among the Han Chinese population and potential histopathology of R4810K variant-related ICASO. Read More