3,777 results match your criteria Moyamoya Disease


Cilostazol may improve cognition better than clopidogrel in non-surgical adult patients with ischemic moyamoya disease: subanalysis of a prospective cohort.

Neurol Res 2019 Feb 18:1-8. 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

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http://dx.doi.org/10.1080/01616412.2019.1580455DOI Listing
February 2019

Efficacy of Direct Revascularization Surgery for Hemorrhagic Moyamoya Syndrome As a Late Complication of Cranial Irradiation for Childhood Craniopharyngioma.

J Stroke Cerebrovasc Dis 2019 Feb 13. 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

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http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2019.01.033DOI Listing
February 2019

dl-3-n-butylphthalide for alleviation of neurological deficit after combined extracranial-intracranial revascularization for moyamoya disease: a propensity score-matched analysis.

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

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http://dx.doi.org/10.3171/2018.10.JNS182152DOI Listing
February 2019

Predictive factors for acute thrombogenesis occurring immediately after bypass procedure for moyamoya disease.

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

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http://dx.doi.org/10.1007/s10143-019-01086-4DOI Listing
February 2019

Modified encephalo-duro-periosteal-synangiosis (EDPS) for the revascularization of anterior cerebral artery territory in moyamoya disease: A single-center experience.

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

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http://dx.doi.org/10.1016/j.clineuro.2019.02.002DOI Listing

Hypercapnic BOLD MRI compared to HO PET/CT for the hemodynamic evaluation of patients with Moyamoya Disease.

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

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http://dx.doi.org/10.1016/j.nicl.2019.101713DOI Listing
February 2019

Arterial spin-labeling cerebral perfusion changes after revascularization surgery in pediatric moyamoya disease and syndrome.

J Neurosurg Pediatr 2019 Feb 8:1-7. Epub 2019 Feb 8.

4Division of Pediatric Neuroradiology, Department of Radiology, Lucile Packard Children's Hospital, Stanford University School of Medicine, Stanford, California.

OBJECTIVEMoyamoya disease is a dynamic cerebrovascular condition that often requires vascular surveillance. Arterial spin labeling (ASL) is an MR perfusion method that is increasingly used for stroke and other various neurovascular pathologies. Unlike perfusion-weighted MRI, ASL uses endogenous water molecules for signal and therefore obviates gadolinium use; and provides direct, not relative, quantitative cerebral blood flow (CBF) measures. Read More

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http://dx.doi.org/10.3171/2018.11.PEDS18498DOI Listing
February 2019
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Effect of choroidal collateral vessels on de novo hemorrhage in moyamoya disease: analysis of nonhemorrhagic hemispheres in the Japan Adult Moyamoya Trial.

J Neurosurg 2019 Feb 8:1-7. Epub 2019 Feb 8.

1Department of Neurosurgery, Kyoto University Graduate School of Medicine, Kyoto.

OBJECTIVEFollowing hemorrhagic stroke in moyamoya disease, de novo intracranial hemorrhage can occur in the previously unaffected nonhemorrhagic hemisphere. In the present analysis the authors intended to determine whether the presence in the nonhemorrhagic hemisphere of choroidal collateral vessels, which have been the focus of attention as a source of bleeding, affects the risk of de novo hemorrhage.METHODSThe subject of focus of the present cohort study was the nonhemorrhagic hemispheres of adult patients with hemorrhagic moyamoya disease enrolled in the Japan Adult Moyamoya Trial and allocated to the nonsurgical arm. Read More

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http://dx.doi.org/10.3171/2018.10.JNS181139DOI Listing
February 2019
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Revascularization surgery for symptomatic non-moyamoya intracranial arterial stenosis or occlusion.

J Neurosurg 2019 Feb 8:1-6. Epub 2019 Feb 8.

2Department of Clinical Medicine, Macquarie University; and.

OBJECTIVEPrevious trials rejected a role of extracranial-to-intracranial bypass surgery for managing symptomatic atheromatous disease. However, hemodynamic insufficiency may still be a rationale for surgery, provided the bypass can be performed with low morbidity and patency is robust.METHODSConsecutive patients undergoing bypass surgery for symptomatic non-moyamoya intracranial arterial stenosis or occlusion were retrospectively identified. Read More

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http://dx.doi.org/10.3171/2018.9.JNS181075DOI Listing
February 2019

Practice Trends in Intracranial Bypass Surgery in a 21-Year Experience.

World Neurosurg 2019 Feb 5. Epub 2019 Feb 5.

Department of Neurological Surgery, University of California San Francisco, San Francisco, CA; Department of Neurological Surgery, Barrow Neurological Institute, Phoenix, AZ. Electronic address:

Introduction: The aim of this study was to analyze practice trends in specific intracranial bypass types in a large, consecutuve bypass experience.

Methods: This retrospective review of a prospectively maintained database included all intracranial bypasses performed by a single surgeon over 21 years. Bypass types were grouped into 7 categories and analyzed in seven 3-year time intervals: type 1 = extracranial-to-intracranial (EC-IC) bypass with scalp arteries as donors; type 2 = EC-IC bypass with interposition graft to cervical carotid arteries; type 3 = arterial reimplantation; type 4 = in situ bypass; type 5 = reanastomosis; type 6 = intracranial-to-intracranial (IC-IC) bypass with interposition graft; and type 7= combination bypass. Read More

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http://dx.doi.org/10.1016/j.wneu.2019.01.161DOI Listing
February 2019

Reply to 'Moyamoya vasculopathy with anti-SCL-70 antibodies: A case report and review of the literature'.

J Clin Neurosci 2019 Feb 4. Epub 2019 Feb 4.

Goethe University, Division of Rheumatology, University Hospital Frankfurt, Frankfurt am Main, Germany.

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http://dx.doi.org/10.1016/j.jocn.2019.01.043DOI Listing
February 2019

Moyamoya disease presenting as thalamic infarction in a patient with neuromyelitis optica spectrum disorder.

CNS Neurosci Ther 2019 Feb 6. Epub 2019 Feb 6.

Department of Neurology, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China.

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http://dx.doi.org/10.1111/cns.13106DOI Listing
February 2019

Characteristics of Moyamoya Syndrome in Sickle-Cell Disease by Magnetic Resonance Angiography: An Adult-Cohort Study.

Front Neurol 2019 22;10:15. Epub 2019 Jan 22.

Unité des Maladies Génétiques du Globule Rouge, Sickle-Cell Referral Center, Internal Medicine, APHP, Hôpital Henri Mondor, UPEC, Créteil, France.

Sickle cell disease (SCD) can be complicated by moyamoya syndrome. Brain magnetic resonance angiography (MRA) is a non-invasive method to diagnose this syndrome and, steno-occlusion and moyamoya vessels (MMV) scores have been proposed to evaluate its severity. Previous studies of SCD moyamoya syndrome did not evaluate the severity according to MRA scores. Read More

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http://dx.doi.org/10.3389/fneur.2019.00015DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6349744PMC
January 2019
1 Read

Clinical Features, Surgical Treatment, and Long-Term Outcome of a Multicenter Cohort of Pediatric Moyamoya.

Front Neurol 2019 22;10:14. Epub 2019 Jan 22.

Department of Neurosurgery, Beijing Tiantan Hospital, China National Clinical Research Center for Neurological Diseases, Capital Medical University, Beijing, China.

This study aims to investigate the clinical features, long-term outcomes, and prognostic predictors of a multicenter cohort of children with moyamoya disease. A series of 303 consecutive pediatric moyamoya disease (MMD) patients were screened in the present study. The clinical characteristics were retrospectively collected, and long-term outcomes was evaluated. Read More

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http://dx.doi.org/10.3389/fneur.2019.00014DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6349739PMC
January 2019
2 Reads

Extracranial-intracranial bypass approach to cerebral revascularization: a historical perspective.

Neurosurg Focus 2019 Feb;46(2):E2

1Department of Neurological Surgery, University of Virginia Health System, Charlottesville, Virginia; and.

While the majority of cerebral revascularization advancements were made in the last century, it is worth noting the humble beginnings of vascular surgery throughout history to appreciate its progression and application to neurovascular pathology in the modern era. Nearly 5000 years of basic human inquiry into the vasculature and its role in neurological disease has resulted in the complex neurosurgical procedures used today to save and improve lives. This paper explores the story of the extracranial-intracranial approach to cerebral revascularization. Read More

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http://dx.doi.org/10.3171/2018.11.FOCUS18527DOI Listing
February 2019
2 Reads

National trends in cerebral bypass surgery in the United States, 2002-2014.

Neurosurg Focus 2019 Feb;46(2):E4

1Department of Neurological Surgery, University of California, San Francisco, California; and.

OBJECTIVECerebral bypass procedures are microsurgical techniques to augment or restore cerebral blood flow when treating a number of brain vascular diseases including moyamoya disease, occlusive vascular disease, and cerebral aneurysms. With advances in endovascular therapy and evolving evidence-based guidelines, it has been suggested that cerebral bypass procedures are in a state of decline. Here, the authors characterize the national trends in cerebral bypass surgery in the United States from 2002 to 2014. Read More

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http://dx.doi.org/10.3171/2018.11.FOCUS18530DOI Listing
February 2019

Effect of revascularization on cognitive outcomes in intracranial steno-occlusive disease: a systematic review.

Neurosurg Focus 2019 Feb;46(2):E14

3Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota.

OBJECTIVESteno-occlusive diseases of the cerebral vasculature have been associated with cognitive decline. The authors performed a systematic review of the existing literature on intracranial steno-occlusive disease, including intracranial atherosclerosis and moyamoya disease (MMD), to determine the extent and quality of evidence for the effect of revascularization on cognitive performance.METHODSA systematic search of PubMed/MEDLINE, the Thomson Reuters Web of Science Core Collection, and the KCI Korean Journal Database was performed to identify randomized controlled trials (RCTs) in the English-language literature and observational studies that compared cognitive outcomes before and after revascularization in patients with steno-occlusive disease of the intracranial vasculature, from which data were extracted and analyzed. Read More

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http://dx.doi.org/10.3171/2018.11.FOCUS18517DOI Listing
February 2019

Wound healing complications after revascularization for moyamoya vasculopathy with reference to different skin incisions.

Neurosurg Focus 2019 Feb;46(2):E12

1Department of Neurosurgery and Center for Stroke Research Berlin, Charité-Universitätsmedizin Berlin (corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health), Berlin.

OBJECTIVEMoyamoya vasculopathy (MMV) is a steno-occlusive cerebrovascular disease that can be treated by a surgical revascularization. All the revascularization techniques influence the blood supply of the scalp, with a risk for wound healing disorders. The authors' aim was to analyze the wound healing process in the patients who underwent a direct or combined bypass surgery with a focus on different skin incisions. Read More

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http://dx.doi.org/10.3171/2018.11.FOCUS18512DOI Listing
February 2019
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Moyamoya syndrome and PHACE syndrome: clinical and radiographic characterization of the intracranial arteriopathy and response to surgical revascularization.

J Neurosurg Pediatr 2019 Feb 1:1-5. Epub 2019 Feb 1.

Departments of1Neurosurgery and.

OBJECTIVEPHACE syndrome (PHACES) has been linked to cervical and cerebral vascular anomalies, including persistent embryonic anastomoses and progressive steno-occlusive disease. However, no prior studies have documented the long-term response of PHACES patients with moyamoya disease to surgical revascularization with pial or myosynangiosis. The authors present their experience with 8 consecutive patients with PHACES and moyamoya disease. Read More

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http://dx.doi.org/10.3171/2018.10.PEDS18582DOI Listing
February 2019
1 Read

The AAA+ ATPase/ubiquitin ligase mysterin stabilizes cytoplasmic lipid droplets.

J Cell Biol 2019 Jan 31. Epub 2019 Jan 31.

Faculty of Life Sciences, Kyoto Sangyo University, Kyoto, Japan.

Mysterin, also known as RNF213, is an intracellular protein that forms large toroidal oligomers. Mysterin was originally identified in genetic studies of moyamoya disease (MMD), a rare cerebrovascular disorder of unknown etiology. While mysterin is known to exert ubiquitin ligase and putative mechanical ATPase activities with a RING finger domain and two adjacent AAA+ modules, its biological role is poorly understood. Read More

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http://dx.doi.org/10.1083/jcb.201712120DOI Listing
January 2019

Retinal microvascular abnormalities in neurofibromatosis type 1.

Br J Ophthalmol 2019 Jan 31. Epub 2019 Jan 31.

Department of Dermatology, Sapienza University of Rome, Rome, Italy.

Purpose: The aim of this study was to provide a classification of the different retinal vascular arrangements in neurofibromatosis 1 (NF1), with appropriate qualitative and quantitative information.

Methods: This study was conducted on 334 consecutive patients with NF1 and 106 sex-matched and age-matched healthy control subjects. Each patient underwent a comprehensive ophthalmological examination inclusive of near-infrared reflectance retinography by using the spectral domain Optical coherence tomography (OCT), a complete dermatological examination and 1. Read More

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http://dx.doi.org/10.1136/bjophthalmol-2018-313002DOI Listing
January 2019
1 Read

Changing Ischemic Lesion Patterns and Hemodynamics of the Posterior Cerebral Artery in Moyamoya Disease.

J Ultrasound Med 2019 Jan 31. Epub 2019 Jan 31.

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

Objective: The aim of this study was to determine how hemodynamics of the posterior cerebral artery (PCA) are associated with cerebral ischemic lesions in moyamoya disease (MMD).

Methods: Thirty-six patients with ischemic MMD (Suzuki grade IV-V) were retrospectively analyzed. Hemodynamic parameters of the PCA were measured by transcranial color-coded sonography. Read More

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http://doi.wiley.com/10.1002/jum.14959
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http://dx.doi.org/10.1002/jum.14959DOI Listing
January 2019
2 Reads

Microstructural damage pattern of vascular cognitive impairment: a comparison between moyamoya disease and cerebrovascular atherosclerotic disease.

Neural Regen Res 2019 May;14(5):858-867

Department of Neurosurgery, Huashan Hospital, Fudan University, Shanghai, China.

Moyamoya disease and cerebrovascular atherosclerotic disease are both chronic ischemic diseases with similar presentations of vascular cognitive impairment. The aim of the present study was to investigate the patterns of microstructural damage associated with vascular cognitive impairment in the two diseases. The study recruited 34 patients with moyamoya disease (age 43. Read More

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http://dx.doi.org/10.4103/1673-5374.249234DOI Listing
May 2019
1 Read

Comparison of acute moyamoya disease-related and idiopathic primary intraventricular hemorrhage in adult patients.

World Neurosurg 2019 Jan 24. Epub 2019 Jan 24.

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

Objective: Primary intraventricular hemorrhage (PIVH) is a rare condition in adult patients. PIVH occurs frequently in adult hemorrhagic MMD. Idiopathic PIVH is defined as PIVH without cerebrovascular abnormalities. Read More

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http://dx.doi.org/10.1016/j.wneu.2019.01.070DOI Listing
January 2019
1 Read

Predictors of neoangiogenesis after indirect revascularization in moyamoya disease: a multicenter retrospective study.

J Neurosurg 2019 Jan 25:1-11. Epub 2019 Jan 25.

OBJECTIVEThe effect of indirect revascularization to improve cerebral perfusion for moyamoya disease (MMD) is based on ingrowth of new vessels into the cortical brain. Preoperative indicators for neoangiogenesis would be helpful to the selection of appropriate procedures for MMD patients but have not yet been investigated. Our study aimed to identify potential predictors for neovascularization after indirect bypass surgery. Read More

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http://dx.doi.org/10.3171/2018.9.JNS181562DOI Listing
January 2019
5 Reads

The Coexistence of Persistent Primitive Trigeminal Artery, Moyamoya Disease, And Multiple Intracranial Aneurysms: A Case Report And Literature Review.

World Neurosurg 2019 Jan 23. Epub 2019 Jan 23.

Department of Neurosurgery, The First Hospital of Jilin University, Changchun, 130021, China. Electronic address:

Background: Persistent primitive trigeminal artery (PPTA) is a rare abnormal carotid-basilar anastomosis. In rare circumstances, PPTA could be associated with some cerebrovascular anomalies including arteriovenous malformation, intracranial aneurysm, carotid cavernous fistula, and moyamoya disease (MMD).

Case Description: In this report, we would like to present an extremely rare case with simultaneous occurrence of PPTA, MMD, and multiple intracranial aneurysms. Read More

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http://dx.doi.org/10.1016/j.wneu.2019.01.030DOI Listing
January 2019
3 Reads

Comparison of TOF-MRA and silent scan-MRA in depicting cerebral arteries in patients with Moyamoya disease.

Acta Radiol 2019 Jan 25:284185118824782. Epub 2019 Jan 25.

Department of Neuroradiology, Radiology, and Neurosurgery, Southern Tohoku Research Institute for Neuroscience, Southern Tohoku General Hospital, Fukushima, Japan.

Background: Silent scan magnetic resonance angiography (silent-MRA) as an arterial spin labeling technique offering sound reduction is insensitive to saturation effect. Time-of-flight (TOF)-MRA has limitations in terms of fine or slow-flow blood vessels due to sensitive saturation effects.

Purpose: Silent-MRA was compared with TOF-MRA for visualizing cerebral arteries in patients with Moyamoya disease (MMD). Read More

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http://dx.doi.org/10.1177/0284185118824782DOI Listing
January 2019

Recovered bone flap tilting after encephalo-myo-synangiosis: A complication.

Clin Neurol Neurosurg 2019 Mar 17;178:13-19. Epub 2019 Jan 17.

Department of Neurosurgery, Second Affiliated Hospital, School of ⁎Medicine, Zhejiang University, Hangzhou, Zhejiang, 310009, China. Electronic address:

Objectives: The aim of this study was to analyze the clinical data concerning the loosening and tilting of the recovered bone flap after encephalo-myo-synangiosis (EMS) and explore the potential underlying mechanisms.

Patients And Methods: Clinical data of all patients who underwent an EMS procedure from January 1, 2014 to April 30, 2018 at our hospital were collected. The cases with postoperative tilting of the recovered bone flap were identified and the clinical characteristics of the patients involved were analyzed. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S03038467193000
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http://dx.doi.org/10.1016/j.clineuro.2019.01.009DOI Listing
March 2019
3 Reads

Association of CD40 SNPs with Moyamoya in a Chinese children population.

Br J Neurosurg 2019 Jan 25:1-4. Epub 2019 Jan 25.

a Department of Pediatric Neurosurgery , Children's Hospital of Fudan University , Shanghai , China.

Background: The etiology of Moyamoya disease (MMD) remains unknown to a large extent. Immune and inflammation dysfunction may play a role in the pathogenesis of this rare disease. Coexisting Kawasaki disease (KD) with MMD were reported and both diseases have a feature of vasculopathy, raising the hypothesis that there may be some common pathologic factors. Read More

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http://dx.doi.org/10.1080/02688697.2018.1559275DOI Listing
January 2019
2 Reads

Unraveling Specific Brain Microstructural Damage in Moyamoya Disease Using Diffusion Magnetic Resonance Imaging and Positron Emission Tomography.

J Stroke Cerebrovasc Dis 2019 Jan 21. Epub 2019 Jan 21.

Department of Neurosurgery, Tokyo Medical and Dental University, Bunkyo-ku, Tokyo, Japan; Team for Neuroimaging Research, Tokyo Metropolitan Institute of Gerontology, Itabashi-ku, Tokyo, Japan.

Background And Purpose: Chronic ischemia may induce brain microstructural damage and lead to neurocognitive dysfunction in patients with Moyamoya disease (MMD). We applied neurite orientation dispersion and density imaging (NODDI) and O-gas positron emission tomography (PET) to elucidate the specific ischemic brain microstructural damage of MMD in the cortex and the white matter.

Materials And Methods: Thirty-one patients (16-63 years old, 9 males) and 20 age- and sex-matched normal controls were enrolled in this study. Read More

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http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2018.12.038DOI Listing
January 2019

In vivo demonstration of blood-brain barrier impairment in Moyamoya disease.

Acta Neurochir (Wien) 2019 Feb 24;161(2):371-378. Epub 2019 Jan 24.

Department of Neurosurgery, Charitè-Universitätsmedizin Berlin, Berlin, Germany.

Background: Moyamoya disease (MMD) is a cerebrovascular disorder characterized by fragile vascular system. Previous studies suggested that the blood-brain barrier (BBB) destabilizing cytokine angiopoietin-2 plays a critical role in increasing vascular plasticity and endothelial disintegration in MMD. The aim of this study was to assess cerebrovascular integrity in vivo in patients affected by MMD. Read More

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http://dx.doi.org/10.1007/s00701-019-03811-wDOI Listing
February 2019

RNF213 Variant Diversity Predisposes Distinct Populations to Dissimilar Cerebrovascular Diseases.

Biomed Res Int 2018 20;2018:6359174. Epub 2018 Dec 20.

Department of Neurology, First Affiliated Hospital, Sun Yat-sen University, Guanghzou, China.

In recent years, the ring finger protein 213 gene (RNF213) has gradually attracted attention, mainly because it has been found that RNF213 c.14429 G>A is associated with moyamoya disease (MMD) in East Asian populations. Recent studies have revealed that RFN213 is not only associated with MMD but is also connected with intracranial major artery stenosis/occlusion (ICASO) and intracranial aneurysm (IA). Read More

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https://www.hindawi.com/journals/bmri/2018/6359174/
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http://dx.doi.org/10.1155/2018/6359174DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6317084PMC
December 2018
3 Reads

Impaired functional recovery of endothelial colony-forming cells from moyamoya disease in a chronic cerebral hypoperfusion rat model.

J Neurosurg Pediatr 2018 Nov 1:1-10. Epub 2018 Nov 1.

1Division of Pediatric Neurosurgery, Pediatric Clinical Neuroscience Center, Seoul National University Children's Hospital.

OBJECTIVEEndothelial colony-forming cells (ECFCs) isolated from pediatric patients with moyamoya disease (MMD) have demonstrated decreased numbers and defective functioning in in vitro experiments. However, the function of ECFCs has not been evaluated using in vivo animal models. In this study, the authors compared normal and MMD ECFCs using a chronic cerebral hypoperfusion (CCH) rat model. Read More

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http://dx.doi.org/10.3171/2018.8.PEDS1883DOI Listing
November 2018
2 Reads

Standardized MR Perfusion Scoring System for Evaluation of Sequential Perfusion Changes and Surgical Outcome of Moyamoya Disease.

AJNR Am J Neuroradiol 2019 Feb 17;40(2):260-266. Epub 2019 Jan 17.

From the Department of Medical Imaging (Y.-H.L., C.-J.L., C.-W.L., Y.-F.C.)

Background And Purpose: Simple-but-precise evaluation of cerebral perfusion is crucial for the treatment of Moyamoya disease. We aimed to develop a standardized scoring system for MR perfusion suitable for Moyamoya disease evaluation and investigate the postoperative serial changes and outcome predictors.

Materials And Methods: From January 2013 to December 2016, patients diagnosed with Moyamoya disease and receiving indirect revascularization were recruited prospectively. Read More

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http://dx.doi.org/10.3174/ajnr.A5945DOI Listing
February 2019
1 Read

[Etiologies and risk factors for young people with intracerebral hemorrhage].

Zhong Nan Da Xue Xue Bao Yi Xue Ban 2018 Nov;43(11):1246-1250

Department of Geriatrics Neurology, Xiangya Hospital, Central South University, Changsha 410008, China.

Objective: To determine the etiologies and risk factors of intracerebral hemorrhage in young people.
 Methods: A total of 401 young patients with intracerebral hemorrhage were enrolled, and they were assigned into a 20-29 , a 30-39, and a 40-45 age group. The differences of various etiologies and risk factors among the three groups were analyzed. Read More

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http://www.csumed.org/xbwk/CN/10.11817/j.issn.1672-7347.2018
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http://dx.doi.org/10.11817/j.issn.1672-7347.2018.11.013DOI Listing
November 2018
5 Reads

Identifying Hypoperfusion in Moyamoya Disease With Arterial Spin Labeling and an [O]-Water Positron Emission Tomography/Magnetic Resonance Imaging Normative Database.

Stroke 2019 Feb;50(2):373-380

From the Department of Radiology (A.P.F., J.G., Y.I., J.R., M.W., J.H.P., B.S., D.H., H.G., T.H., P.S., F.T.C., G.Z.), Stanford University, CA.

Background and Purpose- Noninvasive imaging of brain perfusion has the potential to elucidate pathophysiological mechanisms underlying Moyamoya disease and enable clinical imaging of cerebral blood flow (CBF) to select revascularization therapies for patients. We used hybrid positron emission tomography (PET)/magnetic resonance imaging (MRI) technology to characterize the distribution of hypoperfusion in Moyamoya disease and its relationship to vessel stenosis severity, through comparisons with a normative perfusion database of healthy controls. Methods- To image CBF, we acquired [O]-water PET as a reference and simultaneously acquired arterial spin labeling (ASL) MRI scans in 20 Moyamoya patients and 15 age-matched, healthy controls on a PET/MRI scanner. Read More

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https://www.ahajournals.org/doi/10.1161/STROKEAHA.118.023426
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http://dx.doi.org/10.1161/STROKEAHA.118.023426DOI Listing
February 2019
5 Reads

Balanced single-vector co-delivery of VEGF/PDGF-BB improves functional collateralization in chronic cerebral ischemia.

J Cereb Blood Flow Metab 2019 Jan 9:271678X18818298. Epub 2019 Jan 9.

1 Department of Neurosurgery and Center for Stroke research Berlin (CSB), Charité - Universitätsmedizin Berlin, Berlin, Germany.

The myoblast-mediated delivery of angiogenic genes represents a cell-based approach for targeted induction of therapeutic collateralization. Here, we tested the superiority of myoblast-mediated co-delivery of vascular endothelial growth factor-A (VEGF) together with platelet-derived growth factor-BB (PDGF-BB) on transpial collateralization of an indirect encephalomyosynangiosis (EMS) in a model of chronic cerebral ischemia. Mouse myoblasts expressing a reporter gene alone (empty vector), VEGF, PDGF-BB or VEGF and PDGF-BB through a single bi-cistronic vector (VIP) were implanted into the temporalis muscle of an EMS following permanent ipsilateral internal carotid artery occlusion in adult, male C57BL/6N mice. Read More

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http://dx.doi.org/10.1177/0271678X18818298DOI Listing
January 2019

Direct Bypass Surgery Vs. Combined Bypass Surgery for Hemorrhagic Moyamoya Disease: A Comparison of Angiographic Outcomes.

Front Neurol 2018 20;9:1121. Epub 2018 Dec 20.

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

Extracranial-intracranial bypass is currently recognized as the optimal treatment for hemorrhagic-type moyamoya disease (MMD) which reduces incidence of rebleeding. Recent studies have reported the advantage of combined bypass over direct bypass for the general MMD patients. However, the effect of direct bypass and combined bypass surgery specifically for hemorrhagic-type MMD had not been investigated yet. Read More

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http://dx.doi.org/10.3389/fneur.2018.01121DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6306562PMC
December 2018
2 Reads

Clinical Features and Prognosis of Primary Intraventricular Hemorrhage in Elderly: Single-Center Experience.

World Neurosurg 2019 Jan 3. Epub 2019 Jan 3.

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

Objective: Primary intraventricular hemorrhage (PIVH) is rare in the aging population and remains a challenge for cerebrovascular surgeons. In the present study, the authors reviewed the patient characteristics, angiographic results, and treatments and determined clinical outcomes in 34 patients older than 60 years of age who were treated at West China Hospital between 2010 and 2014.

Methods: The medical records of elderly patients were queried. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S18788750183293
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http://dx.doi.org/10.1016/j.wneu.2018.12.114DOI Listing
January 2019
2 Reads

Correction to: Efficacy of superficial temporal artery-middle cerebral artery double bypass in patients with hemorrhagic moyamoya disease: surgical effects for operated hemispheric sides.

Neurosurg Rev 2019 Jan 4. Epub 2019 Jan 4.

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

Table 2 of the original version of this article contained an added data. Correct Table 2 is presented here. Read More

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http://dx.doi.org/10.1007/s10143-018-01073-1DOI Listing
January 2019

GEN-O-MA project: an Italian network studying clinical course and pathogenic pathways of moyamoya disease-study protocol and preliminary results.

Neurol Sci 2019 Jan 3. Epub 2019 Jan 3.

Cerebrovascular Unit, Neurological Institute "C. Besta" IRCCS Foundation, Milan, Italy.

Background: GENetics of mOyaMoyA (GEN-O-MA) project is a multicenter observational study implemented in Italy aimed at creating a network of centers involved in moyamoya angiopathy (MA) care and research and at collecting a large series and bio-repository of MA patients, finally aimed at describing the disease phenotype and clinical course as well as at identifying biological or cellular markers for disease progression. The present paper resumes the most important study methodological issues and preliminary results.

Methods: Nineteen centers are participating to the study. Read More

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http://link.springer.com/10.1007/s10072-018-3664-z
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http://dx.doi.org/10.1007/s10072-018-3664-zDOI Listing
January 2019
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Angiographic and clinical outcomes of non-patent anastomosis after bypass surgery in adult moyamoya disease.

Acta Neurochir (Wien) 2019 Feb 2;161(2):379-384. Epub 2019 Jan 2.

Department of Neurosurgery, Inje University, Haeundae Paik Hospital, 875, Haeun-daero, Haeundae-gu, Busan, 612-896, Republic of Korea.

Background: The clinical and radiologic outcomes of symptomatic adult moyamoya disease (MMD) patients who have an occluded anastomosis immediately after bypass surgery are poorly studied. The clinical and angiographic outcomes of non-patent anastomosis in symptomatic adult MMD patients were retrospectively reviewed.

Methods: From August 2011 to November 2016, 31 revascularization surgeries, consisting of direct and indirect bypass, were performed on 29 adult MMD patients. Read More

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http://dx.doi.org/10.1007/s00701-018-3733-3DOI Listing
February 2019

Machine learning for detecting moyamoya disease in plain skull radiography using a convolutional neural network.

EBioMedicine 2018 Dec 28. Epub 2018 Dec 28.

Department of Neurosurgery, Seoul National University Bundang Hospital, 82, Gumi-ro 173 Beon-gil, Bundang-gu, Seongnam-si, Gyeonggi-do 13620, Republic of Korea; Department of Neurosurgery, Seoul National University College of Medicine, 101 Daehak-Ro Jongno-Gu, Seoul 03080, Republic of Korea. Electronic address:

Background: Recently, innovative attempts have been made to identify moyamoya disease (MMD) by focusing on the morphological differences in the head of MMD patients. Following the recent revolution in the development of deep learning (DL) algorithms, we designed this study to determine whether DL can distinguish MMD in plain skull radiograph images.

Methods: Three hundred forty-five skull images were collected as an MMD-labeled dataset from patients aged 18 to 50 years with definite MMD. Read More

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http://dx.doi.org/10.1016/j.ebiom.2018.12.043DOI Listing
December 2018

Imaging of Moyamoya Disease and Moyamoya Syndrome: Current Status.

J Comput Assist Tomogr 2018 Dec 26. Epub 2018 Dec 26.

Department of Radiology, Dongfeng People's Hospital, Guangdong, Shongshan, China.

Moyamoya disease (MMD) and Moyamoya syndrome (MMS) are referring to a progressive steno-occlusive vasculopathy at terminal portions of the bilateral internal carotid arteries and their proximal branches with prominent collateral artery formation. They can be found throughout the world and cause irreversible damage to the cerebral hemodynamics due to the progressive nature. Prompt diagnosis and accurate assessment could significantly improve the prognosis of MMD and MMS. Read More

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http://dx.doi.org/10.1097/RCT.0000000000000834DOI Listing
December 2018
10 Reads
1.602 Impact Factor

Sturge-Weber Syndrome Coexisting With Moyamoya Disease in the Fifth Decade: A Case Report and Literature Review.

Neurologist 2019 Jan;24(1):13-16

Department of Neurology, West China Hospital, Sichuan University, Guo Xue Xiang, Chengdu, China.

Sturge-Weber syndrome (SWS) is a sporadically occurring neurocutaneous syndrome characterized by port-wine stain over the face, ocular abnormalities (glaucoma and choroidal hemangioma), and leptomeningeal angiomas. It is usually diagnosed in infancy, but it may occasionally present in adulthood with seizures or stroke-like episodes. Here, we report a 46-year-old male patient, having SWS coexisting with moyamoya disease, attending our hospital due to sudden loss of consciousness. Read More

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http://dx.doi.org/10.1097/NRL.0000000000000207DOI Listing
January 2019

The novel mutation of gene as the cause for Spastic paraplegia 30 in a Japanese case.

eNeurologicalSci 2019 Mar 22;14:34-37. Epub 2018 Nov 22.

Departmentof Neurology, Faculty of Medicine, Kindai University, Japan.

Spastic paraplegia 30 is a recently established autosomal recessive disease characterized by a complex form of spastic paraplegia associated with neuropathy. Homozygous mutations of reportedly lead to hereditary spastic paraplegia or hereditary sensory and autonomic neuropathy type 2 (HSAN2), whereas heterozygous mutations can cause nonsyndromic and syndromic intellectual disability (MRD9). Here we report the case of a 37-year-old female who presented with gait disturbance complicated with moyamoya disease. Read More

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http://dx.doi.org/10.1016/j.ensci.2018.11.026DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6297067PMC
March 2019
4 Reads

Improved White Matter Cerebrovascular Reactivity after Revascularization in Patients with Steno-Occlusive Disease.

AJNR Am J Neuroradiol 2019 Jan 20;40(1):45-50. Epub 2018 Dec 20.

From the Division of Neuroradiology, Joint Department of Medical Imaging (L.M., C.R., D.M.M., K.S., O.S., J.P., E.G., A.C., D.J.M.)

Background And Purpose: One feature that patients with steno-occlusive cerebrovascular disease have in common is the presence of white matter (WM) lesions on MRI. The purpose of this study was to evaluate the effect of direct surgical revascularization on impaired WM cerebrovascular reactivity in patients with steno-occlusive disease.

Materials And Methods: We recruited 35 patients with steno-occlusive disease, Moyamoya disease ( = 24), Moyamoya syndrome ( = 3), atherosclerosis ( = 6), vasculitis ( = 1), and idiopathic stenosis ( = 1), who underwent unilateral brain revascularization using a direct superficial temporal artery-to-MCA bypass (19 women; mean age, 45. Read More

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http://dx.doi.org/10.3174/ajnr.A5912DOI Listing
January 2019
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A Novel Staging System to Evaluate Cerebral Hypoperfusion in Patients With Moyamoya Disease.

Stroke 2018 Dec;49(12):2837-2843

From the Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University (H.Y., X.L., D.Z., Y.Z., R.W., M.Z., J.Z.).

Background and Purpose- The study goal was to evaluate cerebral perfusion in moyamoya patients with a novel staging system and investigate the association between differences of perfusion status and clinical outcomes in patients treated with revascularization. Methods- About 506 consecutive patients from 2009 to 2015 were enrolled. The perfusion status was evaluated by a staging system-the stage of preinfarction period based on the result of computed tomography perfusion. Read More

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https://www.ahajournals.org/doi/10.1161/STROKEAHA.118.022628
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http://dx.doi.org/10.1161/STROKEAHA.118.022628DOI Listing
December 2018
5 Reads