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Pract Neurol 2020 Jul 3. Epub 2020 Jul 3.

Interventional Neuroradiology Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milano, Italy

Comput Math Methods Med 2020 13;2020:2018214. Epub 2020 Jun 13.

Shanghai Key Laboratory of Molecular Imaging, Shanghai University of Medicine & Health Sciences, Shanghai, China.

Background: Moyamoya disease (MMD) is a rare cerebrovascular disease characterized by chronic progressive stenosis or occlusion of the bilateral internal carotid artery (ICA), the anterior cerebral artery (ACA), and the middle cerebral artery (MCA). MMD is secondary to the formation of an abnormal vascular network at the base of the skull. However, the etiology and pathogenesis of MMD remain poorly understood. Read More

Case Rep Neurol 2020 May-Aug;12(2):140-147. Epub 2020 May 20.

Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.

A 31-year-old woman presented with sudden onset of weakness in her left upper limb. Magnetic resonance imaging revealed acute cerebral infarctions in the right frontal and parietal lobes. Magnetic resonance angiography showed stenosis in the proximal portions of the bilateral middle cerebral arteries and terminal portions of the bilateral internal carotid arteries. Read More

Neurol Res 2020 Jun 26:1-5. Epub 2020 Jun 26.

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

Objective: Unilateral movement disorder associated with moyamoya disease is a rare finding and the mechanism remains to be fully elucidated. Theories postulated include contralateral cerebral ischemic or hemorrhagic lesions, and/or hypoperfusion. However, few studies have reported such patients without contralateral lesions nor hypoperfusion. Read More

Neurosurgery 2020 Jun 25. Epub 2020 Jun 25.

Division of Image Science Department of Radiology University of Calgary Calgary, AB, Canada.

World Neurosurg 2020 Jun 22. Epub 2020 Jun 22.

Professor of Neurosurgery, Department of Neurological Surgery, University of Wisconsin-Madison School of Medicine and Public Health, Madison, WI, USA. Electronic address:

Background: Cerebral bypass procedures are complex, require substantial experience and skills, and thorough preoperative planning. Cerebrovascular surgeons face increasingly complex bypass cases because most routine cases are managed by endovascular means, and because increasing numbers of patients have complex medical problems which affect available and suitable bypass conduit options. We present herein several cerebral bypass cases with limited bypass conduit alternatives, and where there were unexpected intraoperative difficulties requiring complex solutions. Read More

Elife 2020 Jun 23;9. Epub 2020 Jun 23.

Research Institute of Molecular Pathology (IMP), Vienna BioCenter, Vienna, Austria.

RNF213 is the major susceptibility factor for Moyamoya disease, a progressive cerebrovascular disorder that often leads to brain stroke in adults and children. Characterization of disease-associated mutations has been complicated by the enormous size of RNF213. Here, we present the cryo-EM structure of mouse RNF213. Read More

No Shinkei Geka 2020 Jun;48(6):533-540

Department of Neurosurgery, Seiwa-kai Nishiarai Hospital.

Aplastic or twig-like middle cerebral artery(Ap/T-MCA)is a rare congenital anomaly, and several cases of ruptured cerebral aneurysm associated with Ap/T-MCA have been reported. Recently, the association of ring finger protein 213(RNF213)mutations with moyamoya disease has been identified, and the involvement of such mutations in intracranial arterial stenosis lesions other than those of moyamoya disease has been suggested. A 53-year-old woman with headache and nausea was admitted to our hospital. Read More

Neurol Res 2020 Jun 20:1-6. Epub 2020 Jun 20.

Department of Neurosurgery, The First Affiliated Hospital of Nanchang University , Nanchang, Jiangxi Province, China.

Objectives: To explore clinical features and compare of the difference between conservative treatment and surgical treatmentin prognosis of intracranial aneurysms associated with moyamoya disease (MMD).

Methods: 104 patients with MMD a ssociated with intracranial aneurysms diagnosed by digital subtraction angiography (DSA) or computed tomography angiography (CTA) were retrospectively analyzed.

Results: All patients of MMD with intracranial aneurysms had 129 aneurysms distributed at different sites. Read More

Br J Neurosurg 2020 Jun 22:1-7. Epub 2020 Jun 22.

Department of Neurosurgery, The Second Hospital, Cheeloo College of Medicine, Shandong University, Jinan, Shandong, China.

The purpose of this study was to present our experience in the management of ruptured intracranial aneurysms associated with moyamoya disease (MMD), and to discuss their treatment strategies and the timing of revascularization surgery. Thirteen patients who had ruptured intracranial aneurysms associated with MMD were enrolled in this study. Different treatment strategies were adopted based on the location of the aneurysms. Read More

Ir J Med Sci 2020 Jun 19. Epub 2020 Jun 19.

National Neurosurgical Centre, Beaumont Hospital, Dublin, Ireland.

Background: There are no previously published reports regarding the epidemiology and characteristics of moyamoya disease or syndrome in Ireland.

Aims: To examine patient demographics, mode of presentation and the outcomes of extracranial-intracranial bypass surgery in the treatment of moyamoya disease and syndrome in Ireland.

Methods: All patients with moyamoya disease and syndrome referred to the National Neurosurgical Centre during January 2012-January 2019 were identified through a prospective database. Read More

Clin Rheumatol 2020 Jun 19. Epub 2020 Jun 19.

Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan.

Moyamoya syndrome (MMS) is a chronic cerebrovascular disorder characterized by occlusion or stenosis of the internal carotid arteries with the formation of abnormal collateral vascular networks. Moreover, the development of MMS, which is a distinct category from "moyamoya disease," is attributed to the underlying disease, while some cases of MMS related to systemic lupus erythematosus (SLE) have been previously reported. Herein, we present the case of a 29-year-old Japanese woman with SLE in whom intracranial hemorrhage ascribable to MMS developed during pregnancy. Read More

World Neurosurg 2020 Jun 16. Epub 2020 Jun 16.

Department of Neurosurgery, Yokohama City University Graduate School of Medicine, Yokohama, Japan.

Background: Cerebral bypass surgery, such as the STA-MCA bypass, is one of the essential procedures for cerebral revascularization. However, very narrow or thin blood vessels increase the risk of anastomotic problems, as seen in Moyamoya disease. For such vessels, we have devised the "lifting method" in the recipient arteriotomy. Read More

CNS Neurol Disord Drug Targets 2020 Jun 18. Epub 2020 Jun 18.

Institute of Cerebrovascular Diseases Research, Department of Neurology, and Department of Neurosurgery of Xuanwu Hospital, Capital Medical University, Beijing. China.

Backgrounds: Due to recent development of non-invasive examinations, more asymptomatic patients with moyamoya disease (MMD) have been diagnosed than ever. However, its underlying molecular mechanisms and clinical intervention guidelines are all still obscure.

Methods: Microarray was used to explore those differentially expressed mRNAs and lncRNAs in peripheral neutrophils of asymptomatic MMD patients. Read More

Ther Adv Neurol Disord 2020 26;13:1756286420921092. Epub 2020 May 26.

Department of Neurology, Cerebrovascular Division, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, USA.

Moyamoya disease (MMD) is a complex cerebrovascular disorder about which little is known. Conventionally, revascularization surgery is recommended for patients, despite an absence of conclusive data from adequate clinical trials. Underscoring the uncertainty that exists in treating MMD patients, investigators continue to present data comparing revascularization with conservative or medical management, most of which originates from East Asia where MMD is most prevalent. Read More

World Neurosurg 2020 Jun;138:586

Department of Anesthesiology and Pain Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea. Electronic address:

World Neurosurg 2020 Jun;138:585

Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan, China. Electronic address:

World Neurosurg 2020 Jun;138:583-584

Department of Neurosurgery, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA. Electronic address:

World Neurosurg 2020 Jun;138:582

Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan, China. Electronic address:

Neurosurgery 2020 Jun 15. Epub 2020 Jun 15.

Department of Neurosurgery Zhongnan Hospital of Wuhan University Wuhan, China.

Surg Radiol Anat 2020 Jun 14. Epub 2020 Jun 14.

Department of Neurosurgery, Neurocenter of the Southern Switzerland, Regional Hospital of Lugano, Via Tesserete 46, CH-6903, Lugano, Switzerland.

Purpose: The persistence of the stapedial artery is a rare vascular variant that could explain the origin of the middle meningeal artery from the petrous segment of the internal carotid artery. The anatomic variations are illustrated and a summary of the possible hypothesis of the origin of the middle meningeal artery from the internal carotid artery is discussed, analysing the embryological works of the Carnegie Institute and the vascular development of the middle meningeal.

Methods And Results: The authors present a young patient showing a particular vascular variant on diagnostic imaging. Read More

Neurol Med Chir (Tokyo) 2020 Jun 12. Epub 2020 Jun 12.

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

Some of the pediatric moyamoya patients spend their childhood without diagnosed as moyamoya disease (MMD) because of their mild ischemic attacks and emerge again with ischemic or hemorrhagic stroke in their adulthood. This study was aimed to clarify the clinical characteristics of adult moyamoya patients with childhood onset and elucidate the impact of long disease period on their clinical features. Present study included 116 untreated hemispheres of 69 adult patients with MMD. Read More

J Neurosurg 2020 Jun 12:1-12. Epub 2020 Jun 12.

1Department of Neurosurgery and Stanford Stroke Center, Stanford University School of Medicine.

Objective: The only effective treatment for ischemic moyamoya disease (iMMD) is cerebral revascularization by an extracranial to intracranial bypass. The preferred revascularization method remains controversial: direct versus indirect bypass. The purpose of this study was to test the hypothesis that method choice should be personalized based on angiographic, hemodynamic, and clinical characteristics to balance the risk of perioperative major stroke against treatment efficacy. Read More

World Neurosurg 2020 Jun 8. Epub 2020 Jun 8.

Department of Neurosurgery, Interdisciplinary Graduate School of Medicine and Engineering, University of Yamanashi, Yamanashi, Japan. Electronic address:

Background: and Importance: Early and late images of single photon emission computed tomography (SPECT) using I-iomazenil (I-IMZ) can demonstrate cerebral blood flow and cortical neuronal viability, respectively. Hyperperfusion syndrome is one of the serious complications after revascularization surgery for moyamoya disease; therefore, the real-time observation of the hemodynamics and neuronal viability is important for the treatment after the revascularization. Here we report, a case of moyamoya disease in whom I-IMZ SPECT had a significant efficacy to delineate the hemodynamics and transient neuronal dysfunction in hyperperfusion state after revascularization. Read More

J Neurointerv Surg 2020 Jun 10. Epub 2020 Jun 10.

Baptist Neurological Institute - Department of Cerebrovascular Surgery, Lyerly Neurosurgery, Jacksonville, Florida, USA

Background: Microcephalic primordial dwarfism (MPD) is a heterogeneous group of rare disorders. Recent studies have reported a significant percentage of patients with MPD suffering from a spectrum of cerebrovascular abnormalities, including intracranial aneurysms (IAs) and moyamoya syndrome. The neurological literature has not as yet specifically assessed IAs in this population. Read More

World Neurosurg 2020 May 8. Epub 2020 May 8.

Department of Neurosurgery, Hokkaido University Graduate School of Medicine, Sapporo, Japan. Electronic address:

Neurosurg Rev 2020 Jun 7. Epub 2020 Jun 7.

Mayo Clinic Department of Radiology, 200 First Street SW, Rochester, MN, 55905, USA.

An embryological association between moyamoya disease (MMD) and cardiac manifestations has been proposed. Data up to this point remains anecdotal, and the prevalence of cardiac manifestations in a western MMD population is uncertain. The objective of this study was to determine the prevalence of cardiac manifestations including coronary artery disease (CAD) and congenital cardiac defects in a mostly Caucasian population of MMD patients and review prior reports of such cases. Read More

Acta Neurochir (Wien) 2020 Jun 6. Epub 2020 Jun 6.

Department of Neurosurgery, Duke University Medical Center, 1000 Trent Drive 4520 Hosp. South, Box 3807, Durham, NC, 27710, USA.

Background: A variety of revascularization techniques have been reported for the management of moyamoya disease to prevent risks of stroke. STA (superficial temporal artery)-MCA (middle cerebral artery) microanastomosis, single or double bypass, with temporal muscle or galeal onlay graft has been the standard operative procedure.

Method: Our rationale of revascularization surgery has been a combination of STA-MCA double bypass using the frontal and the parietal branches of STA and transfer of a highly vascularized thick galeal flap with maintained STA vasculature. Read More

Front Neurol 2020 15;11:338. Epub 2020 May 15.

Eye Center, Second Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou, China.

Moyamoya disease (MMD) is a chronic cerebrovascular disease that frequently results in intracranial ischemia or hemorrhage. Its concurrence with varying ophthalmic findings is relatively rare yet may lead to irreversible blindness. We performed a search and review of the literature to characterize the relevance of MMD (excluding moyamoya syndrome) and ophthalmic findings. Read More

World Neurosurg 2020 May 31. Epub 2020 May 31.

Department of Neurosurgery, Hokkaido University Graduate School of Medicine, North 15 West 7, Kita, Sapporo 060-8638, Japan.

Background: Intracranial hematomas associated with abnormal collateral vessels are observed in certain populations of adult patients with moyamoya disease (MMD). Of these, intraventricular hematomas resulting from rupture of intraventricular aneurysms, which are formed along an abnormal peripheral choroidal artery, are sometimes detected and could be severe. No appropriate treatment option for these ruptured aneurysms has been well established to date. Read More

World Neurosurg 2020 May 31. Epub 2020 May 31.

Department of Neurosurgery, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan.

J Neurosurg Anesthesiol 2020 May 29. Epub 2020 May 29.

Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan.

J Neurosurg Anesthesiol 2020 May 29. Epub 2020 May 29.

Departments of Anesthesiology.

Medicine (Baltimore) 2020 May;99(21):e20229

Department of Neurosurgery, the First Hospital of Jilin University, Changchun, China.

Rationale: The incidence of pure arterial malformations is relatively low, and few cases have been reported. Only 2 cases with pure arterial malformation have been reported to receive surgery or endovascular treatment.

Patient Concerns: We report 3 cases and review the relevant literatures. Read More

Acta Neurochir (Wien) 2020 May 28. Epub 2020 May 28.

Mayo Clinic Department of Neurology, 200 1st St. SW, Rochester, MN, 55905, USA.

Background: Moyamoya Disease (MMD) and moyamoya Syndrome (MMS) have been reported to be associated with pro-thrombotic states in some patients. To date, however, such reports have been limited to case reports or small case series. We sought to determine the prevalence of pro-thrombotic states among a large cohort of both MMD and MMS patients. Read More

Front Neurol 2020 7;11:382. Epub 2020 May 7.

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

To evaluate clinical and radiological outcomes after revascularization of hemorrhagic moyamoya disease (MMD). We retrospectively collected patients with hemorrhagic MMD who received revascularization from January 2011 to June 2018 at a high-volume stroke center. Rebleeding, ischemic stroke, modified Rankin Scale (mRS) and death after revascularization were used to evaluate long-term clinical outcome. Read More

JMIR Mhealth Uhealth 2020 May 25;8(5):e17034. Epub 2020 May 25.

Mo-Im Kim Nursing Research Institute, Yonsei University College of Nursing, Seoul, Republic of Korea.

Background: Moyamoya disease (MMD) is a known progressive obstructive cerebrovascular disorder. Monitoring and managing mood and stress are critical for patients with MMD, as they affect clinical outcomes. The ecological momentary assessment (EMA) method is a longitudinal study design by which multiple variable assessments can be performed over time to detect momentary fluctuations and changes in psychological dimensions such as mood and stress over time. Read More

J Neurosurg 2020 May 22:1-9. Epub 2020 May 22.

Departments of1Neurosurgery.

Objective: This study aimed to clarify the underlying mechanism of pathognomonic angiogenesis between the temporal muscle and neocortex after indirect bypass for moyamoya disease by shedding light on the role of platelet-derived growth factor receptor-α (PDGFRα) in angiogenesis.

Methods: The gene for PDGFRα was systemically inactivated in adult mice (α-KO mice). The Pdgfra-preserving mice (Flox mice) and α-KO mice were exposed to bilateral common carotid artery stenosis (BCAS) by using microcoils. Read More

J Stroke Cerebrovasc Dis 2020 May 18:104846. Epub 2020 May 18.

Department of Neurology, North Huashan hospital, Fudan University, No.108 Lu Xiang Road, Shanghai 201900, China; Department of Neurology, Huashan Hospital, Fudan University, No.12 Wulumuqi Zhong Road, Jing an District, Shanghai 200040, China. Electronic address:

Background: To determine the functional outcomes in each period for adult ischemic and hemorrhagic Moyamoya disease (MMD) patients and identify prognostic factors.

Methods: The current retrospective study reviewed consecutive adult MMD patients surgically treated from January 2012 to June 2017. Perioperative clinical data were collected and follow-up was conducted via telephone interviews. Read More

World Neurosurg 2020 May 10. Epub 2020 May 10.

Department of Neurosurgery, Faculty of Medicine, The University of Tokyo, Tokyo, Japan.

Background: Intracranial arterial dissection (IAD) is known to exhibit various patterns of arterial imaging features such as stenosis and dilation; however, the genetic background of IAD has not been elucidated so far. RNF213 was recently identified as a susceptibility gene for moyamoya disease (MMD) and intracranial artery stenosis (ICAS). More recently, RNF213 p. Read More

Acta Neurol Taiwan 2020 Jun;29(2):64-66

University of California, San Diego, Department of Neurosciences, Stroke Center.

Background Purpose: Moyamoya syndrome is the progressive stenosis of intracranial carotids with secondary collateralization. Whole body cryotherapy (WBC) involves external cooling and is used in holistic and sports medicine, its neurologic effects are unknown.

Case Report: We report a first case of symptoms of moyamoya syndrome presenting following WBC and diagnosed with classic MRI ( "Brush Sign", "Ivy sign") and digital subtracted angiography. Read More

World Neurosurg 2020 May 17;140:233-236. Epub 2020 May 17.

Department of Neurosurgery, Dokkyo Medical University, Tochigi, Japan.

Background: We report a rare case of unruptured middle cerebral artery aneurysm associated with moyamoya disease.

Case Description: A 48-year-old woman with an 8-year history of moyamoya disease developed a de novo aneurysm at the bifurcation of the right middle cerebral artery. The aneurysm showed rapid enlargement in size in 1 year and surgical treatment was performed. Read More

World Neurosurg 2020 May 17;140:276-282. Epub 2020 May 17.

Department of Neurosurgery, Faculty of Medicine, The University of Tokyo, Tokyo, Japan.

Background: Reports of cases diagnosed as Moyamoya disease (MMD) after stereotactic radiosurgery (SRS) for arteriovenous malformation (AVM) are extremely rare. In recent years, ring finger protein 213 (RNF213) has been identified as a susceptibility gene of MMD, but the mechanism by which MMD develops remains unclear. Those cases of de novo development of MMD may provide some clues to clarify the mechanism of progression of MMD. Read More

BMJ Case Rep 2020 May 18;13(5). Epub 2020 May 18.

Department of Paediatrics, Penang General Hospital, Georgetown, Pulau Pinang, Malaysia.

Children with Down syndrome have a higher risk of stroke. Similarly, intravenous immunoglobulin (IV Ig) is also known to cause a stroke. We reported a 3-year-old boy with Down syndrome who presented with severe pneumonia and received IV Ig. Read More

Semin Intervent Radiol 2020 Jun 14;37(2):166-174. Epub 2020 May 14.

Department of Neurosurgery, University of Colorado School of Medicine, Aurora, Colorado.

Neuroangiography (NA) is a minimally invasive procedure used to diagnose patients with neurovascular diseases. Noninvasive imaging has improved dramatically in recent years and is utilized more frequently; however, further evaluation with NA is still required in certain cases. NA indications include intracranial (cerebral aneurysms, arteriovenous malformations, dural arteriovenous fistula, cerebral vasculitis, cerebral vasospasm, ischemic stroke, nontraumatic subarachnoid hemorrhage, intracerebral hemorrhage, Moyamoya, vein of Galen malformation, intracranial tumors, and pseudotumor cerebri) and extracranial (internal and common carotid artery stenosis, vertebral artery stenosis, carotid artery blowout, vertebral artery blowout, epistaxis, oropharyngeal bleeding, and carotid body tumor) pathologies which can help with diagnosis and potential subsequent endovascular treatment. Read More

Zh Vopr Neirokhir Im N N Burdenko 2020 ;84(2):93-102

Burdenko Neurosurgical Center, Moscow, Russia.

An adult patient with progressive chronic cerebral ischemia associated with moyamoya disease who underwent combined revascularization of both cerebral hemispheres in step-by-step fashion is reported in the article. The feature of this case is a large volume of revascularization procedures with double-barrel extra-intracranial anastomoses combined with indirect synangioses. This surgical approach ensured early postoperative development of extensive collateral network and complete compensation of impaired cerebral circulation. Read More

PeerJ 2020 5;8:e8841. Epub 2020 May 5.

Department of Physiology, Nanjing Medical University, Nanjing, Jiangsu, China.

Moyamoya disease (MMD) is a progressive stenosis at the terminal portion of internal carotid artery and frequently occurs in East Asian countries. The etiology of MMD is still largely unknown. We performed a case-control design with whole-exome sequencing analysis on 31 sporadic MMD patients and 10 normal controls with matched age and gender. Read More

Stroke Vasc Neurol 2020 27;5(1):97-102. Epub 2020 Feb 27.

Department of Neurosurgery, Beijing Tiantan Hospital, Beijing, China.

Background: Evidence on the natural angiographic course of moyamoya disease (MMD) is lacking. It takes about 6 months for waiting for revascularisation surgery. The issue of when to perform subtraction angiography (DSA) for follow-up remains unclear. Read More

Stroke Vasc Neurol 2020 25;5(1):86-96. Epub 2020 Mar 25.

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

Objective: Although a few reports suggested that cognitive function impairment could be found in adult patients with moyamoya disease (MMD), there were still many aspects that are unclear. The aim of our study was to assess the cognitive function of adult patients with MMD and its clinical subtypes.

Methods: 49 patients with MMD and 23 healthy controls were asked to take cognitive function tests. Read More

Mediators Inflamm 2020 28;2020:5847478. Epub 2020 Apr 28.

Jining First People's Hospital, Jining Medical University, Jining, China.

Systemic inflammation has been shown to play a pivotal role in the pathogenesis of moyamoya disease (MMD). Brain-gut peptides exhibit regulatory effects in the secretion of proinflammatory cytokines. To investigate the association between brain-gut peptides and inflammation in the occurrence of MMD, 41 patients with MMD, as well as 74 age- and sex-matched healthy individuals were enrolled. Read More