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J Cereb Blood Flow Metab 2022 Jun 25:271678X221110679. Epub 2022 Jun 25.

Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Japan.

RNF213 gene mutations are the cause behind Moyamoya disease, a rare cerebrovascular occlusive disease. However, the function of RNF213 in the vascular system and the impact of its loss of function are not yet comprehended. To understand RNF23 function, we performed gene knockdown (KD) in vascular cells and performed various phenotypical analysis as well as extensive transcriptome and epitranscriptome profiling. Read More

World Neurosurg 2022 Jun 21. Epub 2022 Jun 21.

Nanjing Comprehensive Stroke Center, Affiliated Nanjing Brain Hospital, Nanjing Medical University, 264 Guangzhou Road, Nanjing 210029, PR China. Electronic address:

Objective: The study aimed to investigate the hemodynamic features and independent predictors of neoangiogenesis after revascularization in MMD by pseudo-continuous arterial spin labeling magnetic resonance imaging (pCASL MRI).

Methods: 39 MMD patients were categorized into infarction group, hemorrhagic group and atypical group. All patients underwent combined bypass surgery and pCASL imaging with post-labeling delays (PLD) of 1525ms and 2525ms. Read More

World Neurosurg 2022 Jun 21. Epub 2022 Jun 21.

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

Objective: Cerebral angiography is the gold standard for diagnosing moyamoya disease (MMD), whereas magnetic resonance (MR) imaging/angiography is becoming more popular in the field of cerebrovascular disease due to its low invasiveness. Although there are concerns about using only MR imaging/angiography for preoperative analysis of MMD, considering the underestimation of pre-existing transdural collateral circulations and dangerous collaterals related to the risk of hemorrhage, we retrospectively reviewed our 10-year experience of MRI-first diagnosis and analyzed the perioperative outcomes.

Methods: We analyzed 160 consecutive direct/indirect combined revascularization procedures for MMD preoperatively assessed using MR imaging/angiography alone. Read More

Ophthalmology 2022 Jul;129(7):802

Department of Ophthalmology & Visual Sciences, University of Chicago, Chicago, Illinois.

Ann Med Surg (Lond) 2022 Jun 21;78:103815. Epub 2022 May 21.

Ribat Neurospine Center, Ribat University Hospital, The National Ribat University, Khartoum, Sudan.

Background: Sickle cell disease (SCD) is autosomal recessive hemolytic anemia due to hemoglobinopathy commonly in Sub-Saharan Africa, and particularly in Sudan. The disease induces a pro-inflammatory cascade in the intimal layer that leads to hyperplasia and progressive stenosis in the major vessel of the circle of Willis. This is associated with the development of Moyamoya collaterals. Read More

Ann Med Surg (Lond) 2022 Jun 11;78:103771. Epub 2022 May 11.

University of Hawai'i at Mānoa, John A. Burns School of Medicine, Honolulu, HI, USA.

Introduction: Better characterizing moyamoya disease (MMD) from ischemic strokes of other etiologies may facilitate earlier diagnosis by raising suspicion for a diagnostic work-up.

Methods: To identify associated variables, MMD cases (n = 12) were compared against three sets of controls: age-, sex-, and race-matched controls of patients with general neurological disorders (n = 48), unmatched general controls (n = 48), and unmatched non-MMD ischemic stroke controls (n = 48).

Results: MMD patients were 32 years (p < 0. Read More

J Neurosurg Case Lessons 2022 May 30;3(22):CASE22121. Epub 2022 May 30.

Neurosurgery, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.

Background: Aplastic or twiglike middle cerebral artery (Ap/T-MCA) is a rare anomaly characterized by a unilateral MCA occlusion with plexiform vessels that causes hemorrhagic and (less commonly) ischemic strokes. The reasons for this are rarely discussed, and thus optimal treatment for ischemic Ap/T-MCA remains controversial. Here, the authors report a case of Ap/T-MCA with transient ischemic attacks treated by bypass surgery and discuss the mechanism of ischemic development and treatment methods. Read More

World Neurosurg 2022 Jun 17. Epub 2022 Jun 17.

Department of Neurosurgery, Graduate School of Medical Sciences, Kyushu University.

Objective: The long-term outcomes of cognitive function in moyamoya disease remain unknown. We aimed to assess 5-year changes in cognitive function in adult moyamoya disease patients and to evaluate the value of magnetic resonance angiography (MRA) steno-occlusive score to predict cognitive changes.

Methods: Participants comprised 20 consecutive patients whose cognitive functions had been evaluated using the Frontal Assessment Battery (FAB) and Neurobehavioral Cognitive Status Examination (Cognistat) at baseline and reassessed 5 years later. Read More

Front Aging Neurosci 2022 2;14:788661. Epub 2022 Jun 2.

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

Objective: Ischemic moyamoya (MMD) disease could alter the cerebral structure, but little is known about the topological organization of the structural covariance network (SCN). This study employed structural magnetic resonance imaging and graph theory to evaluate SCN reorganization in ischemic MMD patients.

Method: Forty-nine stroke-free ischemic MMD patients and 49 well-matched healthy controls (HCs) were examined by T1-MPRAGE imaging. Read More

Front Neurol 2022 2;13:902474. Epub 2022 Jun 2.

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

Background And Purposes: The risk factors of poor postoperative angiogenesis in moyamoya disease (MMD) patients remain unknown. We aimed to investigate the association between hyperhomocysteinemia (HHcy) and postoperative angiogenesis of adult patients with MMD.

Methods: A total of 138 adult patients with MMD were prospectively recruited from July 1 to December 31, 2019. Read More

J Stroke Cerebrovasc Dis 2022 Jun 16;31(8):106601. Epub 2022 Jun 16.

Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, N14W5, Kita-Ku, Sapporo 060-8648, Japan.

Objectives: Complement component 6 (C6) deficiency is a very rare genetic defect that leads to significantly diminished synthesis, secretion, or function of C6. In the current report, we demonstrate a previously undescribed, homozygous missense mutation in exon 17 of the C6 gene (c.2545A>G p. Read More

Front Mol Neurosci 2022 31;15:881954. Epub 2022 May 31.

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

Moyamoya disease (MMD) is a rare, progressively steno-occlusive cerebrovascular disorder of unknown etiology. Here, we revealed the gene expression profile of the intracranial arteries in MMD the RNA-sequencing (RNA-seq). We identified 556 differentially expressed genes (DEGs) for MMD, including 449 and 107 significantly upregulated or downregulated genes. Read More

J Thromb Thrombolysis 2022 Jun 14. Epub 2022 Jun 14.

Division of Neurology, Department of Medicine, National University Health System, Singapore, Singapore.

Background And Aims: Patients with Moyamoya disease (MMD) can present with ischaemic or haemorrhagic stroke. There is no good evidence for treatment strategies in MMD-associated acute ischaemic stroke (AIS), particularly for treatments like intravenous thrombolysis (IVT) and endovascular thrombectomy (ET). As the intracranial vessels are friable in MMD, and the risk of bleeding is high, the use of IVT and ET is controversial. Read More

Pediatr Neurosurg 2022 Jun 13. Epub 2022 Jun 13.

Introduction Transdural collaterals (TC) from the external carotid artery (ECA) must be preserved when operating on patients with moyamoya vasculopathy. Several techniques have been used to identify the superficial temporal artery (STA) and middle meningeal artery (MMA) during surgery and prevent their damage. However, the use of neuronavigation for this specific purpose has never been described in the literature. Read More

Magn Reson Med 2022 Jun 13. Epub 2022 Jun 13.

Institute of Science and Technology for Brain-Inspired Intelligence, Fudan University, Shanghai, People's Republic of China.

Purpose: The phase mismatch between odd and even echoes in EPI causes Nyquist ghost artifacts. Existing ghost correction methods often suffer from severe residual artifacts and are ineffective with k-space undersampling data. This study proposed a deep learning-based method (PEC-DL) to correct phase errors for DWI at 7 Tesla. Read More

World Neurosurg 2022 Jun 8. Epub 2022 Jun 8.

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

Cerebrovascular bypass techniques are the current cornerstone methods to achieve cerebral revascularization for Moyamoya disease (MMD) or syndrome and select cases of vascular pathologies, such as intracranial atherosclerotic occlusive disease and complex aneurysms. Factors influencing bypass efficiency include graft patency, short temporary occlusion time, and precise anastomosis. Based mainly on our senior author's vast experience with 1300 bypasses, we recommend performing the anastomosis with the minimal amount of stitches as achievable, to avoid stenosis of the artery's internal lumen that may occur with unnecessary, additional stiches, preserving patency. Read More

Pediatr Blood Cancer 2022 Jun 10:e29830. Epub 2022 Jun 10.

The Division of Hematology and Oncology, St. Louis Children's Hospital, Washington University, School of Medicine in St. Louis, Washington University, St. Louis, Missouri, USA.

Purpose: Primary germ cell tumors (GCTs) are the most common central nervous system (CNS) neoplasm in patients with Down syndrome (DS). However, a standard of care has not been established due to paucity of data.

Methods: A retrospective multi-institutional analysis was conducted, in addition to a comprehensive review of the literature. Read More

Radiol Case Rep 2022 Aug 4;17(8):2798-2801. Epub 2022 Jun 4.

Department of Neurology, Mohammed V Military Instruction Hospital, Rabat, Morocco.

Moyamoya disease is an unusual occlusive cerebrovascular condition commonly seen in children, marked by stenosis of the internal carotid artery and circle of Willis, causing, cerebral ischemia. Moyamoya syndrome is a Moyamoya-like arteriopathy with risk factors including autoimmune disorders, thyroid disease, sickle cell disease, or Down syndrome. Trisomy 21 is a genetic disorder consistent with specific physical and behavioral characteristics, with intellectual impairment. Read More

Curr Stem Cell Res Ther 2022 Jun 7. Epub 2022 Jun 7.

Savaid Medical School, University of Chinese Academy of Sciences, Beijing China.

Moyamoya disease (MMD) is a chronic steno-occlusion cerebrovascular disease, accompanied by the formation of abnormal vascular network at the base of brain. The etiology of MMD is not fully clarified. Lack of pathological specimens hinders the research progress. Read More

J Stroke Cerebrovasc Dis 2022 Jun 4;31(8):106588. Epub 2022 Jun 4.

Department of Neurosurgery, Iwate Medical University, Japan. Electronic address:

Objectives: Although revascularization surgery is recommended for adult patients with moyamoya disease (MMD) who present with ischemic symptoms due to hemodynamic compromise, the clinical course of such patients who are treated with medical management alone remains unclear. Here, we report outcomes of adult patients with cerebral misery perfusion due to ischemic MMD who received medical management alone.

Materials And Methods: We prospectively followed up patients who showed misery perfusion in the symptomatic cerebral hemisphere on O gas positron emission tomography (PET) and received strict medical management alone after refusing revascularization surgery. Read More

Front Genet 2022 20;13:816919. Epub 2022 May 20.

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

MicroRNAs (miRNAs) in exosomes had been implicated differentially expressed in patient with moyamoya disease (MMD), but the miRNAs expression in circulating leukocytes remains unclear. This study was investigated on the differential expression of miRNAs in peripheral leukocytes between MMD patients and healthy adults, and among patients with subtypes of MMD. A total of 30 patients with MMD and 10 healthy adults were enrolled in a stroke center from October 2017 to December 2018. Read More

World J Clin Cases 2022 May;10(14):4617-4624

Department of Neurosurgery, Henan University People's Hospital, Henan Provincial People's Hospital, Zhengzhou 450000, Henan Province, China.

Background: Moyamoya disease is essentially an ischemic cerebrovascular disease. Here, we describe a case of acute recurrent cerebral infarction caused by moyamoya disease with concurrent adenomyosis which, to our knowledge, is the first in the literature. A literature review is also presented. Read More

Dev Med Child Neurol 2022 Jun 5. Epub 2022 Jun 5.

Department of Neurology, Sydney Children's Hospital, Randwick, NSW, Australia.

Aim: To improve delivery of acute therapies for acute ischaemic stroke (AIS).

Method: We identified factors influencing the speed of diagnosis and delivery of acute therapies in a prospective cohort of 21 children with suspected AIS (eight with AIS, 13 stroke mimics) and explored them in a retrospective cohort with confirmed AIS.

Results: Approximately half of the prospective and total AIS cohorts presented with acute, sustained hemiparesis, and were diagnosed relatively quickly. Read More

J Stroke Cerebrovasc Dis 2022 Jun 1;31(8):106581. Epub 2022 Jun 1.

Department of Neurosurgery, Brigham and Women's Hospital, Harvard University, Boston, MA, United States. Electronic address:

Objectives: Moyamoya disease is an idiopathic cerebrovascular disorder in which patients experience recurrent transient ischemic attacks, ischemic or hemorrhagic strokes, headaches, and seizures from progressive stenosis of the vessels of the anterior circulation. The mainstay of treatment in symptomatic patients is surgical revascularization. Here, we present the case of a moyamoya patient in which a failed encephaloduroarteriosynangiosis, after new strokes, is recycled and converted into a combined "double barrel" direct superficial temporal artery to middle cerebral artery bypass with included video. Read More

World Neurosurg 2022 May 31. Epub 2022 May 31.

Department of Neurosurgery, Iwate Medical University School of Medicine, Yahaba, Japan. Electronic address:

Objective: Brain I-iomazenil single-photon emission computed tomography (SPECT) can assess the distribution of the binding potential of central benzodiazepine receptors in the cerebral cortex. This binding potential may reflect neuronal function in viable tissues. The present prospective study using brain I-iomazenil SPECT aimed to determine whether improvements in cognitive function after indirect revascularization surgery alone are associated with postoperative recovery in neurotransmitter receptor function in the affected cerebral hemisphere among adult patients with moyamoya disease accompanied by ischemic presentation due to misery perfusion. Read More

Front Neurol 2022 16;13:890126. Epub 2022 May 16.

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

Objective: In moyamoya disease (MMD) with direct or combined revascularization, the initially hemodynamic recipient features are likely one of the main causes of acute hemodynamic disruption. Previous studies have explored the relationship between recipient diameter or flow velocity and postoperative complications, but there are still no optimal selection criteria with multiple potential recipient vessels. Cerebral edema is one of the most common radiological manifestations in the acute postoperative period. Read More

Front Neurol 2022 13;13:871421. Epub 2022 May 13.

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

Objective: Cerebral ischemia and intracranial hemorrhage are the two main phenotypes of moyamoya disease (MMD). However, the pathophysiological processes of these two MMD phenotypes are still largely unknown. Here, we aimed to use multimodal neuroimaging techniques to explore the brain structural and functional differences between the two MMD subtypes. Read More

Int J Nurs Pract 2022 May 29:e13066. Epub 2022 May 29.

College of Nursing, Korea University, Seoul, South Korea.

Purpose: To identify the risk and protective factors affecting a salutogenic sense of health in adolescents with Moyamoya disease based on Antonovsky's salutogenesis theory.

Methods: This study used a qualitative descriptive design. We interviewed adolescents diagnosed with Moyamoya disease and their parents and experts with experience in treating them from 4 July 2019 to 10 October 2019. Read More

World Neurosurg 2022 May 26. Epub 2022 May 26.

Department of Neurosurgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea.

Objective: We present the relationship between an aneurysm in adult moyamoya disease (MMD) patients and a future stroke event.

Methods: One hundred forty-seven aneurysms were found in 118 adult MMD patients. To find risk factors for future hemorrhagic and ischemic stroke, Kaplan-Meier and Cox-regression analyses were performed on clinical and radiologic factors. Read More

Br J Neurosurg 2022 Jun 1:1-4. Epub 2022 Jun 1.

Outpatient Department of Pediatrics, The First Hospital of Jilin University, Changchun, P.R. China.

Purpose: Moyamoya disease (MMD) is an uncommon chronic and occlusive cerebrovascular disorder involving the development of abnormal collateral vessels. This report aimed to describe a Chinese familial case with a rare variant in the RNF213 gene.

Methods: The present report presents a rare familial case of MMD involving a heterozygous c. Read More