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moyamoya disease AND pediatric

Wuyang Yang, Jose Luis Porras, Risheng Xu, Tomas Garzon-Muvdi, Justin M Caplan, Geoffrey P Colby, Alexander Lewis Coon, Rafael J Tamargo, Judy Huang, Edward Sanghoon Ahn
No abstract text is available yet for this article.
August 1, 2016: Neurosurgery
Juan C Mejia-Munne, Jason A Ellis, Neil A Feldstein, Philip M Meyers, Edward S Connolly
BACKGROUND: Over fifty years have elapsed since Moyamoya was initially described; however, the disease etiology remains unknown. Although certain genetic loci and immunological characteristics are associated with Moyamoya, this does not fully explain its pathophysiology. An association with inflammatory disease has been postulated but not rigorously explored. We sought to examine the epidemiological association of Moyamoya and inflammatory diseases by analyzing data from a large administrative database...
January 13, 2017: World Neurosurgery
Yuki Nakamori, Kenji Yoshitani, Yoshihiko Ohnishi
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
Jacqueline Chan, Fabiola D'Ambrosio Rodriguez, Deepank Sahni, Claudia Boucher-Berry
Moyamoya disease is a rare chronic and progressive cerebrovascular disease of the arteries of the circle of Willis that can affect children and adults. It has been associated with multiple diseases, including immunologic, like Graves' disease, diabetes mellitus, and SLE. Hyperlipidemia has been recognized in patients with Moyamoya disease with an incidence of 27-37%. However, no case in pediatric patients has been reported of the coexistence of Moyamoya disease and hyperlipidemia. Here we present a case of a 9-year-old female diagnosed with Moyamoya disease after a stroke with incidental finding of familial hypercholesterolemia...
2016: Case Reports in Endocrinology
Qian Zhang, Rong Wang, Yaping Liu, Yan Zhang, Shuo Wang, Yong Cao, Yuanli Zhao, Xingju Liu, Jia Wang, Xiaofeng Deng, Faliang Gao, Ziwen Yang, Meng Zhao, Peicong Ge, Yonggang Ma, Jizong Zhao, Dong Zhang
OBJECTIVE: To elucidate the clinical features, risk factors for contralateral progression, and long-term outcomes of patients with unilateral moyamoya disease (MMD). METHODS: We retrospectively reviewed 109 patients with unilateral MMD treated at Beijing Tiantan Hospital. The clinical features, radiological findings and outcomes were analyzed. RESULTS: The mean age at diagnosis was 30.8±14.2 years with a one-peak distribution in the thirties...
September 16, 2016: World Neurosurgery
Shin-Joe Yeh, Sung-Chun Tang, Li-Kai Tsai, Ya-Fang Chen, Hon-Man Liu, Ying-An Chen, Yu-Lin Hsieh, Shih-Hung Yang, Yu-Hsuan Tien, Chi-Cheng Yang, Meng-Fai Kuo, Jiann-Shing Jeng
The marked cerebral hypoperfusion of moyamoya disease (MMD) can be treated with encephaloduroarteriosynangiosis (EDAS), an indirect revascularization surgery. Collateral establishment after the surgery is a gradual process; thus, easy access to serial assessment is of great importance. We prospectively recruited 15 pediatric moyamoya patients who underwent EDAS surgeries on a total of 19 hemispheres. Ultrasonography of extracranial and intracranial arteries was performed pre-operatively and post-operatively at 1, 3 and 6 mo...
December 2016: Ultrasound in Medicine & Biology
Suresh Kumar, Sudhir Sharma, Anupam Jhobta, Ram Gopal Sood
Moyamoya disease (MMD) is a rare cerebrovascular disease characterized by idiopathic occlusion of bilateral internal carotid arteries and the development of characteristic leptomeningeal collateral vessels along anterior or posterior circulation. We present an unusual case of MMD presenting with generalized dystonia as the predominant manifestation.
April 2016: Journal of Pediatric Neurosciences
Cunxin Tan, Ran Duan, Xun Ye, Dong Zhang, Rong Wang
BACKGROUND: and Purpose: Moyamoya disease (MMD) is a chronic cerebrovascular disorder with little known etiology. We aim to propose a new classification system for MMD from the perspective of embryology. METHODS: MMD patients' digital subtraction angiograms were retrospectively analyzed. Every angiogram was analyzed to find the abnormal vessels and from which part of the posterior cerebral artery (PCA) the lesions begin. RESULTS: In 262 MMD cases, 32 pediatric patients had PCA involvement, of which 17 were male and 15 were female; 68 adults had PCA involvement, of which 33 were male and 35 were female...
September 1, 2016: World Neurosurgery
Güliz Acker, Susanne Goerdes, Peter Schmiedek, Marcus Czabanka, Peter Vajkoczy
BACKGROUND: Moyamoya disease (MMD) associated with a potentially underlying disease, such as genetic disorders or other coexisting hematological pathologies, is called quasi-MMD. This very rare disease has been characterized mainly in Asian countries, so far. As MMD reveals several significant ethnic differences, the question is raised whether characteristics of quasi-MMD would also vary among different ethnic backgrounds. Here, we report a series of 61 patients with quasi-MMD and highlight the specific clinical features of this rare disease among European Caucasians...
2016: Cerebrovascular Diseases
Kimon Bekelis, Ian D Connolly, Huy M Do, Omar Choudhri
OBJECTIVE The impact of procedural volume on the outcomes of cerebrovascular surgery in children has not been determined. In this study, the authors investigated the association of operative volume on the outcomes of cerebrovascular neurosurgery in pediatric patients. METHODS The authors performed a cohort study of all pediatric patients who underwent a cerebrovascular procedure between 2003 and 2012 and were registered in the Kids' Inpatient Database (KID). To control for confounding, the authors used multivariable regression models, propensity-score conditioning, and mixed-effects analysis to account for clustering at the hospital level...
November 2016: Journal of Neurosurgery. Pediatrics
Alexander J Ball, Gary K Steinberg, Jorina Elbers
BACKGROUND: Moyamoya disease (MMD) is a progressive intracranial arteriopathy with high risk of stroke. Its impact on quality of life is unstudied. We surveyed children with moyamoya disease and compared their quality of life to chronically ill children and children with stroke to better understand the impact of this diagnosis. METHODS: Children with moyamoya disease aged seven to 17 years from Stanford's Moyamoya Clinic between June 2014 and March 2015 were included...
October 2016: Pediatric Neurology
Luke Macyszyn, Mark Attiah, Tracy S Ma, Zarina Ali, Ryan Faught, Alisha Hossain, Karen Man, Hiren Patel, Rosanna Sobota, Eric L Zager, Sherman C Stein
OBJECTIVE Moyamoya disease (MMD) is a chronic cerebrovascular disease that can lead to devastating neurological outcomes. Surgical intervention is the definitive treatment, with direct, indirect, and combined revascularization procedures currently employed by surgeons. The optimal surgical approach, however, remains unclear. In this decision analysis, the authors compared the effectiveness of revascularization procedures in both adult and pediatric patients with MMD. METHODS A comprehensive literature search was performed for studies of MMD...
July 29, 2016: Journal of Neurosurgery
Wuyang Yang, Jose Luis Porras, Risheng Xu, Tomas Garzon-Muvdi, Justin M Caplan, Geoffrey P Colby, Alexander Lewis Coon, Rafael J Tamargo, Judy Huang, Edward Sanghoon Ahn
INTRODUCTION: Sickle cell disease (SCD) patients with moyamoya syndrome (MMS) represent a rare subset of patients with potentially devastating neurological outcomes. The effectiveness of surgical revascularization in this patient population is currently unclear. We aim to elucidate the effectiveness of surgical intervention in our series of SCD-MMS patients by comparing stroke recurrence in those undergoing revascularization vs conservative transfusion therapy. METHODS: We retrospectively reviewed our database of moyamoya patients at the Johns Hopkins Medical Institution from 1990 to 2013...
August 2016: Neurosurgery
Katie Pricola Fehnel, Micah Duggins-Warf, David Zurakowski, Maxwell McKee-Proctor, Rajarshi Majumder, Michael Raber, Xuezhe Han, Edward R Smith
OBJECTIVE The authors report the use of urinary biomarkers as a novel, noninvasive technique to detect juvenile pilocytic astrocytomas (JPAs), capable of distinguishing JPAs from other CNS diseases, including other brain tumors. Preliminary screening of an array of tumors implicated proteases (including matrix metalloproteinases [MMPs]) and their inhibitors (tissue inhibitors of metalloproteinase [TIMPs]) as well as growth factors (including basic fibroblast growth factor [bFGF]) as candidate biomarkers. These data led the authors to hypothesize that tissue inhibitor of metalloproteinase 3 (TIMP3) and bFGF would represent high-probability candidates as JPA-specific biomarkers...
October 2016: Journal of Neurosurgery. Pediatrics
Peng Liu, Xian-Li Lv, Ai-Hua Liu, Cheng Chen, Hui-Jian Ge, Heng-Wei Jin, Xin Feng, Ming Lv, You-Xiang Li, Lian Duan
BACKGROUND: Moyamoya disease (MMD) in children was rarely associated with intracranial aneurysms. The purpose of this study was to report the clinical characteristics and long-term surgical outcomes of pediatric intracranial aneurysms accompanied with MMD. METHODS: Between October 2002 and October 2013, our department treated 9 pediatric MMD patients (aged ≤17 years) with intracranial aneurysms. Clinical and angiographic features, treatment selection, as well as follow-up information were obtained and analyzed...
October 2016: World Neurosurgery
Yoshito Sugita, Takeshi Funaki, Jun C Takahashi, Yasushi Takagi, Yasutaka Fushimi, Takayuki Kikuchi, Kazumichi Yoshida, Taketo Hatano, Natsuhi Sasaki, Susumu Miyamoto
BACKGROUND: The pathophysiological mechanism of chorea as a presentation of pediatric moyamoya disease remains unknown, although ischemia is suspected as a likely cause. The authors describe two cases of pediatric moyamoya disease, both of which presented with hemichorea in the stable phase after successful bypass surgery. CLINICAL PRESENTATION: Cerebral blood flow was almost normal in one case and decreased in the basal ganglia and watershed area in the other case due to infarcts occurring before surgery...
November 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Sherif Rashad, Miki Fujimura, Kuniyasu Niizuma, Hidenori Endo, Teiji Tominaga
Moyamoya disease (MMD) is a rare occlusive cerebrovascular disease that mainly presents in children as cerebral ischemia. Prompt treatment with either a direct or indirect revascularization procedure is necessary for children with MMD in order to prevent repeated ischemic events. We herein present our experience with combined direct and indirect bypass surgery for the treatment of pediatric MMD as well as our uniquely designed perioperative protocol. Twenty-three patients with MMD, aged between 2 and 16 years old (mean 9...
October 2016: Neurosurgical Review
Muhammad Jamil, Germaine Xin Yi Tan, Mehnaz Huq, Heidi Kang, Zhi Rui Lee, Phua Hwee Tang, Xi Hong Hu, Choon Hwai Yap
BACKGROUND: The Moyamoya disease is a cerebrovascular disease that causes occlusion of the distal end of the internal carotid artery, leading to the formation of multiple tiny collateral arteries. To date, the pathogenesis of Moyamoya is unknown. Improved understanding of the changes to vascular geometry and fluid mechanics of the carotid siphon during disease may improve understanding of the pathogenesis, prognosis techniques and disease management. METHODS: A retrospective analysis of Magnetic Resonance Angiography (MRA) images was performed for Moyamoya pediatric patients (MMD) (n = 23) and control (Ctrl) pediatric patients (n = 20)...
December 2016: Computer Methods in Biomechanics and Biomedical Engineering
Yasushi Takagi, Yulius Hermanto, Jun C Takahashi, Takeshi Funaki, Takayuki Kikuchi, Yohei Mineharu, Kazumichi Yoshida, Susumu Miyamoto
Moyamoya disease (MMD) is a unique progressive steno-occlusive disease of the distal ends of bilateral internal arteries and their proximal branches. The difference in clinical symptoms between adult and children MMD patients has been well recognized. In this study, we sought to investigate the phenomenon through histopathological study. Fifty-one patients underwent surgical procedures for treatment of standard indications of MMD at Kyoto University Hospital. Fifty-nine specimens of MCA were obtained from MMD patients during the surgical procedures...
June 15, 2016: Neurologia Medico-chirurgica
W Lee Titsworth, R Michael Scott, Edward R Smith
BACKGROUND AND PURPOSE: Comprehensive multicenter data on treatment of pediatric moyamoya in the United States is lacking. We sought to identify national trends in the diagnosis and treatment of this disease. METHODS: A total of 2454 moyamoya admissions from 1997 to 2012 were identified from the Kids Inpatient Database. Demographics, inpatient costs, interventions, and discharge status were analyzed. Admissions with and without surgical revascularization were reviewed separately...
May 2016: Stroke; a Journal of Cerebral Circulation
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