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moyamoya disease AND pediatric

Thomas Blauwblomme, Bertrand Mathon, Olivier Naggara, Manoelle Kossorotoff, Marie Bourgeois, Stéphanie Puget, Philippe Meyer, Valentine Brousse, Marianne de Montalembert, Francis Brunelle, Michel Zerah, Christian Sainte-Rose
BACKGROUND: Multiple burr hole (MBH) surgery is a simple, safe, and effective indirect technique of revascularization in moyamoya angiopathy (MM). However, it is not yet recognized as a first-line treatment. OBJECTIVE: To assess the long-term outcome and perioperative complications in a large single-center cohort of children with MM who underwent burr hole surgery. METHODS: This study is a retrospective analysis of children who underwent surgery for MM in a national reference center for pediatric stroke between 1999 and 2015...
February 20, 2017: Neurosurgery
Hime Suzuki, Takeshi Mikami, Tomoyoshi Kuribara, Kazuhisa Yoshifuji, Katsuya Komatsu, Yukinori Akiyama, Hirofumi Ohnishi, Kiyohiro Houkin, Nobuhiro Mikuni
OBJECTIVE Medullary streaks detected on fluid-attenuated inversion recovery (FLAIR) imaging have been considered to be reflected ischemic regions in pediatric moyamoya disease. The purpose of this study was to evaluate these medullary streaks both clinically and radiologically and to discuss associated pathophysiological concerns. METHODS The authors retrospectively reviewed data from 14 consecutive pediatric patients with moyamoya disease treated between April 2009 and June 2016. Clinical and radiological features and postoperative imaging changes were analyzed...
March 10, 2017: Journal of Neurosurgery. Pediatrics
Jonathan Gaillard, Jennifer Klein, Daniel Duran, Armide Storey, R Michael Scott, Kristopher Kahle, Edward R Smith
OBJECTIVE Limited data exist on familial moyamoya in children. The purpose of this study was to characterize presentation and outcomes of pediatric moyamoya patients who have relatives diagnosed with moyamoya. METHODS The authors performed a single-institution retrospective analysis of a case series including all surgically treated children with moyamoya with first- or second-degree relatives with moyamoya. Clinical and radiographic characteristics were analyzed, along with surgical outcomes. RESULTS A total of 537 patients underwent surgery for moyamoya during the study period...
March 10, 2017: Journal of Neurosurgery. Pediatrics
Seigo Korematsu, Hiroshi Yamada, Hiroaki Miyahara, Kenji Ihara
Cerebral infarction in children is rare and often occurs secondary to moyamoya disease, hereditary coagulopathies, vasculitis, antiphospholipid antibody syndrome, heart disease, mitochondrial disease. However, in some cases, the causes of cerebral infarction is unknown. In this study, we detected increased levels of serum anti-phosphatidylcholine and anti-phosphatidylethanolamine IgG antibodies in three pediatric patients with cerebral infarction whose primary disorders are unknown by routine examination. For the five disease control patients of cerebral infarction due to other primary disorders, there was no such increase in these antibodies levels...
February 23, 2017: Brain & Development
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: More than 50 years have elapsed since moyamoya disease was initially described; however, the disease etiology remains unknown. Although certain genetic loci and immunologic characteristics are associated with moyamoya disease, this does not fully explain its pathophysiology. An association with inflammatory disease has been postulated but not rigorously explored. We sought to examine the epidemiologic association of moyamoya and inflammatory diseases by analyzing data from a large administrative database...
April 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. Clinical features, radiologic 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...
December 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
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