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moyamoya disease AND pediatric

Sara C Tho-Calvi, Dominic Thompson, Dawn Saunders, Shakti Agrawal, Anna Basu, Manali Chitre, Gabriel Chow, Frances Gibbon, Anthony Hart, Krishnaraya Kamath Tallur, Fenella Kirkham, Rachel Kneen, Helen McCullagh, Leena Mewasingh, Grace Vassallo, Kayal Vijayakumar, Elizabeth Wraige, Tong Hong Yeo, Vijeya Ganesan
OBJECTIVE: To describe characteristics and course of a large UK cohort of children with moyamoya from multiple centers and examine prognostic predictors. METHODS: Retrospective review of case notes/radiology, with use of logistic regression to explore predictors of outcome. RESULTS: Eighty-eight children (median presentation age 5.1 years) were included. Thirty-six presented with arterial ischemic stroke (AIS) and 29 with TIA. Eighty had bilateral and 8 unilateral carotid circulation disease; 29 patients had posterior circulation involvement...
February 27, 2018: Neurology
Brittany L Ko, John H Unkel
Moyamoya disease (MMD) is a rare cerebrovascular disorder involving progressive constriction of the internal carotid artery and its branches. The disease has a particularly aggressive course in very young patients, and early surgical intervention is often necessary to prevent permanent neurological damage. MMD patients have an increased risk of stroke development, which may be provoked by pain or anxiety. Currently, no reports of pediatric patients with MMD exist in the dental literature. The purpose of this paper was to discuss the dental management of a two-year-old with moyamoya disease who presented with early childhood caries and dental fear, offering recommendations for dental providers with emphasis on stroke prevention, collaboration with the medical team, anesthesia considerations for patients with increased stroke risk, and the challenges to maintain the oral health of a patient undergoing complex medical treatment...
January 1, 2018: Pediatric Dentistry
Shinsuke Muraoka, Yoshio Araki, Goro Kondo, Michihiro Kurimoto, Yoshiki Shiba, Kenji Uda, Shinji Ota, Sho Okamoto, Toshihiko Wakabayashi
OBJECTIVE: Although revascularization surgery for patients with moyamoya disease can effectively prevent ischemic events and thus improve the long-term clinical outcome, the incidence of postoperative ischemic complications affects patients' quality of life. This study aimed to clarify the risk factors associated with postoperative ischemic complications and to discuss the appropriate perioperative management. METHODS: Fifty-eight revascularization operations were performed in 37 children with moyamoya disease...
February 9, 2018: World Neurosurgery
Hiroyuki Akagawa, Maki Mukawa, Tadashi Nariai, Shunsuke Nomura, Yasuo Aihara, Hideaki Onda, Taku Yoneyama, Takumi Kudo, Kazutaka Sumita, Taketoshi Maehara, Takakazu Kawamata, Hidetoshi Kasuya
Moyamoya disease is a progressive steno-occlusive condition of the main intracranial arteries that results in the compensatory formation of fragile moyamoya vessels at the base of the brain. RNF213 is the most significant susceptibility gene and is often found with the p.Arg4810Lys founder variant in East Asian patients. We identified three putatively deleterious variants of this gene from three pediatric patients: two were novel, and one was a recurrent missense variant previously reported in other pediatric patients...
2018: Human Genome Variation
Natália Battisti Serafini, Cássio Battisti Serafini, Alanna Santoro Vinhas, Marcio Barbosa Godinho
Neurofibromatosis type 1 is a multisystem genetic disease of autosomal dominant transmission that reveals important cutaneous manifestations such as café-au-lait spots, multiple neurofibromas, and ephelides in skin fold areas, as well as hamartomatous lesions in the eyes, bones, glands, and central nervous system. Moyamoya disease is a rare progressive vaso-occlusive disorder that occurs with important ischemic cerebrovascular events. Despite the rarity of this association in childhood, children diagnosed with neurofibromatosis type 1 and focal neurologic symptoms should be investigated for moyamoya syndrome...
November 2017: Anais Brasileiros de Dermatologia
Candice D Carpenter, Luke L Linscott, James L Leach, Sudhakar Vadivelu, Todd Abruzzo
BACKGROUND: Alagille syndrome is a pediatric multisystem disease with increased prevalence of cerebrovascular disease. The spectrum of cerebrovascular disease in Alagille syndrome includes cerebral aneurysms, moyamoya arteriopathy and dolichoectasia. The prevalence of cerebrovascular disease in Alagille syndrome varies widely in the literature. OBJECTIVE: To determine the prevalence of cerebrovascular disease in our institution's Alagille patient population by employing a full primary review of all available neuroimaging...
January 23, 2018: Pediatric Radiology
Yusuke S Hori, Yuki Ebisudani, Mizuho Aoi, Toru Fukuhara
BACKGROUND: Quasi-moyamoya disease is a condition that occurs in association with a specific underlying condition or disease such as atherosclerotic disease or neurofibromatosis type 1 (NF1). Pediatric cases are frequently reported, and an ischemic and bilateral presentation is more common than a hemorrhagic and unilateral presentation. CLINICAL PRESENTATION: A 39-year-old woman previously diagnosed with NF1 presented to our department with nausea and left hemiparesis...
January 2, 2018: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
Yeonhee Lee, Young Shin Lim, Sang Taek Lee, Heeyeon Cho
BACKGROUND: Renovascular hypertension (RVH) accounts for 5-10% of pediatric hypertension, and can be associated with underlying disease involving other organs. The purpose of this study was to evaluate the clinical characteristics and assess the treatment outcomes of Korean pediatric patients with RVH. METHODS: The medical records of 25 Korean pediatric patients with RVH were retrospectively reviewed. RESULTS: Twenty-four patients had underlying disease, and the most common cause was Moyamoya disease (MMD) (n = 10, 40%)...
December 27, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
Meng Zhao, Dong Zhang, Shuo Wang, Yan Zhang, Rong Wang, Xiaofeng Deng, Jizong Zhao
The importance of the posterior cerebral artery (PCA) involvement in moyamoya disease has been highlighted in recent years. However, few studies compared the impact of PCA lesions in moyamoya disease between pediatric and adult patients. We conducted this study to summarize the clinical features of moyamoya patients with PCA lesions and describe the difference between pediatric and adult patients. We reviewed the records of 696 consecutive moyamoya vasculopathy patients from 2009 to 2015. The Suzuki and the Miyamoto stages were used to evaluate the steno-occlusive lesions of the anterior and posterior arteries...
November 15, 2017: Acta Neurologica Belgica
Junko Yamanaka, Ikuma Nozaki, Mizue Tanaka, Hideko Uryuu, Noriko Sato, Takeji Matsushita, Hiroyuki Shichino
In the era of Antiretroviral Therapy (ART) in which human immunodeficiency virus type 1 (HIV-1) infection affected children can expect a better prognosis, the importance of careful follow up of pediatric HIV-1 cases for neurological complications has been growing. We present a case of hemorrhagic Moyamoya syndrome in a child with congenital HIV-1 infection. A 10-year-old girl was referred to our hospital for the treatment of Pneumocystis Jirovecii Pneumonia (PCP: Pneumocystis pneumonia). Her HIV-1 control was poor and Moyamoya syndrome was found during the opportunistic infection screening at admission...
November 11, 2017: Journal of Infection and Chemotherapy: Official Journal of the Japan Society of Chemotherapy
Yonggang Ma, Meng Zhao, Qian Zhang, Xingju Liu, Dong Zhang, Shuo Wang, Yan Zhang, Maogui Li, Jizong Zhao
OBJECTIVE: We aimed to explore the risk factors of seizure recurrence and the optimal surgical procedure for epileptic pediatric patients with moyamoya disease (MMD). METHODS: We reviewed 696 consecutive patients with MMD admitted to our hospital from 2009 to 2015. Pediatric patients with MMD who had seizures before revascularization were identified. We summarized the characteristics of seizures in pediatric patients with MMD and analyzed the predictive factors of recurrent seizure events...
November 8, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
Meng Zhao, Dong Zhang, Shuo Wang, Yan Zhang, Xiaofeng Deng, Jizong Zhao
BACKGROUND: The patterns and clinical significance of the presence of collaterals in moyamoya disease has not been elucidated thoroughly. We aimed to summarize the clinical features of collaterals in pediatric moyamoya disease. BASIC PROCEDURES: We reviewed consecutive pediatric patients with moyamoya vasculopathy who were admitted to our hospital from 2009 to 2015. Only pediatric moyamoya patients with digital subtraction angiography examinations were included in this study...
December 2017: Pediatric Neurology
Seong-Eun Park, Ju-Seong Kim, Eun Kyung Park, Kyu-Won Shim, Dong-Seok Kim
OBJECTIVE For patients with moyamoya disease (MMD), surgical intervention is usually required because of progressive occlusion of the internal carotid artery. The indirect bypass method has been widely accepted as the treatment of choice in pediatric patients. However, in adult patients with MMD, the most effective treatment method remains a matter of debate. Here, the authors compared the clinical outcomes from MMD patients treated with either extracranial-intracranial arterial bypass (EIAB; 43 hemispheres) or modified encephaloduroarteriosynangiosis (mEDAS; 75 hemispheres) to investigate whether mEDAS is an effective surgical method for treating adults with symptomatic MMD...
October 27, 2017: Journal of Neurosurgery
Peiji Song, Jing Qin, Han Lun, Penggang Qiao, Anming Xie, Gongjie Li
Because digital subtraction angiography (DSA) is not an ideal angiographic examination for moyamoya disease in the pediatric population, magnetic resonance angiography (MRA) provides a noninvasive contrast-free angiographic examination; whereas magnetic resonance imaging (MRI) provides superior spatial resolution and soft-tissue contrast for lesion assessment. Ninety patients with moyamoya disease were examined by MRI and DSA to assess the distribution of lesions and their diagnostic agreement between modalities...
November 2017: Journal of Child Neurology
Kinya Yokoyama, Mikio Maruwaka, Kazuhiro Yoshikawa, Yoshio Araki, Sho Okamoto, Masaki Sumitomo, Akino Kawamura, Yusuke Sakamoto, Kenzo Shimizu, Takashi Izumi, Toshihiko Wakabayashi
BACKGROUND: In moyamoya disease (MMD), the causes of differences in clinical features between children and adults and of the dramatic temporal changes in moyamoya vessels are poorly understood. We previously discovered elevated levels of m/z 4588 and m/z 4473 peptides in cerebrospinal fluid (CSF) in MMD patients. This study examined the amino acid sequences of these peptides and quantified in specimens. METHODS: The m/z 4588 and m/z 4473 peptides in CSF from MMD patients were purified and concentrated by high-performance liquid chromatography and ultrafiltration...
October 10, 2017: World Neurosurgery
Meng Zhao, Faliang Gao, Dong Zhang, Shuo Wang, Yan Zhang, Rong Wang, Jizong Zhao
Moyamoya disease (MMD) is the most common pediatric cerebrovascular disease in Eastern Asian countries but the etiology is not well understood. Circular RNAs (circRNAs) have been implicated in various biological processes, but their role in the development of MMD remains unclear. To address this issue, we carried out a comparative circRNA microarray analysis of blood samples obtained from patients with MMD and healthy subjects and identified 146 circRNAs that were differentially expressed between the two groups...
October 15, 2017: Journal of the Neurological Sciences
James Murchison, John M Wilson, Coby Ray, Jessica Ginsberg, Laszlo Nagy
BACKGROUND Cerebral hyperperfusion syndrome is a rare complication of indirect revascularization due to moyamoya disease, but has not been reported previously in the pediatric population. We present a case of an 18-month-old girl with moyamoya disease that was treated with bilateral pial synangiosis and had complications consistent with cerebral hyperperfusion syndrome. This case report discusses the pathophysiological mechanisms involved in cerebral hyperperfusion in moyamoya syndrome. CASE REPORT An 18-month-old female Caucasian presented with seizures and weakness of the left side...
October 9, 2017: American Journal of Case Reports
M Wintermark, N K Hills, G A DeVeber, A J Barkovich, T J Bernard, N R Friedman, M T Mackay, A Kirton, G Zhu, C Leiva-Salinas, Q Hou, H J Fullerton
BACKGROUND AND PURPOSE: Childhood arteriopathies are rare but heterogenous, and difficult to diagnose and classify, especially by nonexperts. We quantified clinical and imaging characteristics associated with childhood arteriopathy subtypes to facilitate their diagnosis and classification in research and clinical settings. MATERIALS AND METHODS: The Vascular Effects of Infection in Pediatric Stroke (VIPS) study prospectively enrolled 355 children with arterial ischemic stroke (2010-2014)...
October 5, 2017: AJNR. American Journal of Neuroradiology
Haruto Uchino, Ken Kazumata, Masaki Ito, Naoki Nakayama, Kiyohiro Houkin
OBJECTIVE A specific population of young patients with moyamoya disease (MMD) persistently experience physical symptoms not attributable to focal ischemia. These symptoms, highly suggestive of orthostatic intolerance (also termed "orthostatic dysregulation"), were investigated and reported as potential determinants of quality of life in young MMD patients. METHODS Forty-six patients (6-30 years of age) were selected from a group of 122 patients who were diagnosed with MMD before 18 years of age. The authors administered a structured questionnaire consisting of 11 items based on screening checklists published in the Japanese clinical guidelines for juvenile orthostatic dysregulation in young patients...
November 2017: Journal of Neurosurgery. Pediatrics
Joham Choque-Velasquez, Roberto Colasanti, Danil A Kozyrev, Juha Hernesniemi, Akitsugu Kawashima
BACKGROUND: Pediatric moyamoya cases may be very arduous, even more so in a developing country, where access to specialized centers may be prevented by different factors. CASE DESCRIPTION: Herein we report a challenging case, which was managed in the new Neurosurgical Center of Trujillo, regarding the direct anastomosis between the left superficial temporal artery and a cortical branch of the left middle cerebral artery in a 8-year-old Peruvian boy with moyamoya disease...
December 2017: World Neurosurgery
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