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Moyamoya disease

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https://www.readbyqxmd.com/read/28436815/moyamoya-in-a-patient-with-fires-a-first-case-report
#1
Taylor Kaufman, Andrew White
Febrile infection-related epilepsy syndrome (FIRES) is a form of epileptic encephalopathy with severe refractory epilepsy that presents in previously healthy, school-aged children after significant febrile illness with concomitant rise in body temperature. Suspected causes include genetic or acquired channelopathies, as well as mitochondrial disturbances. In FIRES, the EEG shows diffuse slowing, generalized, and/or multifocal discharges. Seizures are present and resistant to treatment. Moyamoya angiopathy (MMA) is characterized by progressive stenosis of cerebral arteries and subsequent development of a network of collateral circulation that is prone to rupture...
2017: Neurodiagnostic Journal
https://www.readbyqxmd.com/read/28431773/atypical-moyamoya-syndrome-with-brain-calcification-and-stenosis-of-abdominal-aorta-and-renal-arteries
#2
Hideki Uchikawa, Katsunori Fujii, Mayuko Fujita, Tomoko Okunushi, Naoki Shimojo
Moyamoya syndrome is a progressive cerebrovascular disease that is characterized by stenosis of the terminal portion of the internal carotid artery and its main branches, in combination with an accompanying disease. We herein describe an 8-year-old boy exhibiting transient loss of consciousness, who had recurrent seizures in infancy with progressive brain calcification. On admission, he was alert but magnetic resonance angiography showed bilateral stenosis of the whole internal carotid artery and proliferation of vascular collaterals, and brain CT revealed calcification on bilateral putamen...
April 18, 2017: Brain & Development
https://www.readbyqxmd.com/read/28414759/significant-association-of-rnf213-p-r4810k-a-moyamoya-susceptibility-variant-with-coronary-artery-disease
#3
Takaaki Morimoto, Yohei Mineharu, Koh Ono, Masahiro Nakatochi, Sahoko Ichihara, Risako Kabata, Yasushi Takagi, Yang Cao, Lanying Zhao, Hatasu Kobayashi, Kouji H Harada, Katsunobu Takenaka, Takeshi Funaki, Mitsuhiro Yokota, Tatsuaki Matsubara, Ken Yamamoto, Hideo Izawa, Takeshi Kimura, Susumu Miyamoto, Akio Koizumi
BACKGROUND: The genetic architecture of coronary artery disease has not been fully elucidated, especially in Asian countries. Moyamoya disease is a progressive cerebrovascular disease that is reported to be complicated by coronary artery disease. Because most Japanese patients with moyamoya disease carry the p.R4810K variant of the ring finger 213 gene (RNF213), this may also be a risk factor for coronary artery disease; however, this possibility has never been tested. METHODS AND RESULTS: We genotyped the RNF213 p...
2017: PloS One
https://www.readbyqxmd.com/read/28414666/just-a-puff-of-smoke
#4
A Leonard
CASE: A 44 year old woman with a history of stroke s/p aneurysm clipping, seizure, and substance abuse was brought to the hospital after a family member received a call from her friend saying she was acting unusual and may have had a seizure while hanging out on the street. At her baseline, she needed modest assistance in daily activities after her CVA and conversed without issue. Her mother died of a stroke. On exam the patient was afebrile, normotensive with mild tachycardia to 110...
March 2017: Journal of the Louisiana State Medical Society: Official Organ of the Louisiana State Medical Society
https://www.readbyqxmd.com/read/28411258/patient-with-severe-moyamoya-disease-who-presents-with-acute-cortical-blindness
#5
REVIEW
Aparna Sajja, Deki Tsering, Annie C Mooser, Tiffani A DeFreitas, Jessica Carpenter, Suresh N Magge
No abstract text is available yet for this article.
April 14, 2017: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/28409736/angiographic-features-of-hemorrhagic-moyamoya-disease-with-high-recurrence-risk-a-supplementary-analysis-of-the-japan-adult-moyamoya-trial
#6
Takeshi Funaki, Jun C Takahashi, Kiyohiro Houkin, Satoshi Kuroda, Shigekazu Takeuchi, Miki Fujimura, Yasutake Tomata, Susumu Miyamoto
OBJECTIVE In this paper, the authors set out to identify the angiographic features of moyamoya disease with posterior hemorrhage, which is a strong predictor of rebleeding. METHODS This cross-sectional study used the data set of the Japan Adult Moyamoya Trial (clinical trial registration no.: C000000166 [ www.umin.ac.jp/ctr/index.htm ]). The panel designed the ancillary measurement of angiography at onset, classifying the collateral vessels into 3 subtypes: lenticulostriate anastomosis, thalamic anastomosis, and choroidal anastomosis...
April 14, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28408257/ischemic-stroke-in-young-adults-with-moyamoya-disease-prognostic-factors-for-stroke-recurrence-and-functional-outcome-after-revascularization
#7
Meng Zhao, Xiaofeng Deng, Faliang Gao, Dong Zhang, Shuo Wang, Yan Zhang, Rong Wang, Jizong Zhao
OBJECTIVE: Stroke in young adults is uncommon and have rarely been described. Moyamoya disease is one of the leading causes of stroke in young adults. We aimed to study the prognostic factors for stroke recurrences and functional outcomes in young stroke patients with moyamoya disease after revascularization. METHODS: We reviewed 696 consecutive patients with moyamoya disease admitted to our hospital from 2009 to 2015 and identified patients aged 18-45 years with first-ever stroke...
April 10, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28402467/natural-history-and-risk-factor-of-recurrent-hemorrhage-in-hemorrhagic-adult-moyamoya-disease
#8
Kang Min Kim, Jeong Eun Kim, Won-Sang Cho, Hyun-Seung Kang, Young-Je Son, Moon Hee Han, Chang Wan Oh
BACKGROUND: Recurrent hemorrhage is a serious neurosurgical problem in adult moyamoya disease (MMD) patients. OBJECTIVE: To find the natural history and risk factors of recurrent hemorrhage in cases of adult hemorrhagic MMD. METHODS: One hundred seventy-six adult MMD patients presenting with hemorrhage were enrolled. Patients' medical records and radiological images were retrospectively reviewed. Clinical and radiological features of recurrent hemorrhage, and related risk factors were analyzed...
April 11, 2017: Neurosurgery
https://www.readbyqxmd.com/read/28385458/moyamoya-disease-with-occlusion-of-bilateral-vertebral-arteries-and-the-basilar-artery-fed-by-the-collateral-vessels-of-vertebral-arteries-a-rare-case-report
#9
Xiaofeng Deng, Dong Zhang, Yan Zhang, Rong Wang, Bo Wang, Jizong Zhao
The main change of moyamoya disease (MMD) is steno-occlusion at the terminal portion of the bilateral internal carotid arteries (ICAs). Occlusion of distal portions of the bilateral vertebral arteries (VAs) in MMD has never been reported. An 18-year old female was admitted with repeated headache for 13years. Cerebral digital subtraction angiography (DSA) demonstrated occlusion of bilateral ICAs at their terminal portions, moyamoya vessels at the skull base, occlusion of bilateral distal VAs and the basilar artery (BA) fed by the tortuous collateral vessels arising from the cervical segment of VAs...
April 3, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28382613/reduced-internal-carotid-artery-flow-in-color-coded-carotid-duplex-sonography
#10
Fu-Yi Yang, Po-Ren Hsu, Shinn-Kuang Lin
PURPOSE: Reduced flow in the internal carotid artery (ICA) is related to cerebral ischemia. We established a classification of reduced ICA flow through color-coded carotid duplex (CCD) sonography. METHODS: We retrospectively reviewed 25,000 CCD images in sonography laboratory. RESULTS: Reduced ICA flow [flow volume (FV) less than 100 mL/min] was found in 1.2% of all studies, and 270 patients were enrolled. We included 8% patients with lesions proximal to the ICA in Group A, 27% with lesions at the proximal ICA in Group B, 49% with lesions distal to the visible ICA in Group C, and 16% with unknown causes in Group D...
December 15, 2016: Acta Neurologica Taiwanica
https://www.readbyqxmd.com/read/28381201/etiology-and-pathogenesis-of-moyamoya-disease-an-update-on-disease-prevalence
#11
Shuo Huang, Zhen-Ni Guo, Mingchao Shi, Yi Yang, Mingli Rao
Moyamoya disease is a chronic cerebrovascular occlusive disease that is characterized by progressive stenosis of the terminal portion of the internal carotid artery and its main branches. The occurrence of Moyamoya disease is related to immune, genetic, and other factors. Though the research of Moyamoya disease has made great strides in the past 60 years, the etiology and pathogenesis are largely unknown. This review will focus on the genetic pathogenic and inflammation factors of Moyamoya disease.
April 2017: International Journal of Stroke: Official Journal of the International Stroke Society
https://www.readbyqxmd.com/read/28377246/awake-surgery-for-brain-vascular-malformations-and-moyamoya-disease
#12
Rami James N Aoun, Mithun G Sattur, Chandan Krishna, Amen Gupta, Matthew E Welz, Allan D Nanney, Antoun H Koht, Matthew C Tate, Katherine H Noe, Joseph I Sirven, Barrett J Anderies, Patrick B Bolton, Terry L Trentman, Richard S Zimmerman, Kristin R Swanson, Bernard R Bendok
OBJECTIVE: While a significant amount of experience has accumulated for awake procedures for brain tumor, epilepsy and carotid surgery, its utility for intracranial neurovascular indications remains largely undefined. Awake surgery, for select neurovascular cases, offers the advantage of precise brain mapping and robust neurological monitoring during surgery for lesions in eloquent areas, avoidance of potential hemodynamic instability, and possible faster recovery. Additionally, it opens the window for perilesional epileptogenic tissue resection with potentially less risk for iatrogenic injury...
April 1, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28366750/long-term-outcome-after-conservative-treatment-and-direct-bypass-surgery-of-moyamoya-disease-at-late-suzuki-stage
#13
Peicong Ge, Qian Zhang, Xun Ye, Xingju Liu, Xiaofeng Deng, Hao Li, Rong Wang, Yan Zhang, Dong Zhang, Jizong Zhao
OBJECTIVE: To investigate the long-term outcomes after conservative and direct surgical treatment for patients with moyamoya disease (MMD) at late Suzuki stage METHODS: We retrospectively reviewed 82 patients (164 hemispheres) with MMD at late Suzuki Stage at Beijing Tiantan Hospital. Clinical features, radiologic findings, and outcomes were analyzed. RESULTS: The mean age at diagnosis was 36.4±11.7 years. The distribution of the initial Suzuki stage of MMD was as follows: stage 4, n=113; stage 5, n=45; stage 6, n=6, PCA involvement was observed in 41 (25...
March 30, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28357585/uneven-cerebral-hemodynamic-change-as-a-cause-of-neurological-deterioration-in-the-acute-stage-after-direct-revascularization-for-moyamoya-disease-cerebral-hyperperfusion-and-remote-ischemia-caused-by-the-watershed-shift
#14
Xian-Kun Tu, Miki Fujimura, Sherif Rashad, Shunji Mugikura, Hiroyuki Sakata, Kuniyasu Niizuma, Teiji Tominaga
Superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis is the standard surgical treatment for moyamoya disease (MMD). The main potential complications of this treatment are cerebral hyperperfusion (CHP) syndrome and ischemia, and their managements are contradictory to each other. We retrospectively investigated the incidence of the simultaneous manifestation of CHP and infarction after surgery for MMD. Of the 162 consecutive direct revascularization surgeries performed for MMD, we encountered two adult cases (1...
March 29, 2017: Neurosurgical Review
https://www.readbyqxmd.com/read/28345540/epidemiological-features-of-nontraumatic-spontaneous-subarachnoid-hemorrhage-in-china-a-nationwide-hospital-based-multicenter-study
#15
Jian-Ping Song, Wei Ni, Yu-Xiang Gu, Wei Zhu, Liang Chen, Bin Xu, Bin Leng, Yan-Long Tian, Ying Mao
BACKGROUND: Nontraumatic spontaneous subarachnoid hemorrhage (SAH) is associated with a high mortality. This study was conducted to investigate the epidemiological features of nontraumatic spontaneous SAH in China. METHODS: From January 2006 to December 2008, the clinical data of patients with nontraumatic SAH from 32 major neurosurgical centers of China were evaluated. Emergent digital subtraction angiography (DSA) was performed for the diagnosis of SAH sources in the acute stage of SAH (≤3 days)...
April 5, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28343148/de-novo-mutations-in-cbl-causing-early-onset-paediatric-moyamoya-angiopathy
#16
Stéphanie Guey, Lou Grangeon, Francis Brunelle, Françoise Bergametti, Jeanne Amiel, Stanislas Lyonnet, Audrey Delaforge, Minh Arnould, Béatrice Desnous, Céline Bellesme, Dominique Hervé, Jan C Schwitalla, Markus Kraemer, Elisabeth Tournier-Lasserve, Manoelle Kossorotoff
BACKGROUND: Moyamoya angiopathy (MMA) is characterised by a progressive stenosis of the terminal part of the internal carotid arteries and the development of abnormal collateral deep vessels. Its pathophysiology is unknown. MMA can be the sole manifestation of the disease (moyamoya disease) or be associated with various conditions (moyamoya syndrome) including some Mendelian diseases. We aimed to investigate the genetic basis of moyamoya using a whole exome sequencing (WES) approach conducted in sporadic cases without any overt symptom suggestive of a known Mendelian moyamoya syndrome...
March 25, 2017: Journal of Medical Genetics
https://www.readbyqxmd.com/read/28341559/-moyamoya-disease-associated-with-kidney-angiodysplasia-in-a-child
#17
A Dibi, Z Maana, F Jabourik, A Bentahila
INTRODUCTION: Moyamoya disease is a progressive, chronic occlusive vascular disease of the circle of Willis arteries leading to the development of collateral circulation to compensate the occlusion. CASE REPORT: An 11-year-old girl presented an abrupt loss of consciousness and a right-sided motor deficit. Clinical examination found hypertension with 220/120mmHg arterial blood pressure. Brain magnetic resonance imaging showed a large left hemispheric ischemic lesion...
May 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28327981/long-term-outcome-after-multiple-burr-hole-surgery-in-children-with-moyamoya-angiopathy-a-single-center-experience-in-108-hemispheres
#18
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
https://www.readbyqxmd.com/read/28327889/hemodynamic-changes-after-unilateral-revascularization-for-moyamoya-disease-serial-assessment-by-quantitative-magnetic-resonance-angiography
#19
Tackeun Kim, Jae Seung Bang, O-Ki Kwon, Gyojun Hwang, Jeong Eun Kim, Hyun-Seung Kang, Won-Sang Cho, Cheolkyu Jung, Chang Wan Oh
BACKGROUND: Ultrasonic flow meters and quantitative magnetic resonance angiography quantitatively assess flow during hemodynamic evaluation of cerebral ischemia. Although their reliability and reproducibility have been verified, their clinical impact in moyamoya disease has rarely been reported. OBJECTIVE: To investigate flow measurement outcomes in moyamoya disease patients pre- and postoperatively through a retrospective observational study. METHODS: We evaluated 41 patients undergoing their first revascularization surgery who were followed ≥6 mo...
January 2, 2017: Neurosurgery
https://www.readbyqxmd.com/read/28325888/factors-associated-with-clinical-outcomes-in-patients-with-primary-intraventricular-hemorrhage
#20
Sang-Hoon Lee, Kyung-Jae Park, Dong-Hyuk Park, Shin-Hyuk Kang, Jung-Yul Park, Yong-Gu Chung
BACKGROUND Primary intraventricular hemorrhage (PIVH) is an uncommon type of intracerebral hemorrhage. Owing to its rarity, the clinical and radiological factors affecting outcomes in patients with PIVH have not been widely studied. MATERIAL AND METHODS We retrospectively reviewed 112 patients (mean age 53 years) treated for PIVH at our institution from January 2004 to December 2014. Clinical and radiological parameters were analyzed 3 months after initial presentation to identify factors associated with clinical outcomes, as assessed by the Glasgow Outcome Scale (favorable ≥4, unfavorable <4)...
March 22, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
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