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Moyamoya disease

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https://www.readbyqxmd.com/read/28532265/encephaloduroarteriosynangiosis-edas-in-young-patients-with-cerebrovascular-complications-of-sickle-cell-disease-single-institution-experience
#1
Michael Winstead, Peter P Sun, Kenneth Martin, Janice Earl, Lynne Neumayr, Carolyn Hoppe, Elliott Vichinsky
Moyamoya syndrome occurs in sickle cell disease (SCD) as a secondary complication of large-artery stenosis. Moyamoya increases the risk of stroke, but its optimal management in SCD is not established. ‚ÄÉEncephaloduroarteriosynangiosis (EDAS) is a neurosurgical revascularization procedure for moyamoya whose use has been reported in SCD patients. We report the outcomes of 11 patients with SCD systematically evaluated for EDAS by a multidisciplinary team and compare the rate of stroke in patients who received EDAS to those who did not...
May 22, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28520567/persistent-hypotension-and-cerebral-swelling-resulting-from-mesenteric-traction-syndrome-after-omental-to-pial-pedicle-flap-transfer-in-a-young-woman-with-refractory-moyamoya-disease-a-case-report
#2
Harvey J Woehlck, Suneeta Gollapudy, Christopher J Roberts, Akinwunmi Oni-Orisan, Raphael H Sacho, Paul S Pagel
Superficial temporal arterial to middle cerebral arterial anastomosis is often the initial surgical treatment of Moyamoya disease. In refractory cases, placing a pedicle flap of omentum over the ischemic brain has resulted in clinical improvement or stabilization of symptoms. We present a case of persistent mesenteric traction syndrome manifested by hypotension unresponsive to conventional doses of vasopressors during and after pulling the omentum to the brain. As prostacyclin is a major mediator of hypotension from mesenteric traction syndrome and also a cerebral vasodilator, we discuss the possibility that brain swelling may be a manifestation of mesenteric traction syndrome...
May 17, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28516875/reply-to-moyamoya-disease-and-systemic-sclerosis-mosys-syndrome-a-combination-of-two-rare-entities-comment-to-the-authors
#3
Franz Wegner, Ulf Mueller-Ladner, Florian M P Meier
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April 26, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28506590/rnf213-p-r4810k-variant-and-intracranial-arterial-stenosis-or-occlusion-in-relatives-of-patients-with-moyamoya-disease
#4
Yoshiko Matsuda, Yohei Mineharu, Mitsuru Kimura, Yasushi Takagi, Hatasu Kobayashi, Toshiaki Hitomi, Kouji H Harada, Yoshito Uchihashi, Takeshi Funaki, Susumu Miyamoto, Akio Koizumi
BACKGROUND: This study aimed to determine the effectiveness of genetic testing for the p.R4810K variant (rs112735431) of the Mysterin/RNF213 gene, which is associated with moyamoya disease and other intracranial vascular diseases, in the family members of patients with moyamoya disease. METHODS: We performed genotyping of the RNF213 p.R4810K polymorphism and magnetic resonance angiography on 59 relatives of 18 index patients with moyamoya disease. Nineteen individuals had follow-up magnetic resonance angiography with a mean follow-up period of 7...
May 11, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28500366/comparison-of-3d-magnetic-resonance-imaging-and-digital-subtraction-angiography-for-intracranial-artery-stenosis
#5
Ji Eun Park, Seung Chai Jung, Sang Hun Lee, Ji Young Jeon, Ji Ye Lee, Ho Sung Kim, Choong-Gon Choi, Sang Joon Kim, Deok Hee Lee, Seon-Ok Kim, Sun U Kwon, Dong-Wha Kang, Jong S Kim
OBJECTIVES: To compare three-dimensional high-resolution magnetic resonance imaging (3D HR-MRI) and digital subtraction angiography (DSA) for diagnosing and evaluating stenosis in the entire circle of Willis. METHODS: The study included 516 intracranial arteries from 43 patients with intracranial artery stenosis (ICAS) who underwent both 3D HR-MRI and DSA within 1 month. Two readers independently diagnosed atherosclerosis, dissection, moyamoya disease and vasculitis, rated their diagnostic confidence for each vessel and measured the luminal diameters...
May 12, 2017: European Radiology
https://www.readbyqxmd.com/read/28498060/correlation-between-reduction-in-microvascular-transit-time-after-superficial-temporal-artery-middle-cerebral-artery-bypass-surgery-for-moyamoya-disease-and-the-development-of-postoperative-hyperperfusion-syndrome
#6
Tao Yang, Yoshifumi Higashino, Hiroharu Kataoka, Eika Hamano, Daisuke Maruyama, Koji Iihara, Jun C Takahashi
OBJECTIVE Hyperperfusion syndrome (HPS) is a notable complication that causes various neurological symptoms after superficial temporal artery (STA)-middle cerebral artery (MCA) bypass surgery for moyamoya disease (MMD). The authors used intraoperative indocyanine green (ICG) videoangiography to measure the change in microvascular transit time (MVTT) after bypass surgery. An analysis was then conducted to identify the correlation between change in MVTT and presence of postoperative HPS. METHODS This study included 105 hemispheres of 81 patients with MMD who underwent STA-MCA single bypass surgery between January 2010 and January 2015...
May 12, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28497183/rapid-contralateral-progression-of-focal-cerebral-arteriopathy-distinguished-from-rnf213-related-moyamoya-disease-and-fibromuscular-dysplasia
#7
Yoshio Araki, Yasushi Takagi, Yohei Mineharu, Hatasu Kobayashi, Susumu Miyamoto, Toshihiko Wakabayashi
BACKGROUND: Focal cerebral arteriopathy includes unifocal or multifocal lesions that are unilateral or bilateral. Large- and/or medium-sized vessels are involved and can be visualized on angiography. CASE REPORT: We report a case of cerebral infarction in a 9-year-old Japanese female who presented with a transient ischemic attack. Steno-occlusion involving the distal part of the internal carotid artery, proximal middle cerebral artery, and anterior cerebral artery was observed...
May 11, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28487332/neurocognitive-performance-after-cerebral-revascularization-in-adult-moyamoya-disease
#8
Penelope D Zeifert, Peter Karzmark, Teresa E Bell-Stephens, Gary K Steinberg, Leslie J Dorfman
BACKGROUND AND PURPOSE: Cerebral revascularization using EC-IC bypass is widely used to treat moyamoya disease, but the effects of surgery on cognition are unknown. We compared performance on formal neurocognitive testing in adults with moyamoya disease before and after undergoing direct EC-IC bypass. METHODS: We performed a structured battery of 13 neurocognitive tests on 84 adults with moyamoya disease before and 6 months after EC-IC bypass. The results were analyzed using reliable change indices for each test, to minimize test-retest variability and practice effects...
May 9, 2017: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/28484538/moyamoya-disease-presenting-as-bilateral-acute-subdural-hematomas-without-deficits
#9
Mudumba Vijayasaradhi, Vangala B Prasad
We report a patient presenting with imageological features of bilateral acute subdural hematomas and subarachnoid hemorrhage, who was subsequently diagnosed as a case of moyamoya disease. Imageological features, source of hemorrhage, literature review, and management are discussed. We report this case in view of its rarity in presentation with these imageological features.
April 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28479818/moyamoya-disease-as-a-cause-of-stroke-in-a-child-with-tetralogy-of-fallot
#10
Shambhavi, Priyanka Udawat, Sadasivan Sitaraman
No abstract text is available yet for this article.
April 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28475226/molecular-cytogenetic-characterization-of-five-f8-complex-rearrangements-utility-for-haemophilia-a-genetic-counselling
#11
Y Jourdy, N Chatron, M Fretigny, M L Carage, H Chambost, S Claeyssens-Donadel, V Roussel-Robert, C Negrier, D Sanlaville, C Vinciguerra
BACKGROUND: Genomic inversions are usually balanced, but unusual patterns have been described in haemophilia A (HA) patients for intron 22 (Inv22) and intron 1 (Inv1) inversions leading to the hypothesis of more complex rearrangements involving deletions or duplications. AIM: To characterize five abnormal patterns either in Southern blot and long-range PCR for Inv22 or in PCR for Inv1. MATERIALS AND METHODS: All patients were studied using cytogenetic microarray analysis (CMA)...
May 5, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28474994/meta-analysis-of-the-surgical-outcomes-of-symptomatic-moyamoya-disease-in-adults
#12
Jin Pyeong Jeon, Jeong Eun Kim, Won-Sang Cho, Jae Seung Bang, Young-Je Son, Chang Wan Oh
OBJECTIVE The purpose of this study was to evaluate treatment outcomes of future stroke prevention, perioperative complications, and angiographic revascularization in adults with symptomatic moyamoya disease (MMD) according to treatment modalities and surgical techniques. METHODS A systemic literature review was performed based on searches of the PubMed, Embase, and Cochrane Central databases. A fixed-effects model was used in cases of heterogeneity less than 50%. Publication bias was determined by Begg's funnel plot, Egger's test of the intercept, and the Begg and Mazumdar rank correlation test...
May 5, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28450666/anomalies-of-the-middle-cerebral-artery
#13
Naoyuki Uchiyama
There are several anomalies of the middle cerebral artery (MCA) in humans, such as accessory MCA, duplicated MCA, fenestration of MCA, and duplicated origin of MCA. Recently, unfused or twig-like MCA, which indicates MCA trunk occlusion with collateral plexiform arterial network, have been reported. During the embryonic stage, MCA is thought to generate from plexiform arterial twigs arising from the anterior cerebral artery, and these twigs form the definitive MCA by fusion and regression at the end of the development stage...
April 27, 2017: Neurologia Medico-chirurgica
https://www.readbyqxmd.com/read/28448235/intracranial-artery-steno-occlusion-diagnosis-by-using-three-dimensional-parallel-multichannel-transmission-mr-imaging
#14
Mi Sun Chung, Seung Chai Jung, Seon-Ok Kim, Ho Sung Kim, Choong Gon Choi, Sang Joon Kim, Sun U Kwon, Dong-Wha Kang, Jong S Kim
Purpose To determine whether magnetic resonance (MR) imaging by using two-dimensional spatially selective radiofrequency (RF) excitation pulses (zoomed MR imaging) is a reliable method for the diagnosis of intracranial artery steno-occlusion compared with full-field-of-view (FOV) MR imaging. Materials and Methods The institutional review board approved this retrospective study and informed consent was waived. From December 2014 to August 2015, 88 patients who underwent both full-FOV MR imaging and zoomed MR imaging for further evaluation of intracranial artery steno-occlusion at prior luminal evaluation were included...
April 27, 2017: Radiology
https://www.readbyqxmd.com/read/28446622/cortical-venous-redness-represents-tissue-circulation-status-in-patients-with-moyamoya-disease
#15
Toshio Machida, Yoshinori Higuchi, Shigeki Nakano, Satoshi Ishige, Atsushi Fujikawa, Yuichi Akaogi, Junichiro Shimada, Yoichi Yoshida, Sigenori Maru, Junichi Ono
BACKGROUND AND PURPOSE: Venous oxygen saturation (SO2) is measured in medical fields to assess tissue circulation insufficiency. This study aimed to elucidate the use of a cortical venous redness measurement to evaluate hemodynamic changes during revascularization surgery for patients with moyamoya disease. METHODS: In this retrospective case-series analysis, we first quantitatively measured and correlated SO2 and R intensity of 24-bit color digital red-green-blue pictures of blood samples from 3 volunteers...
April 26, 2017: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/28441445/impact-of-aberrant-cerebral-perfusion-on-resting-state-functional-mri-a-preliminary-investigation-of-moyamoya-disease
#16
Yituo Wang, Lubin Wang, Penggang Qiao, Fugeng Sheng, Cong Han, Enmao Ye, Yu Lei, Feng Yan, Shanshan Chen, Yuyang Zhu, Guiyun Mi, Gongjie Li, Zheng Yang
The impact of chronic cerebral hypoperfusion on resting-state blood oxygen level-dependent signal fluctuations remains unknown. We aimed to determine whether chronic ischemia induces changes in amplitude of low-frequency fluctuations (ALFF) and to investigate the correlation between ALFF and perfusion-weighted magnetic resonance imaging (PWI) parameters in patients with moyamoya disease (MMD). Thirty patients with pre- and postoperative resting-state functional magnetic resonance imaging and PWI were included, and thirty normal controls underwent resting-state functional magnetic resonance imaging...
2017: PloS One
https://www.readbyqxmd.com/read/28436815/moyamoya-in-a-patient-with-fires-a-first-case-report
#17
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
#18
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
#19
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
#20
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
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