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Moyamoya disease and syndrome

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https://www.readbyqxmd.com/read/28859027/subarachnoid-hemorrhage-revealing-moyamoya-syndrome-in-a-patient-with-may-hegglin-anomaly
#1
Yannick Béjot, José-Luis Barnay, Adrien Chavent, Benoit Daubail, Agnès Jacquin, Apolline Kazemi, Frédéric Ricolfi, Maurice Giroud
INTRODUCTION: Moyamoya syndrome is a rare progressive cerebrovascular occlusive disease for which several associated conditions have been described. CASE REPORT: We report the case of a 76-year-old woman with a history of May-Hegglin anomaly who presented with an isolated unusual diffuse headache. Initial laboratory investigations showed only thrombocytopenia (platelet count 95000/μL). Unenhanced brain computed tomography scan revealed a small subarachnoid hemorrhage in the left frontal lobe...
September 2017: Neurologist
https://www.readbyqxmd.com/read/28826581/uncommon-causes-of-cerebral-microbleeds
#2
REVIEW
Nariman Noorbakhsh-Sabet, Varun Chandi Pulakanti, Ramin Zand
BACKGROUND: Cerebral microbleeds (CMBs) are small and round perivascular hemosiderin depositions detectable by gradient echo sequences or susceptibility-weighted imaging. Cerebral microbleeds are common among patients with hypertension, cerebral ischemia, or cerebral amyloid angiopathy. In this article, we describe uncommon causes of CMBs. METHODS: We searched Pubmed with the keyword CMBs for relevant studies and looked for different uncommon causes of CMBs. RESULTS: CMBs have several uncommon etiologies including posterior reversible encephalopathy syndrome, infective endocarditis, brain radiation therapy, cocaine abuse, thrombotic thrombocytopenic purpura, traumatic brain injury, intravascular lymphomatosis or proliferating angio-endotheliomatosis, moyamoya disease, sickle cell anemia/β-thalassemia, cerebral autosomal dominant arteriopathy subcortical infarcts, and leukoencephalopathy (CADASIL), genetic syndromes, or obstructive sleep apnea...
August 17, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28814167/cardio-cephalic-neural-crest-syndrome-a-novel-hypothesis-of-vascular-neurocristopathy
#3
M Komiyama
A novel hypothesis proposes that "cardio-cephalic neural crest (NC) syndrome," i.e. cephalic NC including cardiac NC, contributes to the concurrent occurrence of vascular diseases in the cardio- and cerebrovascular regions. NC is a transient structure present in early embryogenesis. Cephalic NC provides mesenchymal cells to the vascular media in these regions. Concurrent cardio- and cerebrovascular lesions have been reported in PHACE syndrome, ACTA2 mutation syndrome, and less frequently in the spontaneous occlusion of the circle of Willis (so-called moyamoya disease)...
January 1, 2017: Interventional Neuroradiology
https://www.readbyqxmd.com/read/28756377/thrombolytic-therapy-for-the-treatment-of-acute-ischaemic-stroke-in-adults-with-homozygous-sickle-cell-disease
#4
Loubna Majhadi, David Calvet, Charlotte Rosso, Pablo Bartolucci
Stroke is a significant cause of morbidity and mortality in patients with homozygous sickle cell disease (SCD). A specific large-vessel vasculopathy is often responsible for both haemorrhagic and ischaemic strokes in patients with SCD. Although intravenous thrombolysis has been considered as a therapeutic option for acute ischaemic strokes in SCD, its use remains debated because of an increased risk of spontaneous intracranial haemorrhage reported in this disease. This risk of haemorrhage is mainly supported by the presence of a Moyamoya syndrome often associated with the specific vasculopathy in patients with homozygous SCD...
July 28, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28715924/moyamoya-disease-in-children-results-from-the-international-pediatric-stroke-study
#5
Sarah Lee, Michael J Rivkin, Adam Kirton, Gabrielle deVeber, Jorina Elbers
This study aimed to describe children with moyamoya disease from an international multicenter stroke database, and explore risk factors for stroke recurrence. We reviewed data of children >28-days old with moyamoya disease enrolled in the International Pediatric Stroke Study from January 2003 to March 2013. A total of 174 children from 32 sites and 14 countries had moyamoya disease; median age 7.4 years, 49% male. Of these, 90% presented with ischemic stroke, 7.5% with transient ischemic attack, and 2.5% with hemorrhagic stroke...
January 1, 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/28686127/effectiveness-of-surgical-revascularization-for-stroke-prevention-in-pediatric-patients-with-sickle-cell-disease-and-moyamoya-syndrome
#6
COMPARATIVE STUDY
Wuyang Yang, Risheng Xu, Jose L Porras, Clifford M Takemoto, Syed Khalid, Tomas Garzon-Muvdi, Justin M Caplan, Geoffrey P Colby, Alexander L Coon, Rafael J Tamargo, Judy Huang, Edward S Ahn
OBJECTIVE Sickle cell disease (SCD) in combination with moyamoya syndrome (MMS) represents a rare complication of SCD, with potentially devastating neurological outcomes. The effectiveness of surgical revascularization in this patient population is currently unclear. The authors' aim was to determine the effectiveness of surgical intervention in their series of SCD-MMS patients by comparing stroke recurrence in those undergoing revascularization and those undergoing conservative transfusion therapy. METHODS The authors performed a retrospective chart review of patients with MMS who were seen at the Johns Hopkins Medical Institution between 1990 and 2013...
September 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28664017/coexistence-of-quasi-moyamoya-disease-and-poems-syndrome-in-a-patient-with-intracranial-hemorrhage-a-case-report-and-literature-review
#7
Izumi Yamaguchi, Junichiro Satomi, Nobuaki Yamamoto, Shotaro Yoshioka, Yoshiteru Tada, Kenji Yagi, Yasuhisa Kanematsu, Shinji Nagahiro
POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare paraneoplastic syndrome elicited by plasma cell dyscrasia. Its clinical manifestations are multiple and stroke is not a recognized feature. A 44-year-old woman with a 3-month history of bilateral lower extremity sensorimotor disturbance was admitted to our hospital. Examinations revealed polyneuropathy, organomegaly, hypothyroidism, monoclonal gammopathy, pelvic plasmacytoma, and elevated serum vascular endothelial growth factor (VEGF) levels...
January 2017: NMC Case Rep J
https://www.readbyqxmd.com/read/28647711/down-syndrome-and-moyamoya-disease-unusual-cause-of-stroke
#8
Carlos Tavares Bello, Catarina Barreiros, Inês Gil, Carlos Vasconcelos
Down syndrome is a frequent clinical entity, being considered one of the most frequent chromosomal aberrations. It is characterised by a typical clinical phenotype and is associated with a heterogeneous group of organ and system-specific abnormalities. The cardiovascular system is commonly affected and if so, it may be associated with an increased morbidity and mortality. Cerebrovascular events in patients with Down syndrome are multifactorial, being possibly related to congenital heart disease, vascular malformations and traditional cardiovascular risk factors...
June 24, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28634314/-a-case-of-moyamoya-disease-associated-with-sapho-syndrome
#9
Nobutaka Horie, Mayuko Baba, Ken Kawada, Yuuki Matsunaga, Eisaku Sadakata, Yoichi Morofuji, Tsuyoshi Izumo, Minoru Morikawa, Takeo Anda, Takayuki Matsuo
Moyamoya disease is a unique occlusive disease of the internal carotid artery(ICA)with moyamoya vessels that can lead to transient ischemic attacks and hemorrhagic stroke. When other inherited or acquired disorders and conditions occur in conjunction with moyamoya disease, the syndrome is known as quasi-moyamoya disease. We report the case of a 34-year-old woman with a past history of SAPHO(Synovitis-Acne-Pustulosis-Hyperostosis-Osteomyelitis)syndrome, who presented with arm weakness and headache. Magnetic resonance angiography revealed severe terminal stenosis of the left ICA with moyamoya vessels, and she was diagnosed as moyamoya disease associated with SAPHO syndrome...
June 2017: No Shinkei Geka. Neurological Surgery
https://www.readbyqxmd.com/read/28620564/moyamoya-syndrome-in-a-child-with-neurofibromatosis-type-1-magnetic-resonance-imaging-as-a-tool-for-clinical-decision-making
#10
Jonathan Mayl, Hanisha Patel, Tushar Chandra
Moyamoya syndrome is a rare cerebrovasculopathy of unknown etiology which is associated with multiple risk factors. Moyamoya was first discovered in Japan and is reported to have an increased prevalence in the Japanese population. The term "Moyamoya" translates into "puff of smoke" and is named after the finding of the collateral cerebral vasculature that develops secondary to occlusion of an internal carotid artery at the entrance into the circle of Willis. This collateral vasculature characterizes the disease...
May 9, 2017: Curēus
https://www.readbyqxmd.com/read/28616936/encephaloduroateriosynangiosis-edas-in-the-management-of-moyamoya-syndrome-in-children-with-sickle-cell-disease
#11
Alexander Alamri, Pennylouise Hever, Jebet Cheserem, Catia Gradil, Sanj Bassi, Christos M Tolias
BACKGROUND: Encephalo-duro-arterio-synangiosis (EDAS) in Moyamoya syndrome (MMS) treatment has been well described in the literature, however in MMS caused by sickle cell anaemia (SCA), EDAS use remains controversial with poor long-term follow-up. We present a case-series of SCA patients who have undergone EDAS for SCA-related MMS and describe their post-operative course as well as provide a literature review of the role of EDAS in the treatment of sickle cell anaemia. METHODS: A retrospective review of all the paediatric EDAS procedures conducted in our institution for SCA from 2007 to 2015...
June 15, 2017: British Journal of Neurosurgery
https://www.readbyqxmd.com/read/28566764/difference-in-cerebral-circulation-time-between-subtypes-of-moyamoya-disease-and-moyamoya-syndrome
#12
Kaijiang Kang, Jingjing Lu, Dong Zhang, Youxiang Li, Dandan Wang, Peng Liu, Bohong Li, Yi Ju, Xingquan Zhao
In this study, we evaluated the differences in hemodynamics between hemorrhagic and non-hemorrhagic moyamoya disease (MMD) and moyamoya syndrome (MMS) by measuring cerebral circulation time (CCT). This case-control study included 136 patients with MMD or MMS diagnosed between April 2015 and July 2016 at Beijing Tian Tan Hospital. Each hemisphere was analyzed separately. The difference in clinical, radiological characteristics and CCT between subtypes of MMD and MMS were analyzed statistically. The results showed that total CCT between hemorrhagic and non-hemorrhagic sides was not statistically different (16...
May 31, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28549641/treatment-of-moyamoya-disease-and-unruptured-intracranial-aneurysm-in-floating-harbor-syndrome
#13
Daniel J Coughlin, Charles A Miller, A Jesse Schuette
BACKGROUND: This is the first report of the successful treatment of moyamoya disease and an unruptured intracranial aneurysm in a patient with Floating-Harbor syndrome. CASE DESCRIPTION: A 35-year-old, phenotypically syndromic woman presented with signs and symptoms consistent with ischemic stroke. Magnetic resonance imaging and catheter angiography confirmed diagnosis of moyamoya and a 6-mm basilar apex artery aneurysm (BAA). She underwent right-sided craniotomy for direct and indirect revascularization by means of superficial temporal artery-middle cerebral artery bypass and encephalo-duro-arterio-synangiosis...
August 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28532265/encephaloduroarteriosynangiosis-edas-in-young-patients-with-cerebrovascular-complications-of-sickle-cell-disease-single-institution-experience
#14
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.  Encephaloduroarteriosynangiosis (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
#15
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...
September 15, 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
#16
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/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
#17
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/28475226/molecular-cytogenetic-characterization-of-five-f8-complex-rearrangements-utility-for-haemophilia-a-genetic-counselling
#18
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/28436815/moyamoya-in-a-patient-with-fires-a-first-case-report
#19
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
#20
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
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