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Moyamoya disease and syndrome

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https://www.readbyqxmd.com/read/29138020/moyamoya-syndrome-in-a-pediatric-patient-with-congenital-human-immunodeficiency-virus-type-1-infection-resulting-in-intracranial-hemorrhage
#1
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
https://www.readbyqxmd.com/read/29028653/adams-oliver-syndrome-with-moyamoya-disease-for-cerebral-revascularisation-surgery
#2
(no author information available yet)
No abstract text is available yet for this article.
October 12, 2017: Journal of Neurosurgical Anesthesiology
https://www.readbyqxmd.com/read/28989169/moyamoya-disease-in-an-18-month-old-female-caucasian-complicated-by-cerebral-hyperperfusion-syndrome-following-indirect-revascularization
#3
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
https://www.readbyqxmd.com/read/28970816/direct-visualization-of-arterial-emboli-in-moyamoya-syndrome
#4
Julie G Shulman, Samuel Snider, Henri Vaitkevicius, Viken L Babikian, Nirav J Patel
BACKGROUND: Hemodynamic insufficiency is often considered the cause of ischemic stroke in patients with moyamoya syndrome. While high-intensity transient signals (HITS) on transcranial Doppler (TCD) have been reported in this population, the relationship between these signals and ischemic symptoms is not clearly established. Accordingly, current treatment is directed at improving perfusion. CLINICAL PRESENTATION: We present two patients with symptoms of cerebral ischemia and angiographic findings of moyamoya syndrome...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28962888/nonsyndromic-peripheral-pulmonary-artery-stenosis-is-associated-with-homozygosity-of-rnf213-p-arg4810lys-regardless-of-co-occurrence-of-moyamoya-disease
#5
Sung-A Chang, Ju Sun Song, Taek Kyu Park, Jeong Hoon Yang, Woo Chan Kwon, So Ree Kim, Sung Mok Kim, Jihoon Cha, Shin Yi Jang, Young Seok Cho, Tae Jung Kim, Oh Young Bang, Jin Young Song, Chang-Seok Ki, Duk-Kyung Kim
BACKGROUND: Peripheral pulmonary arterial stenosis (PPAS) in childhood is frequently associated with other syndromes; however, PPAS in adolescents and adults is rare and its etiology is not well understood. We report the clinical characteristics of adult-onset nonsyndromic PPAS associated with the p.Arg4810Lys variant of the RNF213 gene. METHODS: We recently encountered an index case of severe pulmonary hypertension with multiple PPAS and intra- and extracranial arteriopathy...
September 28, 2017: Chest
https://www.readbyqxmd.com/read/28859027/subarachnoid-hemorrhage-revealing-moyamoya-syndrome-in-a-patient-with-may-hegglin-anomaly
#6
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
#7
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...
October 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
#8
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)...
December 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
#9
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
#10
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...
October 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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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