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Moyamoya disease and syndrome

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https://www.readbyqxmd.com/read/28070115/proposal-for-a-prospective-registry-for-moyamoya-disease-in-japan
#1
Ken Kazumata, Masaki Ito, Haruto Uchino, Hiroshi Nishihara, Kiyohiro Houkin
The number of clinical research papers published worldwide on moyamoya disease (MMD) has increased recently. However, the majority of the literature comprises retrospective single-center studies collecting data on small numbers of patients. Several multi-center studies are ongoing in Japan; however, the current data are insufficient for comprehensively outlining the various characteristics of MMD. To enhance our knowledge on epidemiologic, vascular, and genetic aspects of MMD, a prospective multicenter registry will be established in Japan that will help to streamline clinical research as well as improve clinical treatments and long-term outcomes...
January 6, 2017: Neurologia Medico-chirurgica
https://www.readbyqxmd.com/read/28033272/intraoperative-dexmedetomidine-and-postoperative-cerebral-hyperperfusion-syndrome-in-patients-who-underwent-superficial-temporal-artery-middle-cerebral-artery-anastomosis-for-moyamoya-disease-a-retrospective-observational-study
#2
Hyungseok Seo, Ho-Geol Ryu, Je Do Son, Jeong-Soo Kim, Eun Jin Ha, Jeong-Eun Kim, Hee-Pyoung Park
Dexmedetomidine, a selective α2-agonist, reduces cerebral blood flow and has neuroprotective effects against cerebral ischemia/reperfusion injury in experimental animals. We examined whether intraoperative dexmedetomidine would reduce the incidence of postoperative cerebral hyperperfusion syndrome (CHS) after superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis in patients with moyamoya disease.The electronic medical records of 117 moyamoya patients who underwent STA-MCA anastomosis were reviewed retrospectively...
December 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27960175/moyamoya-disease
#3
Miki Fujimura, Oh Young Bang, Jong S Kim
Moyamoya disease (MMD) is a chronic occlusive cerebrovascular disease characterized by progressive stenosis at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain. Although its etiology is unknown, recent genetic studies have identified RNF213 in the 17q25-ter region as an important susceptibility gene of MMD among East Asian populations. A c.14576G>A polymorphism in RNF213 was identified in 95% of MMD patients with a family history and in 79% of sporadic cases, and patients carrying this polymorphism exhibited significantly earlier disease onset and a more-severe form of MMD...
2016: Frontiers of Neurology and Neuroscience
https://www.readbyqxmd.com/read/27960158/risk-factors
#4
T Uehara, O Y Bang, J S Kim, K Minematsu, R Sacco
Studies investigating risk factors for intracranial atherosclerosis (ICAS) have been infrequent. However, due to recent availability of non-invasive vascular imaging techniques that can assess intracranial cerebral arteries, there are a growing number of studies on risk factors for ICAS. Conventional vascular risk factors such as hypertension, diabetes, hypercholesterolemia and cigarette smoking are risk factors for ICAS. However, it remains uncertain whether there is a difference in risk factors between ICAS and extracranial atherosclerosis (ECAS)...
2016: Frontiers of Neurology and Neuroscience
https://www.readbyqxmd.com/read/27934593/establishment-of-induced-pluripotent-stem-cell-ipsc-line-from-an-8-year-old-female-patient-with-ischemic-moyamoya-disease
#5
Marina Cardano, Fabio Marsoner, Jacopo Zasso, Matteo Marcatili, Thodoris Karnavas, Luigi Andrea Lanterna, Luciano Conti
Peripheral blood mononuclear cells (PBMCs) were collected from an 8-year old female patient affected by ischemic Moyamoya disease (MMD). Patient's PBMCs were reprogrammed using Sendai virus particles delivering the four Yamanaka factors. The footprint free hiPSC line expressed the major pluripotency markers and exhibited a normal karyotype. Cells were competent to give rise to progeny of differentiated cells belonging to the 3 germ layers. This hiPSC line represents a good tool to in vitro model MMD in order to shed light on the cellular and molecular mechanisms responsible for the occurrence of this syndrome...
November 5, 2016: Stem Cell Research
https://www.readbyqxmd.com/read/27876659/surgical-treatment-for-patients-with-moyamoya-syndrome-and-type-1-neurofibromatosis
#6
Jose L Porras, Wuyang Yang, Tomas Garzon-Muvdi, Risheng Xu, Jaishri Blakeley, Allan Belzberg, Justin M Caplan, Syed Khalid, Geoffrey P Colby, Alexander L Coon, Rafael J Tamargo, Edward S Ahn, Judy Huang
INTRODUCTION: The current study describes the impact of surgery in preventing follow-up ipsilateral TIAs/strokes in an East Coast North American cohort of patients with both moyamoya syndrome (MMS) and neurofibromatosis type 1 (NF1) (MMS-NF1). METHODS: We retrospectively reviewed records of patients with MMS and NF1 at the Johns Hopkins Medical Institutions from 1990-2014. Baseline characteristics and follow-up results including subsequent ipsilateral strokes were collected, and compared between a revascularization group (group1) and a conservatively managed group (group2) on a per-hemisphere basis...
November 19, 2016: World Neurosurgery
https://www.readbyqxmd.com/read/27857839/fatal-outcome-in-a-hispanic-woman-with-moyamoya-syndrome-and-graves-disease
#7
Julian Choi, Perin Suthakar, Farbod Farmand
: We describe the case of a young Hispanic female who presented with thyrotoxicosis with seizures and ischemic stroke. She was diagnosed with a rare vasculopathy - moyamoya syndrome. After starting antithyroid therapy, her neurologic symptoms did not improve. Acute neurosurgical intervention had relieved her symptoms in the immediate post-operative period after re-anastomosis surgery. However, 2 post-operative days later, she was found to be in status epilepticus and in hyperthyroid state...
2016: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/27853035/dystonia-myoclonus-and-encephalopathy-in-a-single-patient-a-rare-association-of-moyamoya
#8
Ujjawal Roy, Urmila Das, Ajay Panwar, Prabhat Kumar Lal
Moyamoya disease (MMD) is a rare neurovascular disorder which pathologically, is a chronic cerebrovasculopathy. It is characterized by stenosis of the internal carotid artery (ICA) and the main branches within the circle of Willis with consecutive development of collateral vessels. There are a few cases in the literature which have described movement disorders as a manifestation of MMD; however these have been uncommonly reported in cases of moyamoya syndrome (MMS). We present a 10-year-old boy with dystonia, myoclonus and encephalopathy like features...
October 2016: Annals of African Medicine
https://www.readbyqxmd.com/read/27843469/moyamoya-syndrome-associated-with-henoch-sch%C3%A3-nlein-purpura
#9
Reza Shiari, Seyed Mohamad Hossein Tabatabaei Nodushan, Mohamad Mahdi Mohebbi, Parvaneh Karimzadeh, Mohsen Javadzadeh
Some reports have shown the association between Moyamoya syndrome and autoimmune diseases. Herewith, we present a 3.5 yr old girl with Henoch- Schönleinpurpura (HSP) who was treated with steroids because of sever colicky abdominal pain. However, central nervous system manifestations such as headache, ataxia and vision impairment developed during 6 months of her outpatient follow-up. More evaluation using MRA revealed intracranial stenosis of internal carotid artery and arterial collaterals that were in favor of Moyamoya syndrome...
2016: Iranian Journal of Child Neurology
https://www.readbyqxmd.com/read/27836652/intracranial-vessel-wall-imaging-for-evaluation-of-steno-occlusive-diseases-and-intracranial-aneurysms
#10
REVIEW
Waleed Brinjikji, Mahmud Mossa-Basha, John Huston, Alejandro A Rabinstein, Giuseppe Lanzino, Vance T Lehman
Cerebrovascular diseases have traditionally been classified, diagnosed and managed based on their luminal characteristics. However, over the past several years, several advancements in MRI techniques have ushered in high-resolution vessel wall imaging (HR-VWI), enabling evaluation of intracranial vessel wall pathology. These advancements now allow us to differentiate diseases which have a common angiographic appearance but vastly different natural histories (i.e. moyamoya versus atherosclerosis, reversible cerebral vasoconstriction syndrome versus vasculitis, stable versus unstable intracranial aneurysms)...
November 8, 2016: Journal of Neuroradiology. Journal de Neuroradiologie
https://www.readbyqxmd.com/read/27790125/a-case-of-turner-syndrome-with-multiple-embolic-infarcts
#11
Cindy W Yoon, Eungseok Lee, Byung-Nam Yoon, Hee-Kwon Park, Joung-Ho Rha
Only a few cases of Turner syndrome (TS) with ischemic stroke have been reported. Various arteriopathies of the cerebral arteries, including fibromuscular dysplasia, congenital hypoplasia, moyamoya syndrome, and premature atherosclerosis have been assumed to be the cause of ischemic stroke in TS. There has been no case report of a TS patient presenting with an embolic stroke pattern without any cerebral arteriopathy. A 28-year-old woman with TS was referred to our hospital because of abnormal brain magnetic resonance imaging (MRI) findings...
September 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27787485/moyamoya-vasculopathy-shows-a-genetic-mutational-gradient-decreasing-from-east-to-west
#12
Alessandro Raso, Roberto Biassoni, Samantha Mascelli, Paolo Nozza, Elisabetta Ugolotti, Eddi DI Marco, Patrizia DE Marco, Elisa Merello, Armando Cama, Marco Pavanello, Valeria Capra
BACKGROUND: Moyamoya disease (MMD) is a chronic, occlusive cerebrovascular disease characterized by bilateral steno- occlusive changes at the terminal portion of the internal carotid arteries and an abnormal vascular network at the base of the brain determining stroke in children. Patients with a similar vasculopathy and associated conditions are affected by the moyamoya syndrome (MMS). Most of the studies focused on MMD were carried out on East-Asian population. Ring Finger 213 (RNF213) has been identified as the strongest susceptibility gene for MMD in East-Asian people...
October 27, 2016: Journal of Neurosurgical Sciences
https://www.readbyqxmd.com/read/27693197/multiparametric-imaging-of-brain-hemodynamics-and-function-using-gas-inhalation-mri
#13
Peiying Liu, Babu G Welch, Yang Li, Hong Gu, Darlene King, Yihong Yang, Marco Pinho, Hanzhang Lu
Diagnosis and treatment monitoring of cerebrovascular diseases routinely require hemodynamic imaging of the brain. Current methods either only provide part of the desired information or require the injection of multiple exogenous agents. In this study, we developed a multiparametric imaging scheme for the imaging of brain hemodynamics and function using gas-inhalation MRI. The proposed technique uses a single MRI scan to provide simultaneous measurements of baseline venous cerebral blood volume (vCBV), cerebrovascular reactivity (CVR), bolus arrival time (BAT), and resting-state functional connectivity (fcMRI)...
September 28, 2016: NeuroImage
https://www.readbyqxmd.com/read/27637639/universal-bypass-for-treatment-of-symptomatic-moyamoya-disease-or-moyamoya-syndrome-analysis-of-a-personal-case-series-on-behalf-of-the-italian-moyamoya-association
#14
Luigi A Lanterna, Carlo Brembilla, Paolo Gritti, Claudio Bernucci
BACKGROUND: Moyamoya (MM) is a very rare cerebrovascular disease, particularly in Caucasians. We describe the results of an Italian case series where the mainstay of treatment was a bypass or a combined approach. METHODS: An analysis of a prospectively collected database was carried out. The main objective was to investigate (1) the risk of perioperative stroke and surgical complications, (2) the risk of new ischemic events, and (3) the risk of new hemorrhages at follow-up (mean follow-up: 2...
2016: Acta Neurochirurgica. Supplement
https://www.readbyqxmd.com/read/27606722/moyamoya-disease-and-systemic-sclerosis-mosys-syndrome-a-combination-of-two-rare-entities
#15
LETTER
Franz Wegner, Ulf Mueller-Ladner, Florian M P Meier
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September 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27592246/moyamoya-syndrome-causing-stroke-in-young-women-with-type-1-diabetes
#16
Jing W Hughes, Jennifer A Wyckoff, Abby S Hollander, Colin P Derdeyn, Janet B McGill
CONTEXT: Moyamoya syndrome is an idiopathic brain vasculopathy characterized by stenosis of major intracranial arteries. It often presents in patients with type 1 diabetes or thyroid disease and may have an autoimmune etiology. Moyamoya-related stroke poses a diagnostic challenge as initial symptoms and deficits vary greatly from classic ischemic stroke to encephalopathy, psychiatric, or seizure disorder. CASE DESCRIPTION: We report 4 patients with type 1 diabetes and other autoimmune diseases who developed moyamoya-related stroke at a young age...
November 2016: Journal of Diabetes and its Complications
https://www.readbyqxmd.com/read/27590482/hemodynamic-contribution-of-transdural-collateral-flow-in-adult-patients-with-moyamoya-disease
#17
Ji Man Hong, Yoon Hee Hong, Seong-Joon Lee, Sung Eun Lee, Jin Soo Lee, Dong Hoon Shin
To evaluate the hemodynamic contributions of collateral flow in adult patients with moyamoya disease, neurological deterioration or fluctuation during admission, Suzuki grade, various collateral routes, lesion volume, cerebral blood flow (CBF), and their associations were analyzed. Thirty patients (60 cerebral hemispheres, mean age 45 ± 25 years, and 73.3 % female) who were diagnosed with moyamoya disease or syndrome were enrolled over 3 years. Moyamoya stages from each hemisphere were stratified according to the Suzuki's criteria through six-vessel angiography into internal carotid arteries (ICAs), external carotid arteries (ECAs), and vertebral arteries (VAs)...
December 2016: Neurological Sciences
https://www.readbyqxmd.com/read/27585640/clinical-interpretation-of-high-resolution-vessel-wall-mri-of-intracranial-arterial-diseases
#18
Vance T Lehman, Waleed Brinjikji, David F Kallmes, John Huston, Giuseppe Lanzino, Alejandro A Rabinstein, Ashima Makol, Mahmud Mossa-Bosha
Intracranial arterial pathology has traditionally been evaluated with luminal imaging. Recently, high-resolution vessel wall imaging (HR-VWI) with MRI has facilitated submillimetre evaluation of the arterial walls. This technique can help differentiate various causes of intracranial steno-occlusive disease, identify culprit atherosclerotic plaques with a recent cerebral infarct, locate vessel wall pathology in areas with minimal or no narrowing on luminal imaging, predict aneurysm stability and identify a ruptured aneurysm when multiple aneurysms are present...
November 2016: British Journal of Radiology
https://www.readbyqxmd.com/read/27576769/more-precise-imaging-analysis-and-diagnosis-of-moyamoya-disease-and-moyamoya-syndrome-using-high-resolution-magnetic-resonance-imaging
#19
Dong Zhang, Le-Bao Yu, Huan He, Ji-Zong Zhao, Rong Wang, Qian Zhang, Zhi-Yong Shi, Jun-Shi Shao
No abstract text is available yet for this article.
August 27, 2016: World Neurosurgery
https://www.readbyqxmd.com/read/27570403/-venous-congestion-as-a-cause-of-subcortical-white-matter-t2-hypointensity-on-magnetic-resonance-images
#20
Jayaprakash Harsha Kamble, Krishnan Parameswaran
Subcortical T2 hypointensity is an uncommon finding seen in very limited conditions such as multiple sclerosis, Sturge-Weber syndrome, and meningitis. Some of the conditions such as moyamoya disease, severe ischemic-anoxic insults, early cortical ischemia, and infarcts are of "arterial origin." We describe two conditions in which "venous congestion" plays a major role in T2 hypointensity - cerebral venous sinus thrombosis (CVST) and dural arteriovenous fistula (dAVF). The third case is a case of meningitis, showing T2 hypointensity as well, and can be explained by the "venous congestion" hypothesis...
July 2016: Annals of Indian Academy of Neurology
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