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Moyamoya disease and syndrome

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
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
Franz Wegner, Ulf Mueller-Ladner, Florian M P Meier
September 2016: Clinical and Experimental Rheumatology
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
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)...
September 2, 2016: Neurological Sciences
Vance T Lehman, Waleed Brinjikji, David F Kallmes, John Huston, Giussepe Lanzino, Rabinstein A Alejandro, 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 sub-millimeter 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...
September 2, 2016: British Journal of Radiology
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
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
Jinlu Yu, Lei Shi, Yunbao Guo, Baofeng Xu, Kan Xu
Moyamoya disease (MMD) involves progressive occlusion of the intracranial internal carotid artery resulting in formation of moyamoya-like vessels at the base of the brain. It can be characterized by hemorrhage or ischemia. Direct vascular bypass is the main and most effective treatment of MMD. However, patients with MMD differ from those with normal cerebral vessels. MMD patients have unstable intracranial artery hemodynamics and a poor blood flow reserve; therefore, during the direct bypass of superficial temporal artery (STA)-middle cerebral artery (MCA) anastomosis, perioperative risk factors and anesthesia can affect the hemodynamics of these patients...
2016: International Journal of Medical Sciences
Alexander J Ball, Gary K Steinberg, Jorina Elbers
BACKGROUND: Moyamoya disease (MMD) is a progressive intracranial arteriopathy with high risk of stroke. Its impact on quality of life is unstudied. We surveyed children with moyamoya disease and compared their quality of life to chronically ill children and children with stroke to better understand the impact of this diagnosis. METHODS: Children with moyamoya disease aged seven to 17 years from Stanford's Moyamoya Clinic between June 2014 and March 2015 were included...
October 2016: Pediatric Neurology
Deepa John, Karthik Muthusamy, Bhavagna Bandla, Sniya Valsa Sudhakar, Maya Thomas
Moya Moya Disease (MMD) is characterised by idiopathic vasculopathy affecting the terminal internal carotid arteries resulting in the formation of extensive collaterals at the base of the brain, leptomeninges and parenchymal regions with resultant infarcts and bleeds. Four children presented with clinico-radiological features suggestive of Moyamoya disease/syndrome. This includes global developmental delay, recurrent seizures, transient ischaemic attacks and impaired vision. The first patient had vision of 6/15 in both eyes with bilateral optic disc pallor...
May 2016: Journal of Clinical and Diagnostic Research: JCDR
Wuyang Yang, Jose Luis Porras, Risheng Xu, Tomas Garzon-Muvdi, Justin M Caplan, Geoffrey P Colby, Alexander Lewis Coon, Rafael J Tamargo, Judy Huang, Edward Sanghoon Ahn
INTRODUCTION: Sickle cell disease (SCD) patients with moyamoya syndrome (MMS) represent a rare subset of patients with potentially devastating neurological outcomes. The effectiveness of surgical revascularization in this patient population is currently unclear. We aim to elucidate the effectiveness of surgical intervention in our series of SCD-MMS patients by comparing stroke recurrence in those undergoing revascularization vs conservative transfusion therapy. METHODS: We retrospectively reviewed our database of moyamoya patients at the Johns Hopkins Medical Institution from 1990 to 2013...
August 2016: Neurosurgery
Valérie Ghilain, Elsa Wiame, Edward Fomekong, Marie-Françoise Vincent, Dana Dumitriu, Marie-Cécile Nassogne
INTRODUCTION: Lysinuric protein intolerance (LPI) is a form of inherited aminoaciduria caused by a deficiency in the cationic amino acid transport process on the basolateral membrane of enterocytes and renal tubular cells. Clinical signs include gastrointestinal symptoms, failure to thrive, hepatosplenomegaly, osteoporosis, episodes of coma, intellectual deficiency, lung and renal involvement, bone marrow abnormalities, as well as altered immune response. Moyamoya disease is a cerebrovascular disorder predisposing sufferers to stroke through progressive stenosis of the intracranial internal carotid arteries and their proximal branches...
September 2016: European Journal of Paediatric Neurology: EJPN
Peter Birkeland, Kate Gardner, Rachel Kesse-Adu, John Davies, Jens Lauritsen, Frantz Rom Poulsen, Christos M Tolias, Swee Lay Thein
BACKGROUND AND PURPOSE: Intracranial aneurysms and aneurysmal subarachnoid hemorrhage may occur more frequently in sickle-cell disease (SCD), and this could be related to the sickle genotype and moyamoya syndrome seen in SCD. METHODS: Records from a total of 1002 patients with SCD attending 2 specialized adult hematologic services were retrospectively reviewed. We analyzed data of a cohort of 767 patients attending 1 SCD clinic between 2002 and 2013 and of 235 patients from the other clinic who have had neurovascular imaging between 2007 and 2014...
July 2016: Stroke; a Journal of Cerebral Circulation
Mahmud Mossa-Basha, Adam de Havenon, Kyra J Becker, Danial K Hallam, Michael R Levitt, Wendy A Cohen, Daniel S Hippe, Matthew D Alexander, David L Tirschwell, Thomas Hatsukami, Catherine Amlie-Lefond, Chun Yuan
BACKGROUND AND PURPOSE: Although studies have evaluated the differential imaging of moyamoya disease and atherosclerosis, none have investigated the added value of vessel wall magnetic resonance imaging (MRI). This study evaluates the added diagnostic value of vessel wall MRI in differentiating moyamoya disease, atherosclerotic-moyamoya syndrome (A-MMS), and vasculitic-MMS (V-MMS) with a multicontrast protocol. METHODS: We retrospectively reviewed the carotid artery territories of patients with clinically defined vasculopathies (moyamoya disease, atherosclerosis, and vasculitis) and steno-occlusive intracranial carotid disease...
July 2016: Stroke; a Journal of Cerebral Circulation
Syed S Mahmood, Anju Nohria
Cancer survivors who receive head and neck radiation are at increased risk for cerebrovascular events. This is mediated via damage to the hypothalamus-pituitary axis leading to the metabolic syndrome and extracranial arterial injury leading to carotid artery stenosis. Head and neck radiation can also lead to intracranial injury that can present as moyamoya, especially in children. Survivors require lifelong periodic follow-up for the development of pan-hypopituitarism or its individual components as well as for dyslipidemia and obesity...
July 2016: Current Treatment Options in Cardiovascular Medicine
Jong S Kim, Yeon-Jung Kim, Sung-Ho Ahn, Bum J Kim
Intracranial atherosclerosis is more prevalent in Asian patients, whereas extracranial atherosclerosis is more common in individuals from western countries. The reasons for this discrepancy remain unknown. We reviewed the relevant literature and discussed the currently available information.Although the study population, diagnostic modality, and risk factor definitions differ between studies, hypercholesterolemia is more correlated with extracranial atherosclerosis than intracranial atherosclerosis. The difference in hypercholesterolemia prevalence is one of the main reasons for racial differences...
May 4, 2016: International Journal of Stroke: Official Journal of the International Stroke Society
V Jecko, G Penchet, C Champeaux
BACKGROUND: Different surgical methods may be used for the treatment of moyamoya disease. The authors report their experience with pial synangiosis revascularization. MATERIAL AND METHODS: We reviewed the clinical, surgical as well as radiographic records of all patients who underwent cerebral revascularization surgery using pial synangiosis for a symptomatic moyamoya disease at a single institution, Bordeaux University Hospital. RESULTS: Over a 6-year period (2007-2013), 17 procedures were performed in 9 patients...
August 2016: Neuro-Chirurgie
Shusuke Yamamoto, Masaki Koh, Daina Kashiwazaki, Naoki Akioka, Naoya Kuwayama, Kyo Noguchi, Satoshi Kuroda
BACKGROUND: Quasi-moyamoya disease (MMD) or moyamoya syndrome is based on various underlying diseases and radiologically simulates MMD, but its disease entity is still unclear. Recent studies have proven specific shrinkage of the involved arteries in MMD. Using 3-dimensional constructive interference in steady state (3D-CISS), therefore, this study aimed to analyze the outer diameter of the involved arteries in quasi-MMD. METHODS: This study included 9 patients with quasi-MMD (unilateral type, n = 2; bilateral type, n = 7)...
June 2016: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
Charles K Hammond, Alexander Shapson-Coe, Rajeshree Govender, Ronald van Toorn, Alvin Ndondo, Nicky Wieselthaler, Brian Eley, Lawrence Mubaiwa, Jo M Wilmshurst
A national multicenter study identified 17 South African children with vertically acquired HIV-1 infection and HIV-associated vasculopathy. Five of the children (all indigenous African ancestry) had progressive vascular disease, consistent with moyamoya syndrome. Median presentation age 5.8 years (range 2.2-11). The children with moyamoya syndrome presented with abnormal CD4 counts and raised viral loads. Clinical features included motor deficits, neuroregression, and intellectual disability. Neuroimaging supported progressive vascular disease with preceding clinically silent disease course...
July 2016: Journal of Child Neurology
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