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Gerardo Pedro García-García, Magdalena Martínez-Rubio, Medina-Azahara Moya-Moya, Juan José Pérez-Santonja, Julio Escribano
PURPOSE: To identify the spectrum of disease-causing CYP4V2 variants in Spanish patients with clinically diagnosed Bietti crystalline dystrophy (BCD) over an 8-year period and to analyse the phenotype-genotype correlation of the identified variants. METHODS: Four unrelated Spanish probands with a clinical diagnosis of BCD were recruited. Ophthalmological examination included visual acuity (VA), slit lamp examination, in vivo corneal confocal microscopy, funduscopy and fluoresceinic angiography...
April 25, 2018: Acta Ophthalmologica
P Brandicourt, L Bonnet, Y Béjot, C Drouet, T Moulin, L Thines
INTRODUCTION: Moya-Moya angiopathy is a neurovascular disease that predisposes to ischemic or hemorrhagic strokes. It is generated by a steno-occlusion of the terminal portion of the internal carotid arteries, which induces the development of abnormal neovessels in the deep regions of the brain. Some pathologies such as sickle cell disease, Down syndrome or Graves' disease may be associated with Moya-Moya angiopathy. These syndromic forms harbor several differences compared with idiopathic Moya-Moya disease...
March 2018: Neuro-Chirurgie
Hyacinth I Hyacinth, Courtney L Sugihara, Thomas L Spencer, David R Archer, Andy Y Shih
Stroke is a dramatic complication of sickle cell disease (SCD), which is associated with cerebral vasculopathies including moya moya, intravascular thrombi, cerebral hyperemia, and increased vessel tortuosity. The spontaneous occurrence of these pathologies in the sickle cell mouse model has not been described. Here, we studied Townes humanized sickle cell and age-matched control mice that were 13 months old. We used in vivo two-photon microscopy to assess blood flow dynamics, vascular topology, and evidence of cerebral vasculopathy...
January 1, 2017: Journal of Cerebral Blood Flow and Metabolism
Ahmed Alnemari, Tarek R Mansour, Mohamad Bazerbashi, Mark Buehler, Jason Schroeder, Daniel Gaudin
BACKGROUND: This article illustrates, summarizes, and reviews the multifaceted application of four-dimensional (4D) computed tomography angiography (CTA) in the characterization and diagnosis of various cerebrovascular and tumor-associated vascular pathologies through the presentation of clinical cases, including acute and chronic stroke, arteriovenous malformations, aneurysm, moyamoya disease, and hypervascularity associated with a tumor and its relationship to preoperative embolization...
September 2017: World Neurosurgery
Prastiya Gunawan, Wihasto Suryaningtyas, Darto Saharso, Risky Prasetyo
BACKGROUND: Moya-moya disease in children is a cerebrovascular disorder that may cause cerebral ischemic or hemorrhage. CASE DETAILS: We report an 8-year-old boy that was admitted with the chief complaint of repeated sudden half left body paralyze. MRA showed acute thrombotic infarction in the right hemisphere and internal carotid artery stenosis in the form of puffs of smokes. Indirect revascularization surgical procedure with combination of Encephalo-myo-pial-synangiosis (EMS) and Encephalo-arterio-pial-synangiosis (EAS) was performed...
March 2017: Ethiopian Journal of Health Sciences
Jonathan B Weaver, Avinash B Kumar
DESIGN: Case report. SETTING: Operating room. PATIENT: 25YF, ASA IV E who underwent an emergent decompressive craniectomy for refractory intracranial hypertension secondary to acute intracranial hemorhage. INTERVENTIONS: A 25Y caucasian female presented with acute intracranial hemorrhage with intraventricular extension secondary to Moya Moya disease. Post admisison, she underwent an emergent decompressive craniectomy for medically refractory intracranial hypertension...
February 2017: Journal of Clinical Anesthesia
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
J D Saldaña Garrido, M Martínez Rubio, R Carrión Campo, M A Moya Moya, L Rico Sergado
CLINICAL CASE: A 43-year-old woman in remission from T- cell acute lymphoblastic leukaemia was referred to our hospital with suspected leukaemic retinitis. The funduscopic examination of her left eye revealed multifocal yellow-white peripheral retinitis and retinal haemorrhage. The patient was treated for cytomegalovirus retinitis after an extended haematological investigation showed no abnormalities. Initial improvement was followed by papillitis in the left eye and motility restriction in the right eye...
March 2017: Archivos de la Sociedad Española de Oftalmología
R G Esin, Yu N Isayeva, E A Gorobets, N V Tokareva, O R Esin
Moya-moya is a rare cerebrovascular disease characterized by the progressive occlusion of cerebral vessels with partial switching off the circle of Willis and arteries that feed it. The article provides a review of literature, modern diagnostic criteria and a description of a single clinical case. The onset of the disease in this patient was characterized by headache and speech disorders.An analysis of speech disorders showed that they were systemic. They were registered at all language levels (phonetic, lexical,morphological, syntactic)...
2016: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
M Vinícius Ribeiro E Silva, F Tavares Mendonça, R Garcia Dusi
OBJECTIVE: The objective of this case report is to describe the anesthetic management in a child with moya-moya disease and sickle cell anemia provided in a tertiary hospital. CASE: A 6 year-old patient, diagnosed with moya-moya disease and sickle cell anemia, both conditions associated with a greater incidence of intracranial ischemic events, with a history of two strokes of the ischemic subtype, was submitted to general anesthesia for the execution of multiple cranial burr holes in order to produce the neovascularization in poorly perfused regions...
December 2016: Revista Española de Anestesiología y Reanimación
Anant Mehrotra, Pradeep Sharma, Kuntal Kanti Das, Kamlesh Singh Bhaisora, Jayesh Sardhara, Shruti Gupta, Satyadeo Pandey, Awadhesh Kumar Jaiswal, Rabi Narayan Sahu, Arun Kumar Srivastava, Sanjay Behari
BACKGROUND: To highlight the management nuances in addressing mirror aneurysms (MirAns) in a subset of multiple aneurysms. METHODS: A retrospective study was carried out and all multiple intracranial aneurysms (n = 70) over 9 years were identified. Exclusion criteria were associated arteriovenous malformations, moyamoya disease, and either traumatic or mycotic aneurysms or those managed by endovascular approach alone. Patients were grouped as MirAn and nonmirror aneurysms (nMirAns)...
August 2016: World Neurosurgery
O A Lvova, I V Jevneronok, L V Shalkevich, T S Prusakova
Moyamoya disease in children is a rarely diagnosed entity. Frequent headache is the first symptom of the disease. Headache associated with moya-moya disease (HAMD) is a separated entity reported in the literature. Variants of onset, a spectrum of primary diagnoses and diagnosis verification rate of moyamoya disease in 7 children are presented. A clinical case of moya-moya disease in a 9-year boy, who was primarily diagnosed with migraine with aura, is reported. The results presented by the authors as well as literature data indicate the necessity of using magnetic-resonance angiography in children with headache attacks and focal neurologic symptoms to identify this progressive vascular pathology and timely recommend a surgical treatment...
2016: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
Vince I Madai, Federico C von Samson-Himmelstjerna, Nora Sandow, Florian Weiler, Miriam Bauer, Peter Vajkoczy, Matthias Günther, Petr Dusek, Philipp Von Gottberg, Thoralf Niendorf, Jens Wuerfel, Jan Sobesky
OBJECTIVES: Time-of-flight (TOF) magnetic-resonance-angiography (MRA) identifies vessel pathology in cerebrovascular disease. At 7.0 T, the clinical performance of TOF-MRA is constrained owing to radio frequency power deposition. We studied the diagnostic value of whole-brain MPRAGE-based MRA as an alternative imaging technique in comparison to the clinical standard 3.0 T TOF-MRA. METHODS: Patients with stroke and/or moya-moya disease were included. TOF-MRA was performed at 3...
December 2015: European Journal of Radiology
Sabina Jagdevan, Kamath Sriganesh, Paritosh Pandey, Madhusudan Reddy, G S Umamaheswara Rao
BACKGROUND: Moya Moya disease (MMD) is one of the most common cerebro-vascular diseases in children resulting in stroke. Surgical revascularization aims at improving the perfusion to the 'at-risk' ischemic brain. Several factors including peri-operative anesthetic related ones, affect the outcome in these children. This study was performed with the aim to explore the role of anesthetic techniques, pharmacological agents and perioperative management strategies on the neurological outcome following an indirect revascularization procedure for the treatment of MMD...
September 2015: Neurology India
Raef Minwer-Barakat, Judit Marigó, Salvador Moyà-Solà
OBJECTIVES: The material of Necrolemur (Microchoerinae, Omomyidae, Primates) from the Middle Eocene (Robiacian) locality of Sant Jaume de Frontanyà (Eastern Pyrenees) is described. This is the first confirmable record of this genus from Spain. METHODS: A mandible fragment bearing P4 -M3 and 15 isolated teeth have been carefully described and compared with all the known species of Necrolemur (namely Necrolemur antiquus, Necrolemur zitteli and Necrolemur cf. zitteli from Egerkingen α) and with Nannopithex filholi...
December 2015: American Journal of Physical Anthropology
Moya Comer
No abstract text is available yet for this article.
January 2016: Nurse Education Today
Violeta Moya-Alvarez, Gilles Cottrell, Smaila Ouédraogo, Manfred Accrombessi, Achille Massougbodgi, Michel Cot
Background.  Pregnancy-associated malaria (PAM) remains a significant health concern in sub-Saharan Africa. Cross-sectional studies report that iron might be associated with increased malaria morbidity, raising fears that current iron supplementation policies will cause harm in the present context of increasing resistance against intermittent preventive treatment in pregnancy (IPTp). Therefore, it is necessary to assess the relation of iron levels with malaria risk during the entire pregnancy. Methods.  To investigate the association of maternal iron levels on malaria risk in the context of an IPTp clinical trial, 1005 human immunodeficiency virus-negative, pregnant Beninese women were monitored throughout their pregnancy between January 2010 and May 2011...
April 2015: Open Forum Infectious Diseases
Peter J Fagenholz, Eva Fuentes, Haytham Kaafarani, Catrina Cropano, David King, Marc de Moya, Kathryn Butler, George Velmahos, Yuchiao Chang, D Dante Yeh
BACKGROUND: Ultrasound (US) is the first-line diagnostic study for evaluating gallstone disease and is considered the test of choice for diagnosing acute cholecystitis (AC). However, computed tomography (CT) is used widely for the evaluation of abdominal pain and is often obtained as a first abdominal imaging test, particularly in cases in which typical clinical signs of AC are absent or other possible diagnoses are being considered. We hypothesized that CT is more sensitive than US for diagnosing AC...
October 2015: Surgical Infections
A Cabrera-León, V Lopez-Villaverde, M Rueda, M N Moya-Garrido
STUDY QUESTION: How does the estimated prevalence of infertility among 30- to 49-year-old women vary when using different approaches to its measurement? SUMMARY ANSWER: The prevalence of women with difficulties in conceiving differed widely according to the measurement approach adopted. WHAT IS KNOWN ALREADY: Establishing the true magnitude of infertility as a public health problem is challenging, given that it is not categorized as a disability or chronic condition and may be largely unreported...
November 2015: Human Reproduction
Mélanie Moya
Treatments for head and neck cancers are generally complex and debilitating. Surgery, often mutilating, profoundly affects the relationship between oneself and others and causes verbal communication, breathing and swallowing difficulties. The functional and aesthetic sequelae are a constant reminder to the patient of the disease and make them conscious of their appearance.
September 2015: Soins; la Revue de Référence Infirmière
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