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https://www.readbyqxmd.com/read/28644112/susac-syndrome-misdiagnosed-as-multiple-sclerosis-with-exacerbation-by-interferon-beta-therapy
#1
Hussein Algahtani, Bader Shirah, Muhammad Amin, Eyad Altarazi, Hashem Almarzouki
Susac syndrome is a rare autoimmune disorder characterised by the clinical triad of encephalopathy, retinopathy (branch retinal artery occlusions) and hearing loss. The diagnosis of Susac syndrome may be difficult initially, and it is not uncommon for patients with Susac syndrome to be misdiagnosed with multiple sclerosis. In this case report, we describe a patient who was diagnosed as having multiple sclerosis for three years, with further deterioration after starting treatment with interferon beta-1a. The patient had the triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss...
January 1, 2017: Neuroradiology Journal
https://www.readbyqxmd.com/read/28641772/susac-syndrome-a-differential-diagnosis-of-white-matter-lesions
#2
Mariano Marrodan, Jorge Correale, Lucas Alessandro, Mariela Amaya, Maria Eugenia Fracaro, Alejandro Alfredo Köhler, Marcela Fiol
INTRODUCTION: Susac Syndrome is an autoimmune endotheliopathy affecting capillaries and precapillary arterioles of the brain, inner ear and retina. The classic symptom triad includes visual disturbances, hypoacusia, and encephalopathy, but is rarely fully manifest at onset. The syndrome typically follows an active fluctuating monophasic course. Typical imaging findings on brain magnetic resonance show central fiber microischemias/microinfarctions of the corpus callosum known as "snow balls", as well as lineal infarcts and upper callosal fiber involvement...
July 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28613358/oct-angiography-assessment-of-retinal-ischemia-in-susac-s-syndrome
#3
Karina Spiess, Jesús Ramón García Martínez
Descriptive study of two patients affected by Susac's syndrome (SuS) focusing on the chronic retinal tissue damage assessed by optical coherence tomography (OCT) and OCT angiography (OCTA). To the authors' knowledge, this is the first case series reporting OCTA in SuS. OCTA imaging revealed secondary hypoxia due to branch retinal artery occlusion of the deep capillary plexus, with additional involvement of the superficial capillary network in one case. OCTA is a noninvasive and quick diagnostic tool for detecting hypoxic retinal damage in SuS...
June 1, 2017: Ophthalmic Surgery, Lasers & Imaging Retina
https://www.readbyqxmd.com/read/28494240/cooperating-commensals-restore-colonization-resistance-to-vancomycin-resistant-enterococcus-faecium
#4
Silvia Caballero, Sohn Kim, Rebecca A Carter, Ingrid M Leiner, Bože Sušac, Liza Miller, Grace J Kim, Lilan Ling, Eric G Pamer
Antibiotic-mediated microbiota destruction and the consequent loss of colonization resistance can result in intestinal domination with vancomycin-resistant Enterococcus (VRE), leading to bloodstream infection in hospitalized patients. Clearance of VRE remains a challenging goal that, if achieved, would reduce systemic VRE infections and patient-to-patient transmission. Although obligate anaerobic commensal bacteria have been associated with colonization resistance to VRE, the specific bacterial species involved remain undefined...
May 10, 2017: Cell Host & Microbe
https://www.readbyqxmd.com/read/28368694/autoimmune-mediated-psychosis-a-case-of-susac-syndrome-in-a-drug-user
#5
Pablo Barrio, Mercè Balcells, Delón La Puma, Carles Gaig
OBJECTIVE: Susac syndrome, a rare disorder, is thought to be mediated by autoantibodies. One of the potential targets of these autoantibodies could be an antigen in the microvessels of the brain, the retina, and the inner ear leading to central nervous system (CNS) alterations, visual disturbances, and hearing deficits. Our aim is to expand clinicians' diagnostic options when facing psychosis due to medical conditions. METHODS: A case report was conducted for this study...
April 2017: Journal of Dual Diagnosis
https://www.readbyqxmd.com/read/28182173/evaluation-of-retinal-changes-using-optical-coherence-tomography-in-a-pediatric-case-of-susac-syndrome
#6
Mehmet Kola, Hidayet Erdöl, Sevil Ertuğrul Atasoy, Adem Türk
Susac syndrome is a rare occlusive vasculopathy affecting the retina, inner ear and brain. The cause is unknown, although it generally affects young women. This syndrome can be difficult to diagnose because its signs can only be revealed by detailed examination. These signs are not always concomitant, but may appear at different times. This report describes a pediatric case who was diagnosed with Susac syndrome when retinal lesions were identified in the inactive period with the help of optical coherence tomography (OCT)...
January 2017: Turkish Journal of Ophthalmology
https://www.readbyqxmd.com/read/28103199/diagnostic-criteria-for-susac-syndrome
#7
REVIEW
Ilka Kleffner, Jan Dörr, Marius Ringelstein, Catharina C Gross, Yvonne Böckenfeld, Wolfram Schwindt, Benedikt Sundermann, Hubertus Lohmann, Heike Wersching, Julia Promesberger, Natascha von Königsmarck, Anne Alex, Rainer Guthoff, Catharina J M Frijns, L Jaap Kappelle, Sven Jarius, Brigitte Wildemann, Orhan Aktas, Friedemann Paul, Heinz Wiendl, Thomas Duning
BACKGROUND: Susac syndrome is characterised by the triad of encephalopathy with or without focal neurological signs, branch retinal artery occlusions and hearing loss. Establishment of the diagnosis is often delayed because the triad is complete only in a minority of patients at disease onset. This leads to a critical delay in the initiation of appropriate treatment. Our objective was to establish criteria for diagnosis of either definite or probable Susac syndrome. METHOD: The establishment of diagnostic criteria was based on the following three steps: (1) Definition of a reference group of 32 patients with an unambiguous diagnosis of Susac syndrome as assessed by all interdisciplinary experts of the European Susac Consortium (EuSaC) team (EuSaC cohort); (2) selection of diagnostic criteria, based on common clinical and paraclinical findings in the EuSaC cohort and on a review of the literature; and (3) validation of the proposed criteria in the previously published cohort of all Susac cases reported until 2012...
December 2016: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28094021/neuropsychological-characteristics-of-encephalopathy-in-susac-s-syndrome-case-report
#8
Magdalena Roessler-Górecka, Tadeusz Mendel, Justyna Wiśniowska, Joanna Seniów
Susac's Syndrome (SS) is a rare, autoimmune angiopathy characterized by hearing loss, retinal artery occlusions and encephalopathy, which is usually expressed in multifocal neurological signs and symptoms, confusion state and cognitive impairment. There have been few descriptions of neuropsychological assessment of SS. We present a case study of 29-year-old woman who developed full SS. During the post-acute stage of disease, she was admitted to neurorehabilitation ward to improve her cognitive-behavioral and motor functioning...
March 2017: Neurologia i Neurochirurgia Polska
https://www.readbyqxmd.com/read/27984352/short-follow-up-bias-confounds-estimates-of-the-typical-clinical-course-of-susac-syndrome
#9
Ivana Vodopivec, Sashank Prasad
BACKGROUND: To evaluate the validity of the prevailing concept that Susac syndrome (SS), a rare microvasculopathy of the brain, retina, and inner ear, is a self-limiting disease. METHODS: We performed a literature search to identify all cases of SS reported between 1973 and October 2015. If available, we determined their demographics, duration of follow-up, and the clinical course that was labeled as monocyclic or polycyclic. We attempted to determine the number of relapses and the relapse rate in patients with polycyclic disease...
June 2017: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/27853020/progressive-susac-syndrome-with-bilateral-visual-loss-and-disability
#10
Morteza Entezari, Saeed Karimi, Mohammadali Feizi
Susac syndrome (SS) is a rare retinal-cochlear-cerebral disease with an unclear etiology. A 35-year-old man presented with sudden painless vision loss in the right eye and 2 months later in the left eye with hemiparesis, behavioral changes, and hearing loss. Ophthalmic examinations revealed multiple branch retinal artery occlusions (BRAOs) in both eyes. Brain magnetic resonance imaging showed inflammatory changes with multiple "punched-out" lesions in the corpus callosum which confirmed the diagnosis of SS...
September 2016: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/27828899/ultra-wide-field-fundus-imaging-in-the-diagnosis-and-follow-up-of-susac-syndrome
#11
Panagiotis Salvanos, Morten C Moe, Tor P Utheim, Ragnheiur Bragadóttir, Emilia Kerty
PURPOSE: To present the use of ultra-wide-field (UW) fundus imaging in the diagnosis and follow-up of a patient with Susac syndrome. METHODS: Case report of a myopic patient presenting initially with rhegmatogenous retinal detachment. A significant portion of the retina was found to be avascular bilaterally at presentation. Surgery was performed with scleral buckle. Then, UW color and autofluorescent imaging and UW fluorescein angiography were obtained. RESULTS: Successful retinal reattachment was obtained...
November 8, 2016: Retinal Cases & Brief Reports
https://www.readbyqxmd.com/read/27793972/mid-term-mechanical-circulatory-support-comparison-of-single-centre-data-with-the-euromacs-registry
#12
Katarzyna Zuk, Brigitta Gahl, Martina Susac, Juliane Vierecke, Theo M M H de By, Stephan Windecker, Lars Englberger, Thierry Carrel, Roland Hetzer, Jan Gummert, Paul Mohacsi
OBJECTIVES: Mechanical circulatory support (MCS) is an established therapy for end-stage heart failure. The EUROMACS registry was created to promote research in patients with MCS and became a committee of the European Association for Cardio-Thoracic Surgery (EACTS) in 2014. Since 1 January 2011, increasing numbers of European centres implanting durable MCS have reported their patient data to EUROMACS. The aim of this study is to compare, as an example of internal quality control, data from a single centre (Bern) with those from the EUROMACS database with respect to mortality rates and preoperative patient characteristics and to describe complications in Bern...
January 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/27788613/susac-s-syndrome-clinical-course-and-epidemiology-in-a-central-european-population
#13
Thomas Seifert-Held, Beate J Langner-Wegscheider, Martina Komposch, Philipp Simschitz, Claudia Franta, Barbara Teuchner, Hans Offenbacher, Ferdinand Otto, Johann Sellner, Helmut Rauschka, Franz Fazekas
OBJECTIVE: Susac's syndrome is characterized by inflammation and occlusion of pre-capillary arterioles with the clinical triad of branch retinal artery occlusion (BRAO), encephalopathy and hearing loss. No epidemiological data are available for the disease. METHODS: All neurology departments in Austria were addressed to report adult patients who were on immunosuppressive treatment for a diagnosis of Susac's syndrome between 1 August 2010 and 1 August 2015. Clinical course, treatment regimens, period and point prevalence rates, and annual incidence of Susac's syndrome in Austria in people over 19 years of age are reported...
November 11, 2016: International Journal of Neuroscience
https://www.readbyqxmd.com/read/27787385/susac-syndrome-clinical-characteristics-clinical-classification-and-long-term-prognosis
#14
Vicktoria Vishnevskia-Dai, Joav Chapman, Roee Sheinfeld, Tal Sharon, Ruth Huna-Baron, Riri S Manor, Yehuda Shoenfeld, Ofira Zloto
Susac syndrome is a rare condition characterized by the clinical triad of central nervous system (CNS) dysfunction, sensorineural hearing impairment, and branch retinal artery occlusion (BRAO). The purpose of this study is to examine the demographics, clinical characteristics, treatment, and long-term prognosis of Susac syndrome. The data recorded for all Susac syndrome patients treated at the Sheba Medical Center between 1998 and 2014 included demographics, clinical signs at presentation and during the disease course, imaging findings, treatment, and prognosis...
October 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27781269/migraine-like-headache-as-presentation-symptom-in-susac-syndrome
#15
Fernando Kowacs, Matheus Ferreira Gomes, Taiane Pigozzo, André Luiz Rodrigues Palmeira, Camila Muratt Carpenedo, Marlise Castro Ribeiro
No abstract text is available yet for this article.
November 2016: Headache
https://www.readbyqxmd.com/read/27618323/white-matter-diseases-with-radiologic-pathologic-correlation
#16
Nicolae Sarbu, Robert Y Shih, Robert V Jones, Iren Horkayne-Szakaly, Laura Oleaga, James G Smirniotopoulos
White matter diseases include a wide spectrum of disorders that have in common impairment of normal myelination, either by secondary destruction of previously myelinated structures (demyelinating processes) or by primary abnormalities of myelin formation (dysmyelinating processes). The pathogenesis of many white matter diseases remains poorly understood. Demyelinating disorders are the object of this review and will be further divided into autoimmune, infectious, vascular, and toxic-metabolic processes. Autoimmune processes include multiple sclerosis and related diseases: tumefactive demyelinating lesions, Balo concentric sclerosis, Marburg and Schilder variants, neuromyelitis optica (Devic disease), acute disseminated encephalomyelitis, and acute hemorrhagic leukoencephalopathy (Hurst disease)...
September 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/27484611/lc-ms-profiling-of-glucosinolates-in-the-seeds-of-brassica-elongata-ehrh-and-of-the-two-stenoendemic-b-botteri-vis-and-b-cazzae-ginzb-teyber
#17
Sabine Montaut, Ivica Blažević, Mirko Ruščić, Patrick Rollin
The glucosinolates (GLs) present in seed extracts of Brassica elongata Ehrh., B. botteri Vis and B. cazzae Ginzb. & Teyber from Croatia were identified by LC-MS. 4-Hydroxyindol-3-ylmethyl GL (3) was the major GL in the seeds of B. elongata, along with the four minor GLs 2-(R)-hydroxy-3-butenyl- (1), 3-butenyl- (2), 4-pentenyl- (4) and indol-3-ylmethyl (5). The seeds of B. botteri (Vis island) and B. cazzae (Sušac island) contained 2 as the major GL as well as 1, 3, 5 and 4-methoxyindol-3-ylmethyl GL (6). However, the GLs in B...
January 2017: Natural Product Research
https://www.readbyqxmd.com/read/27475050/is-susac-syndrome-associated-with-bipolar-disorder
#18
Clea Staelens, Kurt Audenaert, Hannelore Tandt, Veroniek Van Driessche, Gilbert Lemmens
No abstract text is available yet for this article.
October 2016: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/27460737/neurological-otolaryngological-and-ophthalmological-implications-of-susac-syndrome-a-case-report
#19
B Grygiel-Górniak, M Puszczewicz, E Czaplicka
Susac syndrome is an endotheliopathy affecting the arterioles of the brain, retina, and inner ear. Many cases of Susac syndrome are underdiagnosed, mainly at the early stages of the disease, while prompt diagnosis enables a speedy recovery. Immediate treatment can halt disease progression and even prevent future disability. We report a case of Susac syndrome, describe the difficulties in the diagnosis of this case, and include a detailed history of a 35-year-old man via the presentation of extensive laboratory work-up and imaging studies...
July 2016: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/27430448/visual-impairment
#20
REVIEW
Carl Ellenberger
This chapter can guide the use of imaging in the evaluation of common visual syndromes: transient visual disturbance, including migraine and amaurosis fugax; acute optic neuropathy complicating multiple sclerosis, neuromyelitis optica spectrum disorder, Leber hereditary optic neuropathy, and Susac syndrome; papilledema and pseudotumor cerebri syndrome; cerebral disturbances of vision, including posterior cerebral arterial occlusion, posterior reversible encephalopathy, hemianopia after anterior temporal lobe resection, posterior cortical atrophy, and conversion blindness...
2016: Handbook of Clinical Neurology
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