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https://www.readbyqxmd.com/read/28182173/evaluation-of-retinal-changes-using-optical-coherence-tomography-in-a-pediatric-case-of-susac-syndrome
#1
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
#2
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
#3
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...
January 10, 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
#4
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...
December 14, 2016: 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
#5
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
#6
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
#7
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
#8
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 is 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(st) August 2010 and 1(st) August 2015...
October 27, 2016: International Journal of Neuroscience
https://www.readbyqxmd.com/read/27787385/susac-syndrome-clinical-characteristics-clinical-classification-and-long-term-prognosis
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
https://www.readbyqxmd.com/read/27402127/-diagnosis-and-treatment-of-rare-causes-of-ischaemic-stroke
#16
Daniel Kondziella
Rare causes of ischaemic stroke comprise a plethora of diagnoses of cardioembolic, inflammatory and genetic origin. The differential diagnosis is challenging but important because these disorders (e.g. monogenetic disorders such as COL4A1 mutations) often affect young individuals, they typically require sophisticated diagnostics (e.g. fluorescence angiography in Susac's syndrome) and they are increasingly treatable (e.g. enzyme replacement for Fabry's disease). This review discusses practical considerations and recent diagnostic and therapeutic advances in uncommon causes of ischaemic stroke...
June 27, 2016: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/27389624/research-registries-a-tool-to-advance-understanding-of-rare-neuro-ophthalmic-diseases
#17
Kimberly D Blankshain, Heather E Moss
BACKGROUND: Medical research registries (MRR) are organized systems used to collect, store, and analyze patient information. They are important tools for medical research with particular application to the study of rare diseases, including those seen in neuro-ophthalmic practice. EVIDENCE ACQUISITION: Evidence for this review was gathered from the writers' experiences creating a comprehensive neuro-ophthalmology registry and review of the literature. RESULTS: MRR are typically observational and prospective databases of de-identified patient information...
September 2016: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/27276498/intravitreal-triamcinolone-in-susac-syndrome
#18
Juan B Yepez, Felipe A Murati, Michele Pettito, J Fernando Arevalo
PURPOSE: To report the use of intravitreal triamcinolone for Susac Syndrome in one patient. METHODS: Case report. RESULTS: A 23-year-old woman presented with presented with sudden visual loss in the left eye with associated neurologic symptoms. Ophthalmic examination and fluorescein angiography showed multiple areas of branch retinal artery occlusion, bilaterally. Magnetic resonance imaging showed dot-like, diffusion-restricted lesions in the corpus callosum, and audiometry showed low-frequency sensory hearing loss, compatible with Susac Syndrome...
June 7, 2016: Retinal Cases & Brief Reports
https://www.readbyqxmd.com/read/27234436/susac-syndrome-with-prominent-dermatological-findings-and-a-prompt-response-to-intravenous-immunoglobulin-steroids-and-rituximab-a-case-report
#19
Elie Gertner, Michael H Rosenbloom
BACKGROUND: Susac syndrome (retinocochleocerebral vasculopathy) is an autoimmune endotheliopathy affecting the precapillary arterioles of the brain, retina, and inner ear. It presents with encephalopathy, branch retinal artery occlusions, and hearing loss. The condition is often under recognized because the clinical symptoms may present at different times and physicians may be unfamiliar with the syndrome. Peripheral findings would be helpful in early diagnosis. There are numerous treatment regimens proposed with varying effectiveness...
May 27, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27207452/susac-s-syndrome-leptomeningeal-enhancement-on-3d-flair-mri
#20
Renate Engisch, David S Titelbaum, Lara Chilver-Stainer, Frauke Kellner-Weldon
BACKGROUND: Contrast-enhanced (ce) fluid-attenuated inversion recovery magnetic resonance imaging (FLAIR MRI) has recently been shown to identify leptomeningeal pathology in multiple sclerosis. OBJECTIVE: To demonstrate leptomeningeal enhancement on three-dimensional (3D) FLAIR in a case of Susac's syndrome. METHODS: Leptomeningeal enhancement was correlated with clinical activity over 20 months and compared to retinal fluorescein angiography...
June 2016: Multiple Sclerosis: Clinical and Laboratory Research
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