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https://www.readbyqxmd.com/read/28933135/headache-in-a-young-woman-do-not-forget-susac-s-syndrome
#1
A Kefi, F Said, T Ben Salem, M Khedher, M Khanfir, I Ben Ghorbel, M Lamloum, M H Houman
Susac syndrome is a rare disease characterized by the clinical triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. This underdiagnosed condition needs to be considered in the differential diagnosis of a broad variety of disorders. An early diagnosis is important as treatment can halt disease progression and prevent permanent disability. Herein, we report a case of Susac syndrome in a 31-year-old woman and we highlight how challenging an early diagnosis was and the importance of an aggressive therapeutic approach, including the combination of steroids and other cytotoxic drugs...
September 21, 2017: Reumatismo
https://www.readbyqxmd.com/read/28922012/neuropsychological-outcome-of-a-case-of-susac-syndrome-a-two-year-follow-up-study
#2
Andrew W Barritt, Mirdhu Wickremaratchi, Stuart J Anderson
Susac syndrome is a rare condition characterised by the clinical triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. Of the few published cases, there is variability with regard to cognitive outcome. We describe the clinical course and neuropsychological performance of a 21-year-old male patient presenting with severe encephalopathy and later developing the full triad fulfilling the diagnosis of Susac syndrome.
September 18, 2017: Applied Neuropsychology. Adult
https://www.readbyqxmd.com/read/28899478/clinically-diagnosed-susac-syndrome-in-a-50-year-old
#3
Francesca M Brett, Seamus Looby, Conor Fearon, Peter Widdess-Walsh
No abstract text is available yet for this article.
September 7, 2017: Clinical Neuropathology
https://www.readbyqxmd.com/read/28893337/optimization-of-three-dimensional-3d-chemical-imaging-by-soft-x-ray-spectro-tomography-using-a-compressed-sensing-algorithm
#4
Juan Wu, Mirna Lerotic, Sean Collins, Rowan Leary, Zineb Saghi, Paul Midgley, Slava Berejnov, Darija Susac, Juergen Stumper, Gurvinder Singh, Adam P Hitchcock
Soft X-ray spectro-tomography provides three-dimensional (3D) chemical mapping based on natural X-ray absorption properties. Since radiation damage is intrinsic to X-ray absorption, it is important to find ways to maximize signal within a given dose. For tomography, using the smallest number of tilt series images that gives a faithful reconstruction is one such method. Compressed sensing (CS) methods have relatively recently been applied to tomographic reconstruction algorithms, providing faithful 3D reconstructions with a much smaller number of projection images than when conventional reconstruction methods are used...
September 12, 2017: Microscopy and Microanalysis
https://www.readbyqxmd.com/read/28885997/susac-syndrome-the-first-case-report-in-peru
#5
Carolina Rivadeneira-Sotelo, María Meza Vega, Darwin Segura-Chávez, Sheila Castro-Suarez
Susac Syndrome is a rare entity, characterized by a triad of subacute encephalopathy, retinal artery occlusion and sensorineural hearing loss. It is more common in women and the age of onset fluctuates between 9-58 years of age. The pathogenesis is presented as microangiopathic changes at the cerebral, retinal and cochlear levels associated with an autoimmune mechanism. We present the case of a 31-year-old woman who started with a diffuse headache, puerile behavior, bradylalia and somnolence. As the disease progressed, she had auditory deficit and arterial obstruction of the right temporal retinal branch in retinal fluorescein angiography...
August 30, 2017: Medwave
https://www.readbyqxmd.com/read/28879939/a-report-of-third-case-of-susac-syndrome-from-india
#6
Kamble J Harsha, Jeevan S Nair
No abstract text is available yet for this article.
September 2017: Neurology India
https://www.readbyqxmd.com/read/28819045/fundus-fluorescein-angiography-in-susac-s-syndrome
#7
EDITORIAL
Gavin L Reynolds, Kevin Talbot, Srilakshmi M Sharma
No abstract text is available yet for this article.
August 17, 2017: Practical Neurology
https://www.readbyqxmd.com/read/28730399/optical-coherence-tomography-angiography-findings-in-susac-s-syndrome-a-case-report
#8
Rodolfo Mastropasqua, Lisa Toto, Alfonso Senatore, Alessandro D'Uffizi, Piergiorgio Neri, Cesare Mariotti, Marica Tina Maccarone, Luca Di Antonio
PURPOSE: To report clinical features of Susac's syndrome (SS) using optical coherence tomography angiography (OCTA). METHODS: Case report. RESULTS: A 25-year-old Caucasian female with a history of non-pulsatile migraine, tinnitus, and verbal apraxia complaining of peripheral scotoma in left eye due to branch retinal arteriole occlusion (BRAO) was diagnosed as having SS after audiometric test, brain magnetic resonance imaging, and multimodal retinal imaging...
July 20, 2017: International Ophthalmology
https://www.readbyqxmd.com/read/28725290/the-discovery-of-the-flammer-syndrome-a-historical-and-personal-perspective
#9
REVIEW
Josef Flammer, Katarzyna Konieczka
This review describes the clinical and basic research that led to the description of Flammer syndrome. It is narrated from a personal perspective. This research was initiated by the observation of an increased long-term fluctuation of visual fields in a subgroup of glaucoma patients. As these patients had strikingly cold hands, peripheral blood flow was tested with a capillary microscopy, and vasospastic syndrome (VS) was diagnosed. Further studies on these patients revealed frequently weakened autoregulation of ocular blood flow and increased flow resistivity in retroocular vessels...
June 2017: EPMA Journal
https://www.readbyqxmd.com/read/28705845/urodynamic-findings-in-susac-syndrome-first-reported-filling-cystometrogram
#10
Tatenda Calvin Nzenza, Nathan Hoag, Derek Barrry Hennessey, Johan Gani
Susac syndrome is a rare neurological disease, with only 300 cases reported in the literature. Lower urinary symptoms are not an uncommon feature of the disease, yet there is no information on specific dysfunction typical urodynamic findings associated with the disease. We present what we believe to be the first reported filling cystometrogram study of Susac syndrome for the evaluation of voiding dysfunction.
July 13, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28644112/susac-syndrome-misdiagnosed-as-multiple-sclerosis-with-exacerbation-by-interferon-beta-therapy
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
REVIEW
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
#20
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
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