Garcia-Carrasco M, Mendoza-Pinto C, Cervera R. Diagnosis and classification of Susac syndrome. Epub 2014 Apr 12. WebOverview What is Susac syndrome? Careers, Unable to load your collection due to an error. Patients were diagnosed with ocular Susacs syndrome if they have met the retinal involvement criterion, as was published by the EuSaC team, i.e., BRAOs or AWH in FA or characteristic signs of branch retinal ischemia in funduscopy or OCT [ 7 ]. Spinal muscular atrophy with lower extremity predominance (SMALED), This page was last edited on 11 August 2022, at 11:21. Ocular manifestations at baseline are described in Table2. OCT angiography: assessment of retinal ischemia in Susacs syndrome. Patient no.4 showed decreased vascular perfusion of both superficial and deep plexuses in the area of the occluded macular arteriole (Fig. Am J Clin Pathol. A minority of patients with SS present with the typical triad. J Neuroophthalmol. This review focuses on recent developments in the diagnosis and management of the condition. It is also still unknown whether they have a pathogenic role in the disease or if they are secondary to the diseases endothelial damage. Dinah Zur, Michaella Goldstein, Barequet, D. et al. Immunosuppressive and immunomodulation therapies seem to be highly effective in the control of disease activity. 2014;3:1106. The presence of AWH is the second angiographic sign for ocular disease activity [7] and was found in all our patients during the study period. In patients with some of the typical features of Susac, who do not fulfill even two of the main organs, the diagnosis should be considered as possible and these patients need careful and frequent follow-up since the diagnosis could evolve in the future. It is recommended to use a FA montage image or wide-field FA in order to allow a thorough assessment of the peripheral retina. WebSusac syndrome is an autoimmune condition that affects the very small blood vessels in A high index of suspicion is needed to make the diagnosis promptly. Unlike other symptoms and deficits, hearing loss in SS is often irreversible, with frequent need for hearing devices or cochlear implants.9,24,25, Diagnosis of SS is based on the clinical presentation and findings from retinal fluorescein angiography (FA), brain magnetic resonance imaging (MRI) and audiometry. We believe that this noveal ocular manifestation may shed a light on disease pathogenesis. https://www.ncbi.nlm.nih.gov/pubmed/29341996, Heng LZ, Bailey C, Lee R, Dick A, Ross A. Patients were diagnosed with ocular Susacs syndrome if they have met the retinal involvement criterion, as was published by the EuSaC team, i.e., BRAOs or AWH in FA or characteristic signs of branch retinal ischemia in funduscopy or OCT [7]. Patient no. CD8+ T cell-mediated endotheliopathy is a targetable mechanism of neuro-inflammation in Susac syndrome. Enroll in databases to allow researchers from participating institutions to find you. WebSusac syndrome (SS) classically presents with the clinical triad of vision loss, hearing loss, and encephalopathy ( 1,2 ), which is caused by vasculo-occlusive disease affecting the retina, inner ear, and brain. A, Color fundus photograph revealing an Notably, none of these eyes had signs of new BRAOs. Regardless of the favourable prognosis, recurrences of the disease have been reported, some several years after the initial diagnosis and others in association with pregnancy.9,36 Lifelong monitoring for disease recurrence is required for these patients. Lavinsky L, Scarton F, Lavinsky-Wolff M, Lavinsky J, Motta LH. 2009;5:6838. J Neuroophthalmol. A study of experts of the European Susac Consortium (EuSaC) team [7], describes established criteria for diagnosis of either definite or probable Susac syndrome. Partial vision loss is often present and caused by branch retinal artery occlusions. The combined ophthalmic and systemic findings raised the suspicion of Susac syndrome. FA montage composite pictures were evaluated (HRA; Heidelberg Engineering, Heidelberg, Germany or FF450; Carl Zeiss Meditec Inc, Jena, Germany). Diagnostic criteria for Susac syndrome. Accessibility Susacs syndrome consists of the clinical triad of encephalopathy, branch Susac syndrome and multifocal motor neuropathy first manifesting in pregnancy, Susacs syndromepathogenesis, clinical variants and treatment approaches. Boukouvala S, Jacob S, Lane M, Denniston AK, Burdon MA. Susac syndrome affects women more than men. Careers. 3,4,7) showed a scotoma within the 10 central degrees (Fig. Provided by the Springer Nature SharedIt content-sharing initiative, Eye (Eye) Susac syndrome: clinical characteristics and treatment in 29 new cases. Web5 Oftalvisc, Valencia, Spain. Susac syndrome is a relatively rare disorder characterized by Patients no. official website and that any information you provide is encrypted Probable Susac defined patients with an unequivocal clinical and/or paraclinical involvement of two of the three main organs. https://www.ncbi.nlm.nih.gov/pubmed/30707148, https://www.ncbi.nlm.nih.gov/pubmed/12654364, https://www.ncbi.nlm.nih.gov/pubmed/20880549, https://www.ncbi.nlm.nih.gov/pubmed/29341996, https://www.ncbi.nlm.nih.gov/pubmed/30703404, https://www.ncbi.nlm.nih.gov/pubmed/18046231, https://www.ncbi.nlm.nih.gov/pubmed/28730399, https://www.ncbi.nlm.nih.gov/pubmed/22221557, https://www.ncbi.nlm.nih.gov/pubmed/29319463, https://www.karger.com/DOI/10.1159/000493489. There is also a pathological similarity between the endotheliopathy in Susac's syndrome with that seen in juvenile dermatomyositis. Recurrence of Susac syndrome (retinocochleocerebral vasculopathy) after remission of 18 years. In comparison, patients with MS and ADEM typically have lesions involving the undersurface of the corpus callosum. In the meantime, to ensure continued support, we are displaying the site without styles Diagnostic criteria for Susac syndrome | Journal of Neurology All had a bilateral ocular disease. 2) during the follow up. Macular OCT scans were evaluated for (1) areas of hyperreflective thickening of retinal nerve fiber layer to the outer plexiform layer which is indicative for tissue swelling due to acute BRAO, and (2) areas of thinning of these layers indicative for previous ischemic damage. One patient presented with extremely severe CNS disease, 3 patients presented with severe CNS disease and the rest had mild-moderated CNS involvement. SS is frequently misdiagnosed and probably underdiagnosed. 2016;36:36674. American Academy of Ophthalmology Susac's syndrome - A new ocular finding and disease outcome Susac Syndrome Eye 36, 781788 (2022). J Ophthalmic Inflamm Infect. Susac Syndrome: A differential diagnosis of white matter lesions. A review and update on the ophthalmic implications of Susac syndrome. Multimodal Imaging in Susac Syndrome: A Case Report and Literature Review. Lian K, Siripurapu R, Yeung R, Hopyan J, Eng K, Aviv RI, Symons SP. official website and that any information you provide is encrypted Aggressive and early immunosuppressive treatment is the modality of choice in all patients with studies showing good results with recovery or stabilisation of disease. Susacs syndrome, encephalopathy, retinal occlusions, hearing loss, Characteristics of Susac syndrome: a review of all reported cases. 1 The Susac Syndrome Consultation Service and Department of Pediatric Rheumatology, Cleveland Clinic, Cleveland, OH, USA. 2 Department of Neurology, Miller 12235 School of Medicine, University of Miami , Miami, FL, USA. 3 Department of Ophthalmology, Cole Eye Institute, Cleveland Clinic, Cleveland, OH, USA. Three patients (no. Correspondence to He had a 2-month history of hypoacusis, disorientation, memory loss, and behavioral changes. Most of our patients (71%) presented with definite Susacs syndrome. Curr Eye Res. An official website of the United States government. Susacs syndrome consists of the clinical triad of encephalopathy, branch retinal artery (BRAO) occlusion and sensorineural hearing loss. Ophthalmic manifestations may be the sole presenting sign of SS. Susac Susac syndrome is an autoimmune condition that affects the very small blood vessels in the brain, retina, and inner ear (cochlea). Six patients (no. Roeser MM, Driscoll CL, Shallop JK, Gifford RH, Kasperbauer JL, Gluth MB. Use of intravitreal corticosteroids has been reported to lead to resolution of a patients symptoms.33. The .gov means its official. Jarius S, Neumayer B, Wandinger KP, Hartmann M, Wildemann B. Anti-endothelial serum antibodies in a patient with Susacs syndrome, Clinical, paraclinical and serological findings in Susac syndrome: an international multicenter study, Diagnostic criteria and treatment algorithm for susac syndrome. Comprehensive literature review The typical triad of encephalopathy, sensorineural hearing loss and multiple recurrent BRAO is seldom seen at presentation in SS. There are two main types of clinical studies: People participate in clinical trials for a variety of reasons. MeSH Funduscopy of the left eye revealed arteriolar narrowing and rarefaction. Currently GARD aims to provide the following information for this disease: Cephalgia, or pain sensed in various parts of the head, not confined to the area of distribution of any nerve. FA findings included extra-macular BRAO in all eyes, macular BRAO in 3 eyes of 3 patients (3/14, 21.4%; no. Susac syndrome: clinical characteristics, clinical classification, and long-term prognosis. 1 showed signs of acute macular BRAO on OCT which was performed 11 months after initial presentation, patient no.2 had no abnormal findings, patient no.3 who had acute macular BRAO at presentation developed bilateral sectorial macular retinal thinning, patient no.4 showed bilateral sectorial macular retinal thinning on OCT 2 month post initial presentation, patient no.6 had no abnormal findings, and patient no. Encephalopathy, visual disturbance and hearing loss-recognizing the symptoms of Susac syndrome. This may account for the low prevalence of the illness. We demonstrate a new ocular finding, which was not previously reported, the presence of retinal microaneurysms in most of the eyes. Many collaborate with medical experts and researchers.Services of patient organizations differ, but may include: Clinical studies are part of clinical research and at the heart of all medical advances, including rare diseases. Cureus. Author(s) (or their employer(s)) 2020. Participants with a disease may participate to help others, but also to possibly receive the newest treatment and additional care from clinical study staff. Disclaimer. Mastropasqua R, Toto L, Senatore A, D'Uffizi A, Neri P, Mariotti C, Maccarone MT, Di Antonio L. Int Ophthalmol. Our study identifies a new retinal finding and evaluates disease outcome. Bagaglia SA, Passani F, Oliverio GW, Inferrera L, Menna F, Meduri A, Mazzotta C. Int J Environ Res Public Health. Susac Syndrome - Treatment and Symptoms - Autoimmune Sisters Susac Syndrome is a rare autoimmune disease in which the immune system attacks the smallest blood vessels in the brain, retina It is caused by a microangiopathy affecting the arterioles of the brain, retina, and cochlea, giving the classic clinical triad of subacute encephalopathy, visual loss secondary to retinal branch occlusions, and sensorineural hearing loss. They may offer online and in-person resources to help people live well with their disease. National Library of Medicine 2, 4, 5). https://www.ncbi.nlm.nih.gov/pubmed/28730399, Mateen FJ, Zubkov AY, Muralidharan R, Fugate JE, Rodriguez FJ, Winters JL, et al. Fluorescein angiography showed delayed perfusion in both eyes, leakage of dye in several branch retinal arteries, and no perfusion distally, suggesting branch retinal artery occlusions (BRAO). In other published case-series, definite Susacs syndrome was found in 13100% of the patients (Drr et al. government site. Epub 2014 Nov 15. 2, 3, 4, 6, 7). Accessibility HHS Vulnerability Disclosure, Help Both patients underwent fluorescein retinal angiography that demonstrated multifocal retinal artery occlusions without evidence of embolic disease. sharing sensitive information, make sure youre on a federal J Neuroinflammation. Patient no.6 had normal visual fields in both eyes. WebEyes: A dark area in your visual field The feeling of a dark curtain drawn over your eyes Loss of peripheral vision Inner Ear: Hearing loss Tinnitus Dizziness and Imbalance How is Susac Syndrome Diagnosed? 3, 4, 7), AWH in 6 eyes of 4 patients (6/14, 42.9%; no. ClinicalTrials.gov, an affiliate of NIH, provides current information on clinical research studies in the United States and abroad. Am J Ophthalmol. doi: 10.7759/cureus.27352. During follow-up, we observed new ocular findings: new extra-macular BRAO in 11 eyes of all 7 patients (11/14, 78.6%), new macular BRAO in 2 eyes of 2 patients (2/14, 14.3%; no.1 and 7), new Gass plaques in 5 eyes of 3 patients (5/14, 35.7%; no. 3 and 7). Please contact GARD if you need help finding additional information or resources on rare diseases, including clinical studies. The site is secure. Susac syndrome - American Academy of Ophthalmology The mean age at presentation was 34.1 years (range 2044). These authors contributed equally: Dinah Zur, Michaella Goldstein. Intravenous immunoglobulins are also a mainstay of treatment, used with concomitant corticosteroids, and has shown great efficacy in acute and prolonged disease.34 Other agents that can be used include mycophenolate mofetil, rituximab, cyclophosphamide, and azathioprine.7,12 Different agents can be combined, in order to achieve better results or tolerability. The cause is unknown but it is theorized that antibodies are produced against endothelial cells in tiny arteries which leads to damage and the symptoms related to the illness. https://www.ncbi.nlm.nih.gov/pubmed/30703404, Martinet N, Fardeau C, Adam R, Bodaghi B, Papo T, Piette J-C, et al. CNS involvement was characterized by both clinical and radiological evidence [7]. These residual "holes" (and sometimes, "spokes") develop as the acute callosal changes resolve. Google Scholar, Egan RA. The mean logMAR at presentation and at final visit were compared. reported the presence of arterio-arterial collaterals as a newly discovered ophthalmological finding in SS. No commercial re-use. This is a rare disease of unknown origin, most likely an autoimmune endotheliopathy, causing an arteriolar microangiopathy of the brain, cochlea, and retina. Susac syndrome tends to affect young women, but it can also occur in men. It manifests as a clinical triad of subacute encephalopathy, hypoacusis, and visual loss caused by multiple BRAO. Macular OCT at final visit was performed in all but one patient (no.5) and showed bilateral thinning of retinal layers in 4 patients (no. Retinal arterial wall plaques in Susac syndrome. In a case report by Azevedo et al., OCTA showed superficial and deep retinal vascular non-perfusion without chorio-capillary vasculature changes in a SS patient. Susac's syndrome: an update - PubMed Overall, neurological and auditory results were good; all patients achieved neurological improvement or stability and all but one patient had stable or improved thresholds on audiometry. 2009;285:25961. Susac Syndrome https://www.ncbi.nlm.nih.gov/pubmed/25877981, Brandt AU, Oberwahrenbrock T, Costello F, Fielden M, Gertz K, Kleffner I, et al. 2020 Oct 30;10(1):27. doi: 10.1186/s12348-020-00217-z. Susac syndrome--a report of cochlear implantation and review of otologic manifestations in twenty-three patients. Cogan's Syndrome CAS An Introduction to Susac Syndrome 2 and no.6. 2018: 17474930177517371747493017751737. The occurrence of only two manifestations of the triad suggests the diagnosis of incomplete SS.8, The main differential diagnosis of SS includes multiple sclerosis (MS) and acute disseminated encephalomyelitis.26, The white matter of the brain is most frequently affected, but the leptomeninges, grey matter, cerebellum and thalamus can also be affected. The PubMed wordmark and PubMed logo are registered trademarks of the U.S. Department of Health and Human Services (HHS). the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Patients typically present with low frequency hearing loss detectable via an audiogram. The authors report no conflict of interest. Bookshelf https://www.ncbi.nlm.nih.gov/pubmed/27276498, Drr J, Radbruch H, Bock M, Wuerfel J, Brggemann A, Wandinger KP, et al. Our Information Specialists are available to you by phone or by filling out our contact form. While encephalopathy and visual disorders can resolve or remit with treatment, hearing loss is usually permanent.9,11 Delays in diagnosis and treatment can lead to important sequelae, with up to 50% of patients developing cognitive impairment in such circumstances.4,28. Further studies are needed to establish the impact of such antibodies in the pathogenesis and diagnosis of the condition. Fluorescein angiography may demonstrate leakage in areas remote from the retinal infarctions. The areas affected on OCTA corresponded to points of low sensitivity on microperimetry.31 OCT and OCTA may show benefit in monitoring disease activity and in differentiating SS from other ophthalmological or neurological entities, such as MS.20,30, Audiometric data is useful at documenting the sensorineural hearing loss and usually demonstrate loss of low and middle frequencies.24. Clinical studies are medical research involving people as participants. volume36,pages 781788 (2022)Cite this article. Six out of 7 patients had additional treatments: Cyclophosphamide (n=4), mycophenolate mofetil (n=2), intravenous immunoglobulins (n=3), azathioprine (n=1). 2011;136:90312. These agents should be considered in the presence of pro-coagulant risk factors.4,34 Plasma exchange can be used as an adjuvant or alternative therapy in corticosteroid-resistant patients.35, Specific treatment for complications or sequelae include cochlear implants and hearing aids for hearing loss and hyperbaric oxygen therapy for retinal occlusions.25,34, Therapy should be monitored with clinical evaluation and regular MRIs and retinal FAs. J Neurol Neurosurg Psychiatry. We suggest that this may shed a light on disease pathogenesis. Researchers from participating institutions use the database to search for and invite patients or healthy volunteers who meet their study criteria to participate. In some cases, subjects can become confused. All 27 patients had corpus callosum lesions. A Novel Quantitative Assessment Method of Disease Activity in Susac's Syndrome Based on Ultra-Wide Field Imaging. It had been initially termed small infarctions of cochlear, retinal, and encephalic tissues (SICRET) syndrome [ 1 2] or retinopathy, encephalopathy, and This is a rare disease of unknown origin, most likely an autoimmune endotheliopathy, causing an arteriolar microangiopathy of the brain, cochlea, and retina. government site. Symptoms may start to appearat any time in life. Susacs syndrome clinical manifestations may vary between patients and may resemble other neurological, ophthalmic and auditory diseases. Problems with memory, senses, or mood may also occur. Susac Syndrome: Symptoms, Causes, Tests & Treatment Vodopivec I, Venna N, Rizzo JF 3rd, Prasad S. Clinical features, diagnostic findings, and treatment of Susac syndrome: a case series. Audiometry revealed complete deafness of the right ear and neurosensory hypoacusis of the left ear. In conclusion, the data provided in the current study highlights a new FA finding, the presence of retinal microaneurysms. Gass plaques were found in 4 eyes of 3 patients (4/14, 28.6%; patients no. Color fundus photography was performed using a conventional fundus photography camera (FF450; Carl Zeiss Meditec Inc, Jena, Germany). Medical treatments were recorded at the first examination, during follow-up and at the last visit. It indicates disease activity and can be considered pathognomonic for retinal involvement in SS.11,12,20 Changes in the vasculature in SS are limited to the retinal vessels with the choroidal vessels being spared. Four eyes of 3 patients (4/14, 28.6%; no. The cause of Susac syndrome is still unknown. However, VA remained stable in these eyes due to partial foveal sparing. 2015;85:6108. FOIA Seven patients (14 eyes) with a mean age of 34.1 years were included. It manifests as a clinical triad of subacute encephalopathy, hypoacusis, and visual loss caused by multiple BRAO. Superficial (D) and deep (E) vessel plexus show reduced vessel density in the area of the BRAO. inflammation; macula; retina. https://www.ncbi.nlm.nih.gov/pubmed/28613358, Vishnevskia-Dai V, Chapman J, Sheinfeld R, Sharon T, Huna-Baron R, Manor RS, et al. Available at:https://www.ncbi.nlm.nih.gov/pubmed/30707148, Egan RA, Ha Nguyen T, Gass JDM, Rizzo JF 3rd, Tivnan J, Susac JO. At presentation, 13 out of 14 eyes had good VA (>20/40 Snellen equivalent) (78.6%, 11/14 eyes had BCVA of 20/20) and one eye had moderate VA. At final visit all eyes had good VA (20/20 in 6 eyes). Retinal arterio-arterial collaterals in susac syndrome. 6 Sydney Hospital and Sydney Eye Hospital, Sydney, New Susacs syndrome is a rare immune-mediated endotheliopathy that mainly affects young women. All had bilateral ocular disease. The condition is characterized by three main symptoms: brain disease (encephalopathy), hearing loss, and vision loss. To find the right clinical study we recommend you: ResearchMatch helps connect people interested in research studieswith researchers from top medical centers across the United States. https://www.ncbi.nlm.nih.gov/pubmed/19643446, Magro CM, Poe JC, Lubow M, Susac JO. Epub 2019 Jan 29. OCT was normal in both eyes of patients no. In addition, we hypothesize that damage to endothelial cells, may be by anti-endothelial cell antibodies which was shown to exist in a subset of patients with definite Susacs syndrome [5], may cause secondary damage to the pericytes that wrap around the endothelial cells. Disease Overview Although pars planitis is generally benign, there can be significant vision loss in extreme cases. Seven patients (14 eyes) with a follow-up time of more than 12 months were included in the study. Department of Ophthalmology, Hospital Pedro Hispano, Matosinhos, Portugal. ISSN 0950-222X (print), Susacs syndrome A new ocular finding and disease outcome, https://doi.org/10.1038/s41433-021-01464-7, https://www.ncbi.nlm.nih.gov/pubmed/8164809, https://www.ncbi.nlm.nih.gov/pubmed/29933288, https://doi.org/10.1038/s41467-019-13593-5, https://www.ncbi.nlm.nih.gov/pubmed/24606999, https://www.ncbi.nlm.nih.gov/pubmed/19643446, https://www.ncbi.nlm.nih.gov/pubmed/22095376, https://www.ncbi.nlm.nih.gov/pubmed/28103199, https://www.ncbi.nlm.nih.gov/pubmed/25877981, https://www.ncbi.nlm.nih.gov/pubmed/26200513, https://www.ncbi.nlm.nih.gov/pubmed/18325301, https://www.ncbi.nlm.nih.gov/pubmed/26203089, https://www.ncbi.nlm.nih.gov/pubmed/27828899, https://www.ncbi.nlm.nih.gov/pubmed/11097286, https://www.ncbi.nlm.nih.gov/pubmed/28613358, https://www.ncbi.nlm.nih.gov/pubmed/27787385, https://www.ncbi.nlm.nih.gov/pubmed/27276498, https://www.ncbi.nlm.nih.gov/pubmed/19953118, https://www.ncbi.nlm.nih.gov/pubmed/23628737. https://www.ncbi.nlm.nih.gov/pubmed/8164809, Article 1, 2, 3, 4, 5), new CNP areas in 11 eyes of 6 patients (11/14, 78.6%; all patients except no.3) and new NVES in 2 eyes of 2 patients (2/14, 14.3%; no. Eye OCT-A was performed in 2 patients (no. Susac Syndrome - Symptoms, Causes, Treatment | NORD Susac syndrome is a rare condition characterized by the clinical triad of Federal government websites often end in .gov or .mil. The equivalent logarithm of the minimum angle of resolution acuity (logMAR) was calculated and used for analysis. https://www.ncbi.nlm.nih.gov/pubmed/22095376, Kleffner I, Drr J, Ringelstein M, Gross CC, Bckenfeld Y, Schwindt W, et al. Patients with BRAO and hearing loss that do not develop encephalopathy during the initial 2 years will most likely not develop encephalopathy. Susac Syndrome Susac syndrome tends to affect young women, but it can also occur in men. The first patient underwent brain biopsy, which revealed sclerosis of the media and adventitia of small pial and cortical vessels, suggestive of a healed angiitis. BRAOs can be bilateral and can involve multiple arterioles.2,19 Affected areas of retina may suffer from ischaemia. Retin Cases Brief Rep. 2018;12:2349. Muscle biopsies from such patients are usually normal, but some have also shown nonspecific signs of inflammation such as dense hyaline material surrounding endomysial capillaries. WebSusac's syndrome is an uncommon neurologic disorder of unknown cause. WebSusac syndrome is an autoimmune endotheliopathy, meaning the bodys immune system mistakenly attacks the endothelium. Susac Syndrome: Clinical characteristics, diagnostic findings and If close attention is not paid to the retina of a patient with vision loss and brain lesions, their symptoms may be mistaken for MS instead of Susac's syndrome. The three main characteristics are encephalopathy, partial or complete branch retinal artery occlusion, and low-frequency hearing loss. The formation of retinal microaneurysms is the result of focal damage to the capillary wall and may be due to loss of pericytes. Mean BCVA at final visit was stable (0.080.07 logMAR, 0-0.2;20/20-20/30). We would like to thank our photographers Mrs. Galit Yair-Pur and Mrs. Oshrat Barzilai. WebPMID: 23628737 DOI: 10.1038/nrneurol.2013.82 Abstract In Susac syndrome, occlusions of microvessels--presumed to be mediated by an autoimmune response to an as yet unknown antigen--lead to a characteristic clinical triad of CNS dysfunction, branch retinal artery occlusions, and sensorineural hearing impairment. 2003;135:4836. VA was measured on Snellen chart. Susacs Syndrome Update. On MRI, 5 patients showed decrease of lesion load (patients no. https://www.karger.com/DOI/10.1159/000493489. Susacs syndrome: the triad of microangiopathy of the brain and retina with hearing loss in young women.

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