The Journal of Maternal-fetal & Neonatal Medicine : the Official Journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians; Ramos-Ruperto L, Martínez-Sánchez N et. al.
Jan 13th, 2020 - Background: Susac syndrome is a vasculopathy that affects the central nervous system, mainly the brain parenchyma, retina and inner ear. It affects mainly young women and. Management is based on expert consensus and in pregnant women the treatment is not well established. It is necessary to start treatment early because of its potential severity and sequelae.Method: We present two cases of Susa...
Nature Communications; Gross CC, Meyer C et. al.
Dec 19th, 2019 - Neuroinflammation is often associated with blood-brain-barrier dysfunction, which contributes to neurological tissue damage. Here, we reveal the pathophysiology of Susac syndrome (SuS), an enigmatic neuroinflammatory disease with central nervous system (CNS) endotheliopathy. By investigating immune cells from the blood, cerebrospinal fluid, and CNS of SuS patients, we demonstrate oligoclonal ex...
Ultrastructural Pathology; Agamanolis DP, Prayson RA et. al.
Nov 18th, 2019 - Susac syndrome is a rare, immune-mediated disease characterized by encephalopathy, branch retinal artery occlusion, and hearing loss. Herein, we describe the electron microscopic findings of three brain biopsies and two brain autopsies performed on five patients whose working clinical diagnosis was Susac syndrome. In all five cases, the key findings were basement membrane thickening and collage...
BMJ Case Reports; Pérez-Lombardo M, Alberdi-Páramo Í et. al.
Aug 29th, 2019 - A 47-year-old woman presented an episode of confusion and disorientation. According to remarkable psychiatric records, she had been treated for major depression and obsessive-compulsive disorder; however, no other relevant background was known. After preliminary examinations, blood analysis and neurological tests were unspecific and inconclusive. Therefore, the case was treated as a possible ps...
Neurology Law LY, Riminton DS et. al.
Aug 26th, 2019 - The presentation of a patient with brainstem symptoms and signs invokes a number of common and less common differential diagnoses, and accurate diagnosis can be challenging. We review the major immune-mediated and inflammatory syndromes that can affect the brainstem including multiple sclerosis, neuromyelitis optica spectrum disorder, neuro-Behçet disease, chronic lymphocytic inflammation with ...
AJNR. American Journal of Neuroradiology; Coulette S, Lecler A et. al.
Jun 29th, 2019 - Leptomeningeal enhancement can be found in a variety of neurologic diseases such as Susac Syndrome. Our aim was to assess its prevalence and significance of leptomeningeal enhancement in Susac syndrome using 3T postcontrast fluid-attenuated inversion recovery MR imaging. From January 2011 to December 2017, nine consecutive patients with Susac syndrome and a control group of 73 patients with mul...
Brain : a Journal of Neurology; Filippi M, Preziosa P et. al.
Jun 16th, 2019 - MRI has improved the diagnostic work-up of multiple sclerosis, but inappropriate image interpretation and application of MRI diagnostic criteria contribute to misdiagnosis. Some diseases, now recognized as conditions distinct from multiple sclerosis, may satisfy the MRI criteria for multiple sclerosis (e.g. neuromyelitis optica spectrum disorders, Susac syndrome), thus making the diagnosis of m...
Multiple Sclerosis and Related Disorders; Zengin Karahan S, Boz C et. al.
Jun 8th, 2019 - Susac's Syndrome (SS), which was first described in 1979, is a rare and presumably autoimmune disorder characterized by encephalopathy, hearing loss, and visual disturbance resulting from branch retinal artery occlusion (BRAO). This study reports 19 SS patients' clinical characteristics, MRI features, CSF analysis, treatment strategies and outcomes. At initial presentation, only three of 19 pat...
Journal of Neuropathology and Experimental Neurology; Agamanolis DP, Klonk C et. al.
May 17th, 2019 - A 24-year-old woman developed encephalopathy, branch retinal artery occlusion, hearing loss, and had "snowball" lesions in the corpus callosum, classic findings of Susac syndrome (SuS). Despite intensive immunosuppressive therapy, she lapsed into a coma, and died 7 months after the onset of her illness. Neuropathological examination, revealed perivascular inflammation and vasculitis involving s...
European Journal of Ophthalmology; Giuffrè C, Miserocchi E et. al.
Apr 9th, 2019 - The aim is to present the changes in ultra-widefield and widefield multimodal imaging, including optical coherence tomography angiography of a 33-year-old woman diagnosed with Susac syndrome, over 1 year of follow-up. Fundus examination and multimodal imaging revealed bilateral arterial occlusion of multiple vascular branches with retinal ischemia. Over 1 year follow-up, best-corrected visual a...
Journal of Neurology, Neurosurgery, and Psychiatry; Triplett JD, Buzzard KA et. al.
Mar 10th, 2019 - The triad of central nervous system symptoms, visual disturbance and hearing impairment is an oft-encountered clinical scenario. A number of immune-mediated diseases should be considered among the differential diagnoses including: Susac syndrome, Cogan syndrome or Vogt-Koyanagi-Harada disease; demyelinating conditions such as multiple sclerosis or neuromyelitis optica spectrum disorder; systemi...
The American Journal of the Medical Sciences; Hertzano R, Tomlinson JA et. al.
Feb 3rd, 2019 - Francisco Goya produced over 1,800 works during his long career, which earned him a reputation as one of the greatest artists of modern times. When he was 47, he developed a mysterious illness that destroyed his hearing, and for the next 35 years left him "deaf as a stump." Of the diagnoses proposed to date, Susac syndrome is the one most consistent with what little we do know of the character ...
Survey of Ophthalmology; Heng LZ, Bailey C et. al.
Jan 31st, 2019 - Susac syndrome is a rare condition presumed to be immune-mediated occlusion of small arterial vasculature principally of the brain, inner ear, and retina. Clinically, the syndrome manifests as a pathognomonic triad of encephalopathy, hearing loss, and branch retinal artery occlusion. Early recognition and diagnosis is important as delayed treatment may be profound and result in deafness, blindn...
Retina (Philadelphia, Pa.); Buelens T, Ossewaarde-van Norel J et. al.
Jan 28th, 2019 - To evaluate the effect of tumor necrosis factor (TNF) inhibitor therapy on ocular relapses in patients with Susac syndrome. Multicenter retrospective cohort study of patients diagnosed with Susac syndrome according to classical clinical criteria. We evaluated the disease activity before and after introduction of anti-TNF therapy and its value as a steroid-sparing agent. Five patients were inclu...
Journal of the Neurological Sciences; Marrodan M, Acosta JN et. al.
Oct 2nd, 2018 - To assess clinical and/or imaging features useful to distinguish between Susac syndrome (SuS) and primary angiitis of central nervous system (PACNS). Multicenter retrospective analysis of two cohorts of Argentine patients diagnosed with SuS and PACNS. 13 patients diagnosed with SuS (6 women and 7 men, mean age 35 ± 10 years) and 15 with PACNS (10 women and 5 men, mean age 44 ± 18 years) were an...
Journal of Neuro-ophthalmology : the Official Journal of the North American Neuro-Ophthalmology Society; Egan RA
Jun 22nd, 2018 - Susac syndrome (SS) classically presents with the clinical triad of retinal artery occlusion, sensorineural hearing loss, and encephalopathy and the neuroimaging triad of white matter lesions, deep gray matter lesions, and leptomeningeal disease. However, patients can present with an incomplete clinical or neuroimaging triads making diagnosis difficult in certain situations. A standard treatmen...
Retinal Cases & Brief Reports; Ammar MJ, Kolomeyer AM et. al.
Jun 5th, 2018 - We report a patient with branch retinal artery occlusion predominant Susac syndrome and review the literature on current diagnostic modalities and therapeutic approaches for this rare condition. Case report. A 23-year-old white male presented with a 5-month history of a scotoma in his right eye vision, headaches, and intermittent paresthesias of the lower extremities. Ophthalmic examination was...
Retinal Cases & Brief Reports; Yang YS, Zhang L et. al.
Mar 29th, 2018 - To report a case of acute macular neuroretinopathy in a patient with Susac syndrome. Case report. A 39-year-old male patient presented with severe headache, photopsias, and a sudden onset of hearing loss in the right ear. Fluorescein angiography of the right eye revealed multiple branch retinal artery occlusions. Clinical presentation of encephalopathy, hearing loss, and branch retinal artery o...
Jan 24th, 2018 - Susac Syndrome is a rare disease characterized by encephalopathy, branch retinal artery occlusion and sensorineural deafness. The pathogenesis is not yet clear, an autoimmune endotheliopathy is discussed. Because of the variable and often incomplete clinical presentation, the establishment of the diagnosis is often delayed or even completely missed. The aim of this study is to identify biomarke...
Journal of Neuro-ophthalmology : the Official Journal of the North American Neuro-Ophthalmology Society; Egan RA, Jirawuthiworavong G et. al.
Jan 17th, 2018 - The ophthalmic findings of Susac syndrome (SS) consist of visual field defects related to branch retinal artery occlusion (BRAO), and fluorescein angiography (FA) reveals a unique staining pattern. To date, retinal arterial collateral development has been described only in a single patient. Given that the immunopathological process in SS induces retinal ischemia, it is conceivable that abnormal...
International Journal of Stroke : Official Journal of the International Stroke Society; Rennebohm RM, Asdaghi N et. al.
Jan 10th, 2018 - Susac syndrome is an immune-mediated, pauci-inflammatory, ischemia-producing, occlusive microvascular endotheliopathy/basement membranopathy that affects the brain, retina, and inner ear. Treatment of Susac syndrome is particularly challenging. The organs involved can easily become irreversibly damaged, and the window of opportunity to protect them is often short. Optimal outcome requires rapid...
Retinal Cases & Brief Reports; Haider AS, Viswanathan D et. al.
Oct 10th, 2017 - To present the first reported case of paracentral acute middle maculopathy in association with Susac syndrome. Case report. A young female patient presented with unilateral sudden loss of vision of her right eye to count fingers. There were numerous cotton wool spots in a multifocal pattern concentrated around the right macula and optic disk. Clinical examination and spectral domain optical coh...
Applied Neuropsychology. Adult; Barritt AW, Wickremaratchi M et. al.
Sep 18th, 2017 - 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...
BMJ Case Reports; Betend R, Humm AM et. al.
Aug 8th, 2017 - A 67-year-old patient, only known for bilateral presbycusis, presented with subacute onset of delirium. Clinical examination showed multifocal neurological deficits, all together suggesting subcortical frontal dysfunction together with cerebellar and corpus callosum involvement.Cerebral MRI demonstrated supratentorial and infratentorial subcortical and periventricular T2-hyperintense lesions wi...
BMJ Case Reports; Nzenza TC, Hoag N et. al.
Jul 14th, 2017 - 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 voidi...
Multiple Sclerosis and Related Disorders; Marrodan M, Correale J et. al.
Jun 23rd, 2017 - 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 centra...
The Neuroradiology Journal; Algahtani H, Shirah B et. al.
Jun 23rd, 2017 - 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 m...
Journal of Neurology, Neurosurgery, and Psychiatry; Kleffner I, Dörr J et. al.
Jan 19th, 2017 - 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...
Journal of Neuro-ophthalmology : the Official Journal of the North American Neuro-Ophthalmology Society; Vodopivec I, Prasad S
Dec 16th, 2016 - 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. 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 ...
Retinal Cases & Brief Reports; Salvanos P, Moe MC et. al.
Nov 9th, 2016 - To present the use of ultra-wide-field (UW) fundus imaging in the diagnosis and follow-up of a patient with Susac syndrome. 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...
Medicine Vishnevskia-Dai V, Chapman J et. al.
Oct 27th, 2016 - 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 ...
Headache Kowacs F, Ferreira Gomes M et. al.
Oct 26th, 2016 - Migraine-Like Headache as Presentation Symptom in Susac Syndrome.|2016|Kowacs F,Ferreira Gomes M,Pigozzo T,Rodrigues Palmeira AL,Muratt Carpenedo C,|complications,diagnostic imaging,complications,diagnostic imaging,physiopathology,
Clinical Neurology and Neurosurgery; Staelens C, Audenaert K et. al.
Jul 31st, 2016 - Is Susac syndrome associated with bipolar disorder?|2016|Staelens C,Audenaert K,Tandt H,Van Driessche V,Lemmens G,|diagnosis,epidemiology,diagnostic imaging,diagnosis,epidemiology,
Handbook of Clinical Neurology; Ellenberger C
Jul 19th, 2016 - 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...
Retinal Cases & Brief Reports; Yepez JB, Murati FA et. al.
Jun 8th, 2016 - To report the use of intravitreal triamcinolone for Susac Syndrome in one patient. Case report. 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-res...
Journal of Medical Case Reports; Gertner E, Rosenbloom MH
May 28th, 2016 - 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....
JAMA Neurology; Kothari N, Kuriyan AE
May 9th, 2016 - Branched Retinal Artery Occlusions and Susac Syndrome.|2016|Kothari N,Kuriyan AE,|diagnostic imaging,complications,diagnostic imaging,complications,diagnostic imaging,
Seminars in Ophthalmology; Kumar K, Jariwala B et. al.
Apr 29th, 2016 - To report a case of Susac syndrome with ocular complications in the chronic stage of the disease. Case report. A 37-year-old male patient of Indian origin presented with painless blurring of vision in the right eye. Ophthalmoscopic examination revealed vitreous hemorrhage in the right eye and NVE in both eyes well-documented angiographically. Old medical records revealed a previous attack of bi...
Arthritis & Rheumatology (Hoboken, N.J.); Bullock DR, Rivera S et. al.
Mar 18th, 2016 - Early Recognition of and Intervention for Susac Syndrome in a Teenager With Encephalopathy.|2016|Bullock DR,Rivera S,Beardsley RM,Bothun ED,Rennebohm RM,|complications,diagnosis,etiology,therapy,
American Journal of Physical Medicine & Rehabilitation; Ramey LN, Lopez E et. al.
Feb 1st, 2016 - A 43-yr-old man presented with headaches, forgetfulness, and personality changes. Based on imaging, there was concern for acute disseminated encephalomyelitis. He improved with steroids and immunoglobulin. When symptoms recurred, magnetic resonance imaging showed new scattered gray and white matter lesions, many within the corpus callosum. Angiogram showed multiple branch retinal artery occlusi...
The Journal of International Advanced Otology; Yurtsever B, Çabalar M et. al.
Sep 18th, 2015 - Susac's syndrome is a rare autoimmune disease, which is characterized by microangiopathic changes that affects the brain, retina, and cochlea. It is mainly characterized by asymptomatic cerebral infarctions, low-and mid-frequency sensorineural hearing loss, and bilateral distal retinal artery occlusions. Otolaryngologists should be familiar with Susac's syndrome because hearing loss may be the ...
Retinal Cases & Brief Reports; Patel KH, Haug SJ et. al.
Sep 9th, 2015 - To describe an atypical presentation of Susac syndrome. Observational case report. A 44-year-old man with no significant medical history presented with inferonasal visual field loss in his left eye of several months of duration. He was found to have bilateral migratory arteritis with focal areas of arteriolar occlusion in both eyes and peripheral ischemia superotemporally in his left eye. An ex...
Neurology Ringelstein M, Albrecht P et. al.
Jul 23rd, 2015 - The aim of this non-interventional study was to characterize retinal layer pathology in Susac syndrome (SuS), a disease with presumably autoimmune-mediated microvessel occlusions in the retina, brain, and inner ear, in comparison to the most important differential diagnosis multiple sclerosis (MS). Seventeen patients with SuS and 17 age- and sex-matched patients with relapsing-remitting MS (RRM...
Retina (Philadelphia, Pa.); Brandt AU, Oberwahrenbrock T et. al.
Jul 22nd, 2015 - To describe retinal lesion development in Susac syndrome during acute, postacute, and late phases of the disease. Cross-sectional study of four patients with Susac syndrome and longitudinal short-interval case study of one additional patient. Retinal changes were analyzed with high-resolution spectral domain optical coherence tomography and retinal fluorescein angiography. Retinal Susac syndrom...
Neurology Francis AW, Kiernan CL et. al.
Jul 21st, 2015 - Clinical Reasoning: An unusual diagnostic triad. Susac syndrome, or retinocochleocerebral vasculopathy.|2015|Francis AW,Kiernan CL,Huvard MJ,Vargas A,Zeidman LA,|diagnosis,psychology,diagnosis,psychology,
Journal of the Neurological Sciences; Vodopivec I, Venna N et. al.
Jul 7th, 2015 - Susac syndrome (SS) is a rare, presumed autoimmune condition characterized by the clinical triad of branch retinal artery occlusions (BRAOs), encephalopathy, and sensorineural hearing loss. The aim of this study was to evaluate clinical features, diagnostic results, treatment, and outcomes in SS. Five patients with SS were referred to three tertiary care centers in Boston. The observation perio...
Journal of Neuro-ophthalmology : the Official Journal of the North American Neuro-Ophthalmology Society; Artunay O, Sengul A et. al.
Mar 18th, 2015 - We report central serous chorioretinopathy (CSC) in a patient with Susac syndrome. The diagnosis of Susac syndrome was based on the results of funduscopy, brain magnetic resonance imaging, and audiometric testing. Our case demonstrates that possible choroidal involvement in Susac syndrome may lead to the development of CSC.
Journal of Stroke and Cerebrovascular Diseases : the Official Journal of National Stroke Association; Star M, Gill R et. al.
Feb 14th, 2015 - To report 2 patients with Susac syndrome presenting with acute confusion and abnormal neuroimaging and cerebrospinal fluid (CSF) findings, both of whom were initially misdiagnosed. Susac syndrome is a rare, though likely underdiagnosed, endotheliopathy defined clinically by the triad of encephalopathy, branch retinal artery occlusions (BRAOs), and sensorineural hearing loss (SNHL). Its pathogen...
Retinal Cases & Brief Reports; Buelens T, Herode L et. al.
Nov 5th, 2014 - Susac syndrome is a rare disease attributed to microangiopathy involving the arterioles of the brain, retina, and cochlea. Understanding the pathogenesis is incomplete, but an immune-mediated process remains the leading hypothesis. Report of a single case of a previously healthy 22-year-old female patient showing the complete clinical triad. Diagnosis of Susac syndrome in this patient was first...
Seminars in Ultrasound, CT, and MR; Filippi CG, Cauley KA
Sep 13th, 2014 - The corpus callosum is the largest white matter tract in the brain, connecting the 2 hemispheres. The functions of the corpus callosum are many and varied, and lesions frequently cause only subtle clinical findings. The range of diseases that can affect the corpus callosum is vast and includes all potential white matter disease. The distribution of lesions in the corpus callosum is disease spec...
Journal of Medical Toxicology : Official Journal of the American College of Medical Toxicology; Hantson P, Di Fazio V et. al.
Aug 21st, 2014 - Toxic leukoencephalopathy is a possible but rare complication of chronic cocaine abuse. The role of adulterants, mainly levamisole, is still debated. We describe an atypical case of fatal leukoencephalopathy mimicking Susac syndrome in a 22-year-old man who was chronically abusing cannabis and cocaine. Exposure to levamisole as adulterant to cocaine was proven by hair analysis. Despite cessatio...
Journal of Alzheimer's Disease : JAD; Dörr J, Ringelstein M et. al.
Mar 25th, 2014 - Susac syndrome (SuS) is a rare endotheliopathy of the brain, the retina, and the inner ear. The underlying pathophysiology is likely an autoimmune mediated occlusion of microvessels resulting in variable degrees of central nervous system (CNS) dysfunction, visual disturbances, and hearing loss. The disease manifests either with a monophasic or polycyclic course. Patients suffering from SuS are ...
Journal of Neuroinflammation; Jarius S, Kleffner I et. al.
Mar 12th, 2014 - Susac syndrome (SuS) is a rare disorder thought to be caused by autoimmune-mediated occlusions of microvessels in the brain, retina and inner ear leading to central nervous system (CNS) dysfunction, visual disturbances due to branch retinal artery occlusions (BRAO), and hearing deficits. Recently, a role for anti-endothelial cell antibodies (AECA) in SuS has been proposed. To report the clinica...
Autoimmunity Reviews; García-Carrasco M, Mendoza-Pinto C et. al.
Jan 15th, 2014 - Susac syndrome (SS) is an autoimmune disease characterized by the clinical triad of encephalopathy, branch retinal artery occlusions and neuro-sensorial hearing loss; it is due to a microangiopathy affecting the precapillary arterioles of the brain, retina and inner ear. SS is characterized by typical radiological features on magnetic resonance imaging (MRI) which, together with clinical sympto...
Journal of the Neurological Sciences; Hua le H, Donlon SL et. al.
Dec 24th, 2013 - We describe a typical case presentation of Susac syndrome with a novel MRI finding of cervical spinal cord involvement. A 25-year-old, 14-week gestation white woman presented with two episodes of encephalopathy, responsive to steroids, with abnormal brain magnetic resonance imaging (MRI) concerning for Susac syndrome. Further studies confirmed the clinical triad of encephalopathy, branch retina...
European Neurology; Kleffner I, Dörr J et. al.
Dec 17th, 2013 - Susac syndrome is a rare disease characterized by the triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss mainly affecting young women. The finding of antibodies against the endothelium in the sera of these patients has supported the hypothesis of an autoimmune endotheliopathy of the brain, inner ear and retina. Because of the rarity of the disease, treatmen...
Journal of the Neurological Sciences; Allmendinger AM, Viswanadhan N et. al.
Jul 12th, 2013 - Susac syndrome is a rare neurologic disorder first described by Susac et al. in 1979. Clinically, Susac syndrome consists of a triad including encephalopathy, branch retinal artery occlusions and sensorineural hearing loss. All three components of the triad usually do not present at the same time, thus delaying time to diagnosis. MRI studies often show characteristic punched out lesions of the ...
AJNR. American Journal of Neuroradiology; Lehman VT, Doolittle DA et. al.
Jul 5th, 2013 - Descriptions of uncommon diseases with intracranial imaging abnormalities are often difficult to find in the radiology literature. We hypothesized that reported imaging findings of such conditions in the recent literature were more frequent in clinical compared with radiology journals. PubMed searches from December 1, 2007 to December 1, 2012 were performed for 5 uncommon CNS diseases with intr...
Journal of Neurovirology; Longbrake EE, Ances BM et. al.
May 21st, 2013 - Susac syndrome in a patient with human immunodeficiency virus infection.|2013|Longbrake EE,Ances BM,Viets RB,Clifford DB,|blood supply,pathology,virology,blood supply,pathology,virology,isolation & purification,complications,diagnosis,pathology,virology,complications,diagnosis,pathology,virology,
Nature Reviews. Neurology; Dörr J, Krautwald S et. al.
Apr 30th, 2013 - 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. Susac syndrome is considered a rare but important differential diagnosis in numerous neurological, psychiatric, ophthalmological, and ear,...
Journal of Neuro-ophthalmology : the Official Journal of the North American Neuro-Ophthalmology Society; Seamone ME, Fielden M
Apr 24th, 2013 - Susac syndrome is characterized by encephalopathy, sensorineural hearing loss, and branch retinal artery occlusion. Additional ocular findings include arteriolar wall hyperfluorescence and Gass plaques. We present a 51-year-old Caucasian woman with ophthalmologic findings indicative of Susac syndrome in the setting of tinnitus and migraine with aura.
The Primary Care Companion for CNS Disorders; Zormann A, Sylle K et. al.
Dec 19th, 2012 - A patient with susac syndrome and bipolar disorder.|2012|Zormann A,Sylle K,Schroller S,Beck J,|
Multiple Sclerosis (Houndmills, Basingstoke, England); Wuerfel J, Sinnecker T et. al.
Jun 19th, 2012 - Although an orphan disease with still obscure aetiopathogenesis, Susac syndrome has to be considered as differential diagnosis in multiple sclerosis (MS), since its clinical presentation and paraclinical features including routine magnetic resonance imaging (MRI) findings partially overlap. We aimed to study a potential benefit of 7T MRI for (i) the differentiation between Susac syndrome and MS...
PloS One; Brandt AU, Zimmermann H et. al.
Jun 15th, 2012 - Susac syndrome, a rare but probably underdiagnosed combination of encephalopathy, hearing loss, and visual deficits due to branch retinal artery occlusion of unknown aetiology has to be considered as differential diagnosis in various conditions. Particularly, differentiation from multiple sclerosis is often challenging since both clinical presentation and diagnostic findings may overlap. Optica...
Journal of the Neurological Sciences; Kleffner I, Duning T et. al.
May 29th, 2012 - Susac syndrome was named after J.O. Susac who first described the syndrome in 1979. It is characterized by the clinical triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. It mainly occurs in young women. This underdiagnosed disease needs to be considered in the differential diagnosis of a broad variety of disorders. In Susac syndrome, autoimmune processes ...
European Journal of Neurology; Mateen FJ, Zubkov AY et. al.
Jan 9th, 2012 - There are few clinical studies on the attempted treatments and outcomes in patients with Susac syndrome (SS) (retinocochleocerebral vasculopathy). A retrospective review was performed of all patients presenting with SS at the Mayo Clinic in Rochester, Minnesota, USA (1 January 1998-1 October 2011). There were 29 cases of SS (24 women, mean age at presentation, 35 years; range, 19-65; full triad...
American Journal of Clinical Pathology; Magro CM, Poe JC et. al.
Nov 18th, 2011 - Susac syndrome (SS) is the triad of encephalopathy, branch retinal artery occlusions (BRAOs), and hearing loss. Migraines may herald and accompany encephalopathy. Little is known about pathogenesis. Based on light microscopic findings in brain biopsy material analogous to anti-endothelial cell antibody (AECA)-mediated microvascular injury, we postulated that SS microangiopathy was attributable ...
Current Opinion in Ophthalmology; Bitra RK, Eggenberger E
Sep 7th, 2011 - The quest to better understand Susac syndrome has led to advancements in different aspects of the disease. The present article reviews the current literature on the clinical presentation, diagnostic modalities and trends in the management of Susac syndrome. The pathogenesis of Susac syndrome still remains unclear. Fluorescein angiography demonstrates arterial wall hyperfluorescence of the arter...
The Journal of Laryngology and Otology; Grover N, Whiteside OJ et. al.
Apr 12th, 2011 - Susac syndrome comprises a triad of vestibulocochlear dysfunction, retinopathy and multifocal encephalopathy, which is characterised pathophysiologically by microangiopathy of the ear, retina and brain. Diagnosis is confirmed by magnetic resonance imaging of the brain and ophthalmological examination, which reveals branch retinal artery occlusion. Hearing loss persists in 90 per cent of patient...
Journal of Clinical Rheumatology : Practical Reports on Rheumatic & Musculoskeletal Diseases; Deane KD, Tyler KN et. al.
Feb 17th, 2011 - Susac syndrome (SS) consists of a triad of vision loss, hearing loss, and encephalopathy due to autoimmune-mediated vascular endothelial injury. Herein we describe a 25-year-old previously healthy woman who presented at 20 weeks' gestation with symptoms of confusion, difficulty walking, and vision and hearing loss. She had branch-retinal artery occlusions on funduscopic examination, and sensori...
Dermatology Online Journal; Harris K, Pho LN et. al.
Nov 10th, 2010 - A 50-year-old woman presented for asymptomatic yellow hyperkeratotic plaques limited to her face. The plaques reportedly arose over the six months prior to her clinic visit. She was healthy prior to the diagnosis of Susac syndrome (retinocochleocerebral vasculopathy) two years before. A punch biopsy was performed and revealed retention hyperkeratosis. Retention hyperkeratosis is a benign and co...
Journal of the Neurological Sciences; Egan RA, Hills WL et. al.
Sep 30th, 2010 - Susac Syndrome (SS) consists of the triad of encephalopathy, branch retinal artery occlusion, and hearing loss. It is an autoimmune endotheliopathy that primarily affects young women. Two funduscopic findings, Gass plaques (GP) and arteriolar wall hyperfluorescence (AWH), have recently been described and are not only useful in making the SS diagnosis but also point to the endothelium as the sit...
Clinical Imaging; Allmendinger AM, Spektor V et. al.
Mar 1st, 2010 - Susac syndrome is a rare neurologic disorder first described by Susac et al. in 1979. Clinically, Susac syndrome consists of a triad including encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. Microinfarction is believed to be the basic histologic feature and MR is considered the best imaging modality. This case documents the typical imaging findings of Susac synd...
Nature Reviews. Neurology; Dörr J, Radbruch H et. al.
Dec 2nd, 2009 - A 23 year-old female presented to a neurology department with a 3 year history of recurrent episodes involving hearing loss, encephalopathy, focal neurological deficits, and visual field deficits. In the 3 years before presentation, the patient had been treated with methylprednisolone for suspected acute demyelinating encephalomyelitis and peripheral otogenic dysfunction from which she made a c...
Survey of Ophthalmology; Bitrian E, Sanchez-Dalmau B et. al.
Jun 22nd, 2009 - A 46-year-old man presented with acute confusional syndrome, ataxia, dysarthria, and right hemiparesthesia. Brain MRI showed small bilateral infarcts and fluorescein angiography revealed multiple peripheral retinal infarcts bilaterally. No visual loss was present, and no other organs were involved. The diagnosis of Susac syndrome (microangiopathy of the brain, retina and cochlea) was made and i...
The Neuroradiology Journal; Shaikh R, Muttikkal TJ et. al.
May 14th, 2009 - Systemic lupus erythematosus (SLE) has diverse imaging features. However, focal lesions in the corpus callosum are extremely rare in SLE with only few cases mentioned in the literature, with no mention of callosal holes in SLE. Callosal holes have been described as a characteristic finding in Susac syndrome and have been mentioned in Nipah virus encephalitis, Marchiafava Bignami disease and per...
Radiology Demir MK
Feb 3rd, 2009 - Case 142: Susac syndrome.|2009|Demir MK,|pathology,pathology,pathology,pathology,
Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology; Roeser MM, Driscoll CL et. al.
Dec 24th, 2008 - Susac syndrome is a disease condition of unknown cause consisting of vestibulocochlear dysfunction, retinopathy, and multifocal encephalopathy derived from microangiopathy of the ear, retina, and brain, respectively. We present a unique case of bilateral cochlear implantation in a Susac syndrome patient and seek to describe in detail the specific nature of the otologic manifestations of this di...
Retina (Philadelphia, Pa.); Postelmans L, Willermain F et. al.
Aug 7th, 2008 - Retinal arterio-arterial collaterals in SUSAC syndrome.|2008|Postelmans L,Willermain F,Guillaume MP,Libert M,Hermanus N,|therapeutic use,pathology,complications,diagnosis,blood supply,pathology,therapeutic use,therapeutic use,complications,complications,diagnosis,drug therapy,physiopathology,complications,physiopathology,etiology,physiopathology,
Ear, Nose, & Throat Journal; Westreich R, Chandrasekhar S
Jul 17th, 2008 - Susac syndrome is a rare disorder caused by the development of microangiopathic lesions in the inner ear, retina, and central nervous system. Although relatively few cases have been reported in the literature, this diagnosis is becoming more frequent as physician awareness increases. A high index of suspicion is needed to make the diagnosis because the signs and symptoms of Susac syndrome are t...
Otolaryngology--head and Neck Surgery : Official Journal of American Academy of Otolaryngology-Head and Neck Surgery; Magliulo G, Al-Ansi W et. al.
Mar 24th, 2008 - Susac syndrome and vestibular-evoked myogenic potentials.|2008|Magliulo G,Al-Ansi W,Parrotto D,Gagliardi S,Stasolla A,|etiology,etiology,etiology,physiopathology,physiology,etiology,etiology,physiopathology,etiology,physiopathology,
What is Susac's syndrome? Susac's syndrome is a rare, autoimmune disease. "Autoimmune" means that a person's immune system mistakenly attacks the person's own tissues. In Susac's syndrome, the immune system attacks the smallest blood vessels in the brain, retina (part of the eye), and inner ear. The blood vessels become blocked. Problems in the brain, retina, and inner ear occur due to decrease...