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Susac syndrome review

Characteristics of Susac syndrome: a review of all

Susac's Syndrome: An Updated Review - PubMe

Susac syndrome is a rare, underdiagnosed, and often misdiagnosed disease that can lead to severe complications such as deafness, vision loss, dementia, and death. It involves the central nervous system and may mimic other neurological and neuro-ophthalmological diseases Susac syndrome is considered a rare but important differential diagnosis in numerous neurological, psychiatric, ophthalmological, and ear, nose and throat disorders. Improved understanding of this.. Susac syndrome is a rare disease of unknown pathogenesis. 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

About two cases and review of the literature <span>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 Susac's syndrome (SS) is a relatively rare cause of multiple recurrent branch retinal arterial occlusions (BRAO). SS is frequently misdiagnosed and probably underdiagnosed. Ophthalmic manifestations may be the sole presenting sign of SS. Comprehensive literature review The typical triad of encephalopathy, sensorineural hearing loss and multiple recurrent BRAO is seldom seen at presentation. Susac syndrome is a rare, autoimmune disease. Autoimmune means that your immune system mistakenly attacks your own tissues. In Susac syndrome, your immune system attacks the smallest blood vessels in your brain, retina (part of the eye), and inner ear. The blood vessels become blocked

Susac syndrome is an autoimmune disease of the small arterioles of the brain, retina, and inner ear that has been successfully treated with immunosuppressants, such as cyclophosphamide or steroids. 3 Contrast enhancement of brain lesions and leptomeninges hints at an inflammatory process Susac syndrome is a rare disease that is characterized by the clinical triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. It has been named after J. O. Susac who described two young women with the typical triad in 1979. Today, about 200 cases have been reported in case reports or small case series CONCLUSION: Susac syndrome is a rare disorder of unknown origin characterized by the triad of encephalopathy, fluctuating hearing loss, and visual loss resulting from microangiopathy of the brain, cochlea, and retina. The multiple organ involvement seen in Susac syndrome raises a differential diagnosis ranging from autoimmune disease, through.

Susac Syndrome: Report of Four Cases and Review of the

  1. ated encephalomyelitis, it is often.
  2. Susac syndrome is an autoimmune condition that affects the very small blood vessels in the brain, retina, and inner ear (cochlea). The condition is characterized by three main symptoms: brain disease (encephalopathy), hearing loss, and vision loss
  3. Susac's syndrome (SS) is a relatively rare cause of multiple recurrent retinal arterial occlusions that is oftenoverlookedandmisdiagnosed.Ahighindexof suspicion is needed to make the diagnosis. Early diagnosis and treatment are important to delay dis-ease progression and prevent blindness, deafness and dementia. Historica

Susac syndrome, also known as retinocochleocerebral vasculopathy or SICRET syndrome (small infarctions of cochlear, retinal and encephalic tissue), is a rare syndrome typically affecting young to middle-age women that is clinically characterized by the triad of acute or subacute encephalopathy, bilateral sensorineural hearing loss, and branch retinal arterial occlusions Susac syndrome is a rare condition involving the brain, retina, and cochlea vasculature causing encephalopathy, visual loss due to retinal branch occlusion, and sensorineural hearing loss. Most evidence support that this disease is an acquired autoimmune disorder Susac syndrome is a rare disease, named after John Susac (1940-2012) who in 1979 described two female patients REVIEW Susac syndrome: a report of four cases and a review of the literature S.M. van der Kooij1*, M.A. van Buchem2, O.M. Overbeek3, G. Dijkman4, T.W.J. Huizinga

Susac syndrome (SuS) is a rare autoimmune endotheliopathy leading to hearing loss, branch retinal artery occlusions and encephalopathy. Young females are more frequently affected than males, making counselling for family planning an important issue 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.. Susac syndrome (SuS) is a rare immune-mediated occlusive microvascular disease. It is characterized by a typical clinical triad of encephalopathy, visual disturbances and hearing loss [ 1, 2, 3, 4, 5 ]

Neuro-ophthalmic manifestations of Susac syndrom

  1. Title: Susac Syndrome: Case & Review Author: Russell Swan MD, Moran Eye Center, PGY 4 Date: 03/09/2016 Keywords/Main Subjects: Susac's syndrome; Branch retinal arterial occlusion Secondary CORE Category: Neuro-ophthalmology / Causes of Decreased Vision / Vascular Disorders Diagnosis: Susac's syndrome
  2. 2. Dorr J, Krautwald S, Wildemann B, et al. Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol. 2013;9(6):307-316. 3. Susac JO. Susac's syndrome: the triad of microangiopathy of the brain and retina with hearing loss in young women. Neurology. 1994;44(4):591-593. 4
  3. Disease. Microangiopathy of brain, retina, and cochlea (i.e., Susac syndrome (SS)) is a rare condition characterized by the clinical triad of encephalopathy, branch retinal artery occlusion (BRAO), and sensorineural hearing loss. However, all three features may not be present concurrently upon initial presentation

Susac syndrome (SuS) is a rare autoimmune endotheliopathy leading to hearing loss, branch retinal artery occlusions and encephalopathy. Young females are more frequently affected than males, making.. Susac's syndrome, a rare autoimmune vasculo-occlusive disease, may pose a diagnostic challenge and result in a devastating ocular and systemic outcome. Our study identifies a new retinal finding.

Discussion of cochlear implantation in Susac's syndrome. In their review of 304 published cases of SS, Dorr et al 5 noted that 99% of patients had hearing loss. In its least injurious form, the SNHL associated with SS may lead to only a mild unilateral loss with minimal functional impact on the patient Susac syndrome (SuS) is a rare autoimmune disease where the body's immune system mistakenly attacks the lining of the smallest blood vessels in the brain, retina, and inner ear. This attack causes blood vessels to be injured and swollen, blocking blood flow, oxygen, and nutrients to the brain, retina, and inner ear

Do Not Forget Susac Syndrome in Patients with Unexplained

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Susac syndrome is an autoimmune endotheliopathy, a disorder in which the body's immune system mistakenly attacks the inside lining (endothelium) of the walls of the very tiny blood vessels that supply blood to the brain, retina, and inner ear. The exact, underlying reason why this occurs is unknown Susac Syndrome: Report of Four Cases and Review of the Literature Tai Hung Do, Christiane Fisch, and Franc¸ois Evoy Summary: Susac syndrome is a rare disease of unknown pathogenesis. It is caused by a microangiopathy affecting the arterioles of the brain, retina, and cochlea, giving the classic clinical triad of subacute encephalopathy, visua

A review of the outcome of Susac syndrome in 9 patients by Aubart-Cohen and coworkers found that all had permanent hearing loss with mean 34 dB (range 15-7-dB). 3 Although thought to be self-limiting after several years (range of period differs with individual patients), recurrence after 18 years of remission has been reported.52, 53 Hence. 1. Curr Opin Neurol. 2005 Jun;18(3):311-4. Update on Susac's syndrome. Gross M(1), Eliashar R. Author information: (1)Department of Otolaryngology/Head and Neck Surgery, The Hebrew University School of Medicine, Hadassah Medical Center, Jerusalem, Israel. drgrossm@hotmail.com PURPOSE OF VIEW: We review recent developments in the clinical course and imaging modalities for Susac's syndrome, a. Susac syndrome is an autoimmune disease of the small arterioles of the brain, retina, and inner ear that has been successfully treated with immunosuppressants, such as cyclophosphamide or steroids. 3 Contrast enhancement of brain lesions and leptomeninges hints at an inflammatory process. Occlusions and hyperfluorescence of retinal arteries are.

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. 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 Susac's syndrome is an uncommon neurologic disorder of unknown cause. It has been described as a clinical triad of encephalopathy, hearing loss, and branch retinal artery occlusions [].Clinically, the diagnosis is difficult when the patient presents with only a portion of the triad

Susac syndrome: report of four cases and review of the

For a review of the natural history of SS, go to About Susac's Syndrome: Clinical Course section. Historical treatment of SS Review of published case reports and case series has revealed a variable response of SS to immunosuppressive therapy. Some patients have improved without receiving any immunosuppressive treatment Susac's syndrome consists of the clinical triad of encephalopathy, branch retinal artery occlusions, and hearing loss. In 1975, I saw two patients with this syndrome within a matter of 3 weeks while serving in the United States Army at Walter Reed Army Hospital. The first patient was presented t Susac syndrome is a rare disease attributed to a microangiopathy involving the arterioles of the brain, retina, and cochlea. This syndrome was first described by John O. Susac in 1979 in two young women presenting with the classic clinical triad of subacute encephalopathy, retinal arteriolar branch occlusions, and sensorineural hearing loss ( 1 )

Susac syndrome and pregnancy: a relationship to clarify

7. Susac JO. Susac's syndrome: the triad of microangiopathy of the brain and retina with hearing loss in young women. Neurology. 1994;44(4):591-593. 8. Dörr J, Krautwald S, Wildemann B, et al. Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol. 2013;9(6):307-316. 9 As a result of the rarity of the disease, controlled therapeutic trials are missing so far. In this review, we want to demonstrate the clinical features, natural history, treatment, and clinical course of Susac syndrome, illustrated by a typical case history Susac syndrome (SS) is an infrequent neurological disorder that is characterized by the clinical triad of encephalopathy, branch retinal artery occlusions (BRAOs) and hearing loss. It is an autoimmune-mediated microangiopathy (precapillary arterioles) that affects the microvasculature of the brain, retina, and inner ear [1] Susac syndrome (SuS) is named after John Susac, who was the first to describe the syndrome of encephalopathy, hearing loss and branch retinal artery occlusions (BRAO). 1, 2 It is a rare disease, with just over 500 cases described worldwide. 3 Diagnostic criteria were proposed by the European Susac Consortium in 2016. 4 The pathophysiology of. 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.

Susac's syndrome: an update British Journal of Ophthalmolog

An additional Conclusion: Susac syndrome is a rare and potentially devastat- review of relevant vestibulocochlear data is undertaken among ing disease. Hearing loss is quite variable. Low- and mid-range the approximately 100 previously reported cases Objective We characterised the clinical and neuro-otological characteristics of patients with Susac syndrome. Methods The medical records of 30 patients with Susac syndrome were reviewed for details of their clinical presentation and course, neuro-otological symptoms, investigation results including audiology and vestibular function tests, treatment and outcomes. Results Our findings. Susac syndrome and pregnancy: a review of published cases and considerations for patient management Barbara Willekens and Ilka Kleffner Abstract: Susac syndrome (SuS) is a rare autoimmune endotheliopathy leading to hearing loss, branch retinal artery occlusions and encephalopathy. Young females are more frequentl 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

Susac Syndrome: Symptoms, Causes, Tests & Treatmen

Purpose of review 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.. Recent findings The pathogenesis of Susac syndrome still remains unclear. . Fluorescein angiography demonstrates. Characteristics of Susac syndrome: a review of all reported cases. Download. Related Papers. Susac's syndrome: 2 cases without hearing loss. By M. Güney Şenol. Aggressive immunosuppressive treatment of Susac's syndrome in an adolescent: using treatment of dermatomyositis as a model

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 The prognosis of Susac syndrome has been described as following 3 different courses: monocyclic, polycyclic, or chronic continuous. The monocyclic course, which is the most common, has an active period lasting less than 2 years, whereas the polycyclic course is characterized by relapses extending beyond 2 years Susac's syndrome is rare with only around 300 cases reported worldwide. Its cause is unknown but it affects the microvasculature of the cochlea, retina and brain, leading to infarcts in each of these organs. The key to its diagnosis is the characteristic fundal changes and brain imaging findings. In the subacute stage the cerebral infarcts. To the Editor: Susac syndrome (SS), a syndrome affecting the brain along with retinal and cochlear microangiopathy, is characterized by acute multiple encephalopathy, branch retinal artery occlusion and sensorineural deafness. This disease was first described in 1979, but it was named formally in 1994. As a rare disease, only approximately 300 cases have ever been reported

Susac Syndrome - Practical Neurolog

Dorr J, Krautwald S, Wildemann B, et al. Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol. 2013;9:307-16. Gross, Catharina C. et al. CD8+ T cell-mediated endotheliopathy is a targetable mechanism of neuro-inflamation in Susac syndrome. Nat Commun. 2019 Dec 18;10(1):577 Conclusions: Susac syndrome is an immune-mediated vasculopathy involving small vessels in the brain, eye, and ear. Classic clinical triad consists of hearing loss, vision changes, and encephalopathy. Imaging findings in SS can be mistakenly identified as demyelinating lesions, and there are reports of patients with SS erroneously being treated for MS. Red flags raising concern for SS include.

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 Susac's syndrome: Intratympanic therapy for hearing loss and a review of the literature Brianna K. Crawley MD Division of Otolaryngology/Head and Neck Surgery, Department of Surgery, University of New Mexico School of Medicine, Albuquerque, New Mexic Susac's syndrome (retinocochleocerebral vasculopathy) is a very rare form of microangiopathy characterized by encephalopathy, branch retinal artery occlusions and hearing loss. The cause is unknown but the current thinking is that antibodies are produced against endothelial cells in tiny arteries which leads to damage and the symptoms related to the illness Susac's syndrome (SS) consists of the triad of encephalopathy, branch retinal artery occlusions (BRAO), and hearing loss. It usually affects women aged 20 to 40, but men are also affected, and the age range extends from 9 to 72 years. It tends to be unrecognized, even in major academic centers. The complete triad may not be present at the onset, which makes diagnosis more difficult

Susac Syndrome, acute

A brief review of Susac syndrome - ScienceDirec

  1. Purpose of review . This review discusses general features and organ-specific presentations of Susac syndrome as well as diagnosis and treatment.. Recent findings . Latest literature regarding demographics, new diagnostic modalities such as optical coherence tomography and treatment options for Susac syndrome are discussed in detail in this review, summarizing the most recent updated information
  2. M, Altarazi E, Almarzouki H Neuroradiol J 2018 Apr;31(2):207-212. Epub 2017 Jun 23 doi: 10.1177/1971400917712265
  3. imal disease burden to both the mother and newborn. She was treated initially using intravenous methylprednisolone followed by oral prednisone.
  4. Susac syndrome (SuS) is a rare disorder that is thought to be caused by autoimmune-mediated occlusions of microvessels in the brain, retina and inner ear [] which lead to a characteristic clinical triad of central nervous system (CNS) dysfunction, visual disturbances and hearing deficits [1-5].Typical findings in patients with SuS include branch retinal artery occlusions (BRAO) detectable on.
  5. e the proportion of cases and their characteristics in a uveitis referral centre. Charts of patients with the diagnosis of Susac syndrome seen in the Uveitis Clinic of the Centre for.
Susac Syndrome: Report of Four Cases and Review of theSusac Syndrome - Retina Image Bank(PDF) Susac syndrome - Clinical insight and strategies ofFebruary 2016 Wills Eye Resident Case Series - DiagnosisNeuro-ophthalmology - School of Medicine Blogs - StanfordGerardo Quiñones-Pesqueira&#39;s research works | Instituto

Susac syndrome, a rare autoimmune disorder first described as a classic triad (encephalopathy, branch retinal artery occlusion, and sensorineuralhearing loss) in 1979 by renowned physician John O. Susac, has been an advancing area of clinical interest and scientific researchover the last several decades. This comprehensive review aims to succinctly highlight the breadth and detail of this. Susac syndrome, also known as retinocochleocerebral vasculopathy or SICRET syndrome (small infarctions of cochlear, retinal and encephalic tissue), is a rare syndrome typically affecting young to middle-age women that is clinically characterised by the triad of acute or subacute encephalopathy, bilateral sensorineural hearing loss, and branch retinal arterial occlusions Susac syndrome (SS) is a rare microangiopathy that involves arterioles of the brain, retina, and cochlea. Diagnosis is extremely difficult because of the rarity of the disease and because the signs and symptoms often occur at different times. Multidisciplinary approaches and multimodal images are mandatory for diagnosis and prompt therapy. In this report, we describe a case of SS and the. Susac syndrome and pregnancy: a relationship to clarify. About two cases and review of the literature. J Matern Fetal Neonatal Med 2020; published ahead of print. doi: 10.1080/14767058.2020.1712712. [Google Scholar