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Susac syndrome prognosis. It mainly affects young women.
- Susac syndrome prognosis. Susac’s syndrome (SS) is a clinical triad of encephalopathy, branch retinal artery occlusion and sensorineural hearing loss and maybe due to an immune-mediated endotheliopathy. Susac syndrome is an immune-mediated, pauci-inflammatory, ischemia-producing, occlusive microvascular endotheliopathy/basement membranopathy that affects the brain, retina, and inner ear. It is named Susac syndrome after Dr. Learn about symptoms, diagnosis, and treatment. This attack blocks or decreases blood flow and causes symptoms like hearing and vision loss. Immunosuppressive therapies Apr 20, 2021 · Susac’s syndrome, a rare autoimmune vasculo-occlusive disease, may pose a diagnostic challenge and result in a devastating ocular and systemic outcome. Sep 4, 2023 · Susac Syndrome is an extremely rare disorder which is characterized by abnormalities in the three most major organs of the body which is the brain, the eyes, and the ears. Our previous systematic review on all cases of SuS This finding was crucial to the diagnosis of Susac's syndrome (SS), a rare autoimmune disease characterized by, endotheliopathy of retina, encephalic tissues and cochlea. Comprehensive literature review The typical triad of encephalopathy, sensorineural hearing loss and multiple recurrent BRAO is seldom seen at presentation Abstract Susac syndrome (SuS) is an orphan microangiopathic disease characterized by a triad of en-cephalopathy, visual disturbances due to branch retinal artery occlusions, and sensorineuronal hearing loss. Our previous systematic review on all cases of SuS reported until 2012 allowed for a better understanding of clinical presentation and diagnostic findings. The triad of multifocal encephalopathy, visual loss, and hearing loss is caused by microangiopathy of the brain, retina, and cochlea. Clinical presentation Susac syndrome classically consists of a clinical triad of: Susac’s Syndrome Susac’s Syndrome (SuS) is a rare autoimmune disorder in which a person’s own immune system is mistakenly attacking the lining of the smallest blood vessels in the brain, the retina of the eye and inner ear. Susac syndrome is a rare autoimmune disorder characterized by a triad of symptoms involving encephalopathy, monocular visual loss (due to underlying branch retinal artery occlusions), and hearing loss. The May 21, 2014 · The diagnosis of Susac syndrome was made in the period after the patient had given birth, when she developed BRAO in addition to the hearing loss. Susac’s syndrome (SS) is a triad of encephalopathy, branch retinal artery occlusion (BRAO), and sensorineural hearing loss as a result of microvascular occlusions of the brain, retina, and inner ear. SS is characterized by typic … Conclusions: Susac syndrome patients are frequently misdiagnosed at initial presentation, de-spite many having symptoms or radiological features that are red flags for the diagnosis. As a result, patients usually experience symptoms such as hearing loss, vision disturbances, and chronic headaches. Mar 21, 2022 · Susac syndrome is a rare form of micro-angiopathy and is thought to be an autoimmune endotheliopathy, with CD8 + T cells implicated in its pathogenesis [1]. Many patients achieve remission with appropriate therapy, though some may experience permanent deficits. Abstract Background: 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. Investigating the pathophysiology of Susac syndrome Improving the diagnosis of Susac syndrome, in particular establishing consensus criteria for the diagnosis. These three events define Susac syndrome and can Susac syndrome features brain encephalopathy, retinal artery occlusion, and hearing loss, mainly in women. SS is frequently misdiagnosed and probably underdiagnosed. Imaging tests, such as magnetic resonance imaging (MRI) or computed tomography (CT), are used to help diagnose the condition and better understand the Sep 12, 2025 · A definite diagnosis of Susac syndrome requires fulfillment of all three criteria and their subcriteria, in other words, 1-i, 1-ii, 2-ii, 3-i, and 3-ii. The condition is characterized by three main symptoms: brain disease (encephalopathy), hearing loss, and vision loss. Onset occurs at mean age 31. Diagnosis is based on the presence of the clinical triad of central nervous system dysfunction, branch retinal artery occl … Susac syndrome patients are frequently misdiagnosed at initial presentation, despite many having symptoms or radiological features that are red flags for the diagnosis. Susac syndrome is considered rare, but the National Organization of Rare Disorders says the true frequency is likely unknown due to misdiagnosis. Symptoms include episodes with headache, encephalopathic symptoms, focal neurologic deficits, sudden hearing loss for middle and low Feb 16, 2024 · Susac syndrome (SuS) is an orphan microangiopathic disease characterized by a triad of encephalopathy, visual disturbances due to branch retinal artery occlusions, and sensorineuronal hearing loss. Establishment of the diagnosis is often delayed because the triad is complete only in a minority of patients at disease onset. Aug 22, 2023 · Susac syndrome is a likely autoimmune microangiopathy affecting the brain, retina and inner ear. We describe a 51-year-old woman with symptoms, signs Diagnosis Susac syndrome: challenges in the diagnosis and treatment. Typical MRI findings of callosal and peri-callosal Susac Syndrome is a rare autoimmune disorder that affects multiple body systems. Susac syndrome (retinocochleocerebral vasculopathy) is a syndrome of unknown pathogenesis. Oct 28, 2025 · Background Susac syndrome is a rare autoimmune endotheliopathy, with a few hundred cases reported. Introduction Susac’s Syndrome is a rare immune-mediated microangiopathic disease affecting arterioles of the cochlea, brain, and retina, which manifest as a triad of hearing loss, encephalopathy, and visual changes. Diagnosing Susac syndrome may be extremely challenging not only due to its rarity, but also due to the variability of its clinical presentation. Because of its rarity and some similarities to other common neurological Susac’s syndrome (SS) is a relatively rare cause of multiple recurrent branch retinal arterial occlusions (BRAO). Detailed overview of Susac's Syndrome, a rare autoimmune disorder affecting brain, retina, and inner ear. Recent updates to diagnostic criteria and treatment guidelines have been made. Treating symptoms early may help prevent permanent damage. The syndrome often presents diagnostic challenges due to its overlapping symptoms with other conditions. It mainly affects young women. A positive finding of Susac's syndrome is based on identifying symptoms and the findings of diagnostic tests. Learn about Susac's Syndrome, a rare autoimmune condition affecting the brain, eyes, and ears. While acute SuS is well-described, long-term cognitive outcomes with current treatments are underknown. eusac. Symptoms vary from person to person and typically correspond to the area of the body affected. Long term follow up on patients with respect to prognosis and outcome of Susac syndrome Biobanking for scientific evaluation of diagnostic and prognostic markers The prognosis of Susac Syndrome varies among individuals, with some experiencing spontaneous recovery while others may face long-term neurological deficits. It affects middle-aged women and is probably underdiagnosed due to National Center for Biotechnology Information Feb 16, 2024 · Abstract Susac syndrome (SuS) is an orphan microangiopathic disease characterized by a triad of en-cephalopathy, visual disturbances due to branch retinal artery occlusions, and sensorineuronal hearing loss. Susac Syndrome is a rare auto-immune endotheliopathy that rarely presents with the full triad of branched retinal artery occlusion, encephalopathy and sensorineural hearing loss. Our objective was to Background and objectives: Susac syndrome (SuS) is a rare disorder characterized by encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss, often accompanied by vertigo. It is characterized by a triad of symptoms: encephalopathy (brain dysfunction), hearing loss, and vision problems. net), we would like to draw attention to another interdisciplinary, albeit rare, differential diagnosis namely the Susac syndrome. Aug 23, 2023 · Susac syndrome is a likely autoimmune microangiopathy affecting the brain, retina and inner ear. Marrodan M, Fiol MP, Correale JBrain 2022 Apr 29;145 (3):858-871. Apr 1, 2022 · The authors also name rarer phenomena, such as visual snow syndrome and posterior reversible encephalopathy syndrome (PRES). This article aims to provide an insight into the pathophysiology, clinical features, diagnosis and management of Susac syndrome. Diagnosis is based on the clinical presentation, brain magnetic resonance imaging, retinal fluorescein a … Jun 1, 2022 · Susac syndrome is a rare disease characterized by an inflammatory microangiopathy limited to the brain, eye, and ear vessels. PMID: 35136969 Heritable and non-heritable uncommon causes of stroke. 1093/brain/awab476. Nov 22, 2024 · ABSTRACT Background: Susac syndrome is a rare autoimmune microangiopathy that afects the small vessels of the retina, brain, and inner ear, leading to a characteristic triad of encephalopathy, branch retinal artery occlusions, and sensorineural hearing loss. This complex syndrome can significantly impact an individual’s quality of life, making early diagnosis and treatment essential. Susac syndrome (SS) is defined as an infrequent neurological disorder characterized by the clinical triad of encephalopathy, branch retinal artery occlusions (BRAOs), and hearing loss, resulting from autoimmune-mediated microangiopathy that affects the microvasculature of the brain, retina, and inner ear. Diagnosed with MRI, retinal angiography, and audiometry. Delayed diagnosis can lead to patient morbidity. The three main characteristics are encephalopathy, partial or complete branch retinal artery occlusion, and low-frequency hearing loss. Susac syndrome Susac syndrome is a rare autoimmune disorder characterized by the clinical triad of encephalopathy, sensorineural hearing loss, and visual disturbance resulting from branch retinal artery occlusion (BRAO) 1. However, patients can present with an incomplete clinical or neuroimaging triads making diagnosis difficult in certain Jan 17, 2023 · Introduction Susac syndrome is a rare autoimmune condition characterized by three main symptoms: altered brain function (encephalopathy), blockage of retinal arteries, and hearing impairment. Our previous systematic review on all cases of SuS We propose diagnostic criteria for Susac syndrome that may help both experts and physicians not familiar with Susac syndrome to make a correct diagnosis and to prevent delayed treatment initiation. Despite this being an extremely rare BRAO is the classic ophthalmological manifestation of Susac syndrome, and sensorineural hearing loss is the predominant vestibular-cochlear finding. Review symptoms, causes, and treatment options. Additional neurological symptoms, which may mimic MS, can include problems with walking, urinary dysfunction, muscle pain, slurred speech and cognitive issues such as memory loss, confusion and, potentially Susac syndrome (SuS) is an orphan microangiopathic disease characterized by a triad of encephalopathy, visual disturbances due to branch retinal artery occlusions, and sensorineuronal hearing loss. Diagnosis Diagnosis of Susac's syndrome usually begins with a physical exam and a review of the patient's medical history and symptoms. Know the causes, symptoms, treatment of Susac Syndrome. Its pathogenesis involves small vessel vasculitis affecting the cerebrum, retina, and cochlea, primarily seen in young women. Owing to the variable presentation and rarity of the Susac syndrome is an autoimmune condition that affects the very small blood vessels in the brain, retina, and inner ear (cochlea). This syndrome is significant not only because of its unique presentation but also due to its Apr 29, 2022 · Susac syndrome is a disorder thought to be mediated by an autoimmune response towards endothelial cells, leading to a characteristic clinical triad of encephalopathy, visual disturbances due to branch arterial occlusions and sensorineural hearing impairment. Jun 1, 2022 · Susac syndrome is a rare disease characterized by an inflammatory microangiopathy limited to the brain, eye, and ear vessels. AI generated definition based on: Autoimmunity Reviews, 2014 Mar 9, 2022 · Background and purpose Susac syndrome (SuS) is an inflammatory condition of the brain, eye and ear. Susac, has been an advancing area of clinical Jan 10, 2018 · Abstract Susac syndrome is an immune-mediated, pauci-inflammatory, ischemia-producing, occlusive microvascular endotheliopathy/basement membranopathy that affects the brain, retina, and inner ear. With the advancement of research in immunology and neurology, there is an increasing focus on identifying specific biomarkers and targeted therapies that can potentially manage the Susac Syndrome is a rare triad of encephalopathy, sensorineural hearing loss, and retinal microangiopathy. 5:1 female:male occurrence). Apr 7, 2025 · Objective: To demonstrate a unique presentation of probable Susac Disease in a patient with concomitant large vessel involvement Background: Susac syndrome (SuS) is a rare autoimmune vasculo-occlusive disease that affects the microvasculature of the Jul 26, 2025 · Once a diagnosis of Susac syndrome is established and appropriate treatment is initiated, 89% and 83% of patients achieve durable clinical control without relapses at 1 and 3 years, respectively. Susac syndrome was diagnosed in 10 patients (age range 30-45 years). May 2, 2022 · Susac syndrome is an autoimmune condition that affects the very small blood vessels in the brain, retina, and inner ear (cochlea). We assessed ten SuS patients treated in accordance with evidence-based guidelines using immunotherapies targeting humoral and cell-mediated pathways Three of the 19 patients (16%) with probable Susac syndrome developed further symptoms and subsequently would have met the criteria for definite Susac syndrome but were misdiagnosed prior to the final diagnosis of Susac syndrome. It is an autoimmune-mediated microangiopathy (precapillary arterioles) that affects the microvasculature of the brain, retina, and inner ear [1]. Based on these data, we Mar 18, 2025 · A definite diagnosis of Susac syndrome requires fulfillment of all three criteria and their subcriteria, in other words, 1-i, 1-ii, 2-ii, 3-i, and 3-ii. Treatment of Susac syndrome is particularly challenging. Susac syndrome occurs due to unknown causes, and the affected people show symptoms other than the main symptoms, including Abstract Susac's syndrome (SuS) is a rare, autoimmune disorder known as a typical triad of sensorineural hearing impairment, central nervous system involvement, and multiple branch retinal artery occlusions (BRAOs). This syndrome is The data recorded for all Susac syndrome patients treated at the Sheba Medical Center between 1998 and 2014 included demographics, clinical signs at presentation and during the disease course, imaging findings, treatment, and prognosis. Although the pathophysiology is not fully elucidated, recent advances favour a primitive vasculitis affecting the cerebral, retinal and cochlear small vessels. Susac syndrome, a rare autoimmune disorder first described as a classic triad (encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss) in 1979 by renowned physician John O. 1,2 It is an autoimmune vasculo-occlusive disease, in which the vascular endothelium is injured by circulating antibodies, and results in arterial microinfarcts affecting the central nervous system (CNS), retina, and inner ear. The purpose of this study is to examine the demographics, clinical characteristics, treatment, and long-term prognosis of Susac syndrome. Pathogenesis is unknown but proposed to be autoimmunity against the endothelium of capillaries with anti-endothelial cell antibodies present in ~25% of cases1, with preference for the pre-capillary arterioles2,3. Early diagnosis is important, and treatment requires immunosuppression. Due to the rarity of this condition, diagnosis and treatment can be challenging. It is also a disorder of autoimmune Susac's Syndrome (SuS) is a rare, immune-mediated disorder characterized by encephalopathy, branch retinal artery occlusions (BRAO), and sensorineural hearing loss, with an estimated 450 diagnosed cases worldwide. This study examines clinical manifestations; disease activity; and risk factors of disability, dependency, and return to work in patients with SuS. The condition is more common in women and typically presents Susac syndrome (SuS) is an orphan disease characterized by the triad of encephalopathy, branch retinal artery occlusions (BRAO), and sensorineural hearing loss. Susac's syndrome (retinocochleocerebral vasculopathy) is a very rare form of microangiopathy characterized by encephalopathy, branch retinal artery occlusions and hearing loss. Delayed diagnosis is associated with worse morbidity and mortality, and the most important predictor of long-term prognosis in the reported cases is the time to diagnosis. The varied ways in which SuS can present, and clinician failure to consider or rec … Susac syndrome is a rare but important differential diagnosis for a range of neurological and psychiatric presentations. The organs involved can easily become irreversibly dama … Sep 16, 2020 · ABSTRACT: Susac syndrome, a relatively rare autoimmune endotheliopathy affecting the brain, retina, and inner ear, is three times more common in women than in men. doi: 10. 6 and women are more often affected (3. 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. Clinical presentation Susac syndrome classically consists of a clinical triad of: Abstract 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). Although it is a rare disease, three reas … Nov 4, 2015 · Abstract 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). Dec 18, 2024 · Susac syndrome (SuS) is a rare disorder characterized by encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss, often accompanied by vertigo. We also reviewed the literature behind diagnosis and treatment. The condition is characterized by three main symptoms: Encephalopathy (for other causes of encephalopathy, read about Guillain-Barre syndrome) Sensorineural hearing loss Vision loss These symptoms usually are not present when the syndrome first occurs Susac Syndrome: Understanding a Rare Neurological Disorder Introduction Susac Syndrome is a rare and complex neurological disorder that primarily affects the brain, eyes, and inner ear. Jul 24, 2024 · Susac's syndrome (SuS) is a rare, autoimmune disorder known as a typical triad of sensorineural hearing impairment, central nervous system involvement, and multiple branch retinal artery occlusions (BRAOs). The diagnosis of Susac can be a challenge due to the variability in presentation, course and severity of disease. MRI confirmed the diagnosis. The exact aetiology is unknown. 1 Jul 24, 2024 · Abstract Susac's syndrome (SuS) is a rare, autoimmune disorder known as a typical triad of sensorineural hearing impairment, central nervous system involvement, and multiple branch retinal artery occlusions (BRAOs). This study examines clinical manifestations; disease activity; and risk factors of disability, dependency, and return to Background Susac syndrome is characterised by the triad of encephalopathy with or without focal neurological signs, branch retinal artery occlusions and hearing loss. John Susac first described it in 1979. It is characterized by a triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. Oct 1, 2025 · Susac syndrome, characterised by branch retinal artery occlusion, encephalopathy and sensorineural hearing loss, is a rare autoimmune condition which was first described by John Susac in 1979. 1 Diagnosis is based on clinical presentation, brain magnetic resonance imaging, retinal fluorescein angiography and audiometry. Aug 18, 2023 · What is Susac syndrome? Susac syndrome is an autoimmune condition where your immune system mistakenly attacks small blood vessels in your brain, eyes (retina) and inner ear (cochlea). Mar 18, 2025 · A definite diagnosis of Susac syndrome requires fulfilment of all three criteria and their subcriteria, in other words, 1-i, 1-ii, 2-ii, 3-i, and 3-ii. It is characterised by the presence of encephalopathy, retinal vaso-occlusive disease and hearing loss. Susac’s syndrome is a rare immune-mediated endotheliopathy that mainly affects young women. Symptoms of Susac’s Syndrome Symptoms and their severity can vary from person to person. A probable diagnosis of Susac syndrome can be made if 2 out of the three criteria are met. Diagnosis of Susac syndrome is often made on the basis of these symptoms, along with findings from brain magnetic resonance imaging (MRI), retinal angiography, and audiometry [4]. Apr 1, 2014 · 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. Apr 1, 2024 · Susac syndrome is a rare immune-mediated condition that affects the brain, eyes, and ears. MRI of the brain shows punctate T2 lesions in the supratentorial and infratentorial brain with a predilection for the corpus callosum where the presence of May 18, 2023 · Susac syndrome is a rare microangiopathy affecting the cerebral arteries, retina, and cochlea (also called retino-cochleo-cerebral dysimmune vasculopathy). What is the long-term outlook for patients with Susac syndrome? Susac syndrome (or SICRET syndrome) is characterized by small infarctions of the Cochlea, Retina and brain (Encephalon) that can be clinically seen as encephalopathy, loss or hearing, and vision loss/peripheral field loss due to branch retinal artery occlusions (BRAO). 3-5 CRITERIA FOR DISEASE DIAGNOSIS A study by the European Susac Consortium 6 Mar 13, 2013 · The diagnosis of Susac’s syndrome has been facilitated greatly by appreciation of distinctive magnetic resonance imaging (MRI) findings. What is Susac syndrome? Susac syndrome is a rare, autoimmune condition that affects the small blood vessels of the brain, retina and inner ear. Susac syndrome is a likely autoimmune microangiopathy affecting the brain, retina and inner ear. A Background and Objectives Susac syndrome (SuS) is a rare disorder characterized by encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss, often accompanied by vertigo. This study examines clinical manifestations; disease activity; and risk factors of disability, dependency, and return to Dec 22, 2021 · Introduction Susac syndrome is a rare disorder, thought to be caused by autoimmune-mediated occlusion of microvessels in the brain, retina and inner ear, which are responsible for the characteristic clinical triad of CNS dysfunction, visual disturbances caused by branch retinal artery occlusions (BRAO) and sensorineural hearing loss (SNHL). May 1, 2020 · Susac's syndrome is a rare immune-mediated endotheliopathy that mainly affects young women. Ophthalmic manifestations may be the sole presenting sign of SS. This leads to a critical delay in the initiation of appropriate treatment. If you or a loved one is affected by this condition, visit NORD to find resources and Jul 25, 2025 · Early diagnosis and aggressive treatment are essential to prevent permanent damage to the brain, retina, and cochlea. The pathology affects the small vessels of the brain, retina, and inner ear, and therefore the main symptoms of the disease include encephalopathy, visual disturbances, and sensorineural hearing loss. Understanding Susac Syndrome is crucial for both patients and healthcare providers, as Nov 22, 2024 · Susac syndrome is a rare autoimmune microangiopathy that affects the small vessels of the retina, brain, and inner ear, leading to a characteristic triad of encephalopathy, branch retinal artery occlusions, and sensorineural hearing loss. Based on these data, we Here we describe an uncommon recurrent stroke in a young female that unfolds to Susac syndrome at the end. Diagnosis can be challenging, and misdiagnosis is common. Nov 4, 2015 · 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 cause of this syndrome is still Introduction Susac Syndrome is a rare autoimmune condition characterized by the triad of encephalopathy, branch retinal artery occlusions (BRAO), and sensorineural hearing loss. Feb 16, 2024 · Susac syndrome is a rare and enigmatic complex neurological disorder primarily affecting small blood vessels in the brain, retina, and inner ear. Diagnosis is based on the presence of the clinical triad of central Jul 27, 2023 · Learn about Susac Syndrome, including symptoms, causes, and treatments. The encephalopathy is manifested by headache, motor deficiencies, sensor deficiencies, aphasia, cognitive impairment, and urinary insufficiency 2. The data recorded for all Susac syndrome patients Susac syndrome (SuS) is a rare disorder characterized by encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss, often accompanied by vertigo. Results An alternative diagnosis prior to SuS was made in 30 patients (94%) with seven patients receiving two May 1, 2022 · S usac syndrome is a rare disease that commonly affects females and young patients. However, patients can present with an incomplete clinical or neuroimaging triads making diagnosis difficult in certain situations. [1] 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. Symptoms include severe headaches, cognitive issues, visual disturbances, hearing loss, and dizziness. It is characterized by the clinical triad of acute or subacute encephalopathy, sensorineural hearing loss, and retinal branch artery occlusions. It is commonly misdiagnosed, which is of critical relevance given the importance of prompt treatment on long-term outcomes. Methods This is a retrospective review of the medical records of 32 adult patients from an Australasian cohort of SuS patients. Clinical presentation Susac syndrome classically consists of a clinical triad of:. Disease presentation varies in symptoms and duration, including vertigo, hearing loss, visual disturbances, migraine-like headaches, and central nervous system dysfunction. Diagnosis is made via patient history, clinical presentation, and specialized tests including Jan 4, 2025 · The Susac syndrome is a microangiopathy causing small infarcts in the brain, cochlea, and retina. 1,2 Typically, it is the combination of encephalopathy, branch retinal artery occlusions, and sensorineural hearing loss. The hearing loss is Background: 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. Sep 12, 2025 · A definite diagnosis of Susac syndrome requires fulfilment of all three criteria and their subcriteria, in other words, 1-i, 1-ii, 2-ii, 3-i, and 3-ii. Though it can affect anyone, it commonly affects women in their prime ages. It is usually misdiagnosed or underdiagnosed because its symptoms may vary at the presentation time. Symptoms of Susac syndrome can mimic those of other inflammatory diseases, such as multiple sclerosis, acute disseminated encephalomyelitis, encephalitis, and vasculitis. The illness tends to be monophasic, and to our knowledge, recurrence after years of remission has not been reported. Diagnosis relies on the recognition of the triad including: 1/subacute Susac’s Syndrome is a rare autoimmune disorder that affects the very small blood vessels in the brain, retina, and inner ear. Dec 19, 2024 · Susac syndrome is a rare autoimmune disorder where the immune system mistakenly attacks small blood vessels in the brain, retina, and inner ear, leading to reduced blood flow and significant neurological and sensory impairments. In most cases, headaches (including migraine-like headaches) may be the first sign of the disease. It primarily targets the small blood vessels in the brain, retina, and inner ear. Susac syndrome is a rare autoimmune disorder where the immune system attacks blood vessels in the brain, eyes, and inner ear, causing various symptoms. Diagnosis is based on the presence of the clinical triad of central nervous system dysfunction, branch retinal artery occlusions and sensorineural hearing loss. As members of the European Susac Consortium (EUSAC; www. Although neuropsychiatric manifestations are common, they remain poorly characterised. Diagnosis relies on the recognition of the triad including: 1/subacute Jan 31, 2023 · Susac syndrome is a rare connective tissue disorder. Susac syndrome is a rare inflammatory vascular endotheliopathy affecting the small vessels of the brain, eye, and ear. Diagnosis is based on the clinical presentation, The delay to diagnosis of Susac syndrome was influenced by extent of brain, eye and ear involvement at presentation with the fastest diagnosis in patients with brain only involvement, and the slowest diagnosis in those with brain and eye involvement. Aug 15, 2024 · Susac syndrome (SuS) presents with encephalopathy, visual disturbances, and hearing loss from immune-mediated microvascular occlusion. ziwku wsq pg2t gf0qq gutiqw 8nsqy gyp l0x7 itoo 547ck