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Otolaryngology--head and Neck Surgery :... Jun 1998
Topics: Adult; Cerebrovascular Disorders; Hearing Disorders; Heart Septal Defects, Atrial; Humans; Male; Retinal Artery Occlusion; Syndrome
PubMed: 9627253
DOI: 10.1016/S0194-5998(98)70285-2 -
Archivos de La Sociedad Espanola de... May 2022A 38-year-old man who attended the emergency department with headache, accompanied by vomiting, bradypsychia and gait instability, for which he was admitted to Neurology...
A 38-year-old man who attended the emergency department with headache, accompanied by vomiting, bradypsychia and gait instability, for which he was admitted to Neurology for study. During his admission, he began to present bilateral hearing loss and blurred vision in the left eye, with areas of arterial occlusion and hyperfluorescence of the arterial wall being observed in the ophthalmological examination. As a result, he was diagnosed with Susac syndrome. He was treated with systemic corticosteroids, as well as with rituximab and subsequently, with intravenous immunoglobulins and mycophenolate mofetil. The patient managed to preserve visual acuity, with gait instability and bilateral hearing loss as sequelae. Early diagnosis of Susac syndrome is important, because a delay in the start of treatment can lead to irreversible sequelae such as deafness, blindness or neurological involvement.
Topics: Adult; Early Diagnosis; Hearing Loss, Bilateral; Humans; Magnetic Resonance Imaging; Male; Susac Syndrome; Vision Disorders
PubMed: 35526953
DOI: 10.1016/j.oftale.2021.02.009 -
Lakartidningen Feb 2020Susac syndrome is an autoimmune disease characterized by the clinical triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss. It most... (Review)
Review
Susac syndrome is an autoimmune disease characterized by the clinical triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss. It most commonly affects young women. Susac syndrome is most likely underdiagnosed, not the least since only 13% have the clinical triad upon presentation. Many are misdiagnosed with multiple sclerosis or another neuroinflammatory entity. Susac syndrome is a microangiopathy affecting the precapillary arterioles causing infarcts of the brain, retina and inner ear. Beside the clinical symptoms, Susac syndrome is diagnosed by typical radiological features on magnetic resonance imaging and branch retinal artery occlusions, which are best evaluated using fluorescein angiography. Early diagnosis and correct immunosuppressive therapy are of utmost importance for clinical improvement and prevention of permanent disability. Diagnosis and treatment of Susac syndrome requires close cooperation between neurologists, radiologists, ophthalmologists and otorhinologists. Here, we present three cases and a review of the literature.
Topics: Brain; Female; Humans; Magnetic Resonance Imaging; Multiple Sclerosis; Retinal Artery Occlusion; Susac Syndrome
PubMed: 32045006
DOI: No ID Found -
Journal of the Neurological Sciences Jun 2007Susac's syndrome (SS) is an immune-mediated endotheliopathy that affects the microvasculature of the brain, retina, and inner ear. SS responds well to immunosuppressive... (Review)
Review
Susac's syndrome (SS) is an immune-mediated endotheliopathy that affects the microvasculature of the brain, retina, and inner ear. SS responds well to immunosuppressive therapies when treatment is prompt, aggressive, and sustained. Striking similarities exist between SS and dermatomyositis (DM), regarding immunopathogenesis, natural history, and treatment needs. We apply lessons learned from study of DM to SS, and offer our current treatment protocol for SS. Since these treatment guidelines are based mainly on anecdotal evidence, they represent only preliminary recommendations.
Topics: Autoimmune Diseases; Brain Diseases; Cerebrovascular Disorders; Endothelial Cells; Hearing Loss; Humans; Immunosuppressive Agents; Intracranial Arteriosclerosis; Microcirculation; Retinal Artery Occlusion; Syndrome
PubMed: 17324441
DOI: 10.1016/j.jns.2007.01.031 -
Iranian Journal of Neurology Oct 2014Susac's syndrome (SS) is a clinical triad of encephalopathy, branch retinal artery occlusion and sensorineural hearing loss and maybe due to an immune-mediated... (Review)
Review
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. Because of its rarity and some similarities to other common neurological conditions such as multiple sclerosis and acute disseminated encephalomyelitis, it is often misdiagnosed and therefore mistreated. To the best of our knowledge, there is only one case report from our country with this diagnosis. Here, we have a short discussion on this issue to introduce it to our colleagues and remind it as a differential diagnosis in patients with unexplained encephalopathy.
PubMed: 25632332
DOI: No ID Found -
Zhurnal Nevrologii I Psikhiatrii Imeni... 2019Susac syndrome (SS) is an extremely rare neurological disorder characterized by symptoms of a clinical triad: encephalopathy, retinopathy and hearing loss. These... (Review)
Review
Susac syndrome (SS) is an extremely rare neurological disorder characterized by symptoms of a clinical triad: encephalopathy, retinopathy and hearing loss. These problems arise from the microangiopathy of blood vessels that supply blood to the brain, the retina and the cochlea. SS is more common in young women. The authors present a literature review on the diagnosis and treatment of SS and the clinical observation of patients with classic signs of the disease triad.
Topics: Brain Diseases; Female; Humans; Retinal Diseases; Susac Syndrome; Vascular Diseases
PubMed: 31156242
DOI: 10.17116/jnevro20191192252 -
Clinical Psychopharmacology and... May 2023SARS-CoV-2 vaccines are not free of side effects and most commonly affect the central or peripheral nervous system (CNS, PNS). This narrative review aims to summarise... (Review)
Review
SARS-CoV-2 vaccines are not free of side effects and most commonly affect the central or peripheral nervous system (CNS, PNS). This narrative review aims to summarise recent advances in the nature, frequency, management, and outcome of neurological side effects from SARS-CoV-2 vaccines. CNS disorders triggered by SARS-CoV-2 vaccines include headache, cerebro-vascular disorders (venous sinus thrombosis [VST], ischemic stroke, intracerebral hemorrhage, subarachnoid bleeding, reversible, cerebral vasoconstriction syndrome, vasculitis, pituitary apoplexy, Susac syndrome), inflammatory diseases (encephalitis, meningitis, demyelinating disorders, transverse myelitis), epilepsy, and a number of other rarely reported CNS conditions. PNS disorders related to SARS-CoV-2 vaccines include neuropathy of cranial nerves, mono-/polyradiculitis (Guillain-Barre syndrome [GBS]), Parsonage-Turner syndrome (plexitis), small fiber neuropathy, myasthenia, myositis/dermatomyositis, rhabdomyolysis, and a number of other conditions. The most common neurological side effects are facial palsy, intracerebral hemorrhage, VST, and GBS. The underlying pathophysiology is poorly understood, but several speculations have been generated to explain the development of CNS/PNS disease after SARS-CoV-2 vaccination. In conclusion, neurological side effects develop with any type of SARS-CoV-2 vaccine and are diverse, can be serious and even fatal, and should be taken seriously to initiate early treatment and improve outcome and avoid fatalities.
PubMed: 37119215
DOI: 10.9758/cpn.2023.21.2.222 -
Autoimmunity Reviews 2014Susac syndrome (SS) is an autoimmune disease characterized by the clinical triad of encephalopathy, branch retinal artery occlusions and neuro-sensorial hearing loss; it... (Review)
Review
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 symptoms, may permit a diagnosis. Branch retinal artery occlusions (BRAOs) are best evaluated using fluorescein angiography (FA) which may show the typical multifocal fluorescence. SS is an autoimmune endotheliopathy that requires treatment with immunosuppressive agents: steroids, azathioprine, mycophenolate mofetil, methotrexate, cyclophosphamide and intravenous immunoglobulin, usually in combination. Plasma exchange is also useful. In addition, antiplatelet agents may be a useful adjunct. Correct immunosuppressive therapy results in significant clinical and radiological improvement. An early diagnosis and treatment are important to delay the disease progression and prevent permanent disability.
Topics: Brain; Hearing Loss; Humans; Magnetic Resonance Imaging; Retinal Artery Occlusion; Susac Syndrome
PubMed: 24424186
DOI: 10.1016/j.autrev.2014.01.038 -
Journal of the Neurological Sciences Jun 2007Susac's syndrome (SS) consists of the clinical triad of encephalopathy, branch retinal artery occlusions (BRAO) and hearing loss. It is due to a microangiopathy... (Review)
Review
Susac's syndrome (SS) consists of the clinical triad of encephalopathy, branch retinal artery occlusions (BRAO) and hearing loss. It is due to a microangiopathy affecting the precapillary arterioles of the brain, retina, and inner ear (cochlea and semicircular canals). Women are more commonly affected than men (3:1); the age of onset ranges from 9 to 58 years; but young women between the ages of 20 and 40 are most vulnerable. The encephalopathy is almost always accompanied by headache which may be the presenting feature. Multifocal neurological signs and symptoms, psychiatric disturbances, cognitive changes, memory loss, and confusion may rapidly progress to dementia. The MRI shows a distinctive white matter disturbance that always affects the corpus callosum. The central callosal fibers are particularly vulnerable and central callosal holes develop as the active lesions resolve. Linear defects (spokes) and rather large round lesions (snowballs) sometime dominate the MRI findings, which include cortical, deep gray (70%) and leptomeningeal involvement (33%). Frequently, the lesions enhance and may be evident on diffusion weighted imaging (DWI). The BRAO are best evaluated with fluorescein angiography, which may show the pathognomonic multifocal fluorescence. Gass plaques are frequently present and reflect endothelial damage. Brain biopsy shows microinfarction to be the basic pathology, but more recent pathological studies have shown endothelial changes that are typical for an antiendothelial cell injury syndrome. Elevated levels of Factor VIII and von Willebrand Factor Antigen reflect the endothelial perturbation. Despite extensive evaluations, a procoagulant state has never been demonstrated. SS is an autoimmune endotheliopathy that requires treatment with immunosuppressants: steroids, cyclophosphamide, and intravenous immunoglobulin, usually in combination. Aspirin is a useful adjunct.
Topics: Autoimmune Diseases of the Nervous System; Brain; Brain Diseases; Cerebrovascular Disorders; Endothelial Cells; Hearing Loss; Humans; Microcirculation; Retinal Artery Occlusion; Syndrome
PubMed: 17331544
DOI: 10.1016/j.jns.2007.01.036 -
Arquivos de Neuro-psiquiatria Dec 2020
Topics: Humans; Magnetic Resonance Imaging; Neuroimaging; Susac Syndrome
PubMed: 33263612
DOI: 10.1590/0004-282X20200106