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Korean Journal of Ophthalmology : KJO Feb 2014
Topics: Female; Humans; Retinal Artery Occlusion; Sneddon Syndrome; Visual Acuity
PubMed: 24505209
DOI: 10.3341/kjo.2014.28.1.113 -
Acta Neurologica Belgica Jun 2021
Topics: Adult; COVID-19; Female; Humans; SARS-CoV-2; Susac Syndrome
PubMed: 33236280
DOI: 10.1007/s13760-020-01554-5 -
The Pan African Medical Journal 2019Susac syndrome is an autoimmune endothelopathy that affects precapillary arterioles of the brain, retina and inner ear. We report for the first time observations of two...
Susac syndrome is an autoimmune endothelopathy that affects precapillary arterioles of the brain, retina and inner ear. We report for the first time observations of two patients with Susac syndrome in Senegal.
Topics: Adult; Brain; Ear, Inner; Female; Humans; Male; Middle Aged; Retinal Vessels; Senegal; Susac Syndrome
PubMed: 31558942
DOI: 10.11604/pamj.2019.33.145.17954 -
Arquivos Brasileiros de Oftalmologia 2014We report a case of a 19-year-old woman presenting bilateral neurosensorial hearing loss, mental abnormalities, and loss of visual field in the left eye. Visual acuity...
We report a case of a 19-year-old woman presenting bilateral neurosensorial hearing loss, mental abnormalities, and loss of visual field in the left eye. Visual acuity was 20/20 in OD and 20/25 in OS. Patient was examined systemically. Audiometry showed sensorineural hearing loss in both ears. The magnetic resonance imaging (MRI) of brain revealed multiple small lesions in the white matter in both cerebral hemispheres and at the corpus callosum. Fundoscopy showed bilateral normal optic disc and sheathing of the arterioles in the middle periphery of OD. Retinal edema and cotton-wool spots were observed. Fluorescein angiography showed bilateral peripheral occlusive arterial vasculopathy. The patient was diagnosed with Susac syndrome and treated with quetiapine fumarate, flunitrazepam, and prednisone, which resulted in stabile outcome. This case shows that a high index of suspicion leading to early recognition and treatment is important to avoid irreversible damage.
Topics: Audiometry; Female; Fluorescein Angiography; Hearing Loss, Bilateral; Humans; Magnetic Resonance Imaging; Susac Syndrome; Visual Acuity; Young Adult
PubMed: 25295909
DOI: 10.5935/0004-2749.20140048 -
Journal of Neurochemistry Jul 2018It is widely accepted that development of autoimmunity in the central nervous system (CNS) is triggered by autoreactive T cells, that are activated in the periphery and... (Review)
Review
It is widely accepted that development of autoimmunity in the central nervous system (CNS) is triggered by autoreactive T cells, that are activated in the periphery and gain the capacity to migrate through endothelial cells at the blood-brain barrier (BBB) into the CNS. Upon local reactivation, an inflammatory cascade is initiated, that subsequently leads to a recruitment of additional immune cells ultimately causing demyelination and axonal damage. Even though the interaction of immune cells with the BBB has been in the focus of research for many years, the exact mechanisms of how immune cells enter and exit the CNS remains poorly understood. In this line, the factors deciding immune cell entry routes, lesion formation, cellular composition as well as distribution within the CNS have also not been elucidated. The following factors have been proposed to represent key determinants for lesion evaluation and distribution: (i) presence and density of (auto) antigens in the CNS, (ii) local immune milieu at sites of lesion development and resolution, (iii) trafficking routes and specific trafficking requirements, especially at the BBB and (iv) characteristics and phenotypes of CNS infiltrating cells and cell subsets (e.g. features of T helper subtypes or CD8 cells). The heterogeneity of lesion development within inflammatory demyelinating diseases remains poorly understood until today, but here especially orphan inflammatory CNS disorders such as neuromyelitis optica spectrum disorder (NMOSD), Rasmussen encephalitis or SUSAC syndrome might give important insights in critical determinants of lesion topography. Finally, investigating the interaction of T cells with the BBB using in vitro approaches or tracking of T cells in vivo in animals or even human patients, as well as the discovery of lymphatic vasculature in the CNS are teaching us new aspects during the development of CNS autoimmunity. In this review, we discuss recent findings which help to unravel mechanisms underlying lesion topography and might lead to new diagnostic or therapeutic approaches in neuroinflammatory disorders including multiple sclerosis (MS).
Topics: Animals; Autoimmunity; Blood-Brain Barrier; Demyelinating Autoimmune Diseases, CNS; Humans
PubMed: 29574788
DOI: 10.1111/jnc.14339 -
Clinical Rheumatology Jun 2023COVID-19 vaccine circulation approval was a turning point for the coronavirus pandemic. The current approved COVID-19 vaccines, including messenger ribonucleic acid... (Review)
Review
COVID-19 vaccine circulation approval was a turning point for the coronavirus pandemic. The current approved COVID-19 vaccines, including messenger ribonucleic acid (mRNA)-based and adenovirus vector-based vaccines, were shown to significantly reduce the disease mortality and severity, and its adverse reactions are mainly mild ones. However, few cases of autoimmune conditions, both flare-ups and new-onset, were described in association with these vaccines. Susac vasculitis (SaS) is a rare autoimmune disease characterized by the clinical triad of encephalopathy, visual disturbances, and sensorineural hearing loss. Its pathogenesis is still not fully understood but is believed to be related to autoimmune processes, including autoantibodies to anti-endothelial cells and cellular immune processes that lead to microvascular damage and, consequently, micro-occlusions of the cerebral, inner ear, and retinal vessels. It has been previously described following vaccination and, most recently, few cases following coronavirus vaccines. We here describe a case of a previously healthy 49-year-old man diagnosed with SaS 5 days following the first dose of the BNT162b2 COVID-19 vaccine.
Topics: Humans; Male; Middle Aged; BNT162 Vaccine; COVID-19; COVID-19 Vaccines; Magnetic Resonance Imaging; Susac Syndrome; Vaccination
PubMed: 36877303
DOI: 10.1007/s10067-023-06564-1 -
Frontiers in Neurology 2022Susac syndrome (SuS) is a rare neuroinflammatory disease that manifests with a triad of hearing loss, branch retinal artery occlusions, and encephalopathy. Patients with...
Susac syndrome (SuS) is a rare neuroinflammatory disease that manifests with a triad of hearing loss, branch retinal artery occlusions, and encephalopathy. Patients with SuS are frequently misdiagnosed because the clinical trial is incompletely present at disease onset. In this report, we present a case of a 29-year-old man manifesting sleepiness, epilepsy, urinary dysfunction, and hemiparesis at the initial stage. Magnetic resonance imaging (MRI) revealed multiple abnormal signals located in the lateral paraventricular, corpus callosal, and pons. In addition, the patient had sustained elevation of CSF pressure and protein. ADEM was considered according to the clinical and radiographic findings. However, symptoms were not significantly improved after methylprednisolone therapy. He showed a vision decline in the third month after the disease onset. It was considered from intracranial hypertension or optic neuritis, and therefore retinal arteriolar impairment was ignored. As the disease progresses, cognitive decline was presented. Brain MRI exhibits multiple significant hyperintensities on the DWI sequence with speck-like gadolinium enhancement. Thus, PACNS was diagnosed. The SuS was not made until the presence of hearing decline in the 4 months after the disease onset. The case will be helpful for clinicians to better recognize the atypical initial manifestation of SuS.
PubMed: 36570458
DOI: 10.3389/fneur.2022.1055038 -
The Primary Care Companion For CNS... 2012
PubMed: 23251866
DOI: 10.4088/PCC.12l01350 -
Indian Journal of Ophthalmology Sep 2016Susac syndrome (SS) is a rare retinal-cochlear-cerebral disease with an unclear etiology. A 35-year-old man presented with sudden painless vision loss in the right eye...
Susac syndrome (SS) is a rare retinal-cochlear-cerebral disease with an unclear etiology. A 35-year-old man presented with sudden painless vision loss in the right eye and 2 months later in the left eye with hemiparesis, behavioral changes, and hearing loss. Ophthalmic examinations revealed multiple branch retinal artery occlusions (BRAOs) in both eyes. Brain magnetic resonance imaging showed inflammatory changes with multiple "punched-out" lesions in the corpus callosum which confirmed the diagnosis of SS. Despite intravenous and oral corticosteroid therapy, the disease progressed with the development of new BRAOs, low vision in both eyes, and disability. Prompt diagnosis and early treatment may save the vision and even patient's life.
Topics: Administration, Oral; Adult; Disability Evaluation; Disease Progression; Drug Combinations; Fluorescein Angiography; Glucocorticoids; Humans; Injections, Intravenous; Magnetic Resonance Imaging; Male; Methylprednisolone; Prednisolone; Retinal Artery Occlusion; Susac Syndrome; Vision, Low; Visually Impaired Persons
PubMed: 27853020
DOI: 10.4103/0301-4738.194334 -
Neurological, otolaryngological and ophthalmological implications of Susac syndrome - a case report.European Review For Medical and... Jul 2016Susac syndrome is an endotheliopathy affecting the arterioles of the brain, retina, and inner ear. Many cases of Susac syndrome are underdiagnosed, mainly at the early...
Susac syndrome is an endotheliopathy affecting the arterioles of the brain, retina, and inner ear. Many cases of Susac syndrome are underdiagnosed, mainly at the early stages of the disease, while prompt diagnosis enables a speedy recovery. Immediate treatment can halt disease progression and even prevent future disability. We report a case of Susac syndrome, describe the difficulties in the diagnosis of this case, and include a detailed history of a 35-year-old man via the presentation of extensive laboratory work-up and imaging studies. Audiometry showed sensorineural hearing loss of about 75 dB in the left ear. Ovoid lesions of the corpus callosum in magnetic resonance (MR) were present as were advanced binocular ophthalmological changes in fluorescent angiography. Methylprednisolone with acetylsalicylic acid and intravenous immunoglobulin (IVIG) were implemented with a positive outcome (clinical and audiometric improvement).
Topics: Adult; Aspirin; Hearing Loss, Sensorineural; Humans; Immunoglobulins, Intravenous; Magnetic Resonance Imaging; Male; Methylprednisolone; Susac Syndrome
PubMed: 27460737
DOI: No ID Found