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Journal of Clinical Neuroscience :... Oct 2022
Topics: Fluorescein Angiography; Humans; Magnetic Resonance Imaging; Retinal Artery Occlusion; Susac Syndrome
PubMed: 35932634
DOI: 10.1016/j.jocn.2022.07.023 -
Journal of Neuro-ophthalmology : the... Dec 2023
Topics: Humans; Child; Susac Syndrome; Magnetic Resonance Imaging; Retinal Artery Occlusion
PubMed: 35830697
DOI: 10.1097/WNO.0000000000001487 -
Indian Journal of Otolaryngology and... Jun 2022Susac Syndrome (SS) is a rare unique autoimmune underdiagnosed disorder characterized by the clinical triad of central nervous system (CNS) dysfunction, sensorineural...
Susac Syndrome (SS) is a rare unique autoimmune underdiagnosed disorder characterized by the clinical triad of central nervous system (CNS) dysfunction, sensorineural hearing impairment, and branch retinal artery occlusion (BRAO). The purpose of reporting the case is to succinctly highlight this enigmatic condition with focus on otological and audiological aspect of SS and also to augment awareness of this disease among otolaryngologists neurologists, psychiatrists and ophthalmologists. Additionally we reviewed the otological menifestations and audiological findings related to SS. A case of 38 year old female is presented with bilateral SNHL as the initial symptom of SS. The review of otological and audiological findings of previous 10 year case reports was executed using the PRISMA protocol on 20 cases. It was seen that inspite of the ear being one of the main organs involved in the SS very few had undergone detailed audiological assessment. Our review of literature showed that only 40% cases had done audiological assessment other than PTA, out of which only 5% had undergone complete audiological workup. 30% of the patients were having auditory symptoms as presenting chief complaint. Otolyryngologists should keep SS in differentials when a case of SNHL of unknown etiology is encountered.We emphasize the use newer noninvasive Powered by Editorial Manager® and ProduXion Manager® from Aries Systems Corporation diagnostic tools like Optical coherence tomography (OCT), MRI and detailed audiological tests for timely diagnosis of SS. Further multidisciplinary assessment protocols should be used to prevent the development of severe sequelae.
PubMed: 35813770
DOI: 10.1007/s12070-021-02853-w -
Deutsches Arzteblatt International Apr 2022
Topics: Brain; Humans; Magnetic Resonance Imaging; Susac Syndrome
PubMed: 35773987
DOI: 10.3238/arztebl.m2022.0059 -
Annals of Agricultural and... Jun 2022Susac's syndrome (SS) is a rare, autoimmune-mediated endoteliopathy characterized by a clinical triad of encephalopathy, branch retinal artery occlusion, and...
INTRODUCTION
Susac's syndrome (SS) is a rare, autoimmune-mediated endoteliopathy characterized by a clinical triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. SS is also characterized by a neuroimaging triad consisting of white matter lesions, grey matter lesions, and leptomeningeal enhancement on magnetic resonance imaging (MRI). Considering the rarity of SS, as well as certain similarity to other, more frequent neurological diseases, such as multiple sclerosis (MS), this syndrome is sometimes incorrectly diagnosed and treated.
OBJECTIVE
The aim of the study is to present the current state of knowledge on SS, with particular consideration for the differential diagnostics between SS and MS, using the latest available imaging techniques, such as brain MRI, optical coherence tomography (OCT), OCT angiography (OCTA) and fluorescein angiography (FA).
REVIEW METHODS
The major electronic databases (PubMed, Google Scholar) were searched manually in order to identify the relevant studies published on SS.
BRIEF DESCRIPTION OF THE STATE OF KNOWLEDGE
Distinguishing SS from MS is a diagnostic challenge. In the majority of cases, patients with SS do not present the complete clinical or neuroimaging triad, and a delay in making the correct diagnosis exposes the patient to the occurrence of complications, resulting from the development of the underlying disease, or/and the application of improper treatment. In the case of SS the results of brain MRI and FA are essential for making the correct diagnosis as they may reveal pathognomonic changes.
SUMMARY
Imaging examinations, such as brain MRI, FA, and OCT complement each other, due to which the diagnosis of SS may be simpler, irrespective of the stage of the disease.
Topics: Brain; Fluorescein Angiography; Humans; Magnetic Resonance Imaging; Retinal Artery Occlusion; Susac Syndrome
PubMed: 35767750
DOI: 10.26444/aaem/149954 -
Cureus May 2022Susac's syndrome (SS) is a rare, autoimmune-mediated vasculitis involving central nervous system (CNS) microvasculature, which typically targets the brain, retina, and...
Susac's syndrome (SS) is a rare, autoimmune-mediated vasculitis involving central nervous system (CNS) microvasculature, which typically targets the brain, retina, and cochlea. The disease pathology in these regions produces the characteristic triad of encephalopathy, visual loss, and hearing loss. Unfortunately, less than 20% of cases present as the full triad, often making diagnosis challenging. Diagnosis is also confounded by the similarity in the clinical presentation of multiple sclerosis (MS), with an overlap also seen in laboratory studies and radiographic imaging. In this report, we present a case of SS in a young and previously healthy adult male that was initially mistaken as MS. We review the characteristics of SS and highlight the key differences between the two diseases that can be used by diagnosing physicians. Lastly, we describe the treatment strategies involved in SS compared to MS.
PubMed: 35765402
DOI: 10.7759/cureus.25366 -
Case Report of Spinal Cord Involvement in Susac Syndrome Masquerading as a Multiple Sclerosis Mimic.The Neurohospitalist Jul 2022We present a unique case of a 59-year-old woman with atypical Susac syndrome and prominent involvement of the spinal cord. She initially presented with progressive...
We present a unique case of a 59-year-old woman with atypical Susac syndrome and prominent involvement of the spinal cord. She initially presented with progressive headaches, lower extremity weakness and hearing loss. Her MRI brain showed multiple enhancing lesions and her MRI spine showed a T8 enhancing lesion as well as a C2 cord infarct. She was treated with IV methylprednisolone with initial stabilization. However, she developed worsening encephalopathy and lower extremity weakness. Her repeat MRI brain showed new bilateral enhancing lesions and subacute infarcts. Given the increased burden of new lesions, she underwent a brain biopsy, which showed perivascular chronic inflammation within a small vessel distribution. Additionally, fluorescein angiography revealed bilateral branch retinal arterial occlusion (BRAO) and an audiogram demonstrated bilateral sensorineural hearing loss. She was diagnosed with Susac syndrome and treated with IV cyclophosphamide with improvement in her clinical exam. Spinal cord involvement is extremely rare for Susac syndrome, which commonly manifests as the classic triad of encephalopathy, BRAO and hearing loss. Her presentation with myelopathy highlights the importance of considering atypical presentations of well-established syndromes for optimal diagnosis and management.
PubMed: 35755220
DOI: 10.1177/19418744221097473 -
South Dakota Medicine : the Journal of... Mar 2022Susac syndrome (SS) is a rare clinical entity that affects primarily young women and might result in significant morbidity. The triad that leads to suspecting the...
Susac syndrome (SS) is a rare clinical entity that affects primarily young women and might result in significant morbidity. The triad that leads to suspecting the disease has classically been involvement of the brain, retina and inner ear. The likely pathology of the disease is thought to be immune mediated endotheliopathy; given its clinical and, possibly, pathological remission with immunosuppressive therapy. Here we describe an uncommon recurrent stroke in a young female that unfolds to Susac syndrome at the end. We also reviewed the literature behind diagnosis and treatment. 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. Therapies tried (with variable success) include corticosteroids, IVIG, plasmapheresis, cyclophosphamide, mycophenolate mofetil, and rituximab. The prognosis of SS is difficult to predict given the absence of strong clinical or radiographic features to suggest better/worse prognosis at the time of initial diagnosis. Brain MRIs and hearing/vision impairment have never normalized in previously studied cohort of patients.
Topics: Brain; Cyclophosphamide; Female; Hearing Loss; Humans; Magnetic Resonance Imaging; Stroke; Susac Syndrome
PubMed: 35708576
DOI: No ID Found -
BMJ Case Reports May 2022We report a case of Susac syndrome after SARS-CoV-2 infection and subsequent vaccination that presented with meningitis and retinal microembolisation in the form of...
We report a case of Susac syndrome after SARS-CoV-2 infection and subsequent vaccination that presented with meningitis and retinal microembolisation in the form of paracentral acute middle maculopathy (PAMM). After presenting with headache, fever and myalgia followed by scotomata, a woman in her 50s was hospitalised for meningitis; she had had mild COVID-19 infection 2 months prior to admission, having received the first vaccine dose 1 month prior to the neurological manifestation. Eye fundus examination and optical coherence tomography were suggestive of PAMM. D-dimer levels and erythrocyte sedimentation rate were elevated. Before infectious investigation results were available, she was started on empirical antibiotic and antiviral treatment. Having ruled out infectious causes, she was started on high-dose prednisolone. After 1 month, there was partial resolution of retinal lesions. This case highlights that exposure to SARS-CoV-2 antigen may be related to this rare syndrome; treatment with steroids may improve central and retinal impairment.
Topics: COVID-19; Female; Fluorescein Angiography; Humans; Macular Degeneration; Retinal Diseases; Retinal Vessels; SARS-CoV-2; Susac Syndrome; Tomography, Optical Coherence
PubMed: 35537770
DOI: 10.1136/bcr-2021-247159 -
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