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Neurology India 2019
Topics: Brain; Female; Humans; Magnetic Resonance Imaging; Retinal Artery Occlusion; Susac Syndrome; Young Adult
PubMed: 31512675
DOI: 10.4103/0028-3886.266278 -
Journal of Ophthalmic Inflammation and... Oct 2020Susac syndrome is a rare microangiopathy of suspected autoimmune origin affecting arteries of the retina, the cochlea and the brain. The aim of the study was to give a... (Review)
Review
BACKGROUND/PURPOSE
Susac syndrome is a rare microangiopathy of suspected autoimmune origin affecting arteries of the retina, the cochlea and the brain. The aim of the study was to give a review of the disease entity and determine the proportion of cases and their characteristics in a uveitis referral centre.
PATIENTS AND METHODS
Charts of patients with the diagnosis of Susac syndrome seen in the Uveitis Clinic of the Centre for Ophthalmic Specialised Care (COS), Lausanne, Switzerland were reviewed retrospectively to determine the frequency of such cases in a uveitis referral centre. Clinical symptoms and signs, functional data, imaging signs and evolution were analysed in the 3 COS cases and one case shared with the Uveitis Clinic of the Department of Ophthalmology, University of Innsbruck, Austria. Characteristic signs were searched possibly allowing a prompt diagnosis.
RESULTS
During the period from 1994 to 2019 (24 years, 2045 patients), 3 charts with the diagnosis of Susac syndrome were found (0.15%). The whole collective, including the additional case, comprised three women aged 28, 32 and 63 at presentation and one man, aged 42. None of the 3 cases that were referred were diagnosed beforehand. The characteristic item found in all 4 cases was the abrupt arterial stop or segmental interruption of arteries and increased staining of arterial wall on angiography more clearly shown on indocyanine green angiography that can potentially be proposed as a crucial diagnostic element. All 4 cases responded to dual steroidal and non-steroidal immunosuppression. Under treatment, all four patients did not show any further evolution.
CONCLUSION
Susac syndrome is a multilocation arteritis of the head that can involve the eye, ear and brain often first diagnosed by the ophthalmologist. The diagnosis is rapidly reached in uveitis referral centres but seems to be missed otherwise, A helpful angiographic sign to be searched is an abrupt or segmental arterial stop and increased staining of the arterial wall more clearly seen on indocyanine green angiography. Patients often present first to the ophthalmologist who should be acting as a whistleblower to avoid severe involvement of the brain.
PubMed: 33125601
DOI: 10.1186/s12348-020-00217-z -
Otolaryngologia Polska = the Polish... Jan 2023Susac syndrome is a rare connective tissue disorder. The pathology affects the small vessels of the brain, retina, and inner ear, and therefore the main symptoms of the... (Review)
Review
Susac syndrome is a rare connective tissue disorder. 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. The aim of this article is to review the current medical knowledge on Susac syndrome and to present our clinical experience regarding this disease entity. In the paper, we are also presenting a case of a 25-year-old patient who was diagnosed with Susac syndrome on the basis of clinical presentation and additional test results. Susac syndrome should be differentiated from multiple sclerosis and other causes of multifocal brain damage as early diagnosis and treatment play a key role in later prognosis.
Topics: Humans; Adult; Susac Syndrome; Retinal Artery Occlusion; Magnetic Resonance Imaging; Brain Diseases; Brain
PubMed: 37772321
DOI: 10.5604/01.3001.0016.2288 -
Medicine Jan 1998Susac syndrome is an occlusive arteriolar disease that provokes infarcts in the cochlea, retina, and brain of young subjects, mostly women. Its cause is unknown. Some... (Review)
Review
Susac syndrome is an occlusive arteriolar disease that provokes infarcts in the cochlea, retina, and brain of young subjects, mostly women. Its cause is unknown. Some infarcts may be asymptomatic and only revealed by ancillary investigation: 1) audiogram that shows bilateral sensorineural hearing loss predominating on low frequencies, 2) funduscopy and fluorescein retinal angiography demonstrating bilateral distal branch retinal artery occlusions, and 3) brain MRI T2-weighted images disclosing small multifocal hyperintensities in white and gray matter. Treatment options are not codified, ranging from antithrombotic drugs to immunomodulatory therapy. Course is self-limited after an active fluctuating phase. Dementia, blindness, and deafness are rare late sequelae, and half of patients return to normal life.
Topics: Adult; Anti-Inflammatory Agents; Audiometry, Pure-Tone; Brain Diseases; Cyclophosphamide; Diagnosis, Differential; Drug Therapy, Combination; Female; Fluorescein Angiography; GABA Agents; Hearing Loss, Sensorineural; Humans; Magnetic Resonance Imaging; Methylprednisolone; Prednisone; Retinal Artery Occlusion; Syndrome; Valproic Acid
PubMed: 9465860
DOI: 10.1097/00005792-199801000-00002 -
Acta Otorhinolaryngologica Italica :... Dec 2018Susac syndrome, a rare autoimmune disorder first described as a classic triad (encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss) in 1979... (Review)
Review
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. Susac, has been an advancing area of clinical interest and scientific research over the last several decades. This comprehensive review aims to succinctly highlight the breadth and detail of this enigmatic disease, with a primary focus on otologic manifestations. Topics discussed include epidemiology, pathophysiology, clinical manifestations, differential diagnoses, classification schema, laboratory investigations, characteristic audiometric findings, high-yield radiographic imaging, temporal bone histopathology, treatment strategies and overall prognosis.
Topics: Ear Diseases; Humans; Susac Syndrome
PubMed: 30623900
DOI: 10.14639/0392-100X-2166 -
Rheumatology Advances in Practice 2022
PubMed: 35356386
DOI: 10.1093/rap/rkac020 -
Susac syndrome and pregnancy: a review of published cases and considerations for patient management.Therapeutic Advances in Neurological... 2021Susac syndrome (SuS) is a rare autoimmune endotheliopathy leading to hearing loss, branch retinal artery occlusions and encephalopathy. Young females are more frequently... (Review)
Review
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. We reviewed published cases on SuS during pregnancy or in the postpartum period, and selected 27 reports describing the details of 33 patients with SuS. Treatment options and implications for pregnancy and breastfeeding are discussed. We propose new areas for research and suggest a management strategy.
PubMed: 33796140
DOI: 10.1177/1756286420981352 -
Journal of Neuroimmunology Jun 2022Susac syndrome is an immune-mediated microvascular disease characterized by the clinical triad of acute multiple encephalopathies, branch retinal artery occlusion, and... (Review)
Review
Susac syndrome is an immune-mediated microvascular disease characterized by the clinical triad of acute multiple encephalopathies, branch retinal artery occlusion, and sensorineural hearing loss. However, the typical clinical triad is not seen in all patients at disease onset. In this study, a 29-year-old male was admitted to our hospital due to aggravation of headache accompanied by retarded reaction. After treatment for a diagnosis of possible central nervous system vasculitis, the patient's retarded reaction and neurological dysfunction were improved. One year after discharge, the patient had no abnormal clinical symptoms and he discontinued taking prednisone voluntarily five months after discharge. Two years later, the patient was admitted to our hospital again owing to a sudden visual field defect in the superonasal quadrant of the left eye for one week, and Susac syndrome was diagnosed. After treatment, the patient's condition became stabilized with no further progress, but the visual field defect did not recover. At the onset of Susac syndrome, the typical clinical triad of Susac syndrome is rare, so this disease is difficult to be recognized at the beginning. The case we report presented the clinical triad two years after the disease onset. We expect that this case report will increase physicians' understanding of Susac syndrome.
Topics: Adult; Humans; Magnetic Resonance Imaging; Male; Retinal Artery Occlusion; Susac Syndrome; Vasculitis, Central Nervous System; Vision Disorders
PubMed: 35351321
DOI: 10.1016/j.jneuroim.2022.577822 -
European Review For Medical and... 2015Susac syndrome is an uncommon autoimmune microangiopathy characterized mainly by neurological disorders and, to date, 304 clinical cases have been described. The... (Review)
Review
Susac syndrome is an uncommon autoimmune microangiopathy characterized mainly by neurological disorders and, to date, 304 clinical cases have been described. The background of this syndrome is an immune-mediated endotheliopathy that affects the microvasculature of the brain, retina, and inner ear resulting in encephalopathy, hearing loss, and branch retinal artery occlusions. However, the cause and the pathogenesis of this microangiopathy remain unclear. Magnetic resonance imaging, retinal fluorescein angiography, and audiography findings enable the diagnosis of this syndrome. In this review, we have demonstrated the epidemiology and pathology of Susac syndrome with detailed description of clinical signs, diagnostic procedures and therapeutic possibilities.
Topics: Brain; Female; Fluorescein Angiography; Hearing Loss; Humans; Magnetic Resonance Imaging; Male; Retina; Susac Syndrome
PubMed: 26004617
DOI: No ID Found -
Practical Neurology Jun 2021
Topics: Humans; Magnetic Resonance Imaging; Retinal Artery Occlusion; Susac Syndrome
PubMed: 33541913
DOI: 10.1136/practneurol-2020-002908