Encephalopathy, hearing loss and retinal occlusions (Susac’s syndrome): a new case

1998 ◽  
Vol 19 (4) ◽  
pp. 225-227 ◽  
Author(s):  
M. Maddestra ◽  
S. Sabatini ◽  
A. Paci
Keyword(s):  
Hearing Loss ◽  
Susac’S Syndrome

Two Sisters with Leukoencephalopathy, Hearing Loss and Retinopathy: A Familial Case of Susac's Syndrome?

2014 ◽  
Vol 4 (36) ◽  
pp. 5818-5824
Author(s):  
Alberto Gajofatto ◽  
Francesca Gobbin ◽  
Silvio Piffer ◽  
Francesca Calabria ◽  
Stefano Forlivesi ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Familial Case ◽  
Susac’S Syndrome

Susac's syndrome: a rare cause of fluctuating sensorineural hearing loss

1997 ◽  
Vol 111 (11) ◽  
pp. 1072-1074 ◽  
Author(s):  
Neil D. Bateman ◽  
Ian J. M. Johnson ◽  
Kevin P. Gibbin
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Sensorineural Hearing ◽  
Clinical Severity ◽  
Susac’S Syndrome ◽  
Retinal Artery ◽  
Clinical Triad ◽  
First Time

AbstractSusac's syndrome is extremely rare and is characterized by the clinical triad of encephalopathy, retinal artery occlusion and deafness. A case of this rare syndrome is presented and for the first time the fluctuating nature of the hearing loss is demonstrated. The site and nature of the hearing loss has been investigated. The value of neural hearing loss as a method of monitoring the clinical severity is discussed.

scholarly journals SUSAC’S SYNDROME : 2 CASES WITHOUT HEARING LOSS

2015 ◽  
Vol 2 (5) ◽  
Author(s):  
Serkan Demir ◽  
Mehmet Guney Senol ◽  
Rifat Erdem Togrol ◽  
Ali Ayata ◽  
Eyup Duzgun
Keyword(s):  
Hearing Loss ◽  
Susac’S Syndrome

Susac’s syndrome: an update

2020 ◽  
pp. bjophthalmol-2019-315597 ◽  
Author(s):  
Johanna Sauma ◽  
Daniela Rivera ◽  
Andres Wu ◽  
Juan Donate-Lopez ◽  
Roberto Gallego-Pinazo ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Hearing Loss ◽  
Arterial Wall ◽  
Intraocular Inflammation ◽  
Retinal Area ◽  
Susac’S Syndrome ◽  
Spectral Domain Oct ◽  
Ophthalmic Manifestations ◽  
Comprehensive Literature Review ◽  
High Index Of Suspicion

Susac’s syndrome (SS) is a relatively rare cause of multiple recurrent branch retinal arterial occlusions (BRAO). SS is frequently misdiagnosed and probably underdiagnosed. Ophthalmic manifestations may be the sole presenting sign of SS. Comprehensive literature review The typical triad of encephalopathy, sensorineural hearing loss and multiple recurrent BRAO is seldom seen at presentation in SS. The characteristic ophthalmological feature in SS is the presence of recurrent multiple BRAO in the absence of intraocular inflammation. Yellow to yellow-white, non-refractile or refractile retinal arterial wall plaques (Gass plaques) found at midarteriolar segments not associated to bifurcations are commonly found in SS. Because of its ability to capture more peripheral retinal area, ultrawide field fluorescein angiography (FA) has definite advantages over conventional FA and its use should be encouraged in patients suspected of having SS. Optical coherence tomography (OCT), particularly spectral domain OCT complements FA. Patients with BRAO and hearing loss that do not develop encephalopathy during the initial 2 years will most likely not develop encephalopathy. However, these patients will be prone to recurrent BRAO and hearing loss. Imunosuppression is the cornerstone of treatment but the best regimen still needs to be defined. A minority of patients with SS present with the typical triad. A high index of suspicion is needed to make the diagnosis promptly. Early diagnosis and treatment are important to delay disease progression and prevent blindness, deafness and dementia.

scholarly journals The Successful Use of Infliximab in a Relapsing Case of Susac’s Syndrome

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Suran L. Fernando ◽  
Therese Boyle ◽  
Annika Smith ◽  
John D.E. Parratt
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Early Recognition ◽  
Delayed Diagnosis ◽  
Sensorineural Hearing ◽  
High Dose ◽  
Susac’S Syndrome ◽  
First Case ◽  
Retinal Artery ◽  
Bilateral Sensorineural Hearing Loss

Susac’s syndrome is a rare and debilitating disease characterized by the triad of encephalopathy, branch retinal artery occlusions, and sensorineural hearing loss. All manifestations may not be clinically apparent at presentation resulting in delayed diagnosis. Early recognition of the syndrome may prevent disease sequelae such as permanent cognitive, visual, and hearing loss. We present such a case of Susac’s syndrome that was also refractory to conventionally prescribed combination of immunosuppressive treatments including high-dose potent corticosteroids, intravenous cyclophosphamide, methotrexate, plasma exchange, rituximab, and mycophenolate. His disease was stabilized with infliximab in combination with a tapering course of low-dose prednisone. After 2 years of remission with TNF treatment, consideration is being given to ceasing therapy. He has the sequelae of bilateral sensorineural hearing loss but no visual impairment or cognitive deficits on follow-up with neuropsychometric testing. This is the first case report to our knowledge of the successful use of infliximab for a patient with Susac’s syndrome that was necessary following treatment with cyclophosphamide and then rituximab.

scholarly journals Susac’s Syndrome: A Case Presentation and Radiological Approach to this Rare Autoimmune Endotheliopathy

Medicina ◽  
2013 ◽  
Vol 49 (1) ◽  
pp. 3
Author(s):  
Goda Randakevičienė ◽  
Rymantė Gleiznienė ◽  
Rūta Nylander
Keyword(s):  
Magnetic Resonance Imaging ◽  
Hearing Loss ◽  
White Matter ◽  
Resonance Imaging ◽  
Neurologic Disorder ◽  
Case Presentation ◽  
Susac’S Syndrome ◽  
Retinal Artery ◽  
Clinical Triad ◽  
The Brain

Susac’s syndrome is an uncommon neurologic disorder, consisting of the clinical triad of encephalopathy, branch retinal artery occlusions, and hearing loss. We report a case of a sudden vision and hearing impairment in a 35-year-old female patient. Magnetic resonance imaging of the brain revealed multiple lesions in the white matter and the corpus callosum, typical of Susac’s syndrome.

scholarly journals Susac's Syndrome in a Patient Diagnosed with MS for 20 Years: A Case Report

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Bijen Nazliel ◽  
Asli Akyol ◽  
Hale Zeynep Batur Caglayan ◽  
Irem Yildirim-Capraz ◽  
Ceyla Irkec
Keyword(s):  
Hearing Loss ◽  
Vision Loss ◽  
Correct Diagnosis ◽  
Sensorineural Deafness ◽  
Active Phase ◽  
Susac’S Syndrome ◽  
Subacute Dementia ◽  
Appropriate Therapy ◽  
Clinical Triad ◽  
High Index Of Suspicion

Susac’s syndrome is an uncommon neurologic disorder of unknown cause. It has been described as a clinical triad of encephalopathy, hearing loss, and branch retinal artery occlusions. Clinically the diagnosis is difficult when the patient presents only a portion of a triad. We present a case with vision loss and sensorineural deafness and who had been diagnosed with MS for 20 years. Susac’s syndrome is presumed to be an autoimmune endotheliopathy. Neurologic symptoms and signs are diffuse and multifocal, acute or subacute in onset, and progress during the active phase of the disease. In some patients the onset was stroke like and in others that of subacute dementia. Headache, often with migrainous features, was a prominent feature initially in more than one half of the patients. A high index of suspicion leading to correct diagnosis and early appropriate therapy may reduce the permanent sequel seen with this disease. Misdiagnosis is common. In patients in whom diagnosis and treatment are delayed permanent morbidity is higher in terms of visual loss, hearing loss, and neurologic debility. In patients in whom rapid diagnosis has led to early administration of immunosuppressive therapy, recovery can be almost complete.

THUR 158 Susac’s syndrome: treatment and outcomes in three cases

2018 ◽  
Vol 89 (10) ◽  
pp. A19.2-A19
Author(s):  
Chua Wei Xin ◽  
Whittam Dan ◽  
Huda Saif ◽  
Rog David ◽  
Wilson Martin ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Disease Onset ◽  
Visual Field Loss ◽  
Published Data ◽  
High Dose ◽  
Acute Therapy ◽  
Susac’S Syndrome ◽  
Free Case ◽  
Retinal Artery ◽  
Electronic Hospital

BackgroundSusac’s syndrome (SS) is a rare, autoimmune microangiopathy causing a triad of branch retinal artery occlusions, sensorineural hearing loss and brain lesions. Published data on immunotherapy and outcomes is very limited.MethodSingle centre case note review. Electronic hospital records between 1996 and 2017 were searched. 277 possible cases were reviewed.Results3 cases (2 female, 1 male) with the full SS triad were identified. Median age of disease onset was 35.2 years with headache (n=3), confusion (n=1), hearing loss (n=3), vertigo (n=2) and visual field loss (n=2). Time from onset to diagnosis with the full triad was 3–23 months. MRIs showed characteristic corpus callosum involvement. All patients received high-dose corticosteroids as acute therapy. Case 1 was treated with Mycophenolate Mofetil (MMF) and Prednisolone for 27 months, and followed up for a further 8 months. She remained relapse-free. Case 2 relapsed on steroid-reduction, so received Rituximab followed by MMF and Prednisolone. She has now been stable for 14 months. Case 3 was intolerant to high-dose corticosteroids, Cyclophoshamide and MMF. He relapsed off treatment then commenced Azathioprine. Duration on Azathioprine is 14 months with one further relapse.ConclusionSS is rare but causes significant morbidity. Recognition of characteristic findings and early immunotherapy improves outcomes.

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