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.

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.

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 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 Insidious Onset of Tetraparesis due to Cervical Epidural Abscess fromEnterococcus faecalis

2013 ◽  
Vol 2013 ◽  
pp. 1-5
Author(s):  
Konstantinos Chr. Soultanis ◽  
Vasileios I. Sakellariou ◽  
Konstantinos A. Starantzis ◽  
Nikolaos A. Stavropoulos ◽  
Panayiotis J. Papagelopoulos
Keyword(s):  
Epidural Abscess ◽  
Clinical Laboratory ◽  
Correct Diagnosis ◽  
Cervical Disc ◽  
Spinal Epidural Abscess ◽  
Neurological Improvement ◽  
Insidious Onset ◽  
Upper And Lower Extremities ◽  
Clinical Triad ◽  
High Index Of Suspicion

We report a case of cervical epidural abscess fromEnterococcus faecalis, which caused an insidious onset of tetraparesis. This 53-year-old female with a history of diabetes mellitus and chronic renal failure under hemodialysis presented with pain and progressive weakness of upper and lower extremities without fever. Although a recent MRI she did at the beginning of symptoms showed no significant pathologies, except for a cervical disc herniation and adjacent spinal degeneration, and stenosis that confused the diagnostic procedure, newer imaging with CT and MRI, which was performed due to progression of tetraparesis, revealed the formation of a cervical epidural abscess. Surgical drainage was done after a complete infection workup. The patient showed immediate neurological improvement after surgery. She received antibiotics intravenously for 3 weeks and orally for another 6 weeks. The patient was free from complications 24 months after surgery. A high index of suspicion is most important in making a rapid and correct diagnosis of spinal epidural abscess. The classic clinical triad (fever, local pain, and neurologic deficits) is not sensitive enough for early detection. Continuous clinical, laboratory, and imaging monitoring are of paramount importance. Early diagnosis and surgical intervention could optimize the final functional outcome.

SUSAC'S SYNDROME: A CASE SERIES FROM A REGIONAL NEUROSCIENCES UNIT

2015 ◽  
Vol 86 (11) ◽  
pp. e4.34-e4 ◽  
Author(s):  
Dominic King ◽  
Jason Philip Appleton ◽  
Saiju Jacob
Keyword(s):  
Hearing Loss ◽  
Literature Review ◽  
Case Series ◽  
Disease Course ◽  
Susac’S Syndrome ◽  
Immune Mediated ◽  
Relapsing Remitting ◽  
Retinal Artery ◽  
Management Techniques ◽  
Clinical Triad

The clinical triad of branch retinal artery occlusions, hearing loss and encephalopathy constitutes Susac's Syndrome. This rare, immune mediated, microangiopathic disorder shows a variable course with a preponderance of 3 to 1 for women, mainly in their 2nd to 4th decade. The condition relies on diagnosis and early aggressive immunosuppressive therapy to avoid severe sequale. Unfortunately, due to the paucity of experience, and a variable disease course, often lacking all elements of the triad concurrently, diagnosis continues to be delayed.We present five cases (Age 34–45, M:F–1:4) seen at our regional neurosciences centre, which highlight the diagnostic differences and challenges and the variety of management techniques adopted. Three patients had a monophasic illness and two had a relapsing remitting course. In addition, we provide an up-to-date literature review on the pathophysiology, diagnosis and management of this rare and fascinating condition.Susac's disease, otherwise described as retinocochleocerebral vasculopathy, RED-M (retinopathy, encephalopathy, deafness associated microangiopathy) and SICRET (small infarcts of cochlear, retinal and encephalic tissues), is a syndromic pathology requiring the recognition of a triad of deficits to make a diagnosis. The relevance of this and the difficulty in diagnosis is exemplified in the cases presented here.

scholarly journals Do Communication Patterns Affect the Association between Cognitive Impairment and Hearing Loss among Older Adults in Vietnam?

2021 ◽  
Vol 18 (4) ◽  
pp. 1603
Author(s):  
Tran Dai Tri Han ◽  
Keiko Nakamura ◽  
Kaoruko Seino ◽  
Vo Nu Hong Duc ◽  
Thang Van Vo
Keyword(s):  
Older Adults ◽  
Hearing Loss ◽  
Cognitive Impairment ◽  
Cognitive Function ◽  
Vision Loss ◽  
Community Dwelling ◽  
Cross Sectional ◽  
Ordinal Logistic Regression Analysis ◽  
Communication Devices ◽  
Community Dwelling Older Adults

This study examined the prevalence of cognitive impairment among older adults in central Vietnam and the roles of communication (with or without communication devices) in the association between cognitive impairment and hearing loss. This cross-sectional study was performed on 725 randomly selected community-dwelling older adults aged ≥60 years from Thua Thien Hue province, Vietnam. Participants attended a face-to-face survey. Sociodemographic characteristics, social interaction with or without communication devices, health status and cognitive function using the Mini-Mental State Examination were reported. Ordinal logistic regression analysis was performed to quantify the association between hearing loss and cognitive function by frequency of communication with and without devices. Mild and severe cognitive impairment had prevalence rates of 23.6% and 19.3%, respectively. Cognitive impairment was more prevalent among older adults with hearing-loss, vision loss and difficulties with instrumental activities of daily living (IADL). The association between hearing loss and cognitive impairment was not significant when older adults had frequent communication with others using devices. This study presented the relatively high prevalence of cognitive impairment in community-dwelling older adults in Vietnam. Frequent communication using devices attenuated the association between hearing loss and cognitive impairment.

scholarly journals IGF-1 Haploinsufficiency Causes Age-Related Chronic Cochlear Inflammation and Increases Noise-Induced Hearing Loss

Cells ◽  
2021 ◽  
Vol 10 (7) ◽  
pp. 1686
Author(s):  
Adelaida M. Celaya ◽  
Lourdes Rodríguez-de la Rosa ◽  
Jose M. Bermúdez-Muñoz ◽  
José M. Zubeldia ◽  
Carlos Romá-Mateo ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inflammatory Cytokines ◽  
Noise Exposure ◽  
Sensorineural Deafness ◽  
Serum Levels ◽  
Genetic Syndromes ◽  
Expression Ratio ◽  
Postnatal Maturation ◽  
Age Related ◽  
Underlying Mechanisms

Insulin-like growth factor 1 (IGF-1) deficiency is an ultrarare syndromic human sensorineural deafness. Accordingly, IGF-1 is essential for the postnatal maturation of the cochlea and the correct wiring of hearing in mice. Less severe decreases in human IGF-1 levels have been associated with other hearing loss rare genetic syndromes, as well as with age-related hearing loss (ARHL). However, the underlying mechanisms linking IGF-1 haploinsufficiency with auditory pathology and ARHL have not been studied. Igf1-heterozygous mice express less Igf1 transcription and have 40% lower IGF-1 serum levels than wild-type mice. Along with ageing, IGF-1 levels decreased concomitantly with the increased expression of inflammatory cytokines, Tgfb1 and Il1b, but there was no associated hearing loss. However, noise exposure of these mice caused increased injury to sensory hair cells and irreversible hearing loss. Concomitantly, there was a significant alteration in the expression ratio of pro- and anti-inflammatory cytokines in Igf1+/− mice. Unbalanced inflammation led to the activation of the stress kinase JNK and the failure to activate AKT. Our data show that IGF-1 haploinsufficiency causes a chronic subclinical proinflammatory age-associated state and, consequently, greater susceptibility to stressors. This work provides the molecular bases to further understand hearing disorders linked to IGF-1 deficiency.

Mycobacterium Fortuitum Infections of the Hand

1992 ◽  
Vol 17 (6) ◽  
pp. 675-677 ◽  
Author(s):  
F. K. IP ◽  
S. P. CHOW
Keyword(s):  
Correct Diagnosis ◽  
High Index ◽  
Mycobacterium Fortuitum ◽  
High Index Of Suspicion

Five cases are reported of infection due to Mycobactenum fortuitum involving the hand following contaminated injection or traumatic wounds. Synovectomy, debridement, or amputation together with prolonged chemotherapy using kanamycin or amikacin were required. Doxycycline and sulphamethoxasole also seemed to be the effective antibiotics for this organism. A high index of suspicion is important in order to obtain the correct diagnosis.

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
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