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 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.

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

scholarly journals Alport Syndrome: The Eye as a Window to the Human Body

2021 ◽  
Vol 33 (3) ◽  
pp. 46-48
Author(s):  
Aysha Tareq Nusef ◽  
Abdulla Almoosa ◽  
Wael Wagih Aly
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Renal Insufficiency ◽  
Sensorineural Hearing Loss ◽  
Alport Syndrome ◽  
Crystalline Lens ◽  
Secondary Hypertension ◽  
Sensorineural Hearing ◽  
Blurred Vision ◽  
Ophthalmic Manifestations

Alport syndrome (AS) is a rare genetic disease affecting type four collagen production, causing renal, auditory, and ophthalmic manifestations. This case report is about a 32-year-old male who was a known case of renal insufficiency and secondary hypertension and was referred to the ophthalmology department due to blurred vision. Based on the patient‘s history and ophthalmological findings, AS was diagnosed. Ophthalmic examination showed anterior lenticonus associated with sensorineural hearing loss (SNHL) and impaired renal function. This clinical case report sheds light on the role of ophthalmology in diagnosing AS. Keywords: Collagen, Crystalline lens, Hereditary nephritis, Ophthalmology, Renal insufficiency, Sensorineural hearing loss

Optical Coherence Tomography and Glaucoma

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Alexi Geevarghese ◽  
Gadi Wollstein ◽  
Hiroshi Ishikawa ◽  
Joel S. Schuman
Keyword(s):  
Optical Coherence Tomography ◽  
Adaptive Optics ◽  
Imaging Modality ◽  
Annual Review ◽  
Publication Date ◽  
Optical Coherence ◽  
Swept Source ◽  
Diagnosis And Management ◽  
Spectral Domain Oct ◽  
Glaucoma Diagnosis

Early detection and monitoring are critical to the diagnosis and management of glaucoma, a progressive optic neuropathy that causes irreversible blindness. Optical coherence tomography (OCT) has become a commonly utilized imaging modality that aids in the detection and monitoring of structural glaucomatous damage. Since its inception in 1991, OCT has progressed through multiple iterations, from time-domain OCT, to spectral-domain OCT, to swept-source OCT, all of which have progressively improved the resolution and speed of scans. Even newer technological advancements and OCT applications, such as adaptive optics, visible-light OCT, and OCT-angiography, have enriched the use of OCT in the evaluation of glaucoma. This article reviews current commercial and state-of-the-art OCT technologies and analytic techniques in the context of their utility for glaucoma diagnosis and management, as well as promising future directions. Expected final online publication date for the Annual Review of Vision Science, Volume 7 is September 2021. Please see http://www.annualreviews.org/page/journal/pubdates for revised estimates.

Macular capillary plexuses after macular hole surgery: an optical coherence tomography angiography study

2017 ◽  
Vol 102 (7) ◽  
pp. 966-970 ◽  
Author(s):  
Yoon Jeon Kim ◽  
Jaehyuck Jo ◽  
Joo Yong Lee ◽  
Young Hee Yoon ◽  
June-Gone Kim
Keyword(s):  
Optical Coherence Tomography ◽  
Macular Hole ◽  
Structural Changes ◽  
Foveal Avascular Zone ◽  
Vascular Density ◽  
Optical Coherence ◽  
Foveal Thickness ◽  
Functional Changes ◽  
Spectral Domain Oct ◽  
En Face

AimsTo investigate the structural changes of the superficial capillary plexuses (SCP) and deep capillary plexuses (DCP) using optical coherence tomography (OCT) angiography (OCTA) in patients with idiopathic macular hole (MH) after surgery, determine the factors related to changes of macular capillary plexuses and evaluate its association with postoperative visual outcomes.MethodsThirty-three patients with unilateral MH who were followed for ≥6 months after surgery were included. Ophthalmologic evaluations included best corrected visual acuity (BCVA) and spectral-domain OCT before surgery and 6 months postsurgery. En face OCTA images were obtained for both eyes at 6 months postsurgery, and the postoperative foveal avascular zone (FAZ) area and parafoveal vascular density were identified.ResultsCompared with fellow eyes, eyes after MH surgery had a smaller FAZ area in both SCP and DCP (p<0.05 for all). The FAZ area was positively correlated with postoperative foveal thickness of the whole, inner and outer layers (p<0.05 for all). In the parafoveal region, eyes after MH surgery had a tendency to have lower parafoveal vascular density, particularly in DCP (p=0.019). The parafoveal vascular density in DCP was positively correlated with retinal thickness of the whole, inner and outer layers (p<0.05 for all). Correlations between BCVA and FAZ area in both SCP and DCP were significant 6 months after MH surgery (p<0.05 for all).ConclusionAssessment of macular capillary plexuses using OCTA may be useful for monitoring retinal structural and functional changes in MH.

scholarly journals Peripapillary and parafoveal vascular network assessment by optical coherence tomography angiography in aquaporin-4 antibody-positive neuromyelitis optica spectrum disorders

2018 ◽  
Vol 103 (6) ◽  
pp. 789-796 ◽  
Author(s):  
Yongheng Huang ◽  
Lei Zhou ◽  
Jingzi ZhangBao ◽  
Tongjia Cai ◽  
Bei Wang ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Neuromyelitis Optica ◽  
Vessel Density ◽  
Aquaporin 4 ◽  
Optical Coherence ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Spectral Domain Oct ◽  
History Of ◽  
Spectrum Disorders ◽  
Aquaporin 4 Antibody

Background/aimsCurrent understanding of the alterations in the retinal vascular network in neuromyelitis optica spectrum disorders (NMOSDs) is limited. We aim to assess the peripapillary and parafoveal vessel density in aquaporin-4 antibody-positive NMOSD patients by optical coherence tomography (OCT) angiography.MethodsA total of 55 aquaporin-4 antibody-positive NMOSD patients with or without a history of optic neuritis (ON) and 33 healthy controls underwent spectral domain OCT and OCT angiography. Clinical histories, Expanded Disability Status Scale score, visual functional system score (VFSS) and disease duration were collected.ResultsPeripapillary and parafoveal vessel density was significantly decreased in NMOSD eyes with or without a history of ON. The decrease in retinal vessel density could occur before ON and retinal nerve fibre layer (RNFL) atrophy. Peripapillary vessel density correlated well with the spectral domain OCT measurements and VFSS in NMOSD eyes with a history of ON.ConclusionSubclinical primary retinal vasculopathy may occur in NMOSD prior to ON and RNFL atrophy. Peripapillary vessel density might be a sensitive predictor of visual outcomes in NMOSD patients with ON.

Morphological and topographical appearance of microaneurysms on optical coherence tomography angiography

2018 ◽  
Vol 103 (5) ◽  
pp. 630-635 ◽  
Author(s):  
Vivian Schreur ◽  
Artin Domanian ◽  
Bart Liefers ◽  
Freerk G Venhuizen ◽  
B Jeroen Klevering ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Macular Oedema ◽  
Optical Coherence Tomography Angiography ◽  
Diabetic Macular Oedema ◽  
Optical Coherence ◽  
Retinal Area ◽  
Fundus Fluorescein Angiography ◽  
Morphological Appearance ◽  
Capillary Plexus ◽  
Retinal Microaneurysms

AimsTo investigate retinal microaneurysms in patients with diabetic macular oedema (DME) by optical coherence tomography angiography (OCTA) according to their location and morphology in relationship to their clinical properties, leakage on fundus fluorescein angiography (FFA) and retinal thickening on structural OCT.MethodsOCTA and FFA images of 31 eyes of 24 subjects were graded for the presence of microaneurysms. The topographical and morphological appearance of microaneurysms on OCTA was evaluated and classified. For each microaneurysm, the presence of focal leakage on FFA and associated retinal thickening on OCT was determined.ResultsOf all microaneurysms flagged on FFA, 295 out of 513 (58%) were also visible on OCTA. Microaneurysms with focal leakage and located in a thickened retinal area were more likely to be detected on OCTA than not leaking microaneurysms in non-thickened retinal areas (p=0.001). Most microaneurysms on OCTA were seen in the intermediate (23%) and deep capillary plexus (22%). Of all microaneurysms visualised on OCTA, saccular microaneurysms were detected most often (31%), as opposed to pedunculated microaneurysms (9%). Irregular, fusiform and mixed fusiform/saccular-shaped microaneurysms had the highest likeliness to leak and to be located in thickened retinal areas (p<0.001, p<0.001 and p=0.001).ConclusionsRetinal microaneurysms in DME could be classified topographically and morphologically by OCTA. OCTA detected less microaneurysms than FFA, and this appeared to be dependent on leakage activity and retinal thickening. Morphological appearance of microaneurysms (irregular, fusiform and mixed saccular/fusiform) was associated with increased leakage activity and retinal thickening.

A triad of retinal signs in Alport syndrome: The ‘stair-case’ fovea, choroidal thinning and peripheral schisis

2019 ◽  
Vol 29 (1_suppl) ◽  
pp. 10-14
Author(s):  
NJ Ghadiri ◽  
N Stanojcic ◽  
MSA Raja ◽  
BJ Burton
Keyword(s):  
Renal Failure ◽  
Optical Coherence Tomography ◽  
Alport Syndrome ◽  
Optical Coherence ◽  
Membrane Structures ◽  
Tomography Imaging ◽  
Type Iv ◽  
Ophthalmic Manifestations ◽  
Optical Coherence Tomography Imaging ◽  
The Right

Background: Alport syndrome is an inherited Type IV collagenopathy characterised by renal failure, hearing loss and ophthalmic manifestations such as lenticonus and dot-and-fleck retinopathy. New signs have been described which can be useful both for diagnosis and for prognosticating the risk of complications. This study examines and describes a triad comprising the unusual ‘stair-case’ foveal sign, together with choroidal thinning and late-stage peripheral schisis in a patient with Alport syndrome. Case presentation: This is a case report of a 49-year-old Caucasian male with a background of X-linked Alport syndrome presenting with gradual and progressive diminution of vision in the left eye with a central blur. He had already undergone three renal allografts, was deaf and suffered from hypertension by the time of his first presentation to ophthalmology. On examination, corrected visual acuity was 6/9.5 in the right eye and 6/30 in the left eye. Optical coherence tomography imaging showed an unusual ‘stair-case’ sign of the fovea in both eyes, together with choroidal thinning. We postulate that an abnormal vitreomacular interface followed by vitreomacular traction and eventually separation, removing layers of the inner retina with the vitreous, led to this unusual appearance. Subsequently, this patient also developed schitic changes more peripherally in the retina which progressed over the following 5 years. Conclusion: The stair-case foveal sign, choroidal thinning and mid-peripheral schisis are three signs that clinicians might expect to encounter on optical coherence tomography imaging of patients with Alport syndrome. These findings can be attributed to unique mutations of collagen IV which lead to a variety of clinical phenotypes affecting basement membrane structures. Identification of these features may not only be useful diagnostically and in forecasting complications such as macular holes, but also predict mode of inheritance and likelihood of early-onset renal failure.

AXIAL SUPERRESOLUTION BY PHASE FILTER IN OPTICAL COHERENCE TOMOGRAPHY

2012 ◽  
Vol 05 (04) ◽  
pp. 1250022 ◽  
Author(s):  
ZHIHUA DING ◽  
YANG NI ◽  
JIE MENG
Keyword(s):  
Optical Coherence Tomography ◽  
Optical Coherence ◽  
Negative Contribution ◽  
Spectral Domain Oct ◽  
Phase Filter ◽  
Depth Discrimination ◽  
Before And After ◽  
Coherence Imaging ◽  
Axial Superresolution ◽  
Depth Response

Axial superresolution in optical coherence tomography (OCT) by a three-zone annular phase filter is demonstrated. In the proposed probe of a spectral domain OCT system, the width of the central lobe of the axial intensity point spread function is apodized by the filter to be within the coherence gate determined by the light source, while its sidelobes are lying outside the coherence gate without contributing to the coherence imaging. By measurement of the depth response of the OCT system before and after inserting the filter, an improvement of about 20% in axial resolution is confirmed. OCT imaging on biological sample of orange fresh is also conducted, demonstrating increased depth discrimination without the negative contribution from sidelobes realized by the phase filter in combination with the coherence gate intrinsic to OCT. It comes to a conclusion that we can obtain axial superresolution by filter in OCT system without the degrading influence of large sidelobes.

scholarly journals Retinal Damage in Multiple Sclerosis Disease Subtypes Measured by High-Resolution Optical Coherence Tomography

2012 ◽  
Vol 2012 ◽  
pp. 1-10 ◽  
Author(s):  
Timm Oberwahrenbrock ◽  
Sven Schippling ◽  
Marius Ringelstein ◽  
Falko Kaufhold ◽  
Hanna Zimmermann ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Optic Neuritis ◽  
Large Scale ◽  
Retinal Nerve Fibre Layer ◽  
Spectral Domain ◽  
Healthy Controls ◽  
Optical Coherence ◽  
Cross Sectional ◽  
Spectral Domain Oct ◽  
Rnfl Thickness

Background.Optical coherence tomography (OCT) has facilitated characterisation of retinal alterations in MS patients. Only scarce and in part conflicting data exists on different MS subtypes.Objective.To analyse patterns of retinal changes in different subtypes of MS with latest spectral-domain technology.Methods.In a three-centre cross-sectional study 414 MS patients and 94 healthy controls underwent spectral-domain OCT examination.Results.Eyes of MS patients without a previous optic neuritis showed a significant reduction of both retinal nerve fibre layer (RNFL) thickness and total macular volume (TMV) compared to healthy controls independent of the MS subtype (P<0.001for all subtypes). RNFL thickness was lower in secondary progressive MS (SPMS) eyes compared to relapsing-remitting MS (RRMS) eyes (P=0.007), and TMV was reduced in SPMS and primary progressive MS (PPMS) eyes compared to RRMS eyes (SPMS:P=0.039, PPMS:P=0.005). Independent of the subtype a more pronounced RNFL thinning and TMV reduction were found in eyes with a previous optic neuritis compared to unaffected eyes.Conclusion.Analysis of this large-scale cross-sectional dataset of MS patients studied with spectral-domain OCT confirmed and allows to generalize previous findings. Furthermore it carves out distinct patterns in different MS subtypes.

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