A Young Man With Visual Field Loss, Decreased Hearing, and Confusion

2021 ◽  
pp. 168-170
Author(s):  
M. Tariq Bhatti ◽  
Eric R. Eggenberger ◽  
Marie D. Acierno ◽  
John J. Chen
Keyword(s):  
Magnetic Resonance Imaging ◽  
Visual Field ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Branch Retinal Artery Occlusion ◽  
Susac Syndrome ◽  
Retinal Artery ◽  
Signal Changes ◽  
The Brain ◽  
Field Defect

A 27-year-old man noted imbalance and staggering when walking. Vertigo, nausea, vomiting, and mild fever developed. This was presumed to be due to an inner ear infection, and antibiotics were prescribed. He began experiencing intermittent left face and arm numbness, bilateral hearing loss and tinnitus. Audiography indicated low-frequency hearing loss in both ears, left worse than right. He reported headaches and neck stiffness, and his family noticed that he was moody, easily aggravated, and confused, with slow mentation. Magnetic resonance imaging showed patchy, nodular, leptomeningeal enhancement involving both cerebral hemispheres and the posterior fossa, with scattered hyperintense T2 signal changes of the internal capsule and prominent abnormal signal changes in the corpus callosum. Cerebrospinal fluid analysis was remarkable for a markedly increased protein concentration and white blood cells. Eye examination showed 20/20 vision in both eyes with a superior visual field defect in the right eye. Retinal whitening was noted in the vascular distribution of the inferotemporal arcade. Intravenous fluorescein angiography showed delayed filling in this region consistent with a branch retinal artery occlusion and scattered areas of arteriolar wall hyperfluorescence. A diagnosis of Susac syndrome was made on the basis of the branch retinal artery occlusion, magnetic resonance imaging findings, and hearing deficit. Intravenous methylprednisolone was given, followed oral prednisone, which resulted in substantial improvement in headaches and cognition. Cyclophosphamide was also started at the same time as intravenous methylprednisolone. A new visual field defect developed due to a branch retinal artery occlusion in the left eye, which prompted initiation of intravenous immunoglobulin and transition from cyclophosphamide to rituximab. He had no recurrent branch retinal artery occlusions or other relapses of his underlying Susac syndrome on this treatment regimen. Susac syndrome was initially described as a microangiopathy of the brain and retina. It is an idiopathic autoimmune disorder that primarily affects the brain, eye, and inner ear.

scholarly journals Susac syndrome presenting with acute hemibody paraesthesia

2019 ◽  
Vol 8 ◽  
pp. 204800401984468
Author(s):  
Philip Campbell ◽  
Diego Kaski ◽  
Tabish A Saifee
Keyword(s):  
Hearing Loss ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Branch Retinal Artery Occlusion ◽  
Stroke Management ◽  
Susac Syndrome ◽  
Hyperacute Stroke ◽  
Appropriate Treatment ◽  
Retinal Artery ◽  
Clinical Triad

Susac syndrome is an orphan disease characterised by encephalopathy, branch retinal artery occlusion and sensorineural hearing loss. As the clinical triad is rarely present at symptom onset, it is often initially misdiagnosed and appropriate treatment is often delayed. Herewith, we report a case of Susac syndrome in a 47-year-old man presenting with acute hemisensory loss and highlight the challenges of early diagnosis, particularly relevant in the era of hyperacute stroke management.

RECURRENT BRANCH RETINAL ARTERY OCCLUSION FROM SUSAC SYNDROME

2018 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Michael J. Ammar ◽  
Anton M. Kolomeyer ◽  
Nirali Bhatt ◽  
Madhura A. Tamhankar ◽  
Michael T. Mullen ◽  
...  
Keyword(s):  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Branch Retinal Artery Occlusion ◽  
Susac Syndrome ◽  
Retinal Artery

scholarly journals Complications of Intra-Arterial tPA for Iatrogenic Branch Retinal Artery Occlusion: A Case Report through Multimodal Imaging and Literature Review

Medicina ◽  
2021 ◽  
Vol 57 (9) ◽  
pp. 963
Author(s):  
Katherine Dalzotto ◽  
Paige Richards ◽  
Tyler D. Boulter ◽  
Marilyn Kay ◽  
Mihai Mititelu
Keyword(s):  
Visual Field ◽  
Multimodal Imaging ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Fundus Photography ◽  
Automated Perimetry ◽  
Branch Retinal Artery Occlusion ◽  
Retinal Artery ◽  
Sd Oct

Background and Objectives: To document, through multimodal imaging, the post-procedural clinical course and visual outcome of a patient who received intra-arterial tissue plasminogen activator (tPA) for acute iatrogenic branch retinal artery occlusion (BRAO), and to review the literature and guidelines regarding the use of tPA for retinal arterial occlusions. Methods: A 28-year-old female patient who sustained an iatrogenic BRAO and subsequently received intra-arterial tPA was followed through her post-interventional course of 3 months with serial exams and multimodal imaging, including color fundus photography, visual field testing, spectral domain optical coherence tomography (SD-OCT), and OCT angiography (OCT-A). Results: A patient with history of left internal cerebral artery (ICA) aneurysm and baseline visual acuity (VA) of 20/20 developed an acutely symptomatic BRAO after undergoing a neuroendovascular procedure and was acutely treated with tPA through the left ophthalmic artery. At two weeks follow-up, a central posterior pole hemorrhage was noted although VA was preserved. A superior altitudinal defect was shown on automated perimetry. VA dropped to 20/50 at 7 weeks follow-up and hyperreflective material deep to the attachment between the posterior hyaloid and the internal limiting membrane (ILM) consistent with hemorrhage was noted on SD-OCT. At 11 weeks follow-up, VA returned to 20/20, SD-OCT revealed a membrane bridging the foveal depression, OCT-A showed decreased vascularity in the inferior macula, and the visual field defect was stable by automated perimetry. Conclusions: Intraocular hemorrhage is a possible complication of intra-arterial tPA administration for BRAO, and a careful analysis of risks, benefits, and goals of this procedure must be considered by both provider and patient before such intervention.

scholarly journals Delayed Vision Recovery after Carotid Vascular Surgery for Branch Retinal Artery Occlusion

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Jessica Ruzicki ◽  
Eric K. Chin ◽  
David Almeida
Keyword(s):  
Risk Factors ◽  
Cardiovascular Risk ◽  
Visual Field ◽  
Carotid Artery ◽  
Visual Field Loss ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Branch Retinal Artery Occlusion ◽  
Carotid Artery Endarterectomy ◽  
Retinal Artery

Branch retinal artery occlusion (BRAO) is typically associated with irreversible vision and peripheral visual field loss. We report a case of a 62-year-old woman with a BRAO related to several cardiovascular risk factors. Our patient encountered gradual but significant vision recovery months following carotid artery endarterectomy for carotid stenosis.

scholarly journals Headache in a young woman: do not forget Susac’s syndrome

Reumatismo ◽  
2017 ◽  
Vol 69 (3) ◽  
pp. 122 ◽  
Author(s):  
A. Kefi ◽  
F. Said ◽  
T. Ben Salem ◽  
M. Khedher ◽  
M. Khanfir ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Early Diagnosis ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Branch Retinal Artery Occlusion ◽  
Permanent Disability ◽  
Susac Syndrome ◽  
Broad Variety ◽  
Retinal Artery ◽  
Clinical Triad

Susac syndrome is a rare disease characterized by the clinical triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. This underdiagnosed condition needs to be considered in the differential diagnosis of a broad variety of disorders. An early diagnosis is important as treatment can halt disease progression and prevent permanent disability. Herein, we report a case of Susac syndrome in a 31-year-old woman and we highlight how challenging an early diagnosis was and the importance of an aggressive therapeutic approach, including the combination of steroids and other cytotoxic drugs.

Susac syndrome: outcome of unilateral cochlear implantation

2011 ◽  
Vol 125 (8) ◽  
pp. 856-858 ◽  
Author(s):  
N Grover ◽  
O J H Whiteside ◽  
J D Ramsden
Keyword(s):  
Magnetic Resonance Imaging ◽  
Hearing Loss ◽  
Magnetic Resonance ◽  
Cochlear Implantation ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Ophthalmological Examination ◽  
Resonance Imaging ◽  
Susac Syndrome ◽  
Retinal Artery

AbstractObjective:Susac syndrome comprises a triad of vestibulocochlear dysfunction, retinopathy and multifocal encephalopathy, which is characterised pathophysiologically by microangiopathy of the ear, retina and brain. Diagnosis is confirmed by magnetic resonance imaging of the brain and ophthalmological examination, which reveals branch retinal artery occlusion. Hearing loss persists in 90 per cent of patients. We present a case of successful hearing rehabilitation by cochlear implantation in a young woman with this syndrome.Clinical presentation:A 36-year-old woman presented with neurological symptoms suggestive of encephalitis. She subsequently developed vestibulocochlear symptoms. The diagnosis was confirmed upon magnetic resonance imaging and fluorescein angiography, which showed multiple peripheral retinal arterial occlusions. Hearing loss was fluctuant but gradually progressive over nine months, to bilateral profound sensorineural hearing loss.Intervention:A left cochlear implant was placed, with a good outcome.Conclusion:In this Susac syndrome patient, the outcome of cochlear implantation was encouraging, notwithstanding the possible involvement of retrocochlear pathways.

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