Susac syndrome is a rare microangiopathy affecting the brain, retina, and cochlea. Individuals affected typically present with a classical clinical triad of branch retinal artery occlusion (BRAO), encephalopathy or white matter lesions, and sensorineural hearing loss. Herein, we report an exceedingly rare case of a middle-aged female patient with multiple presentations related to her visual field complaints. The presence of multiple bilateral BRAOs, in addition to sensorineural hearing impairment and white matter lesions on the brain magnetic resonance imaging, led to the diagnosis of Susac syndrome. Cases of Susac syndrome are few and far between and have seldom been reported in detail. Awareness of this rare presentation by clinicians and radiologists is of utmost importance to aid in correctly diagnosing and treating such patients.
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Susac Syndrome with Classical Triad: A Case Report and Literature Review.
Ann Afr Med
March 2025
Department of Ophthalmology, College of Medicine, Imam Abdulrahman Bin Faisal University, Dammam, Saudi Arabia.
Susac syndrome is a rare microangiopathy affecting the brain, retina, and cochlea. Individuals affected typically present with a classical clinical triad of branch retinal artery occlusion (BRAO), encephalopathy or white matter lesions, and sensorineural hearing loss. Herein, we report an exceedingly rare case of a middle-aged female patient with multiple presentations related to her visual field complaints.
View Article and Find Full Text PDFSuccessful Natalizumab Treatment of Two Female Individuals With Susac Syndrome.
Eur J Neurol
March 2025
Department of Neurology, University Hospital Marburg, Marburg, Germany.
Background: Susac syndrome is a rare autoimmune endotheliopathy that affects the central nervous system, retina, and inner ear, characterized by encephalopathy, branch retinal artery occlusions, and sensorineural hearing loss. Due to the heterogeneity of its presentation, early diagnosis, and treatment remain challenging.
Objective/methods: To evaluate the clinical outcomes and radiological responses in two patients with Susac syndrome treated with natalizumab in an off-label therapeutic approach, clinical assessments and serial magnetic resonance imaging (MRI) were performed over a follow-up period of up to 22 months to monitor disease progression and treatment response.
Susac's syndrome: diagnostic challenges.
Pract Neurol
February 2025
Neurology, Salford Royal Hospitals NHS Trust, Salford, UK.
A 39-year-old woman presented with headaches, slurred speech, facial weakness and brief altered sensations, together with non-specific visual disturbances, unsteadiness and falls. Despite initial treatment with intravenous methylprednisolone, her condition worsened with cognitive decline, confusion, ataxia and incontinence. We diagnosed Susac's syndrome having excluded other causes.
View Article and Find Full Text PDFFluorescein Angiography as a Surrogate Marker of Disease Activity in Susac Syndrome.
Neurol Neuroimmunol Neuroinflamm
March 2025
Department of Neurology, University Hospital of Marseille, France.
Objectives: To evaluate the sensitivity of fluorescein angiography (FA) in detecting disease activity in Susac syndrome.
Methods: We conducted a blinded analysis of all FA, brain MRI, and audiogram examinations performed throughout the follow-up of patients with Susac syndrome.
Results: A total of 79 FA examinations, 85 brain MRI scans, and 49 audiograms were analyzed from 9 patients followed for a mean (SD) period of 6 (4) years.
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