Susac’s syndrome: isolated retinal artery occlusion after 10 years of remission

Abstract

Susac's syndrome, or microangiopathy of the retina, inner ear, and brain, is a rare condition characterized by the clinical triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. The complete triad has been documented in 85% of reported cases. At clinical onset, the most common manifestations are central nervous system symptoms, followed by visual symptoms and hearing disturbances. Although the clinical course of Susac's syndrome is usually self-limiting, fluctuating, and monophasic, clinical polycyclic and chronic courses have also been described. Likewise, recurrences of the full triad after more than 10 years of remission have been reported. We describe a 21-year-old woman who presented with branch retinal artery occlusions and magnetic resonance imaging findings compatible with Susac's syndrome without objective hearing loss. After 10 years of remission, the patient complained of visual field loss due to new retinal ischemia. Neither other symptoms nor neuroimaging or audiometry pathologic findings were found during the clinical course.

Susac syndrome (Retino-cochleo-cerebral vasculitis), the ophthalmologist in the role of the whistleblower

“…In these cases that present ocular involvement first, the ophthalmologist's role is crucial and acts as a whistle blower, by recognising and treating the patients and so avoiding severe cochlear and cerebral complications. Rapid identification of the disease by the ophthalmologist is crucial, as in ophthalmological series, unlike in neurological series, MRI is often negative and ophthalmic manifestations may be the sole presenting sign [29][30][31][32]. This is one more reason to have good ophthalmological disease defining criteria.…”