Behavioral Impairment and Amnesia at the Onset of Susac Syndrome

Abstract

Susac syndrome (SS) is an autoimmune microangiopathy that affects the brain, retina, and inner ear, causing a wide range of clinical manifestations. The triad of encephalopathy, visual disturbances, and hearing loss constitute the classic disease presentation.We describe an original clinical case of a young male with a definitive diagnosis of SS, who presented with disordered behavior and amnesia, initially manifested as a dissociative or anxiety disorder but with a fulminant evolution toward severe encephalopathy associated with retinal infarcts and sensorineural hearing loss.After the diagnosis of SS, aggressive immunosuppressive treatment was started with significant neurological improvement and favorable evolution during the follow-up period. SS is a rare but potentially devastating disease that can cause great disability if not properly diagnosed and treated. The onset of SS with behavioral or psychiatric manifestation can be misleading, causing a diagnostic delay.

Fatal Presentation of Susac Syndrome in a Congenitally Deaf Patient With a Cochlear Implant

“…Ahmad et al (20) described a patient who presented with BRAO and bilateral SNHL without encephalopathy. Other atypical manifestations of SS include psychiatric or subacute behavioral disorders (21)(22)(23). Given the variability of reported presentations, clinicians, and especially otolaryngologists, should consider performing early MRI and requesting fundoscopy in any patient with hearing loss and encephalopathy or in those with two of three SS criteria.…”