Masjuan Marc, Ivanovski Trajche, Sarasibar Ezcurra Helena, Rigo Oliver Elena
Neurology, Hospital Universitario Son Llatzer, Palma de Mallorca, ESP.
Radiology, Hospital Universitario Son Llatzer, Palma de Mallorca, ESP.
Cureus. 2023 Apr 25;15(4):e38089. doi: 10.7759/cureus.38089. eCollection 2023 Apr.
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.
Susac综合征（SS）是一种自身免疫性微血管病，可累及脑、视网膜和内耳，导致广泛的临床表现。脑病、视觉障碍和听力丧失三联征构成了典型的疾病表现。我们描述了一例年轻男性确诊为SS的原始临床病例，该患者表现为行为紊乱和失忆，最初表现为解离性或焦虑症，但迅速演变为与视网膜梗死和感音神经性听力丧失相关的严重脑病。确诊SS后，开始积极的免疫抑制治疗，随访期间神经功能有显著改善且病情进展良好。SS是一种罕见但可能具有毁灭性的疾病，如果未得到正确诊断和治疗，可导致严重残疾。以行为或精神症状为首发表现的SS可能会造成误导，导致诊断延迟。
