Oluwole Olusegun J, Crespo Cuevas Ane M, Lorente Miranda Andrea, Iantorno Vittorio
Neurology, King's College Hospital, Dubai, ARE.
Cureus. 2024 Nov 26;16(11):e74545. doi: 10.7759/cureus.74545. eCollection 2024 Nov.
Susac's syndrome is a rare inflammatory microangiopathy characterized by the triad of retinopathy, encephalopathy, and hearing loss. The syndrome causes recurrent microinfarcts in these organs, which in turn manifests with repeated attacks of visual field loss, hearing loss and tinnitus, and various brain syndromes. These often lead to the significant accumulation of disability over time, particularly if there is a delay or failure in diagnosis. The brain lesions associated with this condition may resemble those of multiple sclerosis, especially to those who are unfamiliar with the disease and its distinctive clinical and radiological features. Such misdiagnosis may have grave clinical consequences. Here, we present the case of a 41-year-old Danish man who presented with the classical triad of Susac's syndrome but was misdiagnosed with multiple sclerosis. It took three years from disease onset before the eventual diagnosis was recognized, and by this time, he had accrued a lot of neurological deficits. With this case report, we aim to draw awareness to this rare but unforgiving entity which if missed can lead to the accumulation of disabilities. We also aim to emphasize the features that help distinguish it from multiple sclerosis, the condition with which it is most often confused.
苏萨克综合征是一种罕见的炎症性微血管病,其特征为视网膜病变、脑病和听力损失三联征。该综合征会导致这些器官反复出现微梗死,进而表现为反复的视野缺损发作、听力损失和耳鸣,以及各种脑部综合征。随着时间的推移,这些往往会导致严重的残疾累积,尤其是在诊断出现延误或失误的情况下。与这种疾病相关的脑部病变可能类似于多发性硬化症的病变,对于那些不熟悉该疾病及其独特临床和放射学特征的人来说尤其如此。这种误诊可能会产生严重的临床后果。在此,我们报告一例41岁的丹麦男性病例,该患者表现出苏萨克综合征的典型三联征,但被误诊为多发性硬化症。从疾病发作到最终确诊历时三年,此时他已经积累了大量神经功能缺损。通过本病例报告,我们旨在提高对这种罕见但严重的疾病的认识,若漏诊可能导致残疾累积。我们还旨在强调有助于将其与最常与之混淆的多发性硬化症相鉴别的特征。