Susac's Syndrome: A Tale of Disability Due to Late Recognition

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.