Susac Syndrome, a Retino-Cochleo-Cerebellar Vasculitis: A Case-Series and Literature Review

Abstract

Susac syndrome is a rare autoimmune disorder of unknown aetiology that is characterised by a clinical triad of subacute encephalopathy, visual loss secondary to retinal branch artery occlusions, and sensorineural hearing loss. The authors report three patients with Susac syndrome and review the clinical presentations and further discuss treatment options. To date, this disorder has remained uncommon worldwide and the authors aim to expand this by reporting a case-series of this rare disease. Early recognition of the disorder is crucial for the initiation of early treatment with long-term systemic immunosuppressant therapies to prevent neurological involvement and ophthalmologic and auditory impairment.

KEYWORDS::

• retinocochleocerebellar vasculitis
• Susac syndrome
• vasculitis

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.