Neuro Behçet’s disease: report of a rare disease with neurologic presentation

Abstract

Introduction: Behçet’s disease is a multisystem disease consisting of oral lesions, genital ulcers and uveitis with unpredictable evolution. Vasculitic and parenchymal involvement of the central nervous system is uncommon. The aim is to describe a case of systemic rheumatologic disease with unusual central involvement in clinical practice. Case report: Woman, 57-year-old, with a previous history of rheumatoid arthritis for 23 years, presented oral and genital ulcers in addition to paresthesia in the right hemiface and subacute change in visual acuity and bilateral ocular pain in 2017. She was diagnosed with Behçet’s Disease with neurological involvement, being submitted to pulse therapy with Cyclophosphamide and maintenance treatment with Azathioprine. She recurred with the same neurological symptoms in 2020, readmitted in the context of severe thrombocytopenia, being diagnosed with idiopathic thrombocytopenic purpura. Pulse therapy with Methylprednisolone was performed, with improvement of symptoms, but thrombocytopenia was maintained. He opted for the maintenance of Immunoglobulin then, with subsequent initiation of Rituximab. Bilateral ocular sequelae persisted with low acuity and hypoesthesia in the path of the trigeminal nerve on the right. Maintains good control of the disease using Rituximab at weaning and maintenance dose of Prednisone. Conclusion: The prognosis of Behçet’s disease is poor when the central nervous system is affected and studies about treatment still have limited evidence. Early identification of the disease is essential to enable rapid and adequate treatment.