A case of infliximab–resistant neurosarcoidosis successfully treated with rituximab

Abstract

A 39–year–old man was admitted to our hospital with vomiting and diplopia. He was found to have external ophthalmoplegia, bilateral hilar lymphadenopathy, and hydrocephalus. Neuroendoscopic biopsy of the ventricular wall revealed a noncaseating epithelioid cell granuloma, confirming the diagnosis of neurosarcoidosis. After endoscopic third ventriculostomy (ETV) and ventriculoperitoneal shunting, the patient was treated with steroids and methotrexate ; however, hydrocephalus recurred after six months. The patient was treated with infliximab, a TNF–α inhibitor. Although the patient remained in remission for three years, he experienced recurrence of multiple cortical–enhanced lesions. After rituximab, an anti–CD20 antibody, was administered, the multiple cortical–enhanced lesions disappeared, and the patient remained in remission > 1 year. Rituximab may be a useful treatment option for patients with neurosarcoidosis that is refractory to steroids, immunosuppressive agents, and infliximab.