Leptomeningeal inflammation in rheumatoid arthritis

Abstract

A 44-year-old man with a 20-year history of seropositive erosive rheumatoid arthritis (RA), diagnosed according to the American Rheumatology Association revised criteria,1 was referred for severe headache and partial simple motor seizures. At the time of presentation, his disease was inactive under a stable dose of methylprednisolone (8 mg/day). The neurologic examination was normal: there were no cranial nerve palsies, sensory motor deficits, gait imbalance, impairment of cognitive functions, or neck stiffness. Fundoscopy did not reveal any abnormality. The major finding on neuroimaging was a marked leptomeningeal enhancement (figure, A, C, and E). CSF analysis showed a normal opening pressure, normal cell count, and a slightly increased immunoglobulin G index; viral markers and culture studies were unremarkable. Biopsy specimen displayed necrotizing granulomatous inflammation of leptomeninges without pachymeningeal involvement (figure, G and H). IV methylprednisolone was prescribed (1 g/day for 3 days) followed by methotrexate in order to enhance immunosuppression and avoid an increase of the steroid load; favorable clinical and radiologic responses were observed (figure, B, D, and F). Acknowledgment: The authors thank Dr. Francesco Sessa (Department of Neuroradiology, University Hospital of Ancona, Italy) and Dr. Sara Zagaglia (Neurological Clinic, University Hospital of Ancona, Italy) for editing assistance.