Rheumatoid meningitis (RM) has been previously recognized to present as spells of neurological dysfunction in patients with longstanding Rheumatoid Arthritis (RA). Additionally, RM has been reported as the presenting manifestation of RA, however spells of neurological dysfunction in the pre-clinical period of the systemic disease have never been reported as manifestation of RM.

Brain MRI with contrast showed diffuse leptomeningeal enhancement in high frontoparietal regions bilaterally with associated T2 FLAIR hyperintensity. EEG did not demonstrate epileptiform abnormalities. Initial workup revealed elevated serum rheumatoid factor (RF) (245 UI/mL) and anti-Cyclic Citrullinated Peptide 3 (CCP) (16.2 U/mL). CSF demonstrated positive RF (292 UI/mL) and CCP (31 U/mL) with no other abnormalities. Diagnostic cerebral angiogram showed no angiographic evidence of cerebral vasculitis. Biopsy of the lesion demonstrated chronic inflammatory cell infiltrate in the leptomeninges and dura, also occasional multinucleated giant cells were demonstrated. Necrotic debris was also seen in the leptomeninges with small focus resembling a rheumatoid nodule. Autoimmune and infectious workup were unremarkable. Clinical improvement was reported after steroids administration. She was started on azathioprine and methotrexate with no recurrence of neurological spells.

The lack of diagnostic criteria and the frequent need for tissue confirmation have made this serious complication of RA a challenging diagnosis. Accordingly, RM should be considered in patients who present with neurological spells of unknown etiology and elevated serum markers in the pre-clinical period of RA.