Rheumatoid Meningitis Presenting With Acute Parkinsonism and Protracted Non-convulsive Seizures: An Unusual Case Presentation and Review of Treatment Strategies

Abstract

Rheumatoid meningitis is a rare complication of rheumatoid arthritis (RA). It is associated with substantial morbidity and mortality. The condition may present in a variety of ways and is therefore diagnostically challenging. Uncertainty still exists regarding the optimal treatment strategy. Herein, we describe the case of a 74-year-old man with a history of well-controlled seropositive RA on low-dose prednisone, hydroxychloroquine, and methotrexate. The patient presented with a several-month history of multiple prolonged episodes of expressive aphasia, right hemiparesis, and encephalopathy. Although no epileptiform activity was recorded on repeated electroencephalography, the symptoms fully resolved following treatment with antiepileptic drugs. He subsequently developed acute asymmetrical parkinsonism of the right hemibody. Magnetic resonance imaging revealed subtle enhancement of the leptomeninges over the left frontoparietal convexity. Cerebrospinal fluid analysis revealed a mild lymphocytic pleocytosis and elevated proteins. Histopathologic analysis of a meningeal biopsy revealed nodular rheumatoid meningitis. The patient was treated with corticosteroids and cyclophosphamide, following which he incompletely recovered. This is the first description of rheumatoid meningitis manifesting with acute parkinsonism and protracted non-convulsive seizures. A summary of cases reported since 2005, including data on pathology, therapy and outcomes, along with a discussion on the efficacy of different treatment strategies are provided.

Keywords: corticosteroids; immunosuppressant; parkinsonism; rheumatoid arthritis; rheumatoid granuloma; rheumatoid meningitis; seizure; vasculitis.

Figure 1

Brain magnetic resonance imaging (MRI)—Rheumatoid meningitis. (A) Axial T1-weighted sequence post-gadolinium shows faint contrast enhancement of the leptomeninges and underlying gyri over the left convexity. (B) Finite areas of diffusion restriction of the left parietal cortex near the vertex on axial diffusion weighted imaging (DWI) sequence. (C) Coronal T1-weighted sequence post-gadolinium shows longitudinal right frontal leptomeningeal and faint left leptomeningeal contrast enhancement. (D) Axial DWI sequence shows new areas of restricted diffusion in the right frontal parafalcine region along with increased volume of restricted diffusion in left parietal cortex near the vertex. (E) Axial T1-weighted sequence post-gadolinium obtained 3 months following immunosuppressive therapy showing no abnormal contrast enhancement, and left frontal postoperative changes. (F) Axial DWI sequence obtained 3 months following immunosuppressive therapy and demonstrating the resolution of previously documented findings.

Figure 2

Meningeal histologic sections—Rheumatoid meningitis. Representative hematoxylin and eosin (H&E) stained sections. (A) Meningothelial hyperplasia (magnification 200). (B) Necrobiotic core surrounded by palisading macrophages (magnification 200). (C) Cluster of inflammatory infiltrate cells consisting mainly in small lymphocytes, mixed with few plasma cells and histiocytic cells (magnification 400). (D) Diffuse meningeal inflammatory infiltrate (magnification 400).