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.

Rheumatoid arthritis with pachymeningitis – a case presentation and review of the literature

“…While common neurological symptoms like headaches, seizures, focal neurological deficits, altered mental condition and cranial nerve palsies that present as blindness or sensorineural deafness were reported over the years [12,14], some rare symptoms have been described in the recent times which include neuropsychiatric presentation [5] and acute parkinsonism [15]. Due to these varied neurological symptoms and a multitude of etiologies for pachymeningitis, diagnosis of rheumatoid meningitis is challenging.…”

“…Treatment for RA was continued or was changed to rituximab which successfully managed the RA progression [1,3,7,25,35]. Elevated serum ACE and β-2 microglobulin were reported by Pellerin et al [15] and McKenna et al [8] although, these findings haven't been clearly associated with RM [8,15]. Some patients had pulmonary manifestations in the form of nodules or pleural effusions which were attributed as extra-articular manifestations of RA after examinations [22,32,35].…”

Meningeal rheumatoid nodules in a 55-year-old man presenting with chronic headaches and oculomotor nerve palsy: an uncommon extra-articular manifestation of rheumatoid arthritis

“…Review of literature showed only n=13 cases of rheumatoid nodules in the CNS reported since 2000 4–15. These are presented in table 1.…”

Inflammatory Manifestations of Systemic Diseases in the Central Nervous System

“…In addition to diseasemodifying therapy, there is evidence that the transient neurological deficits in RA meningoencephalitis should be treated with antiepileptic drugs (AEDs). Even episodes that are not clearly electroclinical seizures seem to respond to AED's [34,39]. Evidence for treatment of neurosarcoidosis (NS) is based on cases series and expert opinion [40•, 41-43].…”

“…Less than half of patients with CNS disease have active synovitis and 34% of patients had no history of RA prior to diagnosis [ 33 ]. The most common brain MRI finding is nodular patchy or lepto-meningeal thickening and enhancement, and biopsy is the gold standard for diagnosis [ 2 •, 34 ].…”