Relapsing polychondritis-associated meningoencephalitis initially presenting as seizure: a case report and literature review

Abstract

Background and purpose: Relapsing polychondritis (RP) is a rare rheumatologic disorder that may affect the neurological system with various presentations. In this study, we present a case and summarize the clinical characteristics of RP-associated meningoencephalitis.

Case presentation: A 48-year-old man presented with first-ever seizures that were well controlled by valproate. Physical examination results were unremarkable, except for binaural deformation. The initial brain magnetic resonance imaging (MRI) without contrast and electroencephalogram (EEG) findings were normal. However, the patient subsequently developed recurrent fever, scleritis, headache, lethargy, and left arm paresis. Repeated brain MRI with contrast demonstrated increased enhancement of the pia mater and abnormal diffusion-weighted imaging (DWI) signals in the bilateral auricles. The cerebrospinal fluid (CSF) analysis showed 2 leukocytes/μL, 736.5 mg/L of protein, and no evidence of infectious disease or autoimmune encephalitis. Meningoencephalitis secondary to RP was considered. The patient's condition improved significantly and quickly with the administration of dexamethasone (10 mg per day). Oral methylprednisolone was continued, and the patient remained well without relapse during the 9-month follow-up period.

Conclusion: RP-associated meningoencephalitis is rare but fatal. Although symptoms vary, red or deformed ears remain the most common and suggestive features. Non-specific parenchymal changes and/or meningeal enhancement can be observed on brain MRI scans. CSF lymphocytic pleocytosis with mild protein elevation was observed in most patients.

Keywords: immunosuppressants; inflammatory meningoencephalitis; neuroimmune disease; relapsing polychondritis; seizure.

Figure 1

Adapted PRISMA 2020 flow diagram.

Figure 2

Deformed ear.

Figure 3

Brain CT scan showing calcification (arrows), which can be seen in the polychondritic ears (A). DWI shows abnormal signals in the bilateral auricles (B, arrows). T1-weighted MRI with contrast showed abnormal signals and thickening of both ears (C, arrows) and increased enhancement of the pia mater (D).