AUTHOR=Zhang Dan , Shi Jiamin , Zhang Xinhua , Wang Jin , Shao Yuquan 

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

JOURNAL=Frontiers in Neurology

VOLUME=Volume 14 - 2023

YEAR=2023

URL=https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2023.1265345

DOI=10.3389/fneur.2023.1265345

ISSN=1664-2295

ABSTRACT=Background and Purpose: Relapsing polychondritis (RP) is a rare rheumatologic disorder which may affect neurological system with various presentations. Herein, 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. Cerebro-spinal fluid (CSF) analysis showed 2 leukocytes/μL, protein 736.5 mg/L, 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 (10mg per day). Oral methylpredisolone was continued, and the patient remained well without relapse during the 9-month follow-up period.
Conclusions: RP-associated meningoencephalitis is rare, but fatal. Although symptoms vary, red or deformed ears remain the most common and suggestive features. Nonspecific 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.