# Ear and Nose Abnormalities in Meningoencephalitis Associated With Relapsing Polychondritis: A Case Report

## TL;DR

A rare case of relapsing polychondritis with meningoencephalitis highlights the importance of checking ear and nose abnormalities for early diagnosis.

## Contribution

This case report emphasizes the diagnostic value of ear and nose deformities in identifying CNS involvement in relapsing polychondritis.

## Key findings

- A 67-year-old woman with meningoencephalitis was diagnosed with relapsing polychondritis after noticing ear and nose deformities.
- Immunotherapy and reduced intrathecal interleukin-6 levels led to significant improvement in the patient's condition.
- Ear abnormalities were a key diagnostic clue for relapsing polychondritis with central nervous system involvement.

## Abstract

Relapsing polychondritis (RPC) is a rare autoimmune disease that affects cartilage and connective tissue, especially the ear and nose. The diagnosis of relapsing polychondritis is challenging due to its low incidence, and cases with meningoencephalitis as the predominant manifestation are particularly difficult to identify. We report a case of meningoencephalitis associated with relapsing polychondritis. A 67-year-old woman who had a 1.5-month history of fever, acute impaired consciousness, and nuchal rigidity was diagnosed with aseptic meningoencephalitis of uncertain etiology and referred to our hospital. Cerebrospinal fluid (CSF) analysis revealed pleocytosis and high levels of interleukin-6 (IL-6). Head magnetic resonance imaging (MRI) revealed a meningeal enhancement. Her ear and nose deformities were noted, and a biopsy from her pinna was conducted. Based on the histological findings, relapsing polychondritis was diagnosed with central nervous system (CNS) involvement. The symptoms were partially relieved by immunotherapy, and intrathecal interleukin-6 decreased. She has significantly improved and was discharged six months after the initiation of treatment. Since the most frequent clinical manifestation of relapsing polychondritis is auricular chondritis, this finding can become a clue to diagnosis. Neurologists, upon the diagnosis of meningoencephalitis of unknown cause, should carefully examine for nasal and, especially, ear abnormalities, considering the potential for central nervous system involvement in relapsing polychondritis.