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In Vivo Morphometric Analysis of Human Cranial Nerves Using Magnetic Resonance Imaging in Menière's Disease Ears and Normal Hearing Ears
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Ear and Nose Abnormalities in Meningoencephalitis Associated With Relapsing Polychondritis: A Case Report

Tomoaki Taguchi1, Soichiro Matsubara1, Keiichi Nakahara1

  • 1Neurology, Kumamoto University, Kumamoto, JPN.

Cureus
|July 4, 2025
PubMed

Insights

Relapsing polychondritis (RPC) can cause meningoencephalitis, a challenging diagnosis. Early recognition of ear and nose deformities aids in identifying this rare autoimmune condition with central nervous system involvement.

Area of Science:

  • Rheumatology
  • Neurology
  • Pathology

Background:

  • Relapsing polychondritis (RPC) is a rare autoimmune disorder primarily affecting cartilage and connective tissues.
  • RPC diagnosis is challenging due to its low incidence and varied presentations.
  • Meningoencephalitis as the dominant symptom of RPC is particularly difficult to diagnose.

Observation:

  • A 67-year-old woman presented with fever, impaired consciousness, and nuchal rigidity, initially diagnosed as aseptic meningoencephalitis.
  • Cerebrospinal fluid analysis showed pleocytosis and elevated Interleukin-6 (IL-6).
  • Head MRI revealed meningeal enhancement, and physical examination noted ear and nose deformities.

Findings:

  • Histopathological examination of a pinna biopsy confirmed relapsing polychondritis.
  • The patient was diagnosed with RPC involving the central nervous system (CNS).
  • Immunotherapy led to partial symptom relief and decreased intrathecal IL-6 levels.

Implications:

  • Auricular chondritis (ear inflammation) in RPC can serve as a crucial diagnostic clue.
  • Neurologists should consider RPC in cases of meningoencephalitis of unknown origin.
  • Prompt recognition of characteristic physical abnormalities can facilitate timely diagnosis and treatment of RPC with CNS involvement.
Keywords:
case reportmeningoencephalitispinna deformityrelapsing polychondritissaddle nose

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