Abstract
BACKGROUND: Recurrent polychondritis is an immune-mediated systemic disease that affects cartilaginous and non-cartilaginous structures. Despite being rare, multiple neurological manifestations have been described, such as involvement of cranial nerves; headache; ataxia; seizures; confusional syndromes; meningitis; limbic encephalitis; cerebral infarcts; psychosis; and dementia. We present a case report of patient with atypical manifestation of recurrent polychondritis. CASE PRESENTATION: A 71-year-old man with history of three episodes of meningitis who was admitted due to headache, walking difficulties, disorientation, loss of sphincter control and prostration. These symptoms were attributed to recurrent lymphocytic meningitis and progressive dementia secondary to relapsing polychondritis with excellent response to treatment with glucocorticoids and methotrexate. CONCLUSION: The accurate identification of atypical manifestations in relapsing polychondritis is essential for the timely implementation of appropriate therapeutic interventions, thereby enhancing the overall quality of life for individuals affected by this pathology.