• Constanza Vera, José Manuel Matamala, Walter Feuerhake, Oscar Neira, Aaron Vidal, and José Luis Castillo.
• Departamento de Ciencias Neurológicas Oriente, Facultad de Medicina, Universidad de Chile, Santiago, Chile.
• Rev Med Chil. 2021 Jul 1; 149 (7): 1085-1089.

AbstractRelapsing polychondritis (RP) is a rare multisystemic autoimmune disorder characterized by the inflammation and destruction of cartilages, with preference for auricular, nasal and laryngotracheal cartilages. RP may also affect proteoglycan-rich structures, such as, blood vessels, eyes, kidneys, and heart. The central nervous system (CNS) is involved in less than 3% of patients. We report a 32-year-old female with RP associated with a progressive subacute encephalopathy characterized by behavioral disturbances, auditory and visual hallucinations. The EEG showed generalized slow activity and a mononuclear pleocytosis with increased protein was found in the cerebrospinal fluid. The brain magnetic resonance imaging showed multiple supra and infratentorial nodular inflammatory lesions. After initiating treatment with corticosteroids and cyclophosphamide, a significant improvement in chondritis and neurological status was observed.

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