Relapsing Polychondritis Presenting with Meningoencephalitis and Dementia: Correlation with Neuroimaging and Clinical Features.

PURPOSE: Relapsing polychondritis (RP) is a rare systemic autoimmune disease affecting cartilaginous and non-cartilaginous structures. Neurological involvement is rarer but results in profound disability. Early identification and treatment of underlying RP may promote neurological recovery.

CASE REPORT: We illustrated a 53-year-old man diagnosed with dementia. Neuroimaging and cerebrospinal fluid studies disclosed meningoencephalitis. "Prominent ear sign" was evident on diffusion-weight magnetic resonance imaging. After glucocortisone administration, the improvement of clinical manifestations was closely correlated subsequent neuroimaging findings.

CONCLUSION

The importance of better understanding of this disease in terms of the prevention of further tissue damage in patients with RP cannot be overemphasized.