Abstract

Objectives: This study aims to describe the clinical manifests, CSF features and brain MRI characters of patients with relapsing polychondritis (RP) and central nervous system (CNS) involvement presented as encephalitis. Methods: Seven cases with RP and CNS involvement were enrolled from 2011 to 2015. RP was diagnosed upon the modified McAdam criteria, and CNS dysfunction secondary to RP was decided by clinical manifests, CSF analysis and brain MRI. Results: Of 7 cases, 3 were confirmed RP, and 4 probable. All of these RP patients with CNS involvement presented as encephalitis. The common clinical features of CNS involvement were fever and headache. Brain MRI findings were in diversity. The CNS symptoms were relieved after steroid. Conclusions: CNS involvement in RP has no specific findings both in symptoms and in brain MRI findings. When one patient presenting with encephalitis has extra-CNS manifestations such as eye and ear abnormalities, CNS involvement of RP should be suspected.

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