A case of relapsing polychondritis with vestibular neuritis and cerebellar ataxia

Abstract

Relapsing polychondritis(RP) is an autoimmune disease characterized by recurrent inflammatory episodes of cartilaginous structures. It is challenge to diagnosis RP, because it is established by clinical manifestations and the disease has diverse nature. We report interesting case, who had recurrent vertigo be seen as vestibular neuritis, diagnosed with RP. A 59-year-old male visited the emergency room with dizziness and ataxia. The symptom was recurrent from 6 months ago, six attacks were occurred. He also had 1-year history of swelling of both ear lobes. He felt repetitive vertigo and gait disturbance spontaneously recovered after few hours. Neurologic exam showed cerebellar ataxia and left spontaneous beating nystagmus. He showed right side deviation in past-pointing test. He had purplish erythema of both ear lobes, diagnosed as polychondritis. The VOG findings were compatible with vestibular neuritis. There were no specific findings on Brain MRI. In laboratory tests, RA was elevated, anticardiolipin Ab IgG was positive. Episodic ataxia gene was negative. In Seoul Neuropsychological Screening Battery, all domain functions were decreased. After treatment with high dose steroid and intravenous immunoglobulin treatment, neurologic deficit and chondritis were recovered. Relapsing polychondritis is a multisystemic autoimmune disorder. Neurological symptoms occur in minor cases but cerebellar symptoms could be presented. Cerebella ataxia could be seen in RP, but there were no vestibular neuritis cases by RP. In this case, the patient was clinically diagnosed as RP with recurrent chondritis of both auricles and vestibular neuritis with good response to immunosuppressive treatment. To confirm recurrent vertigo with RP, long term observation is needed.