Abstract
Relapsing polychondritis (RP) is a rare and, if not treated, potentially lethal autoimmune disorder. Involvement of central nervous system (CNS) in RP is rare and, when present, makes it extremely difficult to diagnose. In this report, we present a case of a 22-year-old Hispanic woman who presented with sudden onset of headache and blurred vision. Magnetic resonance imaging (MRI) of her brain and orbit showed leptomeningeal enhancements in addition to asymmetrical thickening and enhancement of globes. Her lumbar puncture was consistent with aseptic meningitis picture, and she was placed on empirical treatment for presumptive CNS tuberculosis. Her vision deteriorated, and she was diagnosed with RP with CNS and ocular involvement and placed on high-dose steroids with dramatic rapid response. She has been on immunosuppressive treatment, including Sulfasalazine and Methotrexate, since then and her disease has been under control with decreased need for ophthalmic steroid drops. There have been only 19 previous cases found in literature reporting an association of RP with CNS involvement.
Highlights
Relapsing polychondritis (RP) is a rare autoimmune disease that is characterized by inflammation of cartilaginous tissue, affecting the ears, nose, respiratory tract, eyes, and joints.[1]
A 22-year-old Hispanic woman with RP-related chorioretinitis and central nervous system (CNS) involvement manifested by decreased visual acuity and headache is presented
Neurological involvement is an extremely rare complication in RP which occurs in only 3% of patients
Summary
Relapsing polychondritis (RP) is a rare autoimmune disease that is characterized by inflammation of cartilaginous tissue, affecting the ears, nose, respiratory tract, eyes, and joints.[1]. Other clinical manifestations previously reported are stroke, meningoencephalitis, polyneuritis, and dementia.[2] The presence of meningoencephalitis is a very rare neurological manifestation of RP, making the diagnosis extremely difficult for it is often missed or unrecognized for months to years In this case report, a 22-year-old Hispanic woman with RP-related chorioretinitis and central nervous system (CNS) involvement manifested by decreased visual acuity and headache is presented. A 22-year-old Hispanic woman was in her usual state of health until she developed severe headache (8 of 10 scale) and blurred vision for last 5 days She was seen at the ophthalmology clinic with marked reduction in vision of her right eye more than left that slowly progressed over 5 days. In the following 15 days, she did not improve clinically and instead developed new symptoms Her new symptoms included tinnitus in left ear, vertigo, hyperacusis, photophobia, retroorbital pain, and loss of color perception. During her pulse therapy and following days after, her visual acuity improved, color vision returned, and vertigo and hyperacusis resolved
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