Abstract

We present a unique case of a patient with anti–dipeptidyl peptidase-like protein 6 (DPPX) encephalitis in which severe pruritus was the cardinal symptom. Anti-DPPX encephalitis is caused by cell surface autoantigens to DPPX, a subunit of the Kv4.2 potassium channel.1 Most patients had a combination of limbic encephalitis, brainstem dysfunction, diarrhea, and weight loss.1–3 We describe a patient with severe pruritus and provide long-term follow-up, offering recommendations for treatment.

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