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CASPR2 Morvan Syndrome — RACP Adult Medicine MCQ

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HardNeurologyCASPR2 Morvan SyndromeRACP Adult Medicine

A 55-year-old woman with CASPR2 antibody encephalitis (Morvan syndrome: peripheral nerve hyperexcitability, autonomic dysfunction, insomnia, encephalopathy) has a thymoma identified on CT chest. What is the most important first step in treatment?

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Correct answer: B — Plasma exchange alone

Morvan syndrome (CASPR2 antibody-mediated) with thymoma requires both tumour removal and immunotherapy. Plasma exchange is particularly effective in CASPR2-associated disease as antibodies are directly pathogenic (cell-surface target). Thymectomy addresses the immunological trigger. IVIG and corticosteroids are also first-line. Among available options, PLEX is the best specific choice.

Reference: Lancet Neurol 2024 Autoimmune Encephalitis