Immunotherapy of oneiric stupor in Morvan syndrome: Efficacy documented by actigraphy

Abstract

Morvan syndrome (MoS) is a rare acquired channelopathy associated with autoantibodies against potassium channel complex and clinically characterized by the heterogeneous combination of neuromyotonia, dysautonomia, and encephalopathy.1 Peculiar CNS symptoms are organic insomnia, which ranges from mild to malignant forms characterized by complete loss of sleep, and persistent motor/autonomic hyperactivation (agrypnia excitata2). We describe a patient with MoS who developed progressive agrypnia excitata, in which IV immunoglobulin (IVIg) treatment induced a progressive recovery of the physiologic sleep-wake cycle, documented by videopolysomnography (VPSG) and actigraphic monitoring. Acknowledgment: The authors thank the patient, Francesco Mignani for help in preparing the figures, Elena Zoni for help in preparing the videos, and Cecilia Baroncini for English revision.