Clinical Presentation and Outcome in Autoimmune Encephalitis Associated With N-Type Voltage-Gated Calcium Channels in Children.

Abstract

We present the diagnostic and clinical course of the first multicenter case series of pediatric patients with autoimmune encephalitis associated with N-type voltage-gated calcium channel antibodies. Data from 2 university hospitals were retrospectively reviewed and records of 3 patients with autoimmune encephalitis associated with N-type voltage-gated calcium channel antibodies were evaluated. The 3 pediatric patients (all female) had symptoms that spanned the clinical spectrum. All 3, however, had regression of expressive language and agitation. Neuroimaging in all 3 patients was normal; electroencephalographic (EEG) findings varied among the 3 patients. Positive titers against the N-type voltage-gated calcium channel antibody were found in their cerebrospinal fluid. Following administration of intravenous immunoglobulin, all 3 had improvement in their core presenting symptoms. Autoimmune encephalitis associated with N-type voltage-gated calcium channel antibodies in the pediatric population presents with a wide clinical spectrum, although expressive language delay and agitation seem to be common symptoms. Treatment with intravenous immunoglobulin improves core symptoms.