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.

Autoimmune encephalitis may present across a broad phenotypic spectrum that includes seizures, abnormal movements, autonomic dysfunction, memory deficits, hallucinations, and other behavioral alterations.  A growing litany of antibodies have been associated with autoimmune encephalitis. Fortunately, autoimmune encephalitis is often treatable with immunotherapy, including corticosteroids, intravenous immunoglobulin (IVIG), plasmapheresis, and immunomodulatory therapies.  Antibodies against N-type VGCCs have been previously associated with autoimmune encephalitis in several adults and in one child. 

Data from two university hospital were retrospectively reviewed and records of three patients with autoimmune associated with N-type voltage gated calcium channel antibodies were evaluated.

The three pediatric patients (all female) had symptoms that spanned the clinical spectrum.  All three, however, had regression of expressive language and agitation.  Neuroimaging in all three patients was normal; EEG findings varied among the three patients.  Positive titers against the N-type voltage gated calcium channel antibody were found in their cerebrospinal fluid.  Following administration of IVIG, all three 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.  Anti-N-type VGCC antibody encephalitis is responsive to treatment with IVIG and, as such, should be treated promptly upon diagnosis in order to attenuate the potential risks of long-term neurologic sequelae.