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Clinical Presentation and Outcome in Autoimmune Encephalitis Associated With N-Type Voltage-Gated Calcium Channels in
Jeffrey Kornitzer1,2, Firas Taha3,4, Eric Segal3,4
11 Rutgers New Jersey Medical School, Newark, NJ, USA.
Objective:
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.
Methods:
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.
Results:
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.
Conclusion:
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.
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