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Abstract Details

Presence of P/Q Voltage-gated Calcium Channel (VGCC) Antibodies in Pediatric Autoimmune Encephalitis
Autoimmune Neurology
P6 - Poster Session 6 (12:00 PM-1:00 PM)
Neuronal VGCCs are present in the central nervous system and play a role in various neuronal pathways.  Both P/Q and N-type VGCC antibodies have been implicated in various autoimmune neurological disorders, and are commonly paraneoplastic.  These antibodies are uncommonly reported in the pediatric population, and thus the clinical presentation and oncologic association is less clear.   
We present two pediatric patients who presented to Texas Children’s Hospital with varying neurologic symptoms and ultimately diagnosed with VGCC antibody-mediated autoimmune encephalitis.
A retrospective chart review was performed with collection of demographic and clinical data. 
A 12 year old male presented with neurologic exam consistent with cerebellar ataxia, with dysmetria, slowed speech, and behavior change. The second, a 14 year old male with neurologic exam consistent with myelitis, with weakness, hyperreflexia, and sensory level.  Both patients underwent MRI, one demonstrating mild smooth enhancement of the cauda equina.  Laboratory evaluation for both patients included lumbar puncture, one with elevated WBC (7) and elevated protein (55). Both with oligoclonal bands present.  On paraneoplastic panel, both were found to have elevated P/Q-type VGCC antibodies, and diagnosed with VGCC antibody mediated autoimmune encephalitis.  Clinical work up additionally included metabolic studies and further autoimmune studies, which were unremarkable.  Patient 2 was also found to have N-type VGCC antibodies, but at a low level and felt to be non-contributory.  Both patients received immunotherapy (IVMP and IVIG) with improvement in symptoms, but continued to have deficits. Both patients underwent oncologic screening, which was unrevealing.
Pediatric patients with VGCC antibodies can present with an array of neurologic symptoms, including cerebellar ataxia, myelitis, and cognitive changes.  In the pediatric population, there appears to be less correlation with oncologic disease.  Patients show improvements with immune therapy, but with residual deficits in follow up.  
Kristen Fisher, DO (Baylor College of Medicine)
Dr. Fisher has nothing to disclose.
Nikita Shukla, MD (BCM) The institution of Dr. Shukla has received research support from Roche.