Abstract Details

Title Atypical Epilepsy in Common Variable Immunodeficiency: A Single Institution Case Series

Topic Autoimmune Neurology

Presentation(s) P1 - Poster Session 1 (5:30 PM-6:30 PM)

Poster/Presentation
Number 15-044

Objective To report atypical epilepsy presentations associated with CVID.

Background Common variable immunodeficiency (CVID) is defined by hypogammaglobulinemia, poor vaccine response, and onset after the age of four years old in the absence of an alternative explanation for immunodeficiency. Treatment involves replacement intravenous immunoglobulin (IVIG). Patients are at increased risk of recurrent infections, autoimmunity, and malignancy. They also may present with co-existing neurological disorders, in particular neuroinflammatory or neuroinfectious diseases. An underrecognized aspect of CVID is that patients may also develop seizure disorder.

Design/Methods We performed a retrospective chart review of patients within the University of Utah Healthcare system meeting diagnostic criteria for CVID who were also managed by at least one University of Utah Neurology provider for epilepsy. We excluded patients with abnormal spells without abnormal EEG or patients with provoked seizure.

Results Each patient is described in terms of relevant clinical data including age at seizure onset, age at CVID diagnosis, seizure semiology, electroencephalography and imaging findings, time to intravenous immunoglobulin (IVIG) initiation, and treatment response.

Conclusions We describe an association between atypical epilepsy and CVID. The existence of CVID is important to consider in patients with atypical and/or autoimmune mediated epilepsy as they may benefit from IVIG replacement. Likewise, a strong clinical suspicion for atypical epilepsy (and consideration for obtaining EEG) is important in patients with known CVID who present with concern for seizure.

Authors/Disclosures
Jonathan R. Galli, MD (University of Utah) PRESENTER	Dr. Galli has nothing to disclose.
	No disclosure on file
Julia Klein, NP (University of Utah School of Medicine)	An immediate family member of Ms. Klein has received personal compensation for serving as an employee of Amgen. An immediate family member of Ms. Klein has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Amgen.
	No disclosure on file
Jonathan Graff-Radford, MD, FAAN	Dr. Graff-Radford has received personal compensation for serving as an employee of Mayo Clinic. Dr. Graff-Radford has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for NINDS/NIH. Dr. Graff-Radford has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for JAMA Neurology. Dr. Graff-Radford has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Open evidence . The institution of Dr. Graff-Radford has received research support from NIH. The institution of Dr. Graff-Radford has received research support from Eisai. The institution of Dr. Graff-Radford has received research support from Cognition therapeutics. Dr. Graff-Radford has received personal compensation in the range of $5,000-$9,999 for serving as a Faculty Member with IMPACT AD .
John E. Greenlee, MD, FAAN (University of Utah)	Dr. Greenlee has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Medlink. Dr. Greenlee has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Zeigler Cohen Roche. Dr. Greenlee has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Sommers Schwartz PC. Dr. Greenlee has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for St Francis Hospital. Dr. Greenlee has received publishing royalties from a publication relating to health care. Dr. Greenlee has received publishing royalties from a publication relating to health care.
Stacey Clardy, MD, PhD, FAAN (University of Utah)	Dr. Clardy has received personal compensation for serving as an employee of Veterans Health Administration (VHA). Dr. Clardy has received personal compensation for serving as an employee of University of Utah Health. Dr. Clardy has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for AstraZeneca/Alexion. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen/Horizon. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Arialys. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kyverna. Dr. Clardy has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Neurology/AAN Publications. The institution of Dr. Clardy has received research support from NIH/NINDS. The institution of Dr. Clardy has received research support from SRNA. The institution of Dr. Clardy has received research support from Alexion/AstraZeneca. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a AAN Summer Meeting CoDirector Travel and Lodging with AAN. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a Grand Rounds Travel/Lodging/Honoraria with U of Iowa, Miami, Stanford, Barrow, Beaumont Health, CCF, Emory, Penn State, Mayo Clinic, Walter Reed.

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