Abstract Details

Title Neurologic Autoimmunity in the Immunodeficient: A Case of Neuromyelitis Optica Developing in an Individual with Common Variable Immunodeficiency

Topic Autoimmune Neurology

Presentation(s) P7 - Poster Session 7 (11:45 AM-12:45 PM)

Poster/Presentation
Number 14-002

Objective Neuromyelitis optica spectrum disorder (NMOSD) is a relapsing CNS autoimmune disorder most commonly characterized by longitudinally extensive transverse myelitis (LETM) and/or optic neuritis (ON). Common variable immunodeficiency (CVID) is the most common clinically significant primary immunodeficiency, characterized by immune dysregulation and decreased immunoglobulin production. Autoimmunity is common in CVID, however neurologic autoimmunity is exceedingly rare and co-occurrence with NMOSD has not been previously described.

Background We present the case of a 62-year-old man with CVID who presented with LETM and was subsequently diagnosed with NMOSD.

Design/Methods NA

Results Our patient had been treated with intravenous immunoglobulin therapy for CVID for 5 years when he presented with subacute onset of ascending paraparesis, sensory loss, and urinary retention. MRI identified LETM from C3 to T9. Anti-aquaporin 4 (AQP4) serology was markedly elevated at a titer of >1:100,000. He responded well to immunotherapy.

Conclusions Autoimmunity in CVID is thought to be from loss of regulatory T cell function as well as increased peripheral plasmablasts. We highlight the importance of considering autoimmune CNS diseases in primary immunodeficiencies as well as review possible pathophysiology of neurologic autoimmunity in CVID.

Authors/Disclosures
Yujie Wang, MD (UW Northwest) PRESENTER	Dr. Wang has received personal compensation in the range of $500-$4,999 for serving as a Consultant for TG Therapeutics. The institution of Dr. Wang has received research support from Genentech. The institution of Dr. Wang has received research support from uniQure. The institution of Dr. Wang has received research support from NIH/NINDS.
Monica Solorio, MD	Miss Solorio has nothing to disclose.
Evan Luxenberg, MD (Kaiser Permanente Medical Group)	Dr. Luxenberg has nothing to disclose.
Kathleen Munger, MD (Multicare Health System)	Dr. Munger has nothing to disclose.
Andrew Ayers	No disclosure on file

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