Abstract Details

Title Susac Syndrome Associated with Adenovirus Infection

Topic Autoimmune Neurology

Presentation(s) P9 - Poster Session 9 (5:00 PM-6:00 PM)

Poster/Presentation
Number 2-009

Objective To describe a potential novel association between adenovirus infection and the onset of Susac Syndrome (SuS).

Background SuS is a rare autoimmune endotheliopathy of the brain, retina, and inner ear which classically presents with encephalopathy, branch retinal artery occlusion (BRAO), and sensorineural hearing loss. Its pathophysiology is presumed immune-mediated, and infectious triggers have been proposed. Adenovirus has not previously been described as an infectious trigger for SuS.

Design/Methods Case report.

Results A 24-year-old woman presented with two weeks of progressive headaches, fevers, nausea, and cognitive decline. Examination revealed lethargy, disorientation, and left-sided arm/leg weakness. Given the presence of headaches, fevers, and nausea/vomiting, a broad infectious workup was obtained and notable for a positive adenovirus PCR on a respiratory viral panel. MRI brain demonstrated diffusion-restricting lesions in the corpus callosum, deep white matter, and pons. CSF studies showed lymphocytic pleocytosis (WBC 8; 92% lymphocytes, 8% monocytes) and elevated protein (201 mg/dl). Serum and CSF evaluations for infectious, autoimmune, and neoplastic/paraneoplastic etiologies were otherwise unrevealing. Fluorescein angiography revealed left eye periarterial vasculitis and right eye focal BRAO, consistent with SuS. Audiology evaluation was normal. The patient received IV steroids (1000mg methylprednisolone for 7 days) and a course of IV immunoglobulin (IVIg, 2000 mg/kg total over 3 days) for probable SuS. She had marked improvement in symptoms and was continued on maintenance rituximab, IVIg, and a prednisone taper in the outpatient setting.

Conclusions This case highlights a possible link between adenovirus and SuS, suggesting that infection may act as a trigger in susceptible individuals. Infectious triggers have been proposed in SuS, however, an association with adenovirus infection has not yet been reported. SuS often presents with nonspecific clinical features such as encephalopathy, making diagnosis challenging. Recognizing parainfectious presentations may aid in early detection and potentially improve understanding of pathophysiology.

Authors/Disclosures
Joy Suh, MD PRESENTER	Dr. Suh has nothing to disclose.
Hunter Greer, MD (Anschutz Medical Campus)	Dr. Greer has nothing to disclose.
Jeffrey L. Bennett, MD, PhD, FAAN (University of Colorado School of Medicine)	Dr. Bennett has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Amgen. Dr. Bennett has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Alexion. Dr. Bennett has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech. Dr. Bennett has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Roche. Dr. Bennett has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Mitsubishi Tanabe. Dr. Bennett has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Novartis. Dr. Bennett has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Immpact Bio. Dr. Bennett has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Chugai. Dr. Bennett has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Beigene. Dr. Bennett has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Imcyse. Dr. Bennett has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Bennett has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for MIAC. Dr. Bennett has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for CorEvitas. Dr. Bennett has received personal compensation in the range of $50,000-$99,999 for serving on a Speakers Bureau for Alexion. Dr. Bennett has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Vindico. Dr. Bennett has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Touch IME. Dr. Bennett has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Efficient LLC. Dr. Bennett has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Pavich. Dr. Bennett has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Marie Bush. Dr. Bennett has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Marks Gray. Dr. Bennett has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Knight, Nicastro, MacKay. The institution of Dr. Bennett has received research support from Alexion. The institution of Dr. Bennett has received research support from Genentech. Dr. Bennett has received intellectual property interests from a discovery or technology relating to health care. Dr. Bennett has received publishing royalties from a publication relating to health care.
Daniel Pastula, MD, MHS, FAAN (University of Colorado, Department of Neurology)	Dr. Pastula has nothing to disclose.
Andrew Wolf, MD	The institution of Dr. Wolf has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech. The institution of Dr. Wolf has received personal compensation in the range of $500-$4,999 for serving as a Consultant for TG Therapeutics. The institution of Dr. Wolf has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Horizon. The institution of Dr. Wolf has received research support from Genentech. The institution of Dr. Wolf has received research support from Rocky Mountain MS Center. The institution of Dr. Wolf has received research support from Michael J. Fox Foundation. Dr. Wolf has received publishing royalties from a publication relating to health care.

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