Abstract Details

Title DRESS Complicated by Status Epilepticus and Autoimmune Encephalitis Mimicking HHV-6 Encephalitis

Topic Autoimmune Neurology

Presentation(s) P9 - Poster Session 9 (11:45 AM-12:45 PM)

Poster/Presentation
Number 8-015

Objective To report a case of drug reaction with eosinophilia and systemic symptoms (DRESS) with presumed autoimmune encephalitis mimicking HHV (human herpesvirus)-6 encephalitis, complicated by refractory status epilepticus.

Background HHV-6 reactivates in DRESS, raising the question of whether DRESS-associated encephalitis is driven by HHV-6 or represents a secondary autoimmune encephalitis with unrelated HHV-6 reactivation. To our knowledge, refractory status epilepticus has not been reported in DRESS-associated encephalitis.

Design/Methods Case report.

Results Six weeks after unintentional phenobarbital ingestion, a 38-year-old woman presented with a generalized tonic-clonic seizure. Skin biopsy two weeks prior, prompted by fever, maculopapular rash, and eosinophilia, was consistent with DRESS. Electroencephalogram (EEG) showed status epilepticus with right temporal and midline onset, requiring six anti-seizure medications. Brain MRI revealed T2/FLAIR hyperintensities, restricted diffusion, and cortical thickening of bilateral hippocampi. Serum showed HHV-6 viral load of 288,500 copies/mL and a negative autoimmune encephalopathy panel (Mayo clinical labs). Cerebrospinal fluid (CSF) revealed lymphocytic pleocytosis (7 cells/mm3), elevated protein (51 mg/dL), 2 red blood cells/mm3, normal glucose, negative autoimmune encephalopathy panel, and low HHV-6 viral load of 14,000 copies/mL. The CSF/serum replication ratio of <0.05 suggested against HHV-6 as the primary driver of encephalitis in our host with altered immunity. The patient met diagnostic criteria for definite autoimmune limbic encephalitis based on clinical onset, MRI, EEG, and low probability of HHV-6 encephalitis given the low CSF viral load (Graus et al., 2016). Brain MRI after 11 days showed marginal improvement. Acute treatments included IVIg, high-dose methylprednisolone, ganciclovir, and fluid restriction for SIADH. She was maintained on a prolonged prednisone taper with rituximab due to persistent cognitive dysfunction.

Conclusions In the context of DRESS, HHV-6 viral load requires cautious interpretation, and autoimmune encephalitis should be considered even with HHV-6 viremia or CSF viral load. We present a unique case of status epilepticus attributable to autoimmune encephalitis in DRESS.

Authors/Disclosures
Barrie L. Zerwic, MD (University of Colorado, Anschutz Medical Campus) PRESENTER	Dr. Schmitt has nothing to disclose.
Hunter Greer, MD (Anschutz Medical Campus)	Dr. Greer has nothing to disclose.
Aaron Geller, MD (University of Colorado)	Dr. Geller has nothing to disclose.
Lakshmi Chauhan, MD (University of Colorado)	The institution of Dr. Chauhan has received research support from NIH. The institution of an immediate family member of Dr. Chauhan has received research support from University of Colorado.
Brian M. Sauer, MD (University of Colorado)	The institution of Dr. Sauer has received research support from Annexon, Inc.. The institution of Dr. Sauer has received research support from Patient Centered Outcomes Research Center via Mayo Clinic. The institution of Dr. Sauer has received research support from Argenx. The institution of Dr. Sauer has received research support from NINDS & Amgen, Inc.. The institution of Dr. Sauer has received research support from Bristol Myers Squibb; Janssen Pharmaceutical Companies of Johnson & Johnson. The institution of Dr. Sauer has received research support from Hoffmann-La Roche. The institution of Dr. Sauer has received research support from UCB Biopharma SRL. Dr. Sauer has a non-compensated relationship as a Advisory Board Member, Neuroscience Advisor with Donor Alliance, Inc. that is relevant to AAN interests or activities.
Amanda L. Piquet, MD, FAAN (University of Colorado)	The institution of Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Genentech/Roche. The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alexion. The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Kyverna . The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech/Roche. The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kyverna. The institution of Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Sands Anderson PC. Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Joe Jones Law Firm. Dr. Piquet has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Cortez & Associates. Dr. Piquet has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Falk Waas. The institution of Dr. Piquet has received research support from Rocky Mountain MS Center. The institution of Dr. Piquet has received research support from Roche/Genentech. The institution of Dr. Piquet has received research support from NYU. The institution of Dr. Piquet has received research support from Anokion. The institution of Dr. Piquet has received research support from UCB . The institution of Dr. Piquet has received research support from Foundation for Sarcoidosis. The institution of Dr. Piquet has received research support from Kyverna . Dr. Piquet has received publishing royalties from a publication relating to health care. Dr. Piquet has received publishing royalties from a publication relating to health care. Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving as a Litigative Consultant with US-Dept HHS/DICP. Dr. Piquet has a non-compensated relationship as a Medical Advisory Board Member with Autoimmune Encephalitis Alliance (AEA) that is relevant to AAN interests or activities. Dr. Piquet has a non-compensated relationship as a Medical Advisory Board Member with Stiff Person Syndrome Research Foundation (SPSRF) that is relevant to AAN interests or activities.
Daniel Pastula, MD, MHS, FAAN (University of Colorado, Department of Neurology)	Dr. Pastula has nothing to disclose.
Kenneth L. Tyler, MD, FAAN (University of Colorado School of Medicine)	Dr. Tyler has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Protagonist Therapeutics, Newark CA. Dr. Tyler has received personal compensation in the range of $50,000-$99,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for American Neurological Association/Wiley. Dr. Tyler has received intellectual property interests from a discovery or technology relating to health care. Dr. Tyler has received publishing royalties from a publication relating to health care. Dr. Tyler has a non-compensated relationship as a Director (ex officio) with American Neurological association that is relevant to AAN interests or activities. Dr. Tyler has a non-compensated relationship as a Director with International Society for Neurovirology that is relevant to AAN interests or activities.

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