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Abstract Details

Autoimmune Encephalitis Misdiagnosis in Adults; a multicenter observational study of outpatient subspecialty clinics
Autoimmune Neurology
C8 - Approach to Autoimmune Encephalitis/Epilepsy 2 (11:20 AM-11:25 AM)
P2 - Poster Session 2 (9:00 AM-3:00 PM)
041
Misdiagnosis of autoimmune encephalitis (AE) may harm patients.
To determine the diseases misdiagnosed as AE and potential reasons for misdiagnosis.  
Patients with AE misdiagnosis were identified (1/1/2014-12/31/2020) from outpatient AE subspecialty clinics including: Mayo Clinic (n=44); Oxford (n=18); UT Southwestern (n=18); UCSF (n=17); Washington University (n=6); University of Utah (n=4).  Inclusion criteria were adults (≥18 years) with: 1) A prior diagnosis of AE; and 2) An alternative diagnosis made at a participating center. We collected data on clinical features, investigations, fulfillment of possible AE criteria, alternative diagnoses, and potential contributors to misdiagnosis.
We identified 107 patients misdiagnosed with AE. Thirty (28%) fulfilled diagnostic criteria for “possible AE”. Median onset age was 48 years (inter-quartile range, 35.5-60.5) and 65 (61%) were female. Correct diagnoses included: functional neurologic disorder, 27 (25%); neurodegenerative disease, 22 (21%); primary psychiatric disease, 19 (18%); cognitive deficits from comorbidities, 11 (10%); cerebral neoplasm, 10 (9%); and other, 18 (17%). Onset was insidious (>3 months) in 51 (48%). MRI brain was suggestive of encephalitis in 19/104 (18%) and CSF pleocytosis occurred in 16/84 (19%). Thyroid-peroxidase antibodies were elevated in 24/62 (39%). Positive neural autoantibodies were more frequent in serum (48/105[46%]) than CSF (7/91[8%]; p<0.001) and serum antibodies included: GAD65, 14; voltage-gated-potassium-channel-complex [LGI1, CASPR2 negative], 10; NMDA-receptor by cell-based assay only, 10 (6 negative in CSF); and other, 18. Immunotherapy adverse effects were observed in 17/84 (20%). Potential contributors to misdiagnosis included: over-interpretation of a non-specific positive serum antibody, 53 (50%); misinterpretation of functional, psychiatric, or non-specific cognitive dysfunction as encephalopathy, 41 (38%). 
Red flags suggesting alternative diagnoses to AE include lack of fulfillment of “possible autoimmune encephalitis” criteria, positive non-specific serum antibody, and insidious onset. Avoiding AE misdiagnosis will prevent morbidity from unnecessary immunotherapies and delayed treatment of the correct diagnosis.
Authors/Disclosures
Alfonso S. Lopez, MD (Mayo Clinic)
PRESENTER 		Dr. Lopez has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon. Dr. Lopez has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech .
Michael D. Geschwind, MD, PhD, FAAN (UCSF) Dr. Geschwind has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Brainstorm Cell Therapeutics, Inc.. Dr. Geschwind has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Walter Grubb. Dr. Geschwind has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Gerson Lehrman Group. Dr. Geschwind has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Reata Pharmaceuticals, Inc..
Kyle M. Blackburn, MD (University of Texas Southwestern Medical Center) Dr. Blackburn has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics.
Sanchit Turaga, MBBS (John Radcliffe Hospital) Sanchit Turaga has nothing to disclose.
Sophie Binks, MD, MBBS, PhD The institution of Ms. Binks has received research support from Wellcome Trust. The institution of Ms. Binks has received research support from PetSavers. The institution of Ms. Binks has received research support from PetPlan. The institution of Ms. Binks has received research support from NIHR. Ms. Binks has received intellectual property interests from a discovery or technology relating to health care. Ms. Binks has received personal compensation in the range of $0-$499 for serving as a Speaker with ECTRIMS. Ms. Binks has received personal compensation in the range of $0-$499 for serving as a Speaker with Vetmeduni Wien. Ms. Binks has received personal compensation in the range of $0-$499 for serving as a Speaker with ANA. Ms. Binks has a non-compensated relationship as a Speaker with Encephalitis Society UK that is relevant to AAN interests or activities.
Jennifer Zitser-Koren, MD (UCSF) Dr. Zitser-Koren has nothing to disclose.
Jeffrey M. Gelfand, MD, MAS, FAAN (University of California, San Francisco) Dr. Gelfand has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Arialys. Dr. Gelfand has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Ventyx Bio. An immediate family member of Dr. Gelfand 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 Headache: The Journal of Head and Face Pain. The institution of Dr. Gelfand has received research support from Genentech/Roche. The institution of Dr. Gelfand has received research support from Vigil Neurosciences. An immediate family member of Dr. Gelfand has received publishing royalties from a publication relating to health care. Dr. Gelfand has received publishing royalties from a publication relating to health care. Dr. Gelfand has received publishing royalties from a publication relating to health care. Dr. Gelfand has a non-compensated relationship as a Trial Steering Committee Chairperson and member with Roche / Genentech that is relevant to AAN interests or activities.
Gregory S. Day, MD, MSc, FAAN (Mayo Clinic) Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Parabon Nanolabs. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys Therapeutics. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for DynaMed (EBSCO Health). Dr. Day has stock in ANI Pharmaceuticals. The institution of Dr. Day has received research support from National Institutes of Health / NIA. The institution of Dr. Day has received research support from Chan Zuckerberg Initiative. The institution of Dr. Day has received research support from Alzheimer's Association. The institution of Dr. Day has received research support from National Institutes of Health / NINDS. The institution of Dr. Day has received research support from Amgen Pharmaceuticals. The institution of Dr. Day has received research support from AVID Radiopharmaceuticals. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Presenter at Annual Meeting (CME) with 好色先生. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Content Development (CME) with PeerView, Inc. Dr. Day has received personal compensation in the range of $5,000-$9,999 for serving as a Content Development (CME) with Continuing 好色先生, Inc. Dr. Day has received personal compensation in the range of $5,000-$9,999 for serving as a Content Development (CME) with Ionis Pharmaceuticals. Dr. Day has a non-compensated relationship as a Clinical Director with Anti-NMDA Receptor Encephalitis Foundation that is relevant to AAN interests or activities.
Steven R. Dunham, Jr., MD (Washington University School of Medicine) Dr. Dunham has nothing to disclose.
Stefanie J. Rodenbeck, MD (Indiana University) Dr. Rodenbeck 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 Pharmaceuticals. Dr. Rodenbeck has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics. Dr. Rodenbeck has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen.
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.
Andrew Solomon, MD, FAAN Dr. Solomon has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics. Dr. Solomon 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 . Dr. Solomon has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kiniksa Pharmaceuticals . Dr. Solomon has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Bristol Myers Squibb. Dr. Solomon has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi. The institution of Dr. Solomon has received research support from Bristol Meyers Squibb.
Sean J. Pittock, MD, FAAN (Mayo Clinic Dept of Neurology) Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys Therapeutics. The institution of Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Arialys. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. The institution of Dr. Pittock 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/Genentech. The institution of Dr. Pittock has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion/AstraZeneka. The institution of Dr. Pittock has received research support from NIH. The institution of Dr. Pittock has received research support from Alexion/AstraZeneka. The institution of Dr. Pittock has received research support from F. Hoffman/LaRoche/Genentech. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received publishing royalties from a publication relating to health care.
Andrew McKeon, MD (Mayo Clinic) The institution of Dr. McKeon has received research support from National Institutes of Health. Dr. McKeon has received intellectual property interests from a discovery or technology relating to health care. Dr. McKeon has received intellectual property interests from a discovery or technology relating to health care. Dr. McKeon has received publishing royalties from a publication relating to health care.
Divyanshu Dubey, MD, FAAN (Mayo Clinic) The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Argenx. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from UCB. Dr. Dubey has received research support from David J. Tomassoni ALS Research Grant Program . Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care.
Anastasia Zekeridou, MD, PhD, FAAN (Neuroimmunology Laboratory, Mayo Clinic) The institution of Dr. Zekeridou has received research support from Roche/Genentech. Dr. Zekeridou has received intellectual property interests from a discovery or technology relating to health care. Dr. Zekeridou has received intellectual property interests from a discovery or technology relating to health care. Dr. Zekeridou has received intellectual property interests from a discovery or technology relating to health care. Dr. Zekeridou has received intellectual property interests from a discovery or technology relating to health care.
Michel Toledano, MD (Mayo Clinic) Dr. Toledano has nothing to disclose.
No disclosure on file
Steven Vernino, MD, PhD, FAAN (UT Southwestern Medical Center) Dr. Vernino has received personal compensation in the range of $500-$4,999 for serving as a Consultant for antag. Dr. Vernino has received personal compensation in the range of $500-$4,999 for serving as a Consultant for CSL Behring. Dr. Vernino has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for argenx. Dr. Vernino 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. Vernino has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Autonomic Neuroscience (Elsevier). The institution of Dr. Vernino has received research support from Takeda. The institution of Dr. Vernino has received research support from NIH/NHLBI. The institution of Dr. Vernino has received research support from Dysautonomia International. Dr. Vernino has received personal compensation in the range of $10,000-$49,999 for serving as a Content Expert Consultant with Office of Inspector General for Medicare. Dr. Vernino has a non-compensated relationship as a Board of Directors with American Autonomic Society that is relevant to AAN interests or activities.
Sarosh R. Irani, MD, PhD, FRCP, FEAN (Mayo Clinic) Dr. Irani has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for UCB, Immunovant, MedImmun, Roche, Janssen, Cerebral therapeutics, Biogen, Amgen, Argenex, Clarivate, IQVIA, BioHaven therapeutics, ADC therapeutics, Brain, CSL Behring, and ONO Pharma.. Dr. Irani has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. Dr. Irani has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Irani has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Brain. Dr. Irani has received personal compensation in the range of $100,000-$499,999 for serving as an Expert Witness for LLPs. Dr. Irani has received intellectual property interests from a discovery or technology relating to health care. Dr. Irani has received intellectual property interests from a discovery or technology relating to health care. Dr. Irani has received intellectual property interests from a discovery or technology relating to health care.
Eoin P. Flanagan, MBBCh, FAAN (Mayo Clinic) The institution of Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics. The institution of Dr. Flanagan has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Pharmacy times. The institution of Dr. Flanagan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for UCB. The institution of Dr. Flanagan has received research support from Viela Bio. The institution of Dr. Flanagan has received research support from UCB. The institution of Dr. Flanagan has received research support from Roche. Dr. Flanagan has received publishing royalties from a publication relating to health care. Dr. Flanagan has a non-compensated relationship as a Member of medical Advisory Board with The MOG Project that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Journal of The Neurologic Sciences that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Neuroimmunology Reports that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial Board Member with Neurology, Neuroimmunology Neuroinflammation (N2) Journal that is relevant to AAN interests or activities.