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Abstract Details

Initial MRI, EEG, and CSF White Cell Count Are Abnormal in Children with Anti-NMDA Receptor Encephalitis with Isolated Psychiatric Symptoms
Autoimmune Neurology
P6 - Poster Session 6 (8:00 AM-9:00 AM)
14-020

To assess whether initial ancillary tests (brain magnetic resonance imaging-MRI, electroencephalogram-EEG, and/or cerebrospinal fluid-CSF white blood cell count) are abnormal in children with isolated psychiatric symptoms and anti-NMDA receptor encephalitis.

Isolated psychiatric symptoms can be the initial symptom of pediatric anti-NMDA receptor autoimmune encephalitis (pNMDARE). However, the utility of initiating empiric immunotherapy prior to autoantibody test results is poorly understood. Identification of appropriate patients reduces risk of administering unnecessary treatment. Here we assess the characteristics of isolated new onset psychiatric symptoms in pNMDARE. 

This multi-center retrospective cohort study from CONNECT (CONquering Neuroinflammation and Epilepsies ConsorTium) from 14 institutions included children under 18 years old who were diagnosed with pNMDARE from January 1, 2008 – September 1, 2022. Psychiatric symptoms were defined as the presence of catatonia, agitation, hallucinations, confusion, behavioral changes, anxiety, and suicidal ideation. Patients who had non-psychiatric symptoms (seizures, movement disorders, autonomic instability, speech changes, and hypoventilation) were excluded. Descriptive statistics using means and medians and comparisons for continuous versus discrete data were performed.

Out of 249 children included, twelve (5%) had only psychiatric symptoms without other typical clinical features of autoimmune encephalitis at presentation. All but one (11/12=92%) had at least one abnormal finding on initial ancillary testing: 8/12 (67%) had an abnormal EEG, 6/12 (50%) had an abnormal MRI, and 5/12 (42%) demonstrated CSF pleocytosis. The single patient with a normal MRI, EEG and CSF profile had low positive CSF NMDA antibody (titer of 1:1) and symptoms improved without immunotherapy.

Isolated first episode psychiatric symptoms in pNMDARE is uncommon, and the majority of children will exhibit additional abnormalities on diagnostic testing (EEG, MRI, and/or CSF).  Thus, it is reasonable to await diagnostic confirmation with antibody testing prior to starting immunotherapy in a patient with isolated psychiatric symptoms, especially without abnormal ancillary testing.
Authors/Disclosures
Grace Gombolay, MD, FAAN (Emory University/Children'S Healthcare of Atlanta)
PRESENTER
The institution of Dr. Gombolay has received research support from CDC. The institution of Dr. Gombolay has received research support from NIH.
James N. Brenton, MD, FAAN Dr. Brenton has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for I-ACT on a Novartis sponsored project. The institution of Dr. Brenton has received research support from NIH/NINDS. The institution of Dr. Brenton has received research support from Autoimmune Encephalitis Alliance. Dr. Brenton has received publishing royalties from a publication relating to health care. Dr. Brenton has received personal compensation in the range of $500-$4,999 for serving as a Grant Reviewer with Department of Defense. Dr. Brenton has received personal compensation in the range of $0-$499 for serving as a Grant Reviewer with NIH. Dr. Brenton has received personal compensation in the range of $0-$499 for serving as a Grant Reviewer with FDA.
Jonathan Santoro, MD (Department of Neurology, Children's Hospital Los Angeles) Dr. Santoro has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for UCB. Dr. Santoro has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Cycle Pharma. Dr. Santoro has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Dianthus. Dr. Santoro has received personal compensation in the range of $500-$4,999 for serving as a Consultant for National Down Syndrome Society.
Coral M. Stredny, MD (Children's Hospital Boston) Dr. Stredny has stock in Proctor and Gamble. The institution of Dr. Stredny has received research support from Pediatric Epilepsy Research Foundation.
Ryan Kammeyer, MD (Childrens Hospital Colorado) The institution of Dr. Kammeyer has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Amgen. The institution of Dr. Kammeyer has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Novartis. Dr. Kammeyer has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Ogborn-Mihm Trial Lawyers. The institution of Dr. Kammeyer has received research support from Rocky Mountain Multiple Sclerosis Center.
Kristen Fisher, DO (Baylor College of Medicine) Dr. Fisher has nothing to disclose.
Alexander Sandweiss, MD, PhD Dr. Sandweiss has nothing to disclose.
Tim Erickson No disclosure on file
Varun Kannan, MD (Emory/CHOA) Dr. Kannan has nothing to disclose.
Catherine E. Otten, MD The institution of Dr. Otten has received research support from CDC.
NgocHanh H. Vu, MD (Vanderbilt University, Child Neurology) Dr. Vu has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Syneos Health.
Jennifer H. Yang, MD (Rady Childrens Hospital/UCSD) Dr. Yang has received research support from Pediatric Epilepsy Research Foundation. Dr. Yang has received research support from NIH.
Karla Robles Lopez (UNIVERSITY OF TEXAS AT AUSTIN/DMS) Karla Robles Lopez has nothing to disclose.
Robert C. Goodrich III, MD Dr. Goodrich has nothing to disclose.
Scott I. Otallah, MD (Wake Forest) Dr. Otallah has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Merck.
Janetta L. Arellano, MD (CHOC) Dr. Arellano has nothing to disclose.
Andrew Christiana, MD (NYU) Dr. Christiana has nothing to disclose.
Claude Steriade, MD (NYU) The institution of Dr. Steriade has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for The Epilepsy Study Consortium. Dr. Steriade 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. Dr. Steriade has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for DOJ. The institution of Dr. Steriade has received research support from NIH. Dr. Steriade has received personal compensation in the range of $500-$4,999 for serving as a Consultant with Epitel. Dr. Steriade has received personal compensation in the range of $500-$4,999 for serving as a Consultant with Jazz Pharmaceuticals. Dr. Steriade has received personal compensation in the range of $10,000-$49,999 for serving as a Speakers Bureau with SK Life Sciences. Dr. Steriade has received personal compensation in the range of $5,000-$9,999 for serving as a Speakers Bureau with Neurelis. Dr. Steriade has received personal compensation in the range of $5,000-$9,999 for serving as a Advisory Board with Xenon Pharmaceuticals.
Morgan Morris (Emory University) Morgan Morris has nothing to disclose.
Alexandra B. Kornbluh, MD (Children's National Hospital) Dr. Kornbluh has nothing to disclose.
Ilana L. Kahn, MD (Childrens National Medical Center) Dr. Kahn has nothing to disclose.
Leigh N. Sepeta, PhD (Children'S National Health System) Dr. Sepeta has received personal compensation in the range of $500,000-$999,999 for serving as a Grant PI with Nih. Dr. Sepeta has a non-compensated relationship as a Special volunteer with Nih that is relevant to AAN interests or activities.
Yike Jiang Yike Jiang has nothing to disclose.
Eyal Muscal Eyal Muscal has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for sobi. An immediate family member of Eyal Muscal has stock in pfizer.
Kristy Murray (Baylor College of Medicine) No disclosure on file
Manikum Moodley, MD, FAAN Dr. Moodley has nothing to disclose.
Duriel I. Hardy, MD (Dell Children's Specialty Pavillian) Dr. Hardy has nothing to disclose.
Mark Gorman, MD The institution of Dr. Gorman has received research support from Pfizer. The institution of Dr. Gorman has received research support from Roche / Genetech .