Isolated Psychiatric Symptoms in Children With Anti-N-Methyl-d Aspartate Receptor Encephalitis

Grace Gombolay, Department of Pediatrics, Emory University SOM and Children's Healthcare of Atlanta, Atlanta, Georgia. Electronic address: ggombol@emory.edu.
J Nicholas Brenton, Division of Pediatric Neurology, Department of Neurology, University of Virginia Health System, Charlottesville, Virginia.
Jennifer H. Yang, Department of Pediatrics, University of California San Diego and Rady Children's Hospital San Diego, San Diego, California.
Coral M. Stredny, Department of Neurology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts.
Ryan Kammeyer, Department of Pediatrics, University of Colorado SOM and Children's Hospital Colorado, Denver, Colorado.
Kristen S. Fisher, Division of Pediatric Neurology and Developmental Neuroscience, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas.
Alexander J. Sandweiss, Division of Pediatric Neurology and Developmental Neuroscience, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas.
Timothy A. Erickson, Section of Pediatric Tropical Medicine, Department of Pediatrics, Center for Human Immunobiology, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas; Laboratories for Emerging and Tropical Diseases, School of Public Health, Texas A&M University, College Station, Texas.
Varun Kannan, Department of Pediatrics, Emory University SOM and Children's Healthcare of Atlanta, Atlanta, Georgia; Division of Pediatric Neurology and Developmental Neuroscience, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas.
Catherine Otten, Department of Pediatrics, Seattle Children's/University of Washington, Seattle, Washington.
Claude Steriade, Department of Neurology, New York University SOM, New York, New York.
NgocHanh Vu, Department of Neurology, Vanderbilt University Medical Center, Nashville, Tennessee.
Jonathan D. Santoro, Department of Neurology and Pediatrics, Children's Hospital Los Angeles; Keck School of Medicine, University of Southern California, Los Angeles, California.
Karla Robles-Lopez, Department of Pediatrics, University of Texas at Austin and Dell Medical School, Austin, Texas.
Robert Goodrich, Department of Neurology, Atrium Wake Forest Baptist Health, Wake Forest University School of Medicine, Winston-Salem, North Carolina.
Scott Otallah, Department of Neurology, Atrium Wake Forest Baptist Health, Wake Forest University School of Medicine, Winston-Salem, North Carolina.
Janetta Arellano, Pediatric Neurology, Children's Hospital of Orange County, Orange, California.
Andrew Christiana, Department of Neurology, New York University SOM, New York, New York.
Morgan Morris, Department of Pediatrics, Emory University SOM and Children's Healthcare of Atlanta, Atlanta, Georgia.
Mark P. Gorman, Department of Neurology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts.
Alexandra B. Kornbluh, Department of Pediatrics, Children's National Hospital, George Washington University Medical School, Washington, District of Columbia.
Ilana Kahn, Department of Pediatrics, Children's National Hospital, George Washington University Medical School, Washington, District of Columbia.
Leigh Sepeta, Department of Pediatrics, Children's National Hospital, George Washington University Medical School, Washington, District of Columbia.
Yike Jiang, Section of Rheumatology, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas.
Eyal Muscal, Division of Pediatric Neurology and Developmental Neuroscience, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas; Section of Rheumatology, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas.
Kristy O. Murray, Section of Pediatric Tropical Medicine, Department of Pediatrics, Center for Human Immunobiology, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas.
Manikum Moodley, Department of Pediatrics, University of Texas at Austin and Dell Medical School, Austin, Texas.
Duriel Hardy, Department of Pediatrics, University of Texas at Austin and Dell Medical School, Austin, Texas.

Document Type

Journal Article

Publication Date

7-17-2024

Journal

Pediatric neurology

Volume

159

DOI

10.1016/j.pediatrneurol.2024.07.009

Keywords

Anti-NMDA receptor encephalitis; Anti-NMDAR encephalitis; Anti-NMDARE; Autoimmune encephalitis; Encephalitis; Pediatric autoimmune encephalitis

Abstract

BACKGROUND: Isolated psychiatric symptoms can be the initial symptom of pediatric anti-N-methyl-d-aspartate (NMDA) receptor autoimmune encephalitis (pNMDARE). Here we report on the prevalence of isolated psychiatric symptoms in pNMDARE. We also assess whether initial neurodiagnostic tests (brain magnetic resonance imaging [MRI], electroencephalography [EEG], and/or cerebrospinal fluid [CSF] white blood cell count) are abnormal in children with isolated psychiatric symptoms and pNMDARE. METHODS: This multicenter retrospective cohort study from CONNECT (Conquering Neuroinflammation and Epilepsies Consortium) from 14 institutions included children under age 18 years who were diagnosed with pNMDARE. Descriptive statistics using means, medians, and comparisons for continuous versus discrete data was performed. RESULTS: Of 249 children included, 12 (5%) had only psychiatric symptoms without other typical clinical features of autoimmune encephalitis at presentation. All but one (11 of 12 = 92%) had at least one abnormal finding on initial ancillary testing: eight of 12 (67%) had an abnormal EEG, six of 12 (50%) had an abnormal MRI, and five of 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. CONCLUSIONS: Isolated first-episode psychiatric symptoms in pNMDARE are uncommon, and the majority of children will exhibit additional neurodiagnostic abnormalities. Delaying immunotherapy in a child with isolated psychiatric symptoms and normal neurodiagnostic testing may be warranted while awaiting confirmatory antibody testing.

Department

Neurology

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