Melissa Wright1, Suzanne Liu1, Ka-Ho Wong1, Sama Noroozi Gilandehi1, Sarah Shapiro2, Tammy Smith3, Lisa Peterson4, Stacey Clardy1
1University of Utah, 2Kansas City University College of Osteopathic Medicine, 3George E. Wahlen Department of Veterans Affairs Medical Center, 4ARUP Laboratories
Objective:
To describe the progression of symptoms, diagnostic testing, and encounters with the health care system that pediatric patients experience prior to receiving a confirmed diagnosis of anti-NMDAR encephalitis.
Background:
Prompt treatment of anti-NMDAR encephalitis in pediatric patients is associated with improved outcomes, but diagnosis remains challenging. Heterogeneity in presentations, variability based on age, and broad differential diagnoses of associated symptoms in the pediatric population can complicate timely and accurate identification of the disease.
Design/Methods:
A retrospective chart review for patients within Primary Children’s Hospital (PCH) was conducted. ICD10 code G04.81 at PCH between January 2007 to September 2022 was queried to identify pediatric patients with “other encephalitis”. Of these 147 pediatric patients, 18 had confirmed anti-NMDAR encephalitis based on clinical features and positive NMDAR Ig antibody testing in the cerebrospinal fluid
Results:
We identify a cohort of pediatric patients with confirmed anti-NMDAR encephalitis, and discuss duration and progression of symptoms, diagnostic testing, interventions, number of encounters within the health care system, and involved subspecialties prior to a final diagnosis being made. We also review characteristics associated with prolonged times to diagnosis.
Conclusions:
Pediatric patients with anti-NMDAR encephalitis often have an evolution of symptoms, extensive diagnostic evaluations, and multiple encounters with the healthcare system prior to confirmation of diagnosis. Factors associated with prolonged time until diagnosis may aid in identifying those at risk for diagnostic delays. Additionally, recognition of the path to diagnosis these children often take may help physicians better understand the challenges and stressors patients and families face, not only following a diagnosis of anti-NMDAR encephalitis, but also leading up to it.