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Very Long-Term Functional Outcomes and Dependency in Children With Anti-NMDA Receptor Encephalitis
Li-Wen Chen1, Gemma Olivé-Cirera1, Elianet G Fonseca1
1From the Group of Experimental Neuroimmunology (L.-W.C., G.O.-C., E.G.F., T.A., J.D.), Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) and Caixa Research Institute, Barcelona, Spain; Department of Pediatrics (L.-W.C.), National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan; Pediatric Neurology Unit (G.O.-C.), Hospital Parc Taulí de Sabadell; Neurology Service (E.G.F., J.D.), Hospital Clínic Barcelona; Pediatric Neuroimmunology Unit (E.G.F., T.A.), Neurology Department, Sant Joan de Déu Children's Hospital, Barcelona, Spain; Division of Neurology (M.M.S.), Hospital das Clinicas (HCFMUSP), Faculdade de Medicina, University of São Paulo, Brazil; Department of Neurology (T.I.), Kitasato University School of Medicine, Sagamihara, Japan; Centro de Investigación Biomédica en Red (J.D.), Enfermedades Raras (CIBERER), Spain; Department of Neurology (J.D.), Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA; and University of Barcelona (J.D.), Barcelona, Spain.
Insights
Most children with anti-NMDA receptor encephalitis (NMDARe) recover well, but some face lasting behavioral and cognitive issues. Early disease onset in children increases the risk of severe, long-term deficits and dependency.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Autoimmune Encephalitis
Background:
- Anti-NMDA receptor encephalitis (NMDARe) is a severe autoimmune disorder affecting the central nervous system.
- Long-term functional outcomes in pediatric NMDARe survivors are not fully understood.
Purpose of the Study:
- To evaluate the long-term daily functioning of children diagnosed with NMDARe.
- To identify factors influencing functional recovery in pediatric NMDARe patients.
Main Methods:
- Retrospective study of pediatric patients (onset ≤18 years) with NMDARe.
- Assessment of daily function using the 15-domain Liverpool Outcome Score by physicians.
- Minimum follow-up duration of 5 years.
Main Results:
- Of 76 patients, 68 were followed for a mean of 7.1 years; 11% mortality.
- Full recovery observed in 73% of survivors.
- Behavioral/school deficits in 18%, multidomain deficits in 9%.
Conclusions:
- Most pediatric NMDARe patients achieve substantial functional recovery within 5 years.
- A significant minority experience persistent deficits, particularly behavioral and cognitive.
- Younger age at onset is a predictor of poorer outcomes and increased sociofamiliar dependence.
Objectives:
To assess the daily function of children with anti-N-methyl-d-aspartate receptor encephalitis (NMDARe) after a minimal follow-up of 5 years.
Methods:
Patients 18 years and younger by the time of disease onset, whose serum and CSF were studied in our center between 2013 and 2017, were included in the study. Patients' daily life function was assessed by their physicians using a 15-domain question format (Liverpool Outcome Score).
Results:
Of 76 patients, 8 (11%) died and 68 were followed for a mean of 7.1 years (SD 1.5 years, range: 5.0-10.1). Three outcome patterns were identified: full recovery (50; 73%); behavioral and school/working deficits (12; 18%); and multidomain deficits (6; 9%) involving self-care ability, behavioral-cognitive impairment, and seizures. Younger age of disease onset was significantly associated with multidomain deficits (OR 1.6, 95% CI 1.02-2.4, p = 0.04), particularly in children younger than 6 years, among whom 8 of 23 (35%) remained sociofamiliar dependent.
Discussion:
After a minimal follow-up of 5 years, most children with NMDARe had substantial or full functional recovery, but approximately one-fifth remained with behavioral and school/working deficits. The younger the patient at disease onset, the more probable it was to remain with multidomain deficits and dependent on sociofamiliar support.
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