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High-throughput Flow Cytometry Cell-based Assay to Detect Antibodies to N-Methyl-D-aspartate Receptor or Dopamine-2 Receptor in Human Serum
Published on: November 23, 2013
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.
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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