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Published on: January 9, 2018
Antibody Investigations in 2,750 Children With Suspected Autoimmune Encephalitis
Li-Wen Chen1, Mar Guasp1, Gemma Olivé-Cirera1
1From the Neuroimmunology Program (L.-W.C., M.G., G.O.-C., E.M.-H., R.R.G., A.S., T.A., J.D.), Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Spain; Department of Pediatrics (L.-W.C.), National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan; Neurology Department (M.G., E.M.-H., A.S., J.D.), University of Barcelona; Centro de Investigación Biomédica en Red (M.G., J.D.), Enfermedades Raras (CIBERER) Madrid, Spain; Pediatric Neurology Unit (G.O.-C.), Hospital Parc Taulí de Sabadell; Immunology Department (R.R.G., L.N.), Centre de Diagnòstic Biomèdic, Hospital Clínic, Barcelona; Pediatric Neuroimmunology Unit (T.A.), Neurology Department, Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; Department of Neurology (J.D.), Perelman School of Medicine, University of Pennsylvania, Philadelphia; and Catalan Institution for Research and Advanced Studies (ICREA) (J.D.), Barcelona, Spain.
Objectives:
To assess the frequency and types of neuronal and glial (neural) antibodies in children with suspected autoimmune encephalitis (AE).
Methods:
Patients younger than 18 years with suspected AE other than acute disseminated encephalomyelitis, whose serum or CSF samples were examined in our center between January 1, 2011, and April 30, 2022, were included in this study. Samples were systematically examined using brain immunohistochemistry; positive immunostaining was further investigated with cell-based assays (CBA), immunoblot, or live neuronal immunofluorescence.
Results:
Of 2,750 children, serum or CSF samples of 542 (20%) showed brain immunoreactivity, mostly (>90%) against neural cell surface antigens, and 19 had antibodies only identified by CBA. The most frequent targets were N-methyl-d-aspartate receptor (NMDAR, 76%) and myelin oligodendrocyte glycoprotein (MOG, 5%), followed by glutamic acid decarboxylase 65 (2%) and γ-aminobutyric acid A receptor (2%). Antibodies against other known cell surface or intracellular neural antigens (altogether 6% of positive cases) and unknown antigens (9%) were very infrequent.
Discussion:
The repertoire of antibodies in children with AE is different from that of the adults. Except for NMDAR and MOG antibodies, many of the antibodies included in diagnostic panels are rarely positive and their up-front testing in children seems unneeded.
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