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Autoantibody biomarkers in childhood-acquired demyelinating syndromes: results from a national surveillance cohort
  1. Yael Hacohen1,
  2. Michael Absoud2,3,
  3. Mark Woodhall1,
  4. Carole Cummins3,
  5. Christian G De Goede4,
  6. Cheryl Hemingway5,
  7. Philip E Jardine6,
  8. Rachel Kneen7,8,
  9. Michael G Pike9,
  10. William P Whitehouse10,
  11. Evangeline Wassmer11,
  12. Patrick Waters1,
  13. Angela Vincent1,
  14. Ming Lim1,2,
  15. On behalf of UK & Ireland Childhood CNS Inflammatory Demyelination Working Group
  1. 1Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK
  2. 2Children's Neuroscience, Evelina Children's Hospital at Guy's & St Thomas’ NHS Trust, King's Health Partners Academic Health Science Centre, London, UK
  3. 3School of Health & Population Sciences, University of Birmingham, Birmingham, UK
  4. 4Department of Paediatric Neurology, Royal Preston Hospital, Lancashire, UK
  5. 5Department of Paediatric Neurology, Great Ormond Street Hospital for Children, London, UK
  6. 6Department of Paediatric Neurology, Bristol Royal Hospital for Children, Bristol, UK
  7. 7Institute of Infection and Global Health, University of Liverpool, Liverpool, UK
  8. 8Department of Paediatric Neurology, Alder Hey Children's NHS Foundation Trus, Liverpool, UK
  9. 9Department of Paediatric Neurology, John Radcliffe Hospital, Oxford, UK
  10. 10School of Clinical Sciences, University of Nottingham, Nottingham, UK
  11. 11Department of Paediatric Neurology, Birmingham Children's Hospital, Birmingham, UK
  1. Correspondence to Prof Angela Vincent, Level 6, West Wing, Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, OX3 9DU, UK; angela.vincent@ndcn.ox.ac.uk or Dr Ming Lim, Children's Neurosciences, Evelina Children's Hospital @ Guy's and St Thomas’ NHS Foundation Trust, King's Health Partners Academic Health Sciences Centre, Lambeth Palace Road, London SE1 7EH, UK; ming.lim{at}gstt.nhs.uk

Abstract

Background Autoantibodies to glial, myelin and neuronal antigens have been reported in a range of central demyelination syndromes and autoimmune encephalopathies in children, but there has not been a systematic evaluation across the range of central nervous system (CNS) autoantibodies in childhood-acquired demyelinating syndromes (ADS).

Methods Children under the age of 16 years with first-episode ADS were identified from a national prospective surveillance study; serum from 65 patients had been sent for a variety of diagnostic tests. Antibodies to astrocyte, myelin and neuronal antigens were tested or retested in all samples.

Results Fifteen patients (23%) were positive for at least one antibody (Ab): AQ4-Ab was detected in three; two presenting with neuromyelitis optica (NMO) and one with isolated optic neuritis (ON). Myelin oligodendrocyte glycoprotein (MOG)-Ab was detected in seven; two with acute disseminated encephalomyelitis (ADEM), two with ON, one with transverse myelitis (TM) and two with clinically isolated syndrome (CIS). N-Methyl-D-Aspartate receptor (NMDAR)-Ab was found in two; one presenting with ADEM and one with ON. Voltage-gated potassium channel (VGKC)-complex antibodies were positive in three; one presenting with ADEM, one with ON and one with CIS. GlyR-Ab was detected in one patient with TM. All patients were negative for the VGKC-complex-associated proteins LGI1, CASPR2 and contactin-2.

Conclusions A range of CNS-directed autoantibodies were found in association with childhood ADS. Although these antibodies are clinically relevant when associated with the specific neurological syndromes that have been described, further studies are required to evaluate their roles and clinical relevance in demyelinating diseases.

  • Multiple Sclerosis
  • Neuroimmunology
  • Nmda
  • Paediatric
  • Myelin

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