Long term outcome in non-multiple sclerosis paediatric acquired demyelinating syndromes

Evangeline Wassmer; Charly Billaud; Michael Absoud; Omar Abdel-Mannan; Christina Benetou; Carole Cummins; Katharine Forrest; Christian De Goede; Noha Eltantawi; Helga Hickson; Nahin Hussain; Phil Jardine; John H Livingston; Santosh Mordekar; Sithara Ramdas; Micheal Taylor; K Vijayakumar; Siobhan West; William P Whitehouse; Rachel Kneen; Cheryl Hemingway; Ming Lim; Yael Hacohen; Sukhvir Wright; PUDDLS STUDY GROUP

doi:10.1016/j.ejpn.2024.07.002

Long term outcome in non-multiple sclerosis paediatric acquired demyelinating syndromes

Eur J Paediatr Neurol. 2024 Sep:52:52-58. doi: 10.1016/j.ejpn.2024.07.002. Epub 2024 Jul 6.

Authors

Evangeline Wassmer¹, Charly Billaud², Michael Absoud³, Omar Abdel-Mannan⁴, Christina Benetou³, Carole Cummins⁵, Katharine Forrest⁶, Christian De Goede⁷, Noha Eltantawi⁸, Helga Hickson⁹, Nahin Hussain¹⁰, Phil Jardine¹¹, John H Livingston¹², Santosh Mordekar¹³, Sithara Ramdas¹⁴, Micheal Taylor¹², K Vijayakumar¹⁵, Siobhan West¹⁶, William P Whitehouse¹⁷, Rachel Kneen¹⁸, Cheryl Hemingway¹⁹, Ming Lim²⁰, Yael Hacohen⁴, Sukhvir Wright²¹; PUDDLS STUDY GROUP

Affiliations

¹ Birmingham Children's Hospital, Birmingham, UK; Institute of Health and Neurodevelopment, Aston University, Birmingham, UK. Electronic address: [email protected].
² Institute of Health and Neurodevelopment, Aston University, Birmingham, UK.
³ Evelina London Children's Hospital, London, UK.
⁴ Great Ormond Street Hospital, London, UK; Department of Neuroinflammation, Queen Square MS Centre, UCL Institute of Neurology, London, UK.
⁵ Birmingham University, Birmingham, UK.
⁶ University Hospital Southampton, Southampton, UK; Royal Hospital for Children, Glasgow, UK.
⁷ Royal Preston Hospital, Preston, UK.
⁸ Birmingham Children's Hospital, Birmingham, UK; Manoura University, Egypt.
⁹ Birmingham Children's Hospital, Birmingham, UK; Maidston and Tunbridge Wells, UK.
¹⁰ Leicester Royal Infirmary, Leicester, UK.
¹¹ University Hospital Bristol NHS Foundation Trust, Bristol, UK.
¹² Leeds Teaching Hospitals, Leeds, UK.
¹³ Sheffield Children's Hospital, Sheffield, UK.
¹⁴ John Radcliffe Hospital, Oxford, UK.
¹⁵ University Hospital Bristol NHS Foundation Trust, Bristol, UK; Chelsea and Westminster Hospital NHS Foundation Trust, London, UK.
¹⁶ Manchester Children's Hospital, Manchester, UK.
¹⁷ Nottingham University Hospitals, Nottingham, UK; University of Nottingham, Nottingham, UK.
¹⁸ Alder Hey Children's Hospital, Liverpool, UK.
¹⁹ Great Ormond Street Hospital, London, UK.
²⁰ Evelina London Children's Hospital, London, UK; Faculty of Life Sciences and Medicine, King's College, London, UK.
²¹ Birmingham Children's Hospital, Birmingham, UK; Institute of Health and Neurodevelopment, Aston University, Birmingham, UK.

PMID: 39025036
DOI: 10.1016/j.ejpn.2024.07.002

Abstract

Objectives: We aimed to study the risks of relapse and long term disability in children with non-MS acquired demyelinating syndromes (ADS).

Methods: In this prospective, multi-centre study, from the 14 UK pediatric neurology centres, children (<16 years) experiencing a first episode of ADS were recruited from 2010 to 2014. Case report forms were collected prospectively.

Results: A total of 269 children were recruited and followed up for a median of 7.2 years. Median age at onset was 9y (IQR 9.5-14.5, 126 females). At last follow-up, 46 (18 %) had MS, 4 AQP4-Ab NMOSD and 206 (80 %) had other ADS, of which 27 (13 %) relapsed. Relapsing MOGAD was the diagnosis in 12/27, 6 were seronegative and 9 did not have antibodies tested. Frequency of relapse differed according to first presentation in non-MS ADS, being least likely in transverse myelitis (p = 0.025). In the non-MS group, MOG-Ab was predictive of relapse (HR = 8.42; p < 0.001) occurring 8 times as often decreasing over time. Long-term difficulties did not differ between children with monophasic vs relapsing diseases.

Conclusion: The risk of relapse in non-MS ADS depends on initial diagnosis, and MOG-Ab positivity. Long-term difficulties are observed regardless of relapses and are determined by presenting phenotype.

Keywords: AQP4-Ab NMOSD; Acquired demyelinating syndromes; Long term outcomes; MOGAD; Multiple sclerosis.

Publication types

Multicenter Study

MeSH terms

Adolescent
Autoantibodies / blood
Child
Child, Preschool
Demyelinating Diseases / diagnosis
Female
Follow-Up Studies
Humans
Male
Myelin-Oligodendrocyte Glycoprotein / immunology
Prospective Studies
Recurrence*

Substances

Myelin-Oligodendrocyte Glycoprotein
Autoantibodies