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

• DOI: 10.1016/j.ejpn.2024.07.002
• Publication Date: 2024-07-06T16:29:27Z
• Authors: 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. Whiteh...; Rachel Kneen; Cheryl Hemingway; Ming Lim; Yael Hacohen; Sukhvir Wright
• Keywords: Acquired demyelinating syndromes; long term outcomes; multiple sclerosis; AQP4-Ab NMOSD; MOGAD

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