TY - JOUR
T1 - Long term outcome in non-multiple sclerosis paediatric acquired demyelinating syndromes
AU - Wassmer, Evangeline
AU - Billaud, Charly
AU - Absoud, Michael
AU - Abdel-Mannan, Omar
AU - Benetou, Christina
AU - Cummins, Carole
AU - Forrest, Katharine
AU - De Goede, Christian
AU - Eltantawi, Noha
AU - Hickson, Helga
AU - Hussain, Nahin
AU - Jardine, Phil
AU - Livingston, John H
AU - Mordekar, Santosh
AU - Ramdas, Sithara
AU - Taylor, Micheal
AU - Vijayakumar, K
AU - West, Siobhan
AU - Whitehouse, William P
AU - Kneen, Rachel
AU - Hemingway, Cheryl
AU - Lim, Ming
AU - Hacohen, Yael
AU - Wright, Sukhvir
N1 - Copyright © 2024. Published by Elsevier Ltd on behalf of European Paediatric Neurology Society. This work is licensed under a Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by/4.0/).
PY - 2024/9
Y1 - 2024/9
N2 - 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.
AB - 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.
KW - AQP4-Ab NMOSD
KW - Acquired demyelinating syndromes
KW - Long term outcomes
KW - MOGAD
KW - Multiple sclerosis
UR - https://linkinghub.elsevier.com/retrieve/pii/S1090379824000990
UR - http://www.scopus.com/inward/record.url?scp=85198569708&partnerID=8YFLogxK
U2 - 10.1016/j.ejpn.2024.07.002
DO - 10.1016/j.ejpn.2024.07.002
M3 - Article
C2 - 39025036
SN - 1090-3798
VL - 52
SP - 52
EP - 58
JO - European Journal of Paediatric Neurology
JF - European Journal of Paediatric Neurology
ER -