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 , Sukhvir Wright
{"title":"非多发性硬化症儿科获得性脱髓鞘综合征的长期治疗效果","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. Whitehouse , Rachel Kneen , Sukhvir Wright","doi":"10.1016/j.ejpn.2024.07.002","DOIUrl":null,"url":null,"abstract":"<div><h3>Objectives</h3><p>We aimed to study the risks of relapse and long term disability in children with non-MS acquired demyelinating syndromes (ADS).</p></div><div><h3>Methods</h3><p>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.</p></div><div><h3>Results</h3><p>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.</p></div><div><h3>Conclusion</h3><p>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.</p></div>","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"52 ","pages":"Pages 52-58"},"PeriodicalIF":2.3000,"publicationDate":"2024-07-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Long term outcome in non-multiple sclerosis paediatric acquired demyelinating syndromes\",\"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. Whitehouse , Rachel Kneen , Sukhvir Wright\",\"doi\":\"10.1016/j.ejpn.2024.07.002\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><h3>Objectives</h3><p>We aimed to study the risks of relapse and long term disability in children with non-MS acquired demyelinating syndromes (ADS).</p></div><div><h3>Methods</h3><p>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.</p></div><div><h3>Results</h3><p>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.</p></div><div><h3>Conclusion</h3><p>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.</p></div>\",\"PeriodicalId\":50481,\"journal\":{\"name\":\"European Journal of Paediatric Neurology\",\"volume\":\"52 \",\"pages\":\"Pages 52-58\"},\"PeriodicalIF\":2.3000,\"publicationDate\":\"2024-07-06\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"European Journal of Paediatric Neurology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S1090379824000990\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"European Journal of Paediatric Neurology","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S1090379824000990","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
Long term outcome in non-multiple sclerosis paediatric acquired demyelinating syndromes
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.
期刊介绍：
The European Journal of Paediatric Neurology is the Official Journal of the European Paediatric Neurology Society, successor to the long-established European Federation of Child Neurology Societies.
Under the guidance of a prestigious International editorial board, this multi-disciplinary journal publishes exciting clinical and experimental research in this rapidly expanding field. High quality papers written by leading experts encompass all the major diseases including epilepsy, movement disorders, neuromuscular disorders, neurodegenerative disorders and intellectual disability.
Other exciting highlights include articles on brain imaging and neonatal neurology, and the publication of regularly updated tables relating to the main groups of disorders.
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