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Neuropediatrics 2021; 52(04): 337-340
DOI: 10.1055/s-0041-1726289
Short Communication

Progressive Leukodystrophy-Like Demyelinating Syndromes with MOG-Antibodies in Children: A Rare Under-Recognized Phenotype

Elise Yazbeck
1   Pediatric Neurology Department, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Bicêtre Hospital, Le Kremlin Bicêtre, France
,
Hélène Maurey
1   Pediatric Neurology Department, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Bicêtre Hospital, Le Kremlin Bicêtre, France
2   National Referral Center for Rare Inflammatory and Auto-Immune Brain and Spinal Diseases, Le Kremlin-Bicêtre, France
,
Carole Leroy
2   National Referral Center for Rare Inflammatory and Auto-Immune Brain and Spinal Diseases, Le Kremlin-Bicêtre, France
3   Inserm UMR 1184, Immunology of Viral Infections and Autoimmune Diseases, CEA, IDMIT, Le Kremlin Bicêtre, France
,
Philippe Horellou
3   Inserm UMR 1184, Immunology of Viral Infections and Autoimmune Diseases, CEA, IDMIT, Le Kremlin Bicêtre, France
,
Silvia Napuri
4   Pediatric Department, Centre Hospitalo-Universitaire de Rennes - Hôpital Sud, Rennes, France
,
Mohammed Lali
5   Pediatric Department, Centre hospitalier Bretagne Atlantique, Vannes, France
,
Clovis Adam
6   Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris-Saclay, Neuropathology Laboratory, Le Kremlin-Bicêtre, France
,
Beatrice Husson
7   Pediatric Radiology Department, Assistance Publique-Hôpitaux de Paris, Le Kremlin-Bicêtre, France
,
Caroline Sevin
1   Pediatric Neurology Department, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Bicêtre Hospital, Le Kremlin Bicêtre, France
8   National Referral Center for Leucodystrophy, Le Kremlin-Bicêtre, France
,
Kumaran Deiva
1   Pediatric Neurology Department, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Bicêtre Hospital, Le Kremlin Bicêtre, France
2   National Referral Center for Rare Inflammatory and Auto-Immune Brain and Spinal Diseases, Le Kremlin-Bicêtre, France
3   Inserm UMR 1184, Immunology of Viral Infections and Autoimmune Diseases, CEA, IDMIT, Le Kremlin Bicêtre, France
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Abstract

Acquired demyelinating syndromes (ADS) are frequently associated with myelin oligodendrocytes glycoprotein (MOG) antibodies in children. Clinical phenotypes are heterogeneous and may delay the diagnosis, especially when they relapse and are atypical, mimicking diseases such as multiple sclerosis or neuromyelitis optica spectrum disorders . Here, we describe two children: one with a progressive cognitive and behavioral deterioration with seizures after only one relapse and the other with similar clinical impairments associated with multiple relapses. Brain magnetic resonance imaging revealed a subsequent progressive leukodystrophy-like lesion with diffuse bilateral white matter injuries in both patients. Cerebrospinal fluid analysis showed pleiocytosis, increased level of proteins with no oligoclonal bands. Metabolic and inflammatory blood markers were all negative. Brain biopsy was performed in the second child and nonspecific inflammatory lesions with no argument for histiocytosis or tumor were observed. Clinical and radiological stabilization were obtained after active immunotherapy. Retrospective analysis of anti-MOG antibodies in these two children was positive at the earlier stage of the disease and turned negative after treatment and during follow-up. Leukodystrophy-like ADS with anti-MOG-antibodies may display distinct progressive phenotype and have a severe neurological prognosis. Early diagnosis and appropriate treatment may improve outcome in these children.

Keywords

acquired demyelinating syndromes (ADS) - leukodystrophy-like - MOG antibodies - multiple sclerosis - immunotherapy

Ethics and Consent

Both patients are part of the KidbioSEP cohort and parents have given written consent to participate.


Authors Contributions

All authors contributed equally and agreed to the published version of the manuscript.




Publication History

Received: 02 December 2020

Accepted: 29 January 2021

Article published online:
31 March 2021

© 2021. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

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