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MOG antibody-related disorders: common features and uncommon presentations

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Abstract

Myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) have been reported in acute demyelinating encephalomyelitis (ADEM), optic neuritis (ON), and neuromyelitis optica spectrum disorders (NMOSD) in adults and pediatrics. We aimed to delineate the common features of MOG-Ab-related disorders in children and adults, and report uncommon presentations. Twenty-seven consecutive pediatric and adult patients testing positive for MOG-Ab, with a minimum follow-up of 6 months, were included. Comprehensive epidemiological, clinical, radiological, and laboratory data were retrospectively analyzed. Additionally, we compared radiological features between ADEM MOG-Ab-positive patients, and a group of ADEM MOG-Ab-negative ones, recruited during the same period. Among the whole cohort, 13 (48.1%) were pediatric, and 14 (51.9%) were female. MOG-Ab-related disorders comprised eight ADEM, eight ON, five isolated myelitis, four with NMOSD and two patients with multiple sclerosis, at last follow-up. After a median follow-up of 17.8 months, 11 (40.7%) patients presented a relapse. The most frequent clinical phenotype at onset was encephalopathy in pediatrics (53.9%) and myelitis in adults (50%) (p = 0.013). There were no other differences between both groups. When comparing ADEM MOG-Ab positive and negative patients, bilateral thalamic lesions were more often found in the positive group (p = 0.010). Unusual presentations were identified in three patients: patchy spinal cord gadolinium-enhancing lesions, an associated teratoma, and one presented with status epilepticus. MOG-Ab-related disorders shared common clinical and prognostic features, but encompass a spectrum wider than recently reported.

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Acknowledgements

A. Cobo-Calvo is supported by a grant from the Fundación Alfonso Martín Escudero. The authors thank the group of NeuroBioTec from Hôpital Civils de Lyon for supporting this study. This work has been done by using data from the Observatoire Français de la Sclérose en Plaques (OFSEP) which is supported by a grant provided by the French State and handled by the “Agence Nationale de la Recherche”, within the framework of the “Investments for the Future” programme, under the reference ANR-10-COHO-002 Observatoire Français de la Sclérose en plaques (OFSEP), and by the Eugene Devic Foundation against Multiple Sclerosis (EDMUS Foundation).

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Authors and Affiliations

  1. Service de Neurologie A, Hôpital Neurologique Pierre Wertheimer, Hospices Civils de Lyon, 69677, Bron, France

    Álvaro Cobo-Calvo, Nicolas Philippe, Françoise Durand-Dubief, Sandra Vukusic & Romain Marignier

  2. Lyon’s Neuroscience Research Center, U1028 Inserm, UMR5292 Cnrs, FLUID Team, 69008, Lyon, France

    Álvaro Cobo-Calvo, Anne Ruiz, Hyacintha D’Indy & Romain Marignier

  3. Hôpital Femme Mère Enfant, Neurologie pédiatrique, Hospices Civils de Lyon, 69677, Bron, France

    Anne-Lise Poulat, Maryline Carneiro & Vincent Desportes

  4. Service de neuropédiatrie, Hôpital Kremlin-Bicêtre, 78, rue du Général-Leclerc, 94270, Le Kremlin-Bicêtre, France

    Kumaran Deiva

Authors
  1. Álvaro Cobo-Calvo
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  2. Anne Ruiz
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  3. Hyacintha D’Indy
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  4. Anne-Lise Poulat
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  6. Nicolas Philippe
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  7. Françoise Durand-Dubief
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  9. Sandra Vukusic
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  10. Vincent Desportes
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  11. Romain Marignier
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Corresponding author

Correspondence to Álvaro Cobo-Calvo.

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Conflicts of interest

Cobo-Calvo, Ruiz, d’Indy, Poulat, Carneiro, Nicolas and Desportes report no disclosures. Durand-Dubief serves on scientific advisory board for Merck Serono and has received funding for travel and honoraria from Biogen Idec, Merck Serono, Novartis, Sanofi-Genzyme, Roche and Teva. Deiva received travel funding from Biogen Idec, Merck Serono, and Genzyme. Vukusic has received consulting and lecturing fees, travel grants and research support from Biogen, Geneuro, Genzyme, Novartis, Merck Serono, Roche, Sanofi Aventis and Teva Pharma. Marignier  serves on scientific advisory board for MedImmune and has received funding for travel and honoraria from Biogen Idec, Merck Serono, Novartis, Sanofi-Genzyme and Teva.

Ethical standards

The ethics committee of Lyon University Hospital approved this study. All samples were stored at −80 °C at Neurobiotec (Hospices Civils de Lyon, France, no. 0033-00046, AC-2013-1867, NFS96-900).

Informed consent

Informed consent for storage and use of these samples for research was obtained from all patients.

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Cobo-Calvo, Á., Ruiz, A., D’Indy, H. et al. MOG antibody-related disorders: common features and uncommon presentations. J Neurol 264, 1945–1955 (2017). https://doi.org/10.1007/s00415-017-8583-z

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  • DOI: https://doi.org/10.1007/s00415-017-8583-z

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