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MOG-IgG-associated demyelination: focus on atypical features, brain histopathology and concomitant autoimmunity

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Abstract

Introduction

Antibodies to myelin oligodendrocyte glycoprotein (MOG) have been demonstrated in patients with optic neuritis (ON), encephalitis and myelitis.

Objective

To describe the clinical and paraclinical features in patients with MOG-associated demyelination, focusing on unusual cases, brain biopsy and concomitant autoimmunity.

Methods

A single centre retrospective observational case series, analysing demographic, clinical, laboratory, histopathology and radiological data from MOG- positive patients.

Results

We identified 20 adults. The male/female ratio was 1.5. Mean age at onset was 31.6 years and mean disease duration was 7.5 years. The most frequent presentation was myelitis (45%), followed by ON (30%). One case had simultaneous myelitis and ON. Two patients had a cortical syndrome, 1 patient had an encephalopathic presentation and 1 cryptogenic focal epilepsy. Anti-neutrophil cytoplasmic antibodies (ANCA) were found in 3 cases, while 1 patient had an antibody to glutamic acid decarboxylase (GAD). Brain biopsy was performed in 2 patients. Relapsing course was identified in 60% of patients. We also discuss 3 cases with atypical features, brain histopathology and concomitant autoimmunity.

Conclusion

MOG- associated demyelination represents a new disease entity. Unusual cases are reported, expanding the disease spectrum. Elucidating this further should be the focus of prospective studies.

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Acknowledgements

We thank the patients who gave consent for us to report their individual cases. We thank Dr N. Evangelou, Dr C. Gilmore and Dr B. Gran, who provided patients for this study. We thank Dr G. Sare for critically reading the manuscript and useful comments.

Funding

This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Author information

Authors and Affiliations

  1. Department of Neurology, Queen’s Medical Centre, Nottingham University Hospital NHS Trust, Nottingham, NG7 2UH, UK

    Athanasios Papathanasiou, Radu Tanasescu, Maria Francisca Rocha, Sumeet Singhal & Michael F. O’Donoghue

  2. Department of Pathology, Queen’s Medical Centre, Nottingham University Hospital NHS Trust, Nottingham, UK

    Jillian Davis

  3. Division of Clinical Neuroscience, University of Nottingham, Nottingham, UK

    Cris S. Constantinescu

Authors
  1. Athanasios Papathanasiou
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  2. Radu Tanasescu
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  3. Jillian Davis
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  4. Maria Francisca Rocha
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  5. Sumeet Singhal
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  6. Michael F. O’Donoghue
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  7. Cris S. Constantinescu
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Correspondence to Athanasios Papathanasiou.

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The authors declare that there is no conflict of interest.

Ethical standards

The study was approved by the Quality and Safety Committee of Nottingham University Hospitals NHS Trust.

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Papathanasiou, A., Tanasescu, R., Davis, J. et al. MOG-IgG-associated demyelination: focus on atypical features, brain histopathology and concomitant autoimmunity. J Neurol 267, 359–368 (2020). https://doi.org/10.1007/s00415-019-09586-5

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  • DOI: https://doi.org/10.1007/s00415-019-09586-5

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