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

  • Athanasios PapathanasiouEmail author
  • Radu Tanasescu
  • Jillian Davis
  • Maria Francisca Rocha
  • Sumeet Singhal
  • Michael F. O’Donoghue
  • Cris S. Constantinescu
Original Communication
  • 143 Downloads

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.

Keywords

MOG Myelin oligodendrocyte glycoprotein Concomitant autoimmunity Histopathology 

Notes

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.

Compliance with ethical standards

Conflicts of interest

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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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Neurology, Queen’s Medical CentreNottingham University Hospital NHS TrustNottinghamUK
  2. 2.Department of Pathology, Queen’s Medical CentreNottingham University Hospital NHS TrustNottinghamUK
  3. 3.Division of Clinical NeuroscienceUniversity of NottinghamNottinghamUK

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