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



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


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


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


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.


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.


MOG Myelin oligodendrocyte glycoprotein Concomitant autoimmunity Histopathology 



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.


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.


  1. 1.
    Wynford-Thomas R, Jacob A, Tomassini V (2019) Neurological update: MOG antibody disease. J Neurol 266:1280–1286CrossRefGoogle Scholar
  2. 2.
    Reindl M, Jarius S, Rostasy K et al (2017) Myelin oligodendrocyte glycoprotein antibodies: how clinically useful are they? Curr Opin Neurol 30:295–301CrossRefGoogle Scholar
  3. 3.
    Oliveira LM, Apostolos-Pereira SL, Pitombeira MS et al (2018) Persistent MOG-IgG positivity is a predictor of recurrence in MOG-IgG-associated optic neuritis, encephalitis and myelitis. Mult Scler. CrossRefPubMedGoogle Scholar
  4. 4.
    Jurynczyk M, Jacob A, Fujihara K et al (2018) Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease: practical considerations. Pract Neurol 19(3):187–195CrossRefGoogle Scholar
  5. 5.
    Jarius S, Paul F, Aktas O et al (2018) MOG encephalomyelitis: international recommendations on diagnosis and antibody testing. J Neuroinflammation 15:134CrossRefGoogle Scholar
  6. 6.
    Waters P, Woodhall M, O’Connor KC et al (2015) MOG cell-based assay detects non-MS patient with inflammatory neurologic disease. Neurol Neuroimmunol Neuroinflamm 2:e89CrossRefGoogle Scholar
  7. 7.
    Waters P, Komorowski L, Woodhall M et al (2019) A multicentre comparison of MOG-IgG cell-based assays. Neurology 92:e1250–e1255PubMedPubMedCentralGoogle Scholar
  8. 8.
    Wingerchuck DM, Banwell B, Bennett JL et al (2015) International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology 85:177–189CrossRefGoogle Scholar
  9. 9.
    Jurynczyk M, Messina S, Woodhall MR et al (2017) Clinical presentation and prognosis in MOG-antibody disease: a UK study. Brain 140:3128–3138CrossRefGoogle Scholar
  10. 10.
    Cobo-Calvo A, Ruiz A, Maillart E et al (2018) Clinical spectrum and prognostic value of CNS MOG autoimmunity in adults: the MOGADOR study. Neurology 90:e1858–e1869CrossRefGoogle Scholar
  11. 11.
    Ogawa R, Nakashima I, Takahashi T et al (2017) MOG-antibody-positive, benign, unilateral, cerebral cortical encephalitis with epilepsy. Neurol Neuroimmunol Neuroinflamm 4:e322CrossRefGoogle Scholar
  12. 12.
    Hamid SHM, Whittam D, Saviour M et al (2018) Seizures and encephalitis in myelin oligodendrocyte glycoprotein IgG disease vs aquaporin 4 IgG disease. JAMA Neurol 75:65–71CrossRefGoogle Scholar
  13. 13.
    Jarius S, Ruprecht K, Kleiter I et al (2016) MOG-IgG in NMO and related disorders: a multicentre study of 50 patients. Part 2: epidemiology, clinical presentation, radiological and laboratory features, treatment responses, and long-term outcome. J Neuroinflammation 13:280Google Scholar
  14. 14.
    Salma S, Khan M, Levy M et al (2019) Radiological characteristics of myelin oligodendrocyte glycoprotein antibody disease. Mult Scler Relat Disord 29:15–22CrossRefGoogle Scholar
  15. 15.
    Wang L, ZhangBao J, Zhou L et al (2019) Encephalitis is an important clinical component of myelin oligodendrocyte glycoprotein antibody associated demyelination: a single-center cohort study in Shanghai, China. Eur J Neurol 26:168–174CrossRefGoogle Scholar
  16. 16.
    Zhong X, Zhou Y, Chang Y et al (2019) Seizure and myelin oligodendrocyte glycoprotein antibody-associated encephalomyelitis in a retrospective cohort of chinese patients. Front Neurol 10:415CrossRefGoogle Scholar
  17. 17.
    Dos Passos GR, Oliveira LM, da Costa BK et al (2018) MOG-IgG-associated optic neuritis, encephalitis, and myelitis: lessons learned from neuromyelitis optica spectrum disorder. Front Neurol 9:217CrossRefGoogle Scholar
  18. 18.
    Komatsu T, Matsushima S, Kaneko K et al (2019) Perivascular enhancement in anti-MOG antibody demyelinating disease of the CNS. J Neurol Neurosurg Psychiatry 90:111–112CrossRefGoogle Scholar
  19. 19.
    Weber MS, Derfuss T, Metz I et al (2018) Defining distinct features of anti-MOG antibody associated central nervous system demyelination. Ther Adv Neurol Disord 11:1–15CrossRefGoogle Scholar
  20. 20.
    Spadaro M, Gerdes LA, Mayer MC et al (2015) Histopathology and clinical course of MOG-antibody-associated encephalomyelitis. Ann Clin Transl Neurol 2:295–301CrossRefGoogle Scholar
  21. 21.
    Subesinghe S, van Leuven S, Yalakki L et al (2018) Cocaine and ANCA associated vasculitis-like syndromes—a case series. Autoimmun Rev 17:73–77CrossRefGoogle Scholar
  22. 22.
    Marquez J, Aguirre L, Munoz C et al (2017) Cocaine-levamisole-induced vasculitis/vasculopathy syndrome. Curr Rheumatol Rep 19:36CrossRefGoogle Scholar
  23. 23.
    Zheng Y, Zhang Y, Cai M et al (2019) Central Nervous System Involvement in ANCA-Associated Vasculitis: What Neurologists Need to Know. Front Neurol 9:1166CrossRefGoogle Scholar
  24. 24.
    Salama S, Khan M, Pardo S et al (2019) MOG antibody-associated encephalomyelitis/encephalitis. Mult Scler. CrossRefPubMedGoogle Scholar
  25. 25.
    Budhram A, Mirian A, Le C et al (2019) Unilateral cortical FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES): characterization of a distinct clinic-radiographic syndrome. J Neurol. CrossRefPubMedGoogle Scholar
  26. 26.
    Lopez-Chiriboga AS, Majed M, Fryer J et al (2018) Association of MOG-IgG serostatus with relapse after acute disseminated encephalomyelitis and proposed diagnostic criteria for MOG-IgG-associated disorders. JAMA Neurol 75:1355–1363CrossRefGoogle Scholar
  27. 27.
    Spadaro M, Gerdes LA, Krumbholz M et al (2016) Autoantibodies to MOG in a distinct subgroup of adult multiple sclerosis. Neurol Neuroimmunol Neuroinflamm 3:e257CrossRefGoogle Scholar
  28. 28.
    Cobo-Calvo A, Sepulveda M, Bernard-Valnet R et al (2016) Antibodies to myelin oligodendrocyte glycoprotein in aquaporin 4 antibody seronegative longitudinally extensive transverse myelitis: Clinical and prognostic implications. Mult Scler 22:312–319CrossRefGoogle Scholar
  29. 29.
    Baba T, Shinoda K, Watanabe M et al (2019) MOG antibody disease manifesting as progressive cognitive deterioration and behavioural changes with primary central nervous system vasculitis. Mult Scler Relat Disord 30:48–50CrossRefGoogle Scholar
  30. 30.
    Patterson K, Iglesias E, Nasrallah M et al (2019) Anti-MOG encephalitis mimicking small vessel CNS vasculitis. Neurol Neuroimmunol Neuroinflamm 6:e538CrossRefGoogle Scholar
  31. 31.
    Titulaer MJ, Hoftberger R, Iizuka T et al (2014) Overlapping demyelinating syndromes and anti-N-methyl-d-aspartate receptor encephalitis. Ann Neurol 75:411–428CrossRefGoogle Scholar
  32. 32.
    Fan S, Xu Y, Ren H et al (2018) Comparison of myelin oligodendrocyte glycoprotein (MOG)-antibody disease and AQP4-IgG-positive neuromyelitis optica spectrum disorder (NMOSD) when they co-exist with anti-NMDA (N-methyl-d-aspartate) receptor encephalitis. Mult Scler Relat Dosord 20:144–152CrossRefGoogle Scholar
  33. 33.
    Baizabal-Carvallo JF (2019) The neurological syndromes associated with glutamic acid decarboxylase antibodies. J Autoimmun 101:35–47CrossRefGoogle Scholar
  34. 34.
    Macaron G, Ontaneda D (2019) MOG-related disorders: a new cause of imaging-negative myelitis? Mult Scler. CrossRefPubMedGoogle Scholar
  35. 35.
    Sundaram S, Nair SS, Jaganmohan D et al (2019) Relapsing lumbosacral myeloradiculitis: an unusual presentation of MOG antibody disease. Mult Scler. CrossRefPubMedGoogle Scholar
  36. 36.
    Reindl M, Waters P (2019) Myelin oligodendrocyte glycoprotein antibodies in neurological disease. Nat Rev Neurol 15:89–102CrossRefGoogle Scholar

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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