Antimyelin Oligodendrocyle Glycoprotein Demyelination

MOG antibody-related disorders, linked to demyelinating conditions like ADEM and NMOSD, are diagnosed by serum MOG-IgG positivity and CNS symptoms. Prognosis is generally favorable. Treatment includes high-dose methylprednisolone for acute attacks, and immunotherapies like mycophenolate or rituximab for relapsing cases to reduce frequency.

Overview of Myelin Oligodendrocyte Glycoprotein (MOG) Antibody-Associated Disorders:

Myelin oligodendrocyte glycoprotein (MOG) is a protein which is expressed on the outer surface of oligodendrocytes and myelin sheath. The presence of anti- MOG antibodies may represent a unique humoral mediated group of demyelinating conditions. Anti- MOG antibodies are nearly detected in patients with multiphasic disseminated encephalomyelitis (MDEM), acute disseminated encephalomyelitis (ADEM), relapsing optic neuritis and neuromyelitis optica spectrum disorders (NMOSD). (1)

Diagnostic Criteria for MOG Antibody-Associated Disorders:

For diagnosis of MOG antibody-associated disorders, it requires serum positivity for MOG-IgG, a clinical presentation consistent with central nervous system demyelination (ADEM, optic neuritis, transverse myelitis, a brain or brainstem demyelinating syndrome, or any combination of these), serum positivity for MOG- IgG and exclusion of an alternative diagnosis. The outcome for patients with MOG antibodies is generally thought to be favourable and patients usually have good long term outcomes with little disability, although patients with transverse myelitis may have more severe residual deficits. (2, 3, 4, 5)

Management of Acute Attacks in MOG Antibody-Associated Disorders:

Acute attacks are often treated with high dose intravenous methylprednisolone (1 gram daily for three to five consecutive days). For patients unresponsive to glucocorticoids, then therapeutic plasma exchange is the suggested rescue treatment. (6, 7)

Long-Term Treatment and Relapse Prevention in MOG Antibody-Associated Disease:

Among patients who have relapsing MOG antibody-associated disease, retrospective data suggest that immunotherapy with mycophenolate (2 - 3g daily), intravenous rituximab (1000 mg every six months) and immune globulin are associated with a reduced relapse frequency. (7, 8)

References

1- Hennes EM, Baumann M, Schanda K, Anlar B, Bajer-Kornek B, Blaschek A, Brantner-Inthaler S, Diepold K, Eisenkölbl A, Gotwald T, Kuchukhidze G, Gruber-Sedlmayr U, Häusler M, Höftberger R, Karenfort M, Klein A, Koch J, Kraus V, Lechner C, Leiz S, Leypoldt F, Mader S, Marquard K, Poggenburg I, Pohl D, Pritsch M, Raucherzauner M, Schimmel M, Thiels C, Tibussek D, Vieker S, Zeches C, Berger T, Reindl M, Rostásy K, BIOMARKER Study Group: Prognostic relevance of MOG antibodies in children with an acquired demyelinating syndrome. Neurology. 2017;89(9):900. Epub 2017 Aug 2.2-

2-López-Chiriboga AS, Majed M, Fryer J, et al. Association of MOG-IgG Serostatus With Relapse After Acute Disseminated Encephalomyelitis and Proposed Diagnostic Criteria for MOG-IgG-Associated Disorders. JAMA Neurol 2018; 75:1355.

3- Ramanathan S, Dale RC, Brilot F: Anti-MOG antibody: The history, clinical phenotype, and pathogenicity of a serum biomarker for demyelination. Autoimmun Rev. 2016;15(4):307. Epub 2015 Dec 17.Anti-MOG antibody: The history, clinical phenotype, and pathogenicity of a serum biomarker for demyelination.

4-Polman CH, Reingold SC, Banwell B, Clanet M, Cohen JA, Filippi M, et al. . Diagnostic criteria for multiple sclerosis: 2010 revisions to the McDonald criteria. Ann Neurol. (2011) 69:292–302.

5-Kortvelyessy P, Breu M, Pawlitzki M, Metz I, Heinze HJ, Matzke M, et al. . ADEM-like presentation, anti-MOG antibodies, and MS pathology: TWO case reports. Neurol Neuroimmunol Neuroinflamm. (2017) 4:e335.

6-Sato DK, Callegaro D, Lana-Peixoto MA, Waters PJ, de Haidar Jorge FM, Takahashi T, et al. . Distinction between MOG antibody-positive and AQP4 antibody-positive NMO spectrum disorders. Neurology (2014) 82:474–81.

7- Ramanathan S, Mohammad S, Tantsis E, et al. Clinical course, therapeutic responses and outcomes in relapsing MOG antibody- associated demyelination. J Neurol Neurosurg Psychiatry 2018; 89:127.

8-Hacohen Y, Wong YY, Lechner C, et al. Disease Course and Treatment Responses in Children With Relapsing Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease. JAMA Neurol 2018; 75:478.