Myelin oligodendrocyte glycoprotein antibody disease (MOGAD)

[galyea123]

Request to add a new disease: Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD)

Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is an inflammatory demyelinating disease of the central nervous system (CNS) with the presence of conformation-sensitive antibodies against MOG. The spectrum of MOGAD includes monophasic/relapsing optic neuritis, myelitis, neuromyelitis optica spectrum disorder (NMOSD) phenotype without aquaporin 4 (AQP4) antibodies, acute/multiphasic demyelinating encephalomyelitis (ADEM/MDEM)-like presentation, and brainstem and cerebral cortical encephalitis. The prevalence of MOGAD is approximately 1.3–2.5/100,000. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542411/

I noticed Acute disseminated encephalomyelitis with anti-MOG antibodies (ORPHA:592894) and also Neuromyelitis optica spectrum disorder with anti-MOG antibodies (ORPHA: 592856) but you do not have MOGAD listed.

Please let me know if you would consider adding this condition.

Thank you, Gioconda Alyea

[Orphanet-EmilieGaillard]

Dear Gioconda,

This issue has been thoroughly reviewed with a French expert in November 2020. MOGAD term does not refer to a disorder nor to a group of disorders (because in a given disorder, some patients are MOGAD and others are not). MOGAD is a part of several disorders namely:

1. ORPHA:83597 Acute disseminated encephalomyelitis [Disorder]
2. ORPHA:139417 Acute transverse myelitis [Disorder]
3. ORPHA:499096 Isolated optic neuritis [Disorder]
4. ORPHA:71211 Neuromyelitis optica spectrum disorder [Disorder]

Because the presence of the presence of the anti-MOG auto-antibody confers, for each disease, a differential clinical outcome and lead to different management and treatment of positive patients, the presence of anti-MOG defines a new clinical subtype on its own. Accordingly, a specific MOGAD subtype has been created for each of the concerned disease (as well as their non-MOGAD counterparts). These subtypes can easily be found by coders since “MOGAD” has been added as a KW for each of them:

1. ORPHA:592894 Acute disseminated encephalomyelitis with anti-MOG antibodies [Subtype]
2. ORPHA:592873 Acute transverse myelitis with anti-MOG antibodies [Subtype]
3. ORPHA:592856 Neuromyelitis optica spectrum disorder with anti-MOG antibodies [Subtype]

To note, such subtypes have also originally been created for ORPHA:499096 Isolated optic neuritis [Disorder], but they have been obsoleted in Dec 23 because in the case of Isolated optic neuritis there is no clinical differences between patients positive for MOG-antibodies and patients negative for such autoantibody.

Best regards, Emilie

[galyea123]

Thank you Emilie!