MOGAD
Acute Treatments
Treatment guidelines for MOG antibody disease have not been established. The following are possible treatments in the management of an acute event.
Intravenous Steroids
Although there are no clinical trials that support a unique approach to treat those with MOG antibody disease, it is well recognized as a standard of care to give high-dose intravenous methylprednisolone for suspected acute myelitis or optic neuritis, generally for 3 to 5 days, unless there are compelling reasons not to. The decision to offer continued steroids or add a new treatment is often based on the clinical course and MRI appearance at the end of 5 days of steroids. Those with MOG antibody disease seem to respond well to steroids.2,3 An oral steroid taper may be helpful to prevent steroid-withdrawal relapses.
Plasma Exchange (PLEX)
PLEX is believed to work in autoimmune CNS diseases through the removal of specific or nonspecific soluble factors likely to mediate, be responsible for, or contribute to inflammatory-mediated organ damage. PLEX is often recommended for moderate to aggressive forms of TM and ON, as is very often the case with MOG antibody disease, if there is not much improvement after being treated with intravenous steroids. If presenting symptoms are severe, PLEX may be initiated concurrently with steroids. There have been no prospective clinical trials that prove PLEX’s effectiveness in MOG antibody disease, but retrospective studies of TM treated with IV steroids followed by PLEX have shown a beneficial outcome. PLEX also has been shown to be effective in other autoimmune or inflammatory central nervous system disorders. Early treatment is beneficial – PLEX is typically started within days of administering steroids, very often before the course of steroids has finished. Particular benefit has been shown if started within the acute or sub-acute stage of the myelitis or if there is continued active inflammation on MRI.
Intravenous Immunoglobulin (IVIG)
Another option for treating anti-MOG associated acute inflammation is intravenous immunoglobulin (IVIG). Immunoglobulin comes from pooled blood that is donated by thousands of healthy people.16 As the name suggests, IVIG is given intravenously. IVIG is generally well-tolerated. Potential adverse reactions are uncommon, but usually occur during or immediately after an infusion and include headache, nausea, muscle pain, fever, chills, chest discomfort, skin and anaphylactic reactions. Reactions after an infusion can be more serious and include migraine headaches, aseptic meningitis, renal impairment and blood clots.17 Like corticosteroids and PLEX, there are no data from clinical trials confirming the value of IVIG in the setting of acute events. However, several studies in pediatric MOG antibody disease have shown efficacy of 1-5 days of IVIG after an acute attack.18-20
Other Acute Treatments
In cases of no response to either steroids or PLEX therapy and continued presence of active inflammation in the spinal cord, other forms of immune-based interventions may be required. The use of immunosuppressants or immunomodulatory agents may be considered in some cases. Initial presentation with aggressive forms of myelitis, or if particularly refractory to treatment with steroids and/or PLEX, aggressive immunosuppression is considered. Individuals should be monitored carefully as potential complications may arise from immunosuppression. As with all medications, risks versus benefits of aggressive immunosuppression need to be considered and discussed with the clinical care team.
(2) Kezuka T, Ishikawa H. Diagnosis and treatment of anti-myelin oligodendrocyte glycoprotein antibody positive optic neuritis. Jpn J Ophthalmol. 2018 Mar;62(2):101-108. doi: 10.1007/s10384-018-0561-1. Epub 2018 Feb 14.
(3) Dos Passos GR, Oliveira LM, da Costa BK, et al. MOG-IgG-associated optic neuritis, encephalitis, and myelitis: Lessons learned from neuromyelitis optica spectrum disorder. Front Neurol. 2018 Apr 4;9:217. doi: 10.3389/fneur.2018.00217. eCollection 2018.
(16) Guo Y, Tian X, Wang X, Xiao Z. Adverse Effects of Immunoglobulin Therapy. Front Immunol. 2018 Jun 8;9:1299. doi: 10.3389/fimmu.2018.01299. eCollection 2018.
(18) Hacohen Y, Absoud M, Deiva K, et al. Myelin oligodendrocyte glycoprotein antibodies are associated with a non-MS course in children. Neurol Neuroimmunol Neuroinflamm. 2015; 2: e81
(19) 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: 478-487.
(20) Armangue T, Olive-Cirera G, Martinez-Hernandez E, et al. Associations of paediatric demyelinating and encephalitic syndromes with myelin oligodendrocyte glycoprotein antibodies: a multicentre observational study. Lancet Neurol. 2020; 19: 234-246.