Treatment of Anti-MOG Antibodies in CSF
Initiate IV methylprednisolone 1000 mg/day for 3-5 days immediately upon clinical suspicion without waiting for antibody confirmation, followed by a prolonged oral corticosteroid taper over weeks to months to prevent relapse. 1
Acute Phase Treatment
Start high-dose IV methylprednisolone (1000 mg/day for 3-5 days) as first-line therapy for all MOG-antibody associated disease (MOGAD) presentations including optic neuritis, transverse myelitis, brainstem encephalitis, or cortical encephalitis. 1
Begin treatment immediately based on clinical presentation—do not delay for antibody confirmation if the clinical picture is highly suggestive. 1
If no improvement occurs after 3-5 days of IV steroids, escalate to plasma exchange (5-7 exchanges) or immunoadsorption for steroid-refractory cases. 1, 2, 3, 4
IVIG can be used as an alternative or adjunct therapy in acute management. 5, 2
Critical Post-Acute Management
Implement a prolonged oral corticosteroid taper over weeks to months rather than rapid discontinuation, as 50-60% of patients relapse during steroid dose reduction. 1, 6
Rapid steroid cessation carries high risk of flare-ups and should be avoided. 1, 6
Monitor closely during the taper period for any signs of symptom recurrence. 1
Long-Term Maintenance Therapy
Initiate maintenance immunosuppression in patients with relapsing disease (approximately 50-60% of cases, particularly adults who experience relapsing course in at least 80% of cases). 1, 6
B-cell depleting therapies (rituximab, ocrelizumab, ofatumumab) are emerging as first-line maintenance agents with particularly good responses, though relapses can occur immediately after B-cell reconstitution. 1, 7
Alternative maintenance options include IVIG and oral immunosuppressants based on observational data. 6
Note that 28.6% of MOGAD patients may continue to suffer relapses despite anti-CD20 therapy, requiring additional therapeutic adjustments. 8
Monitoring Strategy
Retest MOG-IgG antibodies 6-12 months after the initial attack to assess prognosis, as antibody disappearance may indicate monophasic disease and potentially allow discontinuation of maintenance therapy. 1, 6
Four patients in one cohort converted to negative antibody status with no attacks occurring after conversion. 8
If long-term immunosuppression is discontinued due to seronegativity, maintain close monitoring of MOG-IgG serostatus as transient seroconversion can occur and antibodies may rise again at relapse. 1
Critical Pitfalls to Avoid
Never use interferon-beta or natalizumab, as these MS disease-modifying therapies worsen MOG-positive disease and increase relapse rates. 5, 1, 6
Avoid fingolimod, which is also contraindicated due to differences in immunopathogenesis. 6
Do not treat MOGAD with standard MS therapies—treatment with mainstay MS treatments worsens outcomes. 5
Ensure MOG-IgG was detected by cell-based assay using full-length human MOG as the target antigen, as this is the gold standard methodology. 5, 1
Diagnostic Considerations Supporting Treatment Decisions
CSF in MOGAD typically shows neutrophilic pleocytosis or white cell count >50/μl, distinguishing it from MS. 9, 6, 2, 3
Absence of CSF-restricted oligoclonal bands is characteristic of MOGAD (particularly in continental European patients) and helps differentiate from MS. 9, 6, 8
Elevated IgG synthesis rate and positive CSF-restricted oligoclonal bands were not seen in MOGAD cohorts, with only rare elevation of IgG index. 8
Progressive disease course is very atypical for MOGAD and should prompt reconsideration of the diagnosis. 9, 6
Special Clinical Scenarios
In refractory cases with seizures (FLAMES presentation), rituximab may be required in addition to standard acute therapies. 2
For extensive brainstem involvement, the same treatment approach applies with steroids and immunoglobulins showing complete MRI resolution and near-complete clinical recovery. 4
Rare co-existence with NMDA receptor antibodies may occur, requiring combined treatment approaches with steroid pulse therapy and plasma exchange. 3
Tocilizumab can successfully prevent relapse in severe refractory MOG disease when other therapies fail. 5