Treatment of MOG Antibody Disease
The most effective treatment approach for MOG antibody disease (MOGAD) includes high-dose corticosteroids for acute attacks followed by intravenous immunoglobulin (IVIG) as maintenance therapy to prevent relapses.
Acute Treatment of MOGAD Attacks
MOGAD is an antibody-mediated demyelinating disease of the central nervous system that typically presents with optic neuritis, myelitis, brainstem encephalitis, or ADEM-like presentations. Acute attacks require prompt intervention:
First-Line Therapy
- High-dose intravenous corticosteroids (typically methylprednisolone 1000 mg daily for 3-5 days) 1
- Follow with extended oral corticosteroid taper (typically over 2-3 months) to prevent early relapses 2
For Severe Attacks or Inadequate Response to Steroids
- Plasma exchange (PLEX) should be considered as an adjunctive therapy 1, 3
- Particularly effective for patients with severe disability or poor response to steroids
- Usually 5-7 exchanges over 10-14 days
Long-Term Maintenance Therapy
For patients with relapsing MOGAD, long-term immunotherapy is necessary to prevent further attacks:
First-Line Maintenance Options
- IVIG: Most effective maintenance therapy with lowest annualized relapse rate (ARR 0) 4
- Typical regimen: 0.4 g/kg/day for 5 days initially, followed by 0.4-1 g/kg every 4 weeks
Alternative Maintenance Options (in order of effectiveness)
- Typical dose: 2-3 mg/kg/day
- Monitor blood counts and liver function
Mycophenolate mofetil: ARR 0.11-0.67 4, 5
- Typical dose: 1000-2000 mg/day in divided doses
- Monitor blood counts regularly
- Typical regimen: 375 mg/m² weekly for 4 weeks or 1000 mg given twice, 2 weeks apart
- Monitor CD19/CD20 B-cell counts
Low-dose oral corticosteroids: May be effective but has significant long-term side effects 2
Important Clinical Considerations
Treatment Monitoring
- Regular monitoring of MOG-IgG titers is not recommended for routine clinical decision-making 1
- Monitor for clinical relapses and disability progression using appropriate scales (EDSS, visual acuity)
Treatment Failures
- For patients who fail first-line maintenance therapy, consider switching to another agent
- Tocilizumab (IL-6 inhibitor) has shown promise in highly relapsing, treatment-resistant cases 6
- Complete relapse freedom was observed in 10 patients over an average of 28.6 months
Contraindications
- Multiple sclerosis disease-modifying therapies are ineffective and should be avoided in MOGAD 4
- Patients treated with MS therapies had breakthrough relapses (ARR 1.5)
Special Populations
- Children with MOGAD: IVIG appears particularly effective 1, 2
- Pregnancy: Treatment decisions require careful risk-benefit assessment
Red Flags for Diagnosis Reconsideration
If any of these features are present, reconsider the diagnosis of MOGAD 1:
- Chronic progressive disease course
- Typical MS-like lesions (Dawson's fingers, periventricular ovoid lesions)
- Positive MRZ reaction in CSF
- Combined central and peripheral demyelination
Early diagnosis and appropriate treatment of MOGAD are crucial for preventing relapses and minimizing long-term disability. The treatment approach should be tailored based on attack severity, relapse frequency, and response to previous therapies.