What is the treatment for relapse of Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD) in pediatric patients?

Treatment of Relapse in Pediatric MOGAD

For pediatric patients experiencing a relapse of Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD), high-dose intravenous methylprednisolone followed by a slow oral steroid taper is the first-line treatment, with plasma exchange or intravenous immunoglobulin as effective second-line options for severe attacks or inadequate steroid response. 1

First-Line Treatment for Acute Relapse

High-Dose Corticosteroids

• Begin with high-dose intravenous methylprednisolone (typically 20-30 mg/kg/day, maximum 1000 mg daily for 3-5 days) 1
• Follow with oral prednisone taper:
  • Start with ≥12.5 mg daily (or 0.16 mg/kg/day for children) 2
  • Maintain this dose for at least 3 months 2
  • Taper very slowly thereafter (this approach has been shown to reduce relapse risk by 88% compared to shorter tapers) 2

Early immunotherapy (within 7 days of symptom onset) and corticosteroid treatment for ≥5 weeks are independently associated with a 6.7-fold reduced odds of developing a relapsing disease course in pediatric MOGAD 3

Second-Line Treatments

For patients with:

1. Inadequate response to steroids
2. Severe attacks (significant visual loss, severe myelitis)
3. Contraindications to high-dose steroids

Choose one of the following:

Plasma Exchange (PLEX)

• 5-7 exchanges over 10-14 days
• Particularly effective for severe attacks with poor initial response to steroids 1

Intravenous Immunoglobulin (IVIG)

• Dosing: 1-2 g/kg of ideal body weight
• Usually given over 2 consecutive days (1 g/kg each day) 1
• Particularly effective in pediatric patients 1
• Check serum IgA level before administering to prevent severe reactions in patients with IgA deficiency 1

Treatment Algorithm for Relapse Management

1. Immediate assessment:

   • Confirm relapse with MRI if possible
   • Evaluate severity (visual acuity, motor function, sensory deficits)

2. First-line treatment:

   • Start IV methylprednisolone within 7 days of symptom onset 3
   • Continue for 3-5 days

3. Response evaluation at 5-7 days:

   • If good response → transition to oral prednisone at ≥12.5 mg daily (0.16 mg/kg/day for children) 2
   • If poor/incomplete response → add second-line therapy (PLEX or IVIG)

4. Oral steroid taper:

   • Maintain ≥12.5 mg daily for at least 3 months 2
   • Then taper very slowly over several additional months
   • Total duration of steroid treatment should be ≥5 weeks 3

5. Consider long-term immunotherapy for patients with:

   • Multiple relapses
   • Severe residual disability
   • Options include rituximab, mycophenolate mofetil, or azathioprine 1

Monitoring and Follow-up

• Clinical reassessment after 3-4 months of therapy 1
• Consider repeat MOG antibody testing during treatment-free intervals or 1-3 months after PLEX/IVIG if initially negative but MOGAD is still suspected 1
• Monitor for steroid side effects (growth, bone density, blood pressure, glucose)
• Assess for treatment response through improvement in neurological symptoms

Important Considerations

• MOG-IgG positive patients have a high risk of flare-ups after steroid cessation, making slow tapering critical 1
• Avoid MS-specific treatments like interferon-beta or natalizumab, which may increase relapse rates in MOGAD 1
• MOGAD attacks in children are generally more responsive to treatment than in adults, but require careful management to prevent relapses 4
• Abnormal optic nerves on onset MRI are associated with a 12.5-fold reduced odds of relapse 3
• Higher disease severity at onset (measured by EDSS) is associated with greater risk of final disability 3

Pitfalls to Avoid

• Tapering steroids too quickly - this significantly increases relapse risk 2
• Delaying treatment beyond 7 days from symptom onset - early treatment is associated with better outcomes 3
• Using MS-specific therapies like interferon-beta or natalizumab, which may worsen MOGAD 1
• Discontinuing maintenance therapy too early, as relapses can occur even after prolonged periods of stability 1
• Failing to check for co-existing autoantibodies (e.g., NMDAR antibodies) that might require additional treatment considerations 1