What is the treatment for transverse myelitis?

Treatment of Transverse Myelitis

The first-line treatment for transverse myelitis is high-dose intravenous methylprednisolone (1 g daily for 3-5 days), followed by immunosuppressive therapy with cyclophosphamide in severe cases. 1

Diagnostic Evaluation

Before initiating treatment, a thorough diagnostic workup should be performed:

• Neurologic consultation to assess the severity and characteristics of symptoms 1
• MRI of spine with thin axial cuts through the region of suspected abnormality and MRI of brain to detect T2-weighted hyperintense lesions (70-93% sensitivity) 1
• Lumbar puncture to analyze:
  • Cell count, protein, glucose levels 1
  • Oligoclonal bands 1
  • Viral PCRs to exclude infectious causes 1
  • Cytology and onconeural antibodies 1
• Blood tests for:
  • B12, HIV, RPR, ANA, Ro/La antibodies 1
  • TSH levels 1
  • Aquaporin-4 IgG (to help diagnose co-existing neuromyelitis optica) 1
• Evaluation for urinary retention and constipation 1

Treatment Algorithm Based on Severity

Mild Symptoms (Grade 1)

• Permanently discontinue immune checkpoint inhibitors if that was the cause 1
• Methylprednisolone 2 mg/kg 1
• Monitor for progression of symptoms 1

Moderate Symptoms (Grade 2)

• Higher doses of methylprednisolone (1 g/day for 3-5 days) 1
• Consider intravenous immunoglobulin (IVIG) 1
• Monitor for treatment response within a few days to 3 weeks 1

Severe Symptoms (Grade 3-4)

• Combination of intravenous methylprednisolone pulse therapy (1 g daily for 3-5 days) and intravenous cyclophosphamide 1
• Consider plasma exchange therapy in refractory cases 1
• For patients with antiphospholipid antibodies, anticoagulation therapy may be beneficial 1

Maintenance Therapy

• After acute treatment, maintenance immunosuppressive therapy (typically azathioprine) is recommended due to high relapse rates (50-60%) during corticosteroid dose reduction 1
• Regular monitoring for treatment response and potential relapses 1

Special Considerations

Transverse Myelitis in Systemic Lupus Erythematosus (SLE)

• Combination of intravenous methylprednisolone and intravenous cyclophosphamide is effective if used promptly 1
• Neurological response paralleled by MRI improvement typically occurs within days to 3 weeks 1
• Plasma exchange therapy may be considered in severe cases 1

Transverse Myelitis Associated with Infections

• Rule out infectious causes before starting immunosuppressive therapy 1
• If infection is suspected, antimicrobial/antiviral therapy should be initiated while awaiting confirmation 1

Prognostic Factors

Factors associated with severe neurological deficit include:

• Extensive spinal cord MRI lesions 1
• Reduced muscle strength or sphincter dysfunction at presentation 1
• Presence of antiphospholipid antibodies 1
• Delay (>2 weeks) in the initiation of therapy 1

Treatment Response and Follow-up

• Monitor neurological response and MRI improvement 1
• Be vigilant for relapses, which are common (50-60%) during corticosteroid dose reduction 1
• Maintenance immunosuppressive therapy may be necessary to prevent relapses 1

Cautions and Pitfalls

• Delay in treatment initiation (>2 weeks) is associated with worse outcomes 1
• Always rule out infectious causes of myelitis before starting immunosuppressive therapy 1
• Consider antiphospholipid syndrome in patients with transverse myelitis, as it may require additional anticoagulation therapy 1
• Monitor for side effects of high-dose corticosteroids and immunosuppressive agents 1