What is the treatment for transverse myelitis?

Treatment for Transverse Myelitis

High-dose intravenous methylprednisolone (1g daily for 3-5 days) is the first-line treatment for transverse myelitis and should be initiated promptly, with the addition of IVIG for moderate to severe cases. 1

Diagnostic Approach

• Obtain prompt neurologic consultation to guide diagnosis and management 1
• Perform MRI of the spine with thin axial cuts to detect characteristic T2-weighted hyperintense lesions 1, 2
• Conduct lumbar puncture to analyze cell count, protein, glucose, oligoclonal bands, viral PCRs, and onconeural antibodies 1
• Order blood tests including B12, HIV, RPR, ANA, Ro/La, TSH, and aquaporin-4 IgG to rule out other causes and identify associated conditions 1, 2
• Evaluate for urinary retention and constipation, which are common autonomic manifestations 1

Treatment Algorithm

First-Line Treatment

• Immediately discontinue any potential causative agents (such as immune checkpoint inhibitors if applicable) 3, 1
• Administer high-dose intravenous methylprednisolone 1g daily for 3-5 days 3, 1
• For moderate to severe cases with significant weakness or sensory changes, combine corticosteroids with IVIG 2g/kg over 5 days 3, 1

Second-Line Treatment (for refractory cases)

• Consider plasma exchange therapy for patients who do not respond adequately to corticosteroids and IVIG 1, 4
• For systemic lupus erythematosus (SLE)-associated transverse myelitis, combine intravenous methylprednisolone with intravenous cyclophosphamide for more effective treatment 3, 5
• Consider rituximab in cases with positive autoimmune encephalopathy antibodies or inadequate response to other therapies 3, 1

Maintenance Therapy

• Implement maintenance immunosuppressive therapy (such as azathioprine) after acute treatment to prevent relapses, which occur in 50-60% of cases during corticosteroid dose reduction 3, 1

Special Considerations

Etiology-Specific Treatment

• For infectious causes:

  • Administer appropriate antimicrobial therapy (e.g., intravenous acyclovir for varicella-zoster virus) alongside corticosteroids 6
  • Rule out infectious myelitis before starting immunosuppressive therapy 3

• For autoimmune-associated transverse myelitis:

  • In SLE or Sjögren's syndrome-associated myelitis, aggressive immunosuppression with cyclophosphamide has shown better outcomes 3, 7, 5
  • For antiphospholipid antibody-positive myelopathy, consider anticoagulation therapy in addition to immunosuppressive treatment 3, 1

• For immune checkpoint inhibitor-related transverse myelitis:

  • Permanently discontinue the immune checkpoint inhibitor 3
  • For grade 1 (mild): methylprednisolone 2 mg/kg 3
  • For grade 2 (moderate): strongly consider higher doses of 1g/day for 3-5 days and IVIG 3
  • For grade 3-4 (severe): high-dose steroids plus IVIG 3

Prognostic Factors and Pitfalls

Poor Prognostic Indicators

• Extensive spinal cord MRI lesions, especially longitudinally extensive transverse myelitis affecting ≥3 vertebral segments 1, 2
• Reduced muscle strength or sphincter dysfunction at presentation 1
• Delay (>2 weeks) in the initiation of therapy 3, 1
• Presence of antiphospholipid antibodies 3

Common Pitfalls to Avoid

• Delaying treatment while awaiting complete diagnostic workup - early intervention is critical for better outcomes 1, 4
• Failing to distinguish transverse myelitis (characterized by spastic paralysis with increased deep tendon reflexes) from conditions with flaccid paralysis like Guillain-Barré syndrome 2, 8
• Overlooking the need for maintenance therapy after acute treatment, leading to high relapse rates 3, 1
• Missing associated systemic autoimmune disorders that may require specific treatment approaches 7, 8