What is the treatment for transverse myelitis?

Treatment of Transverse Myelitis

High-dose intravenous corticosteroids (methylprednisolone 1g/day for 3-5 days) followed by immunosuppressive therapy is the first-line treatment for transverse myelitis, with additional therapies such as IVIG or plasma exchange for moderate to severe cases. 1, 2

Diagnostic Approach

• Neurologic consultation should be obtained promptly for all patients with suspected transverse myelitis 1, 2
• MRI of the spine with thin axial cuts through the region of suspected abnormality is essential to confirm diagnosis and rule out cord compression 1
• Lumbar puncture should be performed to check cell count, protein, glucose, oligoclonal bands, viral PCRs, and onconeural antibodies 1, 2
• Blood tests should include B12, HIV, RPR, ANA, Ro/La, TSH, and aquaporin-4 IgG to rule out other causes 1
• Evaluation for urinary retention and constipation is important as these are common autonomic manifestations 1, 2

Treatment Algorithm

First-Line Treatment

• Permanently discontinue any immune checkpoint inhibitors if they were the triggering cause 1
• Administer high-dose intravenous methylprednisolone (1g/day for 3-5 days) 1, 2
• This should be initiated promptly, ideally within the first few hours of symptom onset 1

Second-Line Treatment (for moderate to severe cases or inadequate response)

• Add intravenous immunoglobulin (IVIG) 2g/kg over 5 days 1, 2
• Consider combination with cyclophosphamide, especially in cases associated with systemic lupus erythematosus 1, 3

Refractory Cases

• Consider plasma exchange therapy 1, 2
• For autoimmune encephalopathy antibody-positive cases with limited improvement, consider rituximab 1
• Maintenance immunosuppressive therapy is often needed to prevent relapses 1

Special Considerations

Systemic Lupus Erythematosus-Associated Transverse Myelitis

• Combination of intravenous methylprednisolone and intravenous cyclophosphamide has shown significant improvement in SLE-related myelitis 1, 3
• Neurological response paralleled by MRI improvement typically occurs within a few days to 3 weeks 1
• Maintenance immunosuppressive therapy is crucial as relapses are common (50-60%) during corticosteroid dose reduction 1, 4

Antiphospholipid Antibody-Associated Myelopathy

• Consider anticoagulation therapy in antiphospholipid-positive myelopathy cases 1
• This approach has shown good results, particularly in cases not responding to immunosuppressive therapy 1

Infectious or Parainfectious Causes

• Rule out infectious causes before initiating immunosuppressive therapy 1
• For schistosomiasis-associated myelopathy, praziquantel is recommended along with corticosteroids 1
• For neurotoxocariasis presenting with myelitis, treatment may include albendazole for 3-4 weeks with corticosteroids 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, 4
  • Delay (>2 weeks) in the initiation of therapy 1

• Early aggressive treatment significantly improves outcomes, particularly in autoimmune-associated transverse myelitis 4, 3

Follow-up and Monitoring

• Monitor for relapses, which are common (up to 50-60%) during corticosteroid dose reduction 1
• Evaluate neurological response with follow-up MRI to document improvement 1
• Assess for recovery of motor function, sensory changes, and autonomic symptoms (bladder/bowel control) 3