What is the treatment for transverse myelitis?

Treatment of Transverse Myelitis

High-dose intravenous methylprednisolone (1g/day for 3-5 days) should be initiated as soon as possible after diagnosis of transverse myelitis, ideally within the first few hours, even while awaiting MRI confirmation. 1

First-Line Treatment

• Begin with IV methylprednisolone 1g/day for 3-5 days 2, 1
• Consider higher doses (up to 1g/day for 3-5 days) in severe cases 2
• This treatment should be started promptly as delay (>2 weeks) in therapy initiation is associated with severe neurological deficits 2

Second-Line Treatment (if no improvement after 3 days or worsening symptoms)

• Add one of the following:
  • Intravenous immunoglobulin (IVIG): 2g/kg divided over 5 days 2, 1
  • Plasma exchange: 5-10 sessions every other day 2, 1

Severe or Life-Threatening Cases

• Consider combination therapy from the beginning:
  • Methylprednisolone plus IVIG or plasma exchange 1
  • For SLE-associated transverse myelitis: IV methylprednisolone combined with IV cyclophosphamide 2
• Admit to ICU for close monitoring 1

Risk Factors for Severe Neurological Deficit

• Extensive spinal cord MRI lesions
• Reduced muscle strength or sphincter dysfunction at presentation
• Positive antiphospholipid antibodies
• Delayed treatment initiation (>2 weeks) 2, 1

Maintenance Therapy

• Taper steroids over at least 4-6 weeks 1
• Maintenance immunosuppressive therapy is crucial to prevent relapses (which occur in 50-60% of patients during corticosteroid dose reduction) 2, 1
• Options for maintenance therapy:
  • Cyclophosphamide (especially for SLE-associated myelitis) 2
  • Rituximab for antibody-mediated autoimmunity 1
  • Azathioprine has shown efficacy in some cases 3

Symptomatic Management

• Neuropathic pain: pregabalin, gabapentin, or duloxetine 1
• Spasticity: baclofen, tizanidine, physical therapy 1
• Monitor for autonomic dysfunction (cardiac arrhythmias, blood pressure fluctuations, urinary retention) 1

Special Considerations

• For antiphospholipid antibody-positive patients: consider anticoagulation therapy 2
• For immune checkpoint inhibitor-related myelitis: permanently discontinue the immunotherapy agent 2
• Always rule out infectious causes before starting immunosuppression 1

Prognosis

• Early aggressive treatment significantly improves outcomes 4
• Factors associated with better prognosis include:
  • Early treatment initiation (within hours of symptom onset)
  • Prompt neurological response (usually within days to 3 weeks) 2
  • Absence of extensive MRI lesions 2

Common Pitfalls to Avoid

• Delaying treatment while awaiting complete diagnostic workup - start methylprednisolone early while continuing evaluation 1
• Failing to rule out infectious causes before immunosuppression
• Inadequate duration of maintenance therapy leading to relapses
• Not monitoring for autonomic dysfunction which can be life-threatening
• Missing associated conditions (like SLE, Sjögren's syndrome, or antiphospholipid antibodies) that may require specific additional treatments 5