What is the treatment for Transverse Myelitis?

Treatment of Transverse Myelitis

Immediately initiate high-dose intravenous methylprednisolone 1g daily for 3-5 days as first-line therapy, and for moderate to severe cases, combine this with IVIG 2g/kg over 5 days. 1, 2

Immediate Management Steps

Discontinue Causative Agents

• Stop any potential triggering medications immediately, particularly immune checkpoint inhibitors if the patient is receiving cancer immunotherapy 1, 2
• This step must occur before or concurrent with immunosuppressive treatment 3

First-Line Immunosuppressive Therapy

For all cases:

• Administer intravenous methylprednisolone 1g daily for 3-5 days 1, 4, 2
• This represents the standard acute treatment endorsed across multiple guidelines 5, 6

For moderate to severe presentations (significant weakness, sensory changes, or sphincter dysfunction):

• Add IVIG 2g/kg divided over 5 days in combination with corticosteroids 1, 2
• The combination approach is particularly important when patients present with reduced muscle strength or autonomic dysfunction 1

Second-Line Therapy for Refractory Cases

If inadequate response within 7-10 days:

• Initiate plasma exchange therapy 1, 2, 5
• Consider rituximab, especially if autoimmune encephalopathy antibodies are positive 1, 2
• For severe cases associated with systemic lupus erythematosus, early aggressive treatment with IV methylprednisolone pulses combined with cyclophosphamide may improve prognosis 6

Diagnostic Workup (Concurrent with Treatment)

Neuroimaging:

• MRI of spine with thin axial cuts through suspected abnormality to identify T2-weighted hyperintense lesions 1, 4, 7
• Lesions extending ≥3 vertebral segments (longitudinally extensive transverse myelitis) suggest neuromyelitis optica spectrum disorder and require more aggressive immunosuppression 1, 2

Laboratory evaluation:

• Lumbar puncture: cell count, protein, glucose, oligoclonal bands, viral PCRs, onconeural antibodies 1, 4, 2
• Blood tests: aquaporin-4 IgG (for NMOSD), MOG-IgG, B12, HIV, RPR, ANA, Ro/La, TSH 1, 4, 2
• Antiphospholipid antibodies if considering thrombotic etiology 1, 6

Autonomic assessment:

• Evaluate for urinary retention and constipation, which are common manifestations requiring specific management 1, 4, 2

Special Clinical Scenarios

NMOSD-Associated Transverse Myelitis

• If aquaporin-4 IgG positive, more aggressive immunosuppression is required 1, 2
• These patients need long-term maintenance therapy to prevent relapses 1

Antiphospholipid Antibody-Positive Cases

• Add anticoagulation therapy to immunosuppressive treatment 1
• This addresses the thrombotic component of pathophysiology 6

Immune Checkpoint Inhibitor-Related

• Permanently discontinue the checkpoint inhibitor 3, 2
• Consider hospitalization for severe weakness, respiratory compromise, or dysphagia 3
• May require escalation to methylprednisolone 1-2 mg/kg IV or higher dose bolus 3

Maintenance and Long-Term Management

Preventing relapses:

• Maintenance immunosuppressive therapy (such as azathioprine) is necessary after acute treatment 1
• Relapses occur in 50-60% of cases during corticosteroid dose reduction, making maintenance critical 1
• For autoimmune-associated cases, continued immunosuppression prevents recurrence 1

Prognostic Factors and Treatment Timing

Poor prognostic indicators:

• Extensive spinal cord MRI lesions, especially longitudinally extensive transverse myelitis (≥3 vertebral segments) 1, 2
• Reduced muscle strength or sphincter dysfunction at presentation 1
• Delay >2 weeks in initiating therapy significantly worsens outcomes 1

Key distinguishing feature:

• Transverse myelitis causes spastic paralysis with increased deep tendon reflexes (upper motor neuron signs), not flaccid paralysis 4, 2
• This distinguishes it from Guillain-Barré syndrome, which presents with flaccid paralysis and reduced/absent reflexes 4

Critical Pitfalls to Avoid

• Do not delay treatment waiting for complete diagnostic workup—begin methylprednisolone immediately while investigations proceed 1, 5
• Do not use corticosteroids alone for moderate-severe cases; combination with IVIG improves outcomes 1, 2
• Do not miss NMOSD by failing to test aquaporin-4 IgG, as these patients require different long-term management 1, 2
• Do not overlook autonomic dysfunction (urinary retention, constipation), which requires specific supportive management 1, 4