Treatment of Transverse Myelitis
Immediately initiate high-dose intravenous methylprednisolone 1 gram daily for 3-5 days as first-line therapy for acute transverse myelitis, and for moderate to severe cases (significant weakness, sensory changes, or dysautonomia), combine this with IVIG 2 g/kg divided over 5 days. 1, 2, 3
Immediate Actions Upon Presentation
- Discontinue any potential causative medications immediately, particularly immune checkpoint inhibitors or other immunosuppressive agents that may trigger immune-mediated myelitis 1, 2, 3
- Obtain urgent neurologic consultation to guide diagnosis and management 2
- Perform MRI of the entire spine with and without contrast using thin axial cuts to confirm T2-weighted hyperintense lesions and assess extent (particularly if ≥3 vertebral segments, indicating longitudinally extensive transverse myelitis) 1, 3
First-Line Immunotherapy
Standard Cases
- Administer IV methylprednisolone 1 gram daily for 3-5 days as pulse dosing 1, 2, 3, 4
- This represents the cornerstone of acute treatment across all guidelines 5
Moderate to Severe Cases
- Simultaneously combine IV methylprednisolone with IVIG 2 g/kg divided over 5 days (0.4 g/kg/day) when patients present with significant motor weakness, sensory deficits, or autonomic dysfunction 1, 2, 3
- The combination approach is specifically indicated when severe dysautonomia is present (blood pressure instability, temperature dysregulation, cardiac arrhythmias), requiring more aggressive treatment 1
Second-Line Therapy for Refractory Cases
- Initiate plasma exchange (PLEX) with 5-10 sessions every other day if no clinical improvement occurs within 7-10 days of combined corticosteroid and IVIG therapy 1, 2, 3
- Consider starting PLEX earlier (within 3 days) if symptoms actively worsen or progress despite initial therapy 1
- Plasma exchange is the established second-line option when first-line immunotherapy fails 5
Third-Line Options
- Rituximab may be beneficial in cases with positive autoimmune encephalopathy antibodies (particularly anti-aquaporin-4 or anti-MOG antibodies) or inadequate response to corticosteroids, IVIG, and plasma exchange 2, 3
- Cyclophosphamide can be considered in severe refractory cases, particularly in systemic lupus erythematosus-associated transverse myelitis, where pulse cyclophosphamide for approximately 6 months following acute treatment has shown benefit 6
Maintenance Immunosuppression
- After acute treatment, maintenance immunosuppressive therapy (such as azathioprine) is necessary in 50-60% of cases to prevent relapses during corticosteroid dose reduction 2
- For autoimmune-associated transverse myelitis, continued immunosuppression may be required long-term to prevent recurrence 2
Critical Diagnostic Work-Up (Concurrent with Treatment)
Do not delay treatment while awaiting diagnostic results - initiate immunotherapy based on clinical presentation and MRI findings 1
Imaging
- MRI of entire spine with and without contrast, including cervical and thoracic regions, to assess full extent of involvement 1, 3
- Thin axial cuts through regions of suspected abnormality are essential 2, 3
Laboratory Studies
- Lumbar puncture for CSF analysis: cell count, protein, glucose, oligoclonal bands, IgG index, viral PCRs, and onconeural antibodies 1, 2, 3
- Blood tests: aquaporin-4 IgG antibodies (for neuromyelitis optica spectrum disorder), MOG-IgG antibodies, ANA, anti-Ro/La, antiphospholipid antibodies, vitamin B12, HIV, RPR, TSH 2, 3
Special Population Considerations
Neuromyelitis Optica Spectrum Disorder (NMOSD)
- For NMOSD-associated transverse myelitis (positive aquaporin-4 IgG), more aggressive immunosuppression is required with earlier consideration of rituximab or other long-term immunosuppressive agents 2, 3
Antiphospholipid Antibody Syndrome
- Add anticoagulation therapy in addition to immunosuppressive treatment for transverse myelitis associated with antiphospholipid antibodies 2
Infectious Etiology
- For infectious causes (such as schistosomiasis), administer appropriate antimicrobial treatment alongside corticosteroids 2
Systemic Lupus Erythematosus
- Pulse methylprednisolone followed by pulse cyclophosphamide for approximately 6 months has demonstrated superior outcomes compared to oral prednisone alone 6
Management of Dysautonomia and Complications
- Avoid medications that worsen autonomic dysfunction: beta-blockers, IV magnesium, and certain antibiotics 1
- Monitor orthostatic vital signs regularly to assess severity of autonomic involvement 1
- Evaluate and manage urinary retention with intermittent catheterization if needed 1, 2
- Address constipation, which is a common autonomic manifestation 1, 2
- Perform frequent neurologic examinations and pulmonary function monitoring to assess for respiratory compromise 1
Critical Pitfalls to Avoid
- Do not use corticosteroids alone in severe presentations with dysautonomia or extensive spinal involvement - combination therapy with IVIG is indicated 1
- Do not delay treatment initiation beyond 2 weeks, as this is associated with poor prognosis 2, 3
- Do not overlook concurrent autoimmune conditions such as myasthenia gravis or myositis, particularly in immune checkpoint inhibitor-related cases, which require specific additional management 1
- Do not abruptly discontinue corticosteroids - gradual tapering is necessary to prevent adrenal insufficiency 4
Prognostic Factors Indicating Poor Outcomes
- Extensive spinal cord MRI lesions, particularly longitudinally extensive transverse myelitis (≥3 vertebral segments) 2, 3
- Severe muscle weakness or sphincter dysfunction at presentation 1, 2, 3
- Delay in treatment initiation beyond 2 weeks 2, 3
Monitoring During Corticosteroid Therapy
- Monitor for hyperglycemia and adjust antidiabetic medications as needed 4
- Watch for signs of infection, as corticosteroids increase infection risk 4
- If steroid therapy continues beyond 6 weeks, monitor intraocular pressure 4
- Monitor for hypokalemia, particularly if using concomitant potassium-depleting agents 4