What is the treatment for transverse myelitis?

Treatment of Transverse Myelitis

Immediate First-Line Treatment

Initiate high-dose intravenous methylprednisolone 1 gram daily for 3-5 days immediately upon diagnosis, combined with IVIG 2 g/kg divided over 5 days (0.4 g/kg/day) for moderate to severe presentations. 1, 2

Corticosteroid Therapy

• Administer IV methylprednisolone 1 gram daily for 3-5 days as pulse dosing for all cases of transverse myelitis 3, 1, 2
• This represents the cornerstone of acute treatment and should not be delayed while awaiting diagnostic test results 2
• Early aggressive treatment with high-dose methylprednisolone significantly reduces time to independent walking (23 vs 97 days) and increases full recovery rates (80% vs 10%) compared to no treatment 4

IVIG Combination Therapy

• Add IVIG 2 g/kg over 5 days to corticosteroids for moderate to severe cases, particularly those with significant weakness, sensory changes, or dysautonomia 1, 2
• The combination approach is specifically indicated when severe dysautonomia (blood pressure instability, temperature dysregulation, cardiac arrhythmias) is present 1
• IVIG is more readily accessible and less costly than plasma exchange, making it a practical second agent 5

Discontinue Causative Agents

• Permanently discontinue immune checkpoint inhibitors or other potential causative medications immediately upon presentation 3, 1, 2
• This applies to any medication that could trigger immune-mediated myelitis 3

Second-Line Treatment for Refractory Cases

Plasma Exchange (PLEX)

• Consider plasma exchange (5-10 sessions every other day) if no clinical improvement occurs within 7-10 days of combined corticosteroid and IVIG therapy 1, 6
• Initiate PLEX earlier (within 3 days) if symptoms worsen or progress despite initial therapy 1
• PLEX is technically challenging and costly but can be beneficial when first-line therapies fail 5, 7

Rituximab

• Consider rituximab for cases with positive autoimmune encephalopathy antibodies or inadequate response to corticosteroids, IVIG, and PLEX 2, 6

Essential Diagnostic Work-Up (Concurrent with Treatment)

Neuroimaging

• Obtain MRI of the entire spine with and without contrast using thin axial cuts through the region of suspected abnormality to confirm T2-weighted hyperintense lesions 3, 1, 2
• Include cervical and thoracic spine imaging to assess full extent of involvement 1
• Longitudinally extensive transverse myelitis (LETM) is defined by lesions extending ≥3 vertebral segments 6

Lumbar Puncture

• Perform CSF analysis including cell count, protein, glucose, oligoclonal bands, IgG index, viral PCRs, cytology, and onconeural antibodies 3, 1, 2
• CSF analysis helps rule out leptomeningeal metastasis and infectious etiologies 3

Blood Tests

• Check B12, HIV, rapid plasma reagin, ANA, Ro/La, TSH, and aquaporin-4 immunoglobulin G 3, 2
• Test for MOG-IgG antibodies and antiphospholipid antibodies, as these guide specific treatment approaches 2, 6, 8

Special Considerations by Etiology

Neuromyelitis Optica Spectrum Disorder (NMOSD)

• For aquaporin-4 IgG-positive cases, more aggressive immunosuppression is required beyond standard treatment 2, 6
• These patients need long-term maintenance immunosuppression to prevent relapses 2

Antiphospholipid Antibody-Associated TM

• Add anticoagulation therapy to immunosuppressive treatment when antiphospholipid antibodies are present 2, 8

Infectious Etiology

• For schistosomiasis-related transverse myelitis, administer praziquantel 40 mg/kg twice daily for 5 days plus dexamethasone 4 mg four times daily, reducing after 7 days over 2-6 weeks total 3
• Treat with corticosteroids alone initially in acute neuroschistosomiasis (Katayama syndrome) to avoid neurological complications 3

Autoimmune Disease-Associated TM

• For systemic lupus erythematosus or Sjögren's syndrome-associated TM, consider IV pulses of methylprednisolone and cyclophosphamide 8

Maintenance Therapy

• After acute treatment, initiate maintenance immunosuppressive therapy (such as azathioprine) to prevent relapses, which occur in 50-60% of cases during corticosteroid dose reduction 2
• Continued immunosuppression may be necessary long-term for autoimmune-associated transverse myelitis 2

Critical Monitoring Requirements

Neurologic Monitoring

• Perform frequent neurologic examinations and pulmonary function monitoring to assess for respiratory compromise 1
• Monitor closely for autonomic dysfunction manifestations including blood pressure instability, temperature dysregulation, and cardiac arrhythmias 1
• Evaluate and manage urinary retention and constipation, which are common autonomic complications 3, 1, 2
• Monitor orthostatic vital signs regularly to assess severity of autonomic involvement 1

Medications to Avoid

• Avoid medications that can worsen autonomic dysfunction, including beta-blockers, IV magnesium, and certain antibiotics 1

Prognostic Factors Indicating Poor Outcomes

• Extensive spinal cord lesions on MRI, particularly LETM affecting ≥3 vertebral segments 1, 2, 6
• Severe muscle weakness or sphincter dysfunction at presentation 1, 2, 6
• Delayed treatment initiation (>2 weeks from symptom onset) 2
• Reduced deep tendon reflexes suggest a different diagnosis like Guillain-Barré syndrome rather than transverse myelitis 6

Critical Pitfalls to Avoid

• Do not delay treatment while awaiting antibody results - initiate immunotherapy based on clinical and MRI findings alone 2
• Do not use corticosteroids alone in severe presentations with dysautonomia - combination therapy with IVIG is indicated 1, 2
• Do not overlook concurrent myasthenia gravis or myositis, which can occur with immune checkpoint inhibitor-related cases and requires specific management 1
• Do not wait for neurologic consultation to begin treatment - start methylprednisolone immediately while arranging consultation 3, 2