What is the management approach for transverse myelitis?

Management of Transverse Myelitis

Immediate Treatment Approach

Administer high-dose intravenous methylprednisolone 1g daily for 3-5 days as first-line therapy, and strongly consider adding IVIG 2g/kg over 5 days for moderate to severe cases. 1, 2

First-Line Therapy Algorithm

• Start methylprednisolone 1-2 mg/kg IV immediately (or pulse dose 1g daily for 3-5 days) while awaiting diagnostic confirmation, particularly if infection has been ruled out 3, 1, 2
• Add IVIG 2g/kg over 5 days for patients with significant weakness, sensory changes, or sphincter dysfunction at presentation 1, 2
• Combine methylprednisolone with cyclophosphamide IV in cases associated with systemic lupus erythematosus, as this combination is effective when initiated within the first few hours 3, 4
• Treatment must be initiated within 2 weeks of symptom onset—delays beyond this timeframe are associated with severe neurological deficits and poor outcomes 3, 1

Second-Line Therapy for Refractory Cases

• Initiate plasma exchange therapy if no adequate response occurs within 7-10 days of corticosteroids and IVIG 3, 1, 2
• Consider rituximab for patients with positive autoimmune encephalopathy or paraneoplastic antibodies who show limited improvement with standard therapy 3, 1, 2
• Plasma exchange has demonstrated good results in severe cases, particularly when combined with early corticosteroid therapy 3

Essential Diagnostic Workup

Before or concurrent with treatment initiation, obtain:

• Neurologic consultation immediately to guide diagnosis and management 3, 1, 5
• MRI of spine with thin axial cuts through the suspected region (with and without contrast) to identify T2-weighted hyperintense lesions 3, 1, 5
• MRI of brain to evaluate for demyelinating disorders and rule out other neuropsychiatric manifestations 3
• Lumbar puncture for cell count, protein, glucose, oligoclonal bands, viral PCRs, cytology, and onconeural antibodies 3, 1, 5
• Blood tests: aquaporin-4 IgG (for neuromyelitis optica spectrum disorder), MOG-IgG, B12, HIV, RPR, ANA, Ro/La, TSH 3, 1, 5, 2
• Assess for urinary retention and constipation, which are common autonomic manifestations requiring management 3, 1, 5

Critical Diagnostic Considerations

• Mild-to-moderate CSF abnormalities occur in 50-70% of cases but are non-specific 3
• Microbiological studies are essential to exclude infectious myelitis before committing to immunosuppression 3
• If CSF resembles bacterial or HSV meningitis, administer antimicrobial/antiviral therapy while awaiting MRI confirmation 3
• Longitudinally extensive transverse myelitis (≥3 vertebral segments) should prompt aquaporin-4 IgG testing for neuromyelitis optica spectrum disorder 1, 5, 2

Maintenance Immunosuppression

After acute treatment, initiate maintenance immunosuppressive therapy to prevent relapses, which occur in 50-60% of cases during corticosteroid dose reduction. 3, 1

• Taper corticosteroids slowly over 4-6 weeks after recovery 3
• Consider azathioprine, mycophenolate mofetil, or other immunosuppressants for long-term maintenance 3, 1
• Do not restart immune checkpoint inhibitors if they were the causative agent—permanent discontinuation is required 3, 1, 2

Special Clinical Scenarios

Antiphospholipid Antibody-Positive Myelopathy

• Add anticoagulation therapy in addition to immunosuppressive treatment for patients with positive antiphospholipid antibodies 3, 1
• This combination has shown good results, particularly in ischemic/thrombotic mechanisms 3

SLE-Associated Transverse Myelitis

• Use combination therapy with IV methylprednisolone plus IV cyclophosphamide if initiated promptly within the first few hours 3, 4
• Neurological response paralleled by MRI improvement typically occurs within days to 3 weeks 3
• Pulse cyclophosphamide should continue for approximately 6 months as maintenance 4

Immune Checkpoint Inhibitor-Related Myelitis

• Permanently discontinue the checkpoint inhibitor immediately 3, 1, 2
• Administer methylprednisolone 2 mg/kg, with strong consideration for higher doses of 1g daily for 3-5 days 3
• Strongly consider adding IVIG early in the treatment course 3

Neuromyelitis Optica Spectrum Disorder (NMOSD)

• More aggressive immunosuppression is required for aquaporin-4 IgG-positive cases 1, 2
• Consider rituximab earlier in the treatment algorithm for NMOSD-associated transverse myelitis 1, 2

Prognostic Factors and Pitfalls

Poor Prognostic Indicators

• Extensive spinal cord MRI lesions (particularly longitudinally extensive transverse myelitis affecting ≥3 vertebral segments) 3, 1, 2
• Reduced muscle strength or sphincter dysfunction at presentation 3, 1
• Delay >2 weeks in initiating therapy—this is the most modifiable risk factor 3, 1
• Presence of antiphospholipid antibodies 3

Common Pitfalls to Avoid

• Do not delay treatment while awaiting complete diagnostic workup if infection has been reasonably excluded 3, 1
• Do not use oral corticosteroids alone for moderate-to-severe cases—IV high-dose therapy is required 1, 2, 6
• Do not taper corticosteroids too rapidly—relapses are common during dose reduction, necessitating slow tapers over 4-6 weeks or longer 3
• Do not overlook the need for maintenance immunosuppression after acute treatment 3, 1

Key Clinical Pearl

• Transverse myelitis presents with spastic paralysis and increased deep tendon reflexes (upper motor neuron signs), distinguishing it from Guillain-Barré syndrome which causes flaccid paralysis with reduced/absent reflexes 5, 2