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 with significant weakness or sensory changes, 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 applicable 1, 2

First-Line Treatment Protocol

• Administer IV methylprednisolone 1g daily for 3-5 days as the cornerstone of acute treatment 1, 2
• Add IVIG 2g/kg over 5 days for patients presenting with moderate to severe disease (significant motor weakness, sensory deficits, or sphincter dysfunction) 1, 2
• This combination approach is particularly important for longitudinally extensive transverse myelitis (LETM) affecting ≥3 vertebral segments 2

Second-Line Therapies for Refractory Cases

Plasma Exchange

• Initiate plasma exchange therapy if patients fail to respond adequately to corticosteroids and IVIG within 7-10 days 1, 2
• This is particularly effective in cases with severe, progressive neurologic decline 3

Rituximab

• Consider rituximab for patients with positive autoimmune encephalopathy antibodies or those with inadequate response to standard therapies 1, 2

Cyclophosphamide

• IV pulse cyclophosphamide may be beneficial, particularly in SLE-associated transverse myelitis, following acute methylprednisolone treatment 4, 5
• Historical data shows improved outcomes with aggressive cyclophosphamide therapy for a mean of 6 months in SLE-related cases 5

Etiology-Specific Considerations

Infectious Causes

• For VZV-associated transverse myelitis: Combine IV acyclovir with corticosteroids 6, 7
• For parasitic infections (e.g., schistosomiasis): Administer appropriate antiparasitic treatment alongside corticosteroids 1

Autoimmune-Associated Disease

• For antiphospholipid antibody-positive cases: Add anticoagulation therapy to immunosuppressive treatment 1
• For NMOSD-associated transverse myelitis (aquaporin-4 IgG positive): More aggressive immunosuppression is required 1, 2
• For SLE or Sjögren's syndrome: Early aggressive treatment with IV methylprednisolone pulses followed by cyclophosphamide improves prognosis 4

Maintenance Therapy

• Initiate maintenance immunosuppression (such as azathioprine) after acute treatment to prevent relapses, which occur in 50-60% of cases during corticosteroid dose reduction 1
• For autoimmune-associated cases, continued immunosuppression may be necessary long-term 1

Critical Diagnostic Workup (Parallel to Treatment Initiation)

Neuroimaging

• Obtain MRI of the spine with thin axial cuts showing T2-weighted hyperintense lesions 1, 2, 8
• Look for wedge-shaped lesions on axial views and cigar-shaped lesions on sagittal views 8

Laboratory Studies

• CSF analysis: Cell count, protein, glucose, oligoclonal bands, viral PCRs, onconeural antibodies 1, 2, 8
• Blood tests: Aquaporin-4 IgG, MOG-IgG, B12, HIV, RPR, ANA, Ro/La, TSH 1, 2, 8

Autonomic Assessment

• Evaluate for urinary retention and constipation, which are common autonomic manifestations requiring management 1, 2, 8

Prognostic Indicators and Treatment Urgency

Poor Prognostic Factors

• Extensive spinal cord MRI lesions, especially LETM affecting ≥3 vertebral segments 1, 2
• Reduced muscle strength or sphincter dysfunction at presentation 1, 2
• Delay >2 weeks in initiating therapy 1

Key Clinical Pearl

• The presence of increased deep tendon reflexes (spastic paralysis) distinguishes transverse myelitis from conditions causing flaccid paralysis like Guillain-Barré syndrome 8

Critical Pitfalls to Avoid

• Do not delay treatment waiting for complete diagnostic workup—initiate corticosteroids promptly once transverse myelitis is suspected 1, 2
• Do not use acyclovir alone for non-infectious transverse myelitis; it is not recommended except for confirmed viral etiologies like VZV 6, 7
• Do not undertreat moderate-to-severe cases with corticosteroids alone—add IVIG from the outset 1, 2
• For immune checkpoint inhibitor-related cases, permanently discontinue the offending agent, not just temporarily 2