What is the management of transverse myelitis?

Management of Transverse Myelitis

Initiate high-dose intravenous methylprednisolone 1 gram daily for 3-5 days immediately upon diagnosis, and strongly consider adding IVIG 2g/kg over 5 days for moderate to severe cases. 1, 2

Immediate Diagnostic Workup

Before initiating treatment, rapidly complete the following essential evaluations:

• Obtain urgent neurology consultation to guide diagnosis and management 1, 3
• MRI of spine with thin axial cuts through suspected abnormality areas (looking for T2-weighted hyperintense lesions) 1, 2, 3
• MRI of brain with and without contrast to evaluate for demyelinating lesions and rule out multifocal disease 4, 3
• Lumbar puncture for cell count, protein, glucose, oligoclonal bands, viral PCRs, cytology, and onconeural antibodies 4, 1
• Blood tests: B12, HIV, RPR, ANA, Ro/La, TSH, and aquaporin-4 IgG (critical for NMOSD diagnosis) 4, 1, 2
• Assess for urinary retention and constipation, which are common autonomic manifestations 4, 1, 2

First-Line Treatment Algorithm

Standard Acute Treatment

Administer intravenous methylprednisolone 1 gram daily for 3-5 days as the cornerstone of acute therapy 1, 2, 5. This should be initiated within hours of diagnosis, as delays beyond 2 weeks are associated with worse neurological outcomes 4, 1.

For patients with moderate to severe disease (significant weakness, sensory changes, or sphincter dysfunction):

• Combine methylprednisolone with IVIG 2g/kg over 5 days 1, 2
• The combination of intravenous methylprednisolone and intravenous cyclophosphamide can be effective if used promptly within the first few hours 4

Context-Specific Modifications

For SLE-associated myelitis: Use combination therapy with intravenous methylprednisolone PLUS intravenous cyclophosphamide from the outset, as neurological response parallels MRI improvement within days to 3 weeks 4, 6, 7

For antiphospholipid antibody-positive patients: Add anticoagulation therapy in addition to immunosuppressive treatment, particularly if there is inadequate response to immunosuppression alone 4, 1, 6

For immune checkpoint inhibitor-related cases: Permanently discontinue the causative agent and administer methylprednisolone 2 mg/kg (strongly consider higher doses of 1g daily for 3-5 days) 4, 2

Second-Line Treatment for Refractory Cases

If patients fail to respond adequately to corticosteroids and IVIG within 7-10 days:

• Plasma exchange therapy should be initiated 4, 1, 2
• Rituximab may be beneficial in cases with positive autoimmune encephalopathy antibodies or inadequate response to other therapies 4, 1, 2

Maintenance Immunosuppression

Critical pitfall: Relapses occur in 50-60% of cases during corticosteroid dose reduction 4, 1. Therefore:

• Initiate maintenance immunosuppressive therapy (such as azathioprine) after acute treatment to prevent relapses 1
• For NMOSD-associated transverse myelitis (aquaporin-4 IgG positive), more aggressive long-term immunosuppression is required 1, 2

Special Populations and Etiologies

Longitudinally Extensive Transverse Myelitis (LETM) affecting ≥3 vertebral segments:

• Requires the same aggressive approach with high-dose methylprednisolone 1g daily for 3-5 days combined with IVIG 2
• These patients have worse prognosis and require particularly prompt treatment 1, 2
• Consider NMOSD or MOGAD as underlying etiologies in young adults rather than multiple sclerosis 3

Infectious etiologies: Administer appropriate antimicrobial/antiparasitic treatment alongside corticosteroids 4, 1

Prognostic Factors Requiring Aggressive Management

Factors associated with severe neurological deficit and poor outcomes include:

• Extensive spinal cord MRI lesions (especially LETM ≥3 vertebral segments) 1, 2
• Reduced muscle strength or sphincter dysfunction at presentation 4, 1, 2
• Antiphospholipid antibodies 4, 6
• Delay >2 weeks in initiating therapy 4, 1

When these factors are present, use combination therapy from the outset rather than sequential monotherapy 4, 6.

Common Pitfalls to Avoid

• Do not delay treatment while awaiting complete diagnostic workup—begin methylprednisolone once infection is reasonably excluded 4
• Do not taper corticosteroids without maintenance immunosuppression in place, as relapse rates are 50-60% 4, 1
• Do not miss aquaporin-4 IgG testing, as NMOSD requires different long-term management 1, 2, 3
• Do not assume multiple sclerosis in young adults with LETM—consider MOGAD or NMOSD first 3