What is the urgent management of transverse myelitis?

Urgent Management 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 severe presentations with significant weakness, sensory changes, or dysautonomia, combine this with IVIG 2 g/kg divided over 5 days. 1, 2

Immediate Actions

Discontinue Causative Agents

• Permanently stop any immune checkpoint inhibitors or other potentially causative medications immediately upon presentation 3, 2
• This is critical as these agents can trigger immune-mediated myelitis and continuing them worsens outcomes 3

Obtain Urgent Neurology Consultation

• Consult neurology immediately to guide diagnosis and management 3, 1
• Early specialist involvement is essential for optimizing treatment decisions 3

First-Line Immunotherapy Protocol

Standard Presentation

• Administer IV methylprednisolone 1 gram daily for 3-5 days as pulse dosing 3, 1, 2
• This represents the cornerstone of acute treatment across all major guidelines 1

Severe Presentation (Significant Weakness, Sensory Changes, or Dysautonomia)

• Combine IV methylprednisolone 1 gram daily for 3-5 days with IVIG 2 g/kg divided over 5 days (0.4 g/kg/day) 1, 2
• The combination approach is specifically indicated when severe dysautonomia, extensive weakness, or longitudinally extensive transverse myelitis (≥3 vertebral segments) is present 2
• This aggressive dual therapy improves outcomes in severe cases compared to corticosteroids alone 2

Critical Diagnostic Work-Up (Performed Concurrently)

Neuroimaging

• Obtain MRI of the entire spine with and without contrast using thin axial cuts through the region of suspected abnormality to detect T2-weighted hyperintense lesions 1, 4
• Include cervical and thoracic spine imaging to assess full extent of involvement 2
• MRI of brain with and without contrast should be obtained to evaluate for demyelinating lesions and rule out multiple sclerosis 4

Lumbar Puncture

• Perform lumbar puncture for CSF analysis including cell count, protein, glucose, oligoclonal bands, IgG index, viral PCRs (HSV, VZV, enterovirus), and onconeural antibodies 1, 2
• Check opening pressure during the procedure 3

Serological Testing

• Draw blood for B12, HIV, RPR, ANA, anti-Ro/La, TSH, and aquaporin-4 IgG to rule out other causes 1, 4
• For suspected MOG-associated disease, obtain serum MOG antibodies using cell-based assays (IFT/FACS) as the gold standard 4
• Check antiphospholipid antibodies, as their presence may require anticoagulation therapy in addition to immunosuppression 1, 5

Monitoring Requirements

Neurologic Surveillance

• Perform frequent neurologic examinations to assess for progression 2
• Monitor pulmonary function closely to detect respiratory compromise early 2
• Evaluate for urinary retention and constipation, which are common autonomic manifestations requiring management 1, 2

Autonomic Monitoring

• Monitor blood pressure for instability, temperature dysregulation, and cardiac arrhythmias 2
• Check orthostatic vital signs regularly to assess severity of autonomic involvement 2
• Avoid medications that worsen autonomic dysfunction including beta-blockers, IV magnesium, and certain antibiotics 2

Escalation Therapy for Refractory Cases

Second-Line Treatment

• 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, 2
• Initiate PLEX earlier (within 3 days) if symptoms worsen or progress despite initial therapy 2
• Plasma exchange is the established second-line therapy for steroid-refractory cases 1

Third-Line Options

• Rituximab may be beneficial in cases with positive autoimmune encephalopathy antibodies or inadequate response to corticosteroids, IVIG, and plasma exchange 1
• This is particularly relevant for antibody-mediated cases 1

Special Considerations by Etiology

Neuromyelitis Optica Spectrum Disorder (NMOSD)

• If aquaporin-4 IgG is positive or longitudinally extensive transverse myelitis (≥3 vertebral segments) is present, more aggressive immunosuppression is required 1, 4
• These patients require long-term maintenance immunosuppression to prevent relapses 1

Antiphospholipid Antibody-Associated

• Add anticoagulation therapy in addition to immunosuppressive treatment if antiphospholipid antibodies are present 1, 5
• This dual approach addresses both the inflammatory and thrombotic components 5

Infectious Etiology

• If infectious cause is identified (such as schistosomiasis), administer appropriate antiparasitic or antimicrobial treatment along with corticosteroids 1
• Consider empirical IV acyclovir until viral PCR results are negative 3

Maintenance Therapy

Long-Term Immunosuppression

• After acute treatment, initiate maintenance immunosuppressive therapy (such as azathioprine) to prevent relapses, which occur in 50-60% of cases during corticosteroid dose reduction 1
• Taper corticosteroids very slowly over 6 weeks or more after recovery 3
• Relapses during steroid tapering are common and require careful monitoring 3

Critical Pitfalls to Avoid

Treatment Delays

• Do not delay treatment while awaiting antibody results—initiate immunotherapy based on clinical and MRI findings 2
• Delay in treatment initiation (>2 weeks) is associated with poor prognosis 1
• Early aggressive treatment improves outcomes even before final etiologic diagnosis 6, 7

Inadequate Initial Therapy

• Do not use corticosteroids alone in severe presentations with dysautonomia or extensive spinal cord involvement—combination therapy with IVIG is indicated 2
• Monotherapy is insufficient for severe cases 2

Missed Concurrent Conditions

• Do not overlook concurrent myasthenia gravis or myositis, which can occur with immune checkpoint inhibitor-related cases and requires specific management 2
• Screen for these conditions in checkpoint inhibitor-associated cases 3

Misdiagnosis

• Rule out compressive myelopathy first, as it is clinically indistinguishable from transverse myelitis but requires emergent surgical intervention 7
• Hyperreflexia distinguishes transverse myelitis from Guillain-Barré syndrome, which presents with areflexia 4

Prognostic Factors

Poor Prognosis Indicators

• Extensive spinal cord MRI lesions, especially longitudinally extensive transverse myelitis affecting ≥3 vertebral segments 1, 2
• Severe muscle weakness or sphincter dysfunction at presentation 1, 2
• Delay (>2 weeks) in initiation of therapy 1
• Up to two-thirds of patients have moderate to severe residual disability despite treatment 7, 8