What is the treatment for transverse myelitis?

Treatment for Transverse Myelitis

Immediately initiate high-dose intravenous methylprednisolone 1g daily for 3-5 days as first-line therapy, and for moderate to severe cases (significant weakness or sensory changes), combine this with IVIG 2g/kg over 5 days. 1, 2

Immediate Actions

• Discontinue any potential causative agents (such as immune checkpoint inhibitors, if applicable) before starting immunotherapy 1, 2
• Obtain urgent neurologic consultation to guide diagnosis and management 1, 3
• Initiate treatment promptly—delays beyond 2 weeks are associated with worse neurological outcomes 4, 1

First-Line Treatment Algorithm

Standard Cases

• Administer IV methylprednisolone 1g daily for 3-5 days as the cornerstone of acute therapy 1, 2, 5
• This regimen has demonstrated effectiveness when initiated within hours to days of symptom onset 4

Moderate to Severe Cases

• Combine corticosteroids with IVIG 2g/kg over 5 days for patients presenting with significant motor weakness, sensory deficits, or sphincter dysfunction 1, 2
• The combination of IV methylprednisolone and IV cyclophosphamide can be particularly effective in SLE-associated myelitis when used promptly within the first few hours 4, 5
• Neurological response typically occurs within a few days to 3 weeks, paralleled by MRI improvement 4

Second-Line Treatment for Refractory Cases

• Plasma exchange therapy should be considered for patients who fail to respond adequately to corticosteroids and IVIG within 7-10 days 1, 2
• Plasma exchange has been used successfully in severe cases and in antiphospholipid-positive myelopathy 4
• Rituximab may be beneficial in cases with positive autoimmune encephalopathy antibodies or inadequate response to other therapies 1, 2

Maintenance Immunosuppression

• After acute treatment, maintenance immunosuppressive therapy (such as azathioprine) is necessary to prevent relapses, which occur in 50-60% of cases during corticosteroid dose reduction 4, 1
• Chronic immunosuppressive therapy is particularly important given the high relapse rate 4

Special Clinical Scenarios

Antiphospholipid Antibody-Positive Cases

• Add anticoagulation therapy to immunosuppressive treatment in patients with antiphospholipid antibodies who present with myelopathy 4, 1
• Anticoagulation may be considered in antiphospholipid-positive patients not responding to immunosuppressive therapy alone 4

Neuromyelitis Optica Spectrum Disorder (NMOSD)

• More aggressive immunosuppression is required for aquaporin-4 IgG-positive transverse myelitis 1, 2
• Longitudinally extensive transverse myelitis (≥3 vertebral segments) should raise suspicion for NMOSD and prompt testing for aquaporin-4 IgG antibodies 1, 3

Infectious Etiology

• For viral causes (such as VZV), combine antiviral therapy (IV acyclovir) with corticosteroids 6
• For parasitic causes (such as schistosomiasis), administer appropriate antiparasitic treatment along with corticosteroids 1

Prognostic Factors Associated with Poor Outcomes

• Extensive spinal cord MRI lesions (especially longitudinally extensive transverse myelitis affecting ≥3 vertebral segments) 1, 2
• Reduced muscle strength or sphincter dysfunction at presentation 4, 1, 2
• Delay in therapy initiation beyond 2 weeks 4, 1, 2
• Presence of antiphospholipid antibodies 4

Critical Diagnostic Workup

Neuroimaging

• MRI of the spine with thin axial cuts showing T2-weighted hyperintense lesions is essential 1, 2, 3
• Brain MRI should be performed when other neuropsychiatric symptoms co-exist or to evaluate for demyelinating disorders 4

Laboratory Studies

• Lumbar puncture: check cell count, protein, glucose, oligoclonal bands, viral PCRs, and onconeural antibodies 1, 2, 3
• Blood tests: aquaporin-4 IgG, MOG-IgG, B12, HIV, RPR, ANA, Ro/La, TSH to identify underlying causes 1, 2, 3

Autonomic Assessment

• Evaluate for urinary retention and constipation, which are common autonomic manifestations requiring management 1, 2, 3

Common Pitfalls to Avoid

• Do not delay treatment while awaiting complete diagnostic workup—initiate corticosteroids early if infection has been ruled out 4, 1
• Do not use oral prednisone alone—historical data shows inadequate control with medium to high doses of oral prednisone, leading to permanent disability 5
• Do not forget maintenance therapy—the 50-60% relapse rate during corticosteroid taper necessitates long-term immunosuppression 4, 1
• Recognize upper motor neuron signs (spastic paralysis with increased deep tendon reflexes) to distinguish transverse myelitis from conditions like Guillain-Barré syndrome, which presents with flaccid paralysis 3