What is the management of transverse myelitis?

Management of Transverse Myelitis

High-dose intravenous methylprednisolone (1g/day for 3-5 days) should be administered as soon as possible after diagnosis of transverse myelitis, ideally within the first few hours, to prevent severe neurological deficits. 1

Diagnosis and Clinical Presentation

Transverse myelitis typically presents with:

• Motor weakness
• Sensory symptoms with a defined sensory level
• Bowel and bladder dysfunction
• Autonomic dysfunction

Acute Treatment Algorithm

First-Line Treatment

• Immediate immunosuppression:
  • IV methylprednisolone 1g/day for 3-5 days 1
  • Consider higher doses in severe cases 1
  • Delay in therapy initiation (>2 weeks) is associated with severe neurological deficits 1

Second-Line Options (if inadequate response to steroids)

• Plasma exchange: 5-10 sessions every other day 1
• Intravenous immunoglobulin (IVIG): 2g/kg divided over 5 days 1

Third-Line Options

• Cyclophosphamide: Particularly effective for SLE-associated myelitis 1
• Rituximab: Consider for antibody-mediated autoimmunity 1

Etiology-Specific Management

Immune Checkpoint Inhibitor-Related Myelitis

• Permanently discontinue the immunotherapy agent 1
• Use high-dose corticosteroids as first-line treatment 1
• Taper corticosteroids slowly over 6+ weeks to prevent relapses 1

Infectious Causes

• Rule out infectious causes before starting immunosuppression 1
• For parasitic infections:
  • Schistosomiasis: Praziquantel 40mg/kg twice daily for 5 days plus dexamethasone 1
  • Toxocariasis: Albendazole plus corticosteroids for 3-4 weeks 1
  • Neurocysticercosis: Dexamethasone starting one day before praziquantel 1

Autoimmune-Associated Myelitis

• For antiphospholipid antibody-positive patients: Consider anticoagulation therapy 1
• For SLE-associated myelitis: Cyclophosphamide may be particularly effective 1

Maintenance Therapy

• Crucial to prevent relapses, which occur in 50-60% of patients during corticosteroid dose reduction 1
• Options include:
  • Cyclophosphamide
  • Rituximab
  • Other immunosuppressants based on underlying etiology

Symptomatic Management

• Neuropathic pain: Pregabalin, gabapentin, or duloxetine 1
• Spasticity: Baclofen, tizanidine, or physical therapy 1
• Autonomic dysfunction: Monitor for cardiac arrhythmias, blood pressure fluctuations, and urinary retention 1
• Respiratory failure: Consider early intubation if vital capacity falls below 15 ml/kg or negative inspiratory force is less than -20 cm H₂O 1

Prognostic Factors

Favorable Prognostic Factors

• Early treatment initiation
• Prompt neurological response
• Absence of extensive MRI lesions 1

Poor Prognostic Factors

• Extensive lesions on spinal MRI
• Reduced muscle strength or sphincter dysfunction at onset
• Positive antiphospholipid antibodies
• Delayed initiation of treatment (>2 weeks) 1

Special Considerations

• Pregnant patients: IVIG may be preferred over steroids 1
• Early pilot studies have shown that high-dose methylprednisolone significantly reduced time to independent walking (23 vs 97 days) and increased full recovery rates (80% vs 10%) 2

Clinical Pitfalls to Avoid

1. Delaying treatment: Do not wait for complete diagnostic workup before initiating treatment if transverse myelitis is strongly suspected
2. Inadequate dosing: Ensure full high-dose steroid regimen is completed
3. Premature steroid tapering: Taper slowly over 6+ weeks to prevent relapses
4. Missing underlying causes: Complete thorough workup for systemic autoimmune disorders, demyelinating diseases, infections, and malignancies
5. Neglecting symptomatic management: Address pain, spasticity, and autonomic dysfunction proactively