What is the therapy for transverse myelitis?

Therapy for 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 of symptom onset. 1

First-Line Treatment

Acute Phase Management

1. High-dose corticosteroids

   • Methylprednisolone 1g/day IV for 3-5 days 1
   • Begin immediately after diagnosis, as delay in therapy initiation (>2 weeks) is associated with severe neurological deficits 1
   • Consider higher doses in severe cases 1

2. Rule out infectious causes before starting immunosuppression 1

   • Perform appropriate diagnostic workup including CSF analysis, MRI, and serology

3. For patients who show inadequate response to corticosteroids:

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

Second-Line Treatments

For patients with inadequate response to first-line therapy or those with specific underlying conditions:

1. Cyclophosphamide

   • Particularly effective for SLE-associated myelitis 1, 2
   • Early aggressive treatment with cyclophosphamide might improve prognosis in autoimmune-associated transverse myelitis 2

2. Rituximab

   • Consider for antibody-mediated autoimmunity 1

3. For immune checkpoint inhibitor-related myelitis

   • Permanently discontinue the immunotherapy agent 1, 3
   • Use high-dose corticosteroids as first-line treatment 3

Maintenance Therapy

Maintenance immunosuppressive therapy is crucial to prevent relapses, which occur in 50-60% of patients during corticosteroid dose reduction 1:

1. Tapering of corticosteroids

   • Slow and careful tapering over 6 weeks or more 3
   • Monitor closely for relapse during tapering

2. Long-term immunosuppression options

   • Cyclophosphamide for cell-mediated autoimmunity 1
   • Rituximab for antibody-mediated autoimmunity 1
   • For antiphospholipid antibody-positive patients, consider anticoagulation therapy 1

Symptomatic Management

1. Neuropathic pain

   • Pregabalin, gabapentin, or duloxetine 1

2. Spasticity

   • Baclofen, tizanidine, or physical therapy 1

3. Autonomic dysfunction

   • Monitor for cardiac arrhythmias, blood pressure fluctuations, and urinary retention 1

4. Respiratory failure

   • Consider early intubation if vital capacity falls below 15 ml/kg or negative inspiratory force is less than -20 cm H₂O 1

Special Considerations

1. Pregnancy

   • IVIG may be preferred over steroids in pregnant patients 1

2. Infectious causes of transverse myelitis

   • Parasitic infections (e.g., 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

3. Cytomegalovirus-associated transverse myelitis

   • High-dose corticosteroids have shown good outcomes in immunocompetent patients 4

Prognostic Factors

Factors associated with better prognosis include:

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

Factors associated with poorer prognosis include:

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

Early aggressive treatment is critical as it significantly improves outcomes. A study in children showed that high-dose methylprednisolone reduced median time to walk independently (23 vs 97 days) and increased the proportion of patients with full recovery within 12 months (80% vs 10%) 5.