What are the causes and treatment options for transverse myelitis?

Causes and Treatment of Transverse Myelitis

High-dose intravenous methylprednisolone (1g/day for 3-5 days) should be initiated as soon as possible after diagnosis of transverse myelitis, ideally within the first few hours, as delay in therapy beyond 2 weeks is associated with severe neurological deficits. 1

Causes of Transverse Myelitis

Transverse myelitis can result from various etiologies, including:

Immune-Mediated Causes

• Autoimmune disorders:
  • Systemic lupus erythematosus (SLE) 2
  • Sjögren's syndrome 2
  • Mixed connective tissue disease 3
  • Antiphospholipid antibody syndrome 1
• Immune checkpoint inhibitor therapy 1, 4
• Post-infectious immune response

Infectious Causes

• Viral infections:
  • Cytomegalovirus 5
• Parasitic infections:
  • Schistosomiasis (S. mansoni, S. haematobium, S. japonicum) 4
  • Toxocariasis (T. canis, T. catis) 4
  • Neurocysticercosis (Taenia solium) 4
  • Baylisascaris procyonsis 4
  • Paragonimiasis 4

Idiopathic

• A significant proportion of cases remain idiopathic despite thorough evaluation 6

Treatment Approach

Acute Management

1. First-line therapy:

   • High-dose intravenous methylprednisolone (1g/day for 3-5 days) 1
   • Important: Rule out infectious causes before starting immunosuppression 1
   • For severe cases, consider higher doses 1

2. Additional therapies (if inadequate response to steroids):

   • Intravenous immunoglobulin (IVIG) at 2g/kg divided over 5 days 1
   • Plasma exchange: 5-10 sessions every other day 1
   • Combination therapy may be considered from the beginning in severe cases 1

3. Specific etiologic considerations:

   • For SLE-associated transverse myelitis: IV methylprednisolone combined with IV cyclophosphamide 1, 2
   • For antiphospholipid antibody-positive patients: Consider anticoagulation therapy 1
   • For immune checkpoint inhibitor-related myelitis: Permanently discontinue the immunotherapy agent 4, 1
   • For infectious causes:
     • Schistosomiasis: Praziquantel 40 mg/kg twice daily for 5 days plus dexamethasone 4 mg four times daily, reducing after 7 days, over 2-6 weeks 4
     • Toxocariasis: Albendazole plus corticosteroids 4
     • Neurocysticercosis: Dexamethasone starting one day before praziquantel 4

Maintenance Therapy

• Essential to prevent relapses, which occur in 50-60% of patients during corticosteroid dose reduction 1
• Options include:
  • Cyclophosphamide (especially for SLE-associated myelitis) 1, 2
  • Rituximab for antibody-mediated autoimmunity 1
  • Azathioprine has been reported effective in some cases 3

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 1

Prognostic Factors

• Better prognosis associated with:

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

• Poorer prognosis associated with:

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

Important Considerations

• Early aggressive treatment significantly improves outcomes 1
• For pregnant patients, IVIG may be preferred over steroids 1
• In immunocompetent patients with CMV-associated transverse myelitis, high-dose corticosteroids have shown good outcomes 5
• Monitor for potential complications of high-dose corticosteroids, including blood glucose fluctuations and gastric irritation 4