What is the treatment for transverse myelitis in children?

Treatment of Transverse Myelitis in Children

The first-line treatment for transverse myelitis in children is high-dose intravenous corticosteroids, typically methylprednisolone, followed by plasma exchange or intravenous immunoglobulin (IVIG) for patients who do not respond adequately to steroids. 1, 2

Initial Management

• Prompt recognition and early therapeutic intervention are critical to reduce the harmful effects of inflammation in transverse myelitis 2
• High-dose intravenous methylprednisolone (typically 30 mg/kg/day for 3-5 days, maximum 1 gram daily) should be initiated as soon as possible after diagnosis 3
• Before initiating treatment, it is essential to rule out spinal cord compression and other treatable conditions through:
  • Spinal MRI with and without contrast 4
  • Brain MRI with and without contrast (to evaluate for demyelinating lesions) 4
  • CSF examination 3
  • Electrophysiologic testing 3

Second-Line Therapies

• For patients who show inadequate response to corticosteroids within 24-48 hours:
  • Plasma exchange (PLEX) should be considered (typically 5-7 sessions) 1, 5
  • Intravenous immunoglobulin (IVIG) at 2 g/kg divided over 2-5 days is an alternative when PLEX is unavailable or contraindicated 1, 2

Special Considerations

• It is crucial to distinguish between inflammatory transverse myelitis and acute flaccid myelitis (AFM):

  • AFM is increasingly linked to enterovirus infections and may worsen with corticosteroid treatment 1
  • Distinguishing features include:
    • MRI appearance (central gray matter involvement in AFM)
    • Flaccid weakness pattern
    • Absence of sensory level in some cases of AFM 1, 6

• For refractory cases, consider:

  • Combination therapy with both PLEX and IVIG 2
  • Cyclophosphamide for severe, non-responsive cases 2

Monitoring and Follow-up

• Regular assessment of motor function, sensory symptoms, and bladder/bowel function 6
• MRI follow-up to evaluate resolution of inflammation 4
• Screening for underlying conditions that may present with transverse myelitis:
  • Multiple sclerosis (MS)
  • Neuromyelitis optica (NMO)
  • Anti-MOG antibody-associated disease 6

Prognosis

• Children generally have better outcomes than adults, with approximately 50% making complete recovery by 2 years 6
• Residual symptoms are common and may include:
  • Motor weakness
  • Sensory abnormalities
  • Bladder/bowel dysfunction 3

Pitfalls and Caveats

• Delay in treatment initiation can lead to worse outcomes; therapy should begin even before a final etiologic diagnosis is reached 2
• Asymptomatic brain MRI lesions are seen in more than one-third of pediatric transverse myelitis cases and may predict development of MS or NMO 6
• Longitudinally extensive transverse myelitis (lesions spanning ≥3 vertebral segments) is more common in children and may indicate NMO or MOG antibody-associated disease rather than MS 6
• COVID-19 has been reported as a potential trigger for transverse myelitis in children, requiring appropriate testing in suspicious cases 5