What is the clinical examination and treatment approach for transverse myelitis in children?

Clinical Examination and Treatment Approach for Transverse Myelitis in Children

The clinical examination of pediatric transverse myelitis should include thorough neurological assessment focusing on motor weakness, sensory changes, and autonomic dysfunction, followed by prompt MRI of the brain and complete spine with and without contrast as the primary diagnostic imaging modality.

Clinical Presentation and Examination

Motor Assessment

• Evaluate for acute or subacute onset of bilateral weakness, which is a hallmark feature of transverse myelitis 1, 2
• Assess deep tendon reflexes, which are often increased in transverse myelitis 1, 3
• Document distribution and pattern of weakness (symmetrical vs asymmetrical) 2, 4

Sensory Assessment

• Determine presence of a sensory level, though this may not be clearly evident in all pediatric patients 2
• Evaluate for paresthesias, numbness, or pain below the level of the lesion 3, 4
• Test for proprioception and vibration sense abnormalities 4, 5

Autonomic Function Assessment

• Assess for bowel and bladder dysfunction, which is common in transverse myelitis 1, 6
• Evaluate for temperature dysregulation or episodes of hypertension 5
• Document any respiratory compromise, especially with high cervical lesions 3

Diagnostic Workup

Neuroimaging

• MRI of the complete spine with and without contrast is the gold standard imaging modality 1
• Look for T2 hyperintense signal in the spinal cord, often centrally located involving gray and neighboring white matter 2
• Longitudinally extensive lesions (spanning ≥3 vertebral segments) are common in pediatric transverse myelitis 2, 4
• MRI of the brain should also be performed to evaluate for additional demyelinating lesions that may suggest multiple sclerosis or neuromyelitis optica spectrum disorders 1, 2

Cerebrospinal Fluid Analysis

• Perform lumbar puncture to analyze:
  • Cell count (often shows pleocytosis with lymphocytic predominance) 1, 5
  • Protein (typically elevated) 1, 5
  • Glucose levels 1
  • Oligoclonal bands (may indicate MS if present) 1, 2
  • PCR for viral pathogens 1, 3
  • Cytology to rule out malignancy 1

Serological Testing

• Test for:
  • Aquaporin-4 IgG antibodies (for neuromyelitis optica) 1, 2
  • Myelin oligodendrocyte glycoprotein (MOG) antibodies 2
  • B12 levels, HIV, syphilis serology 1
  • Autoimmune markers (ANA, Ro/La antibodies) 1
  • Thyroid function tests 1

Differential Diagnosis

• Rule out compressive myelopathy (which requires urgent surgical intervention) 5
• Consider other demyelinating disorders:
  • Multiple sclerosis 1, 2
  • Neuromyelitis optica spectrum disorders 1, 2
  • Acute disseminated encephalomyelitis 1, 3
• Evaluate for infectious causes of myelitis 3, 4
• Consider paraneoplastic syndromes 1, 5

Treatment Approach

Acute Management

• First-line treatment is high-dose intravenous methylprednisolone (typically 30 mg/kg/day for 3-5 days, maximum 1g/day) 1, 6
• For patients with severe symptoms or inadequate response to steroids, consider:
  • Plasma exchange (5-7 exchanges over 10-14 days) 6, 3
  • Intravenous immunoglobulin (2 g/kg divided over 2-5 days) 1, 6
• In cases with suspected autoimmune etiology and limited improvement, consider:
  • Rituximab or cyclophosphamide in consultation with neurology 1, 6

Supportive Care

• Monitor closely for respiratory compromise, especially with cervical lesions 3
• Manage urinary retention with catheterization as needed 1, 3
• Address constipation with appropriate bowel regimen 1
• Provide appropriate pain management for neuropathic pain 3, 5

Rehabilitation

• Initiate early physical therapy to prevent contractures and maintain muscle strength 3
• Occupational therapy for activities of daily living 3
• Speech therapy if bulbar muscles are affected 4

Prognosis and Follow-up

• Children generally have better outcomes than adults, with approximately 50% making complete recovery by 2 years 2
• Risk factors for poorer outcomes include:
  • Severe initial presentation 2, 3
  • Longer time to treatment initiation 6, 3
  • Presence of spinal shock 3
• Regular follow-up is essential to:
  • Monitor for recurrence 2, 4
  • Assess for development of other demyelinating disorders (approximately 20% of children with transverse myelitis may later develop MS or neuromyelitis optica) 2, 3
  • Address long-term complications such as urinary, motor, or sensory dysfunction 3

Important Caveats

• Delay in diagnosis and treatment can significantly worsen outcomes; prompt recognition and intervention are critical 6, 5
• Distinguishing between idiopathic transverse myelitis and the first presentation of a relapsing demyelinating disorder is crucial for long-term management 2, 4
• Asymptomatic brain lesions on MRI in a child with transverse myelitis may predict future development of MS or neuromyelitis optica 2
• There are currently no robust controlled trials specifically in children to guide optimal treatment, highlighting the need for standardized assessment and investigation protocols 2