What is the treatment for acquired hypertrophic neuropathy associated with spinal cord infection?

Treatment of Acquired Hypertrophic Neuropathy Associated with Spinal Cord Infection

High-dose intravenous methylprednisolone (1000 mg daily for 3-5 days) should be initiated promptly within the first few hours after diagnosis, followed by immunosuppressive therapy with intravenous cyclophosphamide and maintenance immunosuppression to treat acquired hypertrophic neuropathy associated with spinal cord infection. 1

Diagnostic Approach

Imaging

• Contrast-enhanced spinal MRI is essential to:
  • Identify T2-weighted hyperintense lesions spanning multiple segments
  • Evaluate for involvement of more than three spinal cord segments (longitudinal myelopathy)
  • Rule out cord compression from hypertrophic nerve roots 1
  • Exclude other causes of myelopathy such as tumor or abscess 2

Laboratory Testing

• CSF analysis should include:
  • Cell count and protein (typically shows mild-to-moderate abnormalities in 50-70% of cases)
  • Glucose levels
  • Gram stain and culture to rule out bacterial infection
  • Viral PCR testing
  • Oligoclonal bands
  • NMO IgG (aquaporin) antibodies if longitudinal myelopathy is present 1, 2
• Serum studies should include:
  • Inflammatory markers (ESR, CRP)
  • Autoimmune panel (ANA, Ro/La, antiphospholipid antibodies)
  • Infectious workup 2

Treatment Protocol

Acute Phase Management

1. Immediate Immunosuppression:

   • High-dose IV methylprednisolone (1000 mg daily for 3-5 days) 1
   • Begin within the first few hours of diagnosis to maximize neurological recovery 2
   • Continue with oral prednisone taper over 4-6 weeks 2

2. Additional Immunotherapy (for severe or refractory cases):

   • Intravenous cyclophosphamide in combination with methylprednisolone 2
   • Plasma exchange therapy for severe cases not responding to initial treatment 1, 2
   • IVIG (2 g/kg over 5 days) may be considered as an alternative 2

3. Antimicrobial Therapy:

   • If infectious etiology is suspected, empiric antimicrobial coverage should be initiated while awaiting culture results 2
   • Continue appropriate antimicrobial therapy based on culture results and sensitivities 2

Surgical Intervention

• Surgical consultation should be obtained for all patients with:

  • Spinal instability
  • Progressive neurologic deficits
  • Spinal cord or nerve root compression
  • Significant sequestered paraspinal abscess 2, 1

• Surgical procedures may include:

  • Decompressive laminectomy to relieve pressure on the spinal cord 3, 4
  • Dural expansion with patch if significant dural thickening is present 5
  • Drainage of any associated abscess 2

Maintenance Therapy

• Long-term immunosuppression is typically required to prevent relapses:
  • Azathioprine or mycophenolate mofetil are common maintenance agents 1
  • Rituximab may be considered for cases with inadequate response to first-line therapy 1
  • Anticoagulation therapy should be considered if antiphospholipid antibodies are positive 2, 1

Monitoring and Follow-up

• Regular neurological assessment to monitor recovery and detect early signs of relapse
• Follow inflammatory markers (ESR, CRP) approximately every 4 weeks during treatment 1
• Follow-up MRI if clinical response is poor or symptoms worsen 1
• Monitor for respiratory involvement, as progressive weakness can affect respiratory muscles 2

Prognostic Factors

Poor prognostic factors include:

• Extensive spinal cord MRI lesions
• Reduced muscle strength or sphincter dysfunction at presentation
• Antiphospholipid antibodies
• Delay (>2 weeks) in the initiation of therapy 2, 1

Special Considerations

• Relapses are common (50-60%) during corticosteroid dose reduction, emphasizing the need for maintenance immunosuppressive therapy 2, 1
• For cases associated with chronic inflammatory demyelinating polyneuropathy (CIDP), consider gabapentinoids for neuropathic pain management 2, 3
• Early rehabilitation is essential to maintain joint mobility and prevent contractures 2

Pitfalls and Caveats

• Hypertrophic neuropathy can be misdiagnosed as spinal nerve neoplasia on imaging 6
• Delay in treatment initiation (>2 weeks) significantly worsens neurological outcomes 1
• Infectious causes must be ruled out before initiating immunosuppressive therapy to avoid worsening infection 2
• Consider underlying systemic conditions that may cause hypertrophic neuropathy, such as multiple sclerosis or CIDP 7, 3