Management of Longitudinally Extensive Transverse Myelitis with Severe Complications
This patient requires immediate high-dose intravenous methylprednisolone 1g daily for 3-5 days combined with IVIG 2g/kg over 5 days, with urgent neurology consultation and ICU-level monitoring given the severe presentation with complete urinary/bowel retention and bilateral lower extremity paralysis. 1, 2
Immediate Actions (Within Hours)
Discontinue Potentially Causative Medications
- Stop warfarin and unfractionated heparin immediately - these anticoagulants pose significant hemorrhagic risk in the setting of acute spinal cord inflammation and the patient already has concerning leukopenia 3, 1
- Continue prednisolone temporarily until high-dose pulse steroids are initiated 1
Initiate Aggressive Immunosuppressive Therapy
- Administer methylprednisolone 1g IV daily for 3-5 days as first-line treatment - this is the cornerstone of acute LETM management and delays beyond 2 weeks worsen prognosis 1, 2, 4
- Add IVIG 2g/kg divided over 5 days (0.4g/kg/day) concurrently - combination therapy is recommended for severe presentations with motor weakness and sphincter dysfunction 3, 1, 2
- The severity of this presentation (bilateral paralysis, complete urinary/fecal retention, paresthesia) mandates combination therapy rather than steroids alone 1, 2
Critical Monitoring and Support
- Transfer to ICU-level care immediately - patients with LETM can develop ascending paralysis affecting respiratory muscles, particularly when lesions extend to cervical segments 3, 4
- Monitor respiratory function closely with serial vital capacity and negative inspiratory force measurements 3
- The case report of a patient developing type 2 respiratory arrest 30 hours after admission with LETM extending from C2 to conus medullaris demonstrates this risk 4
Diagnostic Workup (Concurrent with Treatment)
Essential Serological Testing
- Aquaporin-4 IgG antibodies - positive results indicate neuromyelitis optica spectrum disorder (NMOSD) requiring more aggressive long-term immunosuppression 1, 2, 5
- MOG-IgG antibodies - identifies MOG-antibody disease with different treatment implications 2
- Complete autoimmune panel including ANA, Ro/La, anti-dsDNA 3, 1
- Infectious workup: HIV, RPR, viral PCRs 3, 1
- Vitamin B12, TSH, thyroid peroxidase antibodies 3, 1
CSF Analysis via Lumbar Puncture
- Cell count, protein, glucose, oligoclonal bands 3, 1, 6
- Viral PCRs including JCV to exclude progressive multifocal leukoencephalopathy 3
- Onconeural antibodies and paraneoplastic panel 3, 1
Imaging Confirmation
- MRI already shows LETM (≥3 vertebral segments involved), which is the defining radiologic feature 2, 7, 5
- Ensure brain MRI with contrast is obtained to evaluate for additional demyelinating lesions suggesting multiple sclerosis or NMOSD 3, 1
Management of Concurrent Complications
Hyponatremia Correction
- Correct hyponatremia cautiously (no faster than 8-10 mEq/L per 24 hours) to avoid osmotic demyelination syndrome 1
- This is particularly critical given existing spinal cord demyelination 1
Leukopenia Investigation
- Urgent hematology consultation - leukopenia may represent bone marrow suppression, autoimmune process, or infection 1
- Hold bisacodyl and metoclopramide temporarily as these can rarely cause cytopenias 1
- Check peripheral smear, reticulocyte count, and consider bone marrow biopsy if severe or unexplained 1
Bladder and Bowel Management
- Initiate intermittent catheterization for urinary retention rather than indwelling catheter to reduce infection risk 3, 1, 8
- Aggressive bowel regimen with scheduled enemas given complete fecal retention 3, 1
- Evaluation for autonomic dysfunction is essential as this affects up to 90% of transverse myelitis patients 8, 9, 6
Pain Management
- Continue morphine for neuropathic pain initially 1
- Consider transitioning to neuropathic pain agents (gabapentin, pregabalin, or duloxetine) once acute phase stabilizes 3
Second-Line Therapies (If No Response in 3-7 Days)
Plasma Exchange
- Initiate plasmapheresis if no improvement or worsening after 3 days of methylprednisolone plus IVIG 3, 1, 2
- Plasma exchange has shown benefit in steroid-refractory cases 1, 2
Rituximab Consideration
- Reserve for cases with positive autoimmune antibodies (aquaporin-4, MOG) or inadequate response to steroids, IVIG, and plasma exchange 3, 1, 2
Steroid Tapering and Maintenance
Acute Phase Taper
- After 3-5 days of pulse methylprednisolone, transition to oral prednisone 1mg/kg daily 3, 1
- Taper over minimum 4-6 weeks - rapid tapering increases relapse risk which occurs in 50-60% of cases 3, 1
Long-Term Immunosuppression
- Maintenance therapy with azathioprine or other immunosuppressant is necessary to prevent relapses during steroid taper 1
- If aquaporin-4 positive (NMOSD), consider rituximab, azathioprine, or mycophenolate mofetil for long-term prevention 1, 2, 5
Prognostic Considerations
Poor Prognostic Indicators Present in This Case
- Longitudinally extensive lesion (≥3 vertebral segments) - associated with worse outcomes 1, 2, 5
- Complete sphincter dysfunction at presentation - indicates severe cord involvement 1, 8
- Bilateral lower extremity paralysis - severe motor deficit predicts incomplete recovery 1, 9, 6
Critical Timing Factor
- Delay beyond 2 weeks in initiating therapy significantly worsens prognosis - this patient requires treatment within hours, not days 1
- Two-thirds of transverse myelitis patients have moderate to severe residual disability, emphasizing the urgency of aggressive early treatment 9, 6
Key Clinical Pitfalls to Avoid
- Do not wait for complete diagnostic workup before initiating treatment - the combination of clinical presentation and MRI findings is sufficient to begin therapy 1, 4, 6
- Do not use steroids alone in severe cases - this patient's presentation mandates combination therapy with IVIG 1, 2
- Do not overlook respiratory monitoring - ascending paralysis can occur rapidly, particularly with cervical involvement 3, 4
- Do not continue anticoagulation without compelling indication - hemorrhagic transformation of spinal cord lesions is a recognized complication 1