What is the recommended diagnostic workup and acute treatment for a patient with rapid onset bilateral weakness, a sensory level, and bowel/bladder dysfunction suggestive of transverse myelitis?

Transverse Myelitis: Diagnostic Workup and Acute Treatment

For a patient presenting with rapid onset bilateral weakness, sensory level, and bowel/bladder dysfunction suggestive of transverse myelitis, immediately initiate high-dose intravenous methylprednisolone 1g daily for 3-5 days while simultaneously pursuing urgent diagnostic workup with spine MRI and lumbar puncture. 1

Immediate Diagnostic Workup

Neuroimaging (Priority #1)

• Obtain urgent MRI of the spine with thin axial cuts through the region of suspected abnormality to identify T2-weighted hyperintense lesions 1, 2
• This imaging is essential to confirm the diagnosis and rule out compressive lesions that require surgical intervention 3
• Extensive spinal cord lesions (≥3 vertebral segments) indicate worse prognosis and may suggest neuromyelitis optica spectrum disorder 1, 2

Lumbar Puncture

• Perform lumbar puncture to evaluate CSF cell count, protein, glucose, oligoclonal bands, viral PCRs, and onconeural antibodies 1, 2
• Mild-to-moderate CSF abnormalities occur in 50-70% of cases but are non-specific 4
• Microbiological studies are critical to exclude infectious myelitis 4

Serology Testing

• Order blood tests including B12, HIV, RPR, ANA, Ro/La, TSH, and aquaporin-4 IgG 1, 2
• Aquaporin-4 IgG positivity indicates neuromyelitis optica spectrum disorder and requires more aggressive immunosuppression 1
• Antiphospholipid antibodies suggest thrombotic mechanism and may warrant anticoagulation 4, 1

Neurologic Consultation

• Obtain prompt neurologic consultation to guide diagnosis and management 1, 2

Acute Treatment Algorithm

First-Line Treatment (Initiate Immediately)

• Administer high-dose intravenous methylprednisolone 1g daily for 3-5 days 1
• For moderate to severe cases with significant weakness or sensory changes, combine corticosteroids with IVIG 2g/kg over 5 days 1
• The combination of intravenous methylprednisolone and immunotherapy can be effective if used promptly within the first few hours 4
• Neurological response paralleled by MRI improvement occurs within a few days to 3 weeks 4

Critical Timing Consideration

• Delay >2 weeks in initiating therapy is associated with severe neurological deficit and worse outcomes 4, 1, 2
• Even if awaiting MRI confirmation or infectious workup results, high-dose glucocorticoids may be given early and continued if infection is ruled out 4

Second-Line Treatment (For Refractory Cases)

• Plasma exchange therapy should be considered for patients who do not respond adequately to corticosteroids and IVIG 1
• Plasma exchange has been used in severe cases with good results 4
• Rituximab may be beneficial in cases with positive autoimmune encephalopathy antibodies or inadequate response to other therapies 1

Maintenance Therapy

• After acute treatment, maintenance immunosuppressive therapy (such as azathioprine) is necessary to prevent relapses 1
• Relapses are common (50-60%) during corticosteroid dose reduction, underscoring the need for continued immunosuppression 4, 1

Special Clinical Scenarios

Antiphospholipid Antibody-Positive Myelopathy

• Consider anticoagulation therapy in addition to immunosuppressive treatment 4, 1
• Anticoagulation may be considered in antiphospholipid-positive patients not responding to immunosuppressive therapy 4

Neuromyelitis Optica Spectrum Disorder (Aquaporin-4 IgG Positive)

• More aggressive immunosuppression is required 1
• These patients require long-term maintenance therapy to prevent devastating relapses 1

Infectious Etiology

• If infectious myelitis is suspected, appropriate antimicrobial/antiviral therapy should be administered 4
• For parasitic causes like schistosomiasis, antiparasitic treatment should be given along with corticosteroids 1

Prognostic Factors Associated with Poor Outcome

The following factors predict severe neurological deficit and warrant more aggressive treatment:

• Extensive spinal cord MRI lesions (≥3 vertebral segments) 4, 1, 2
• Reduced muscle strength or sphincter dysfunction at presentation 4, 1, 2
• Delay (>2 weeks) in initiation of therapy 4, 1, 2
• Presence of antiphospholipid antibodies 4

Critical Pitfalls to Avoid

Do Not Delay Treatment

• Do not wait for complete diagnostic workup before initiating corticosteroids 4, 1
• The window for optimal treatment is narrow, and early intervention within hours significantly improves outcomes 4

Rule Out Compressive Lesions

• Always obtain urgent MRI to exclude compressive myelopathy requiring surgical decompression 3
• Compressive lesions are a neurosurgical emergency and require immediate intervention 3

Distinguish from Guillain-Barré Syndrome

• GBS presents with ascending weakness, areflexia, and absence of CNS involvement or sensory level 4
• Unlike transverse myelitis, GBS does not cause bowel/bladder dysfunction early in the disease course 4
• The presence of a sensory level strongly favors transverse myelitis over GBS 2

Monitor for Autonomic Complications

• Evaluate for urinary retention and constipation, which are common autonomic manifestations 1
• Dysautonomia can include blood pressure or heart rate instability requiring intensive monitoring 4