What is the treatment for acute transverse myelitis?

Treatment Protocol for Acute Transverse Myelitis

Immediately initiate high-dose intravenous methylprednisolone 1 gram daily for 3-5 days as first-line therapy for acute transverse myelitis. 1, 2, 3

Immediate Actions Upon Diagnosis

• Discontinue any potential causative medications immediately, particularly immune checkpoint inhibitors if the patient is receiving cancer immunotherapy 1, 2, 3
• Obtain urgent neurology consultation to guide management 1
• Confirm diagnosis with MRI of the entire spine (with and without contrast) using thin axial cuts to identify T2-weighted hyperintense lesions 1, 2, 3

First-Line Treatment Algorithm

Standard Cases

• Administer IV methylprednisolone 1 gram daily for 3-5 days as pulse dosing 1, 2, 3, 4
• This represents the cornerstone of acute treatment and has demonstrated significant benefit in reducing time to independent walking (23 vs 97 days) and achieving full recovery (80% vs 10% at 12 months) compared to no treatment 4

Severe Cases (with significant weakness, sensory changes, or dysautonomia)

• Combine corticosteroids with IVIG 2 g/kg divided over 5 days (0.4 g/kg/day) administered simultaneously with methylprednisolone 1, 2, 3
• This combination approach is specifically indicated when severe dysautonomia (blood pressure instability, temperature dysregulation, cardiac arrhythmias) is present 2
• For longitudinally extensive transverse myelitis (LETM) affecting ≥3 vertebral segments, combination therapy is preferred over corticosteroids alone 2, 3

Second-Line Treatment for Refractory Cases

• Initiate plasma exchange (PLEX) if no clinical improvement occurs within 7-10 days of combined corticosteroid and IVIG therapy 1, 2, 3
• Administer 5-10 sessions of PLEX every other day 2
• Consider earlier PLEX initiation (within 3 days) if symptoms worsen or progress despite initial therapy 2
• Plasma exchange has demonstrated clinical benefit in retrospective studies, particularly in patients who fail corticosteroid therapy 5

Third-Line Options

• Rituximab may be beneficial in cases with positive autoimmune encephalopathy antibodies or inadequate response to corticosteroids, IVIG, and plasma exchange 1, 3
• Cyclophosphamide can be considered in refractory cases, with retrospective data showing benefit in select patient subsets 5

Essential Diagnostic Workup (Perform Concurrently with Treatment)

Do not delay treatment while awaiting test results - initiate immunotherapy based on clinical presentation and MRI findings 2

Imaging

• MRI of entire spine including cervical and thoracic regions to assess full extent of involvement 2
• Look specifically for lesions extending ≥3 vertebral segments (LETM), which indicates more aggressive disease requiring combination therapy 2, 3

Laboratory Studies

• Lumbar puncture for CSF analysis: cell count, protein, glucose, oligoclonal bands, IgG index, viral PCRs, and onconeural antibodies 1, 2, 3
• Blood tests: aquaporin-4 IgG (for neuromyelitis optica spectrum disorder), MOG-IgG antibodies, B12, HIV, RPR, ANA, Ro/La, TSH 1, 3

Maintenance Therapy

• After acute treatment, initiate maintenance immunosuppressive therapy (such as azathioprine) to prevent relapses, which occur in 50-60% of cases during corticosteroid dose reduction 1
• For NMOSD-associated transverse myelitis (positive aquaporin-4 IgG), more aggressive long-term immunosuppression is required 1, 3

Critical Monitoring Requirements

• Perform frequent neurologic examinations and pulmonary function monitoring to assess for respiratory compromise 2
• Monitor orthostatic vital signs regularly to assess severity of autonomic involvement 2
• Evaluate and manage urinary retention and constipation, which are common autonomic complications requiring bladder catheterization and bowel regimens 1, 2, 3
• Avoid medications that worsen autonomic dysfunction: beta-blockers, IV magnesium, and certain antibiotics 2

Special Clinical Scenarios

Transverse Myelitis with Antiphospholipid Antibodies

• Add anticoagulation therapy in addition to immunosuppressive treatment 1

Infectious Etiology (e.g., Schistosomiasis)

• Administer appropriate antiparasitic treatment along with corticosteroids 1

Immune Checkpoint Inhibitor-Related Cases

• Permanently discontinue the immune checkpoint inhibitor 2, 3
• Screen for concurrent myasthenia gravis or myositis, which can occur simultaneously and requires specific management 2

Prognostic Factors Indicating Poor Outcomes

• Extensive spinal cord MRI lesions, particularly LETM affecting ≥3 vertebral segments 1, 2, 3
• Severe muscle weakness or sphincter dysfunction at presentation 1, 2
• Delay >2 weeks in initiation of therapy - this emphasizes the critical importance of immediate treatment 1

Key Clinical Pitfalls to Avoid

• Do not use corticosteroids alone in severe presentations with dysautonomia or LETM - combination therapy with IVIG is indicated 2, 3
• Do not delay treatment awaiting antibody results - clinical and MRI findings are sufficient to initiate therapy 2
• Do not overlook the need for long-term immunosuppression after acute treatment, as relapse rates are high (50-60%) 1
• Distinguish transverse myelitis from Guillain-Barré syndrome by checking deep tendon reflexes - increased reflexes favor transverse myelitis, while absent reflexes suggest GBS 3