What is transverse myelitis and how is it treated?

Understanding Transverse Myelitis: A Simplified Guide

What Is Transverse Myelitis?

Transverse myelitis is an acute inflammatory disease of the spinal cord that causes rapid-onset bilateral weakness, sensory loss, and bladder/bowel dysfunction due to immune-mediated damage to spinal cord tissue. 1, 2

The condition develops over hours to days, with most patients reaching maximum severity within 10 days of symptom onset 2. The term "transverse" refers to the inflammation extending across the width of the spinal cord, affecting both sides of the body 3.

How Does It Affect the Body?

Motor (Movement) Problems

• Patients develop spastic paralysis with stiff, rigid muscles and increased reflexes (not floppy weakness like in Guillain-Barré syndrome) 1
• This occurs because the spinal cord damage creates upper motor neuron injury, leading to hyperreflexia rather than the flaccid paralysis seen in peripheral nerve disorders 1
• At peak severity, approximately 50% of patients become completely paraplegic with no voluntary leg movements 2, 4

Sensory (Feeling) Problems

• 80-94% of patients experience numbness, tingling, or band-like pain sensations around the trunk 4
• Sensory changes are typically bilateral and often have a distinct "sensory level" where normal sensation stops 3

Autonomic (Automatic Body Functions) Problems

• Nearly all patients develop bladder dysfunction, often requiring catheterization 2
• Bowel problems including constipation are common 5
• Some patients experience blood pressure instability, temperature regulation problems, and cardiac rhythm disturbances 6

What Causes It?

The causes are diverse and sometimes difficult to pinpoint 3:

• Infections or post-infectious reactions (most common identifiable cause) 3
• Autoimmune diseases like lupus or neuromyelitis optica 5, 7
• Demyelinating conditions like multiple sclerosis 8
• Medications, particularly immune checkpoint inhibitors used in cancer treatment 5
• Idiopathic (unknown cause despite thorough testing) in 10-40% of cases 3

How Is It Diagnosed?

Imaging

• MRI of the spine is essential, showing bright T2-weighted lesions that appear wedge-shaped on cross-section and cigar-shaped lengthwise 1
• Brain MRI should also be performed to check for other areas of inflammation 5

Spinal Fluid Analysis

• Lumbar puncture reveals elevated white blood cells (lymphocytes) and protein in 50-70% of cases 5
• Testing includes checking for infections, oligoclonal bands, and autoimmune antibodies 5, 1

Blood Tests

• Check for vitamin B12, HIV, syphilis (RPR), autoimmune markers (ANA, Ro/La), thyroid function, and aquaporin-4 antibodies (for neuromyelitis optica) 5, 1

How Is It Treated?

First-Line Treatment (Start Immediately)

High-dose intravenous methylprednisolone 1 gram daily for 3-5 days is the cornerstone of initial therapy 5, 7, 6

• For moderate to severe cases with significant weakness or sensory changes, add IVIG 2 g/kg divided over 5 days 7, 6
• If immune checkpoint inhibitors or other causative medications are involved, permanently discontinue them immediately 5, 6

Second-Line Treatment (If No Improvement)

• Plasma exchange therapy (5-10 sessions) should be considered if patients don't respond to steroids and IVIG within 7-10 days 7, 6
• Rituximab may be beneficial in refractory cases with positive autoimmune antibodies 5, 7

Maintenance Therapy

• Long-term immunosuppression is often necessary because relapses occur in 50-60% of patients during steroid tapering 5, 7
• Options include azathioprine or other immunosuppressive agents 7

Special Situations

• For antiphospholipid antibody-positive patients, add anticoagulation therapy alongside immunosuppression 5, 7
• For neuromyelitis optica spectrum disorder (positive aquaporin-4 antibodies), more aggressive immunosuppression is required 7

What Is the Prognosis?

Recovery is divided roughly into thirds 2, 4:

• One-third recover with minimal or no disability
• One-third have moderate permanent disability
• One-third have severe disability

Poor Prognostic Factors

• Extensive spinal cord lesions on MRI, especially longitudinally extensive transverse myelitis (≥3 vertebral segments) 7, 6
• Severe muscle weakness or sphincter dysfunction at presentation 5, 7
• Delay in treatment beyond 2 weeks 5, 7

Critical Pitfalls to Avoid

• Do not wait for antibody results before starting treatment—begin immunotherapy based on clinical presentation and MRI findings 6
• Do not use corticosteroids alone in severe presentations with autonomic dysfunction—combination therapy with IVIG is indicated 6
• Do not delay treatment—prompt initiation within hours to days significantly improves outcomes 5
• Do not overlook urinary retention and constipation—these require active management to prevent complications 5, 1

Key Distinguishing Features

The presence of increased deep tendon reflexes (hyperreflexia) with spastic paralysis is the hallmark that distinguishes transverse myelitis from conditions like Guillain-Barré syndrome, which causes flaccid paralysis with reduced or absent reflexes 1. This occurs because transverse myelitis damages the spinal cord itself (upper motor neuron), while Guillain-Barré affects peripheral nerves (lower motor neuron) 1.