Transverse Myelitis
Definition
Transverse myelitis (TM) is an acute or subacute inflammatory disorder of the spinal cord causing motor, sensory, and autonomic dysfunction below the level of the lesion. It is usually immune-mediated and may be idiopathic or secondary to another disease.
Etiology
- Idiopathic
- Demyelinating disorders
- Multiple sclerosis (MS)
- Neuromyelitis optica spectrum disorder (NMOSD), associated with AQP4-IgG
- MOG antibody-associated disease (MOGAD)
- Autoimmune/connective-tissue diseases
- SLE
- Sjögren syndrome
- Vasculitis
- Sarcoidosis
- Post-infectious or infective
- Viral, bacterial, tuberculosis, HIV, syphilis
- Vascular mimics
- Spinal cord infarction
- Dural arteriovenous fistula
Clinical features
Symptoms develop over hours to days, usually reaching maximum severity within 4 hours to 21 days.
1. Motor
- Bilateral weakness of legs, usually paraparesis/paraplegia
- Quadriparesis if cervical cord is involved
- Initially flaccid weakness with absent reflexes due to spinal shock
- Later: spasticity, hyperreflexia, extensor plantar response
2. Sensory
- Numbness, tingling, dysesthesia
- A definite sensory level on the trunk
- Back pain or radicular limb pain may occur
3. Autonomic
- Urinary retention or incontinence
- Constipation or fecal incontinence
- Sexual dysfunction
- In cervical/high thoracic lesions, autonomic instability may occur.
In severe disease, there can be complete motor and sensory loss below the lesion with bowel, bladder, and sexual dysfunction. Goldman-Cecil Medicine, p. 3995.
Diagnosis
Diagnostic criteria
Diagnosis is based on:
- Clinical features of acute/subacute spinal cord dysfunction
- Bilateral motor, sensory, or autonomic deficits
- Clearly defined sensory level
- Evidence of spinal cord inflammation
- Exclusion of a compressive spinal lesion
- Progression to nadir usually within 4 hours to 21 days
Investigations
1. MRI spine with gadolinium - investigation of choice
- Excludes cord compression
- T2 hyperintense intramedullary lesion
- Cord swelling and contrast enhancement may be seen
- A lesion spanning 3 or more vertebral segments is longitudinally extensive transverse myelitis (LETM) and suggests NMOSD. Goldman-Cecil Medicine, p. 3995.
2. MRI brain
- To look for lesions suggestive of MS or other demyelinating disease.
3. CSF examination
- Mild lymphocytic pleocytosis
- Raised protein
- Oligoclonal bands may suggest MS
- Microbiological testing when infection is suspected
4. Blood tests
- CBC, ESR/CRP, blood glucose, renal/liver function
- Vitamin B12, HIV, VDRL, ANA, dsDNA, ENA
- Serum AQP4-IgG and MOG-IgG
- Infectious tests as indicated
Differential diagnosis
- Acute compressive myelopathy: prolapsed disc, tumor, epidural abscess, hematoma
- Spinal cord infarction
- MS, NMOSD, MOGAD
- Subacute combined degeneration due to vitamin B12 deficiency
- Guillain-Barré syndrome
- Cauda equina syndrome
- Spinal dural AV fistula
Important point: MRI must urgently exclude compression because compressive myelopathy may require emergency surgery.
Treatment
Acute treatment
- Admit and obtain urgent neurology opinion.
- High-dose IV corticosteroid
- IV methylprednisolone 1 g/day for 3-5 days
- Plasma exchange (PLEX)
- For severe disease or inadequate response to steroids
- IVIG
- Consider when plasma exchange is unavailable or unsuitable.
- Treat the underlying cause:
- NMOSD: acute immunotherapy and long-term relapse prevention
- SLE/vasculitis: steroids plus appropriate immunosuppressant, often cyclophosphamide
- Infection: targeted antimicrobial treatment
Textbook management supports IV methylprednisolone followed by cause-specific treatment; plasma exchange, IVIG, or cyclophosphamide can be considered in steroid-refractory cases. Goldman-Cecil Medicine, p. 3995.
Supportive management
- Bladder catheterization and bladder training
- Bowel care
- Prevention of pressure sores and DVT
- Physiotherapy and rehabilitation
- Management of spasticity, neuropathic pain, depression, and sexual dysfunction
Prognosis
- Variable: complete recovery to permanent paraplegia.
- Recovery commonly begins within weeks to months.
- Poor prognostic factors:
- Severe weakness at onset
- Rapid progression
- Cervical lesion
- Longitudinally extensive lesion
- Lack of early improvement
- NMOSD-associated disease
One-line viva answer
Transverse myelitis is an acute inflammatory myelopathy producing bilateral motor weakness, a sensory level, and bladder-bowel dysfunction, diagnosed by MRI and CSF after excluding spinal cord compression, and treated initially with high-dose IV methylprednisolone with plasma exchange for refractory cases.