Transverse Myelitis

Reviewed and revised 16 January 2014

OVERVIEW

• myelitis is a rare inflammatory condition of spinal cord characterised by pyramidal (motor), sensory, and/or autonomic dysfunction to varying degrees
• deficits are not always bilateral and do not necessarily affect all long tracts within the cord
• the term “transverse” originally referred to the clinical finding of a band-like horizontal area of altered sensation usually at the dermatomal level of the lesion within the cord, more recently it is used simply describes the position of the inflammation, that is, across the width of the spinal cord
• affects any age, according to underlying cause

CAUSES

Most common

• multiple sclerosis
• neuromyelitis optica (NMO) aka Devic syndrome (autoantibodies against aequaporin-4)
• post infections or vaccinations
• Idiopathic in about 30%

Other causes

• Demyelinating, e.g. MS, NMO, Acute demyelinating encephalomyelitis (ADEM)
• viral infection e.g. CMV, HZV, HIV, enterovirus, HSV
• parasitic infection e.g. schistosomiasis
• bacterial infection e.g. cat scratch disease, Tb
• fungal infection e.g. cryptococcus
• autoimmune/ inflammatory e.g. SLE, sarcoidosis
• paraneoplastic autoantibodies

Click here for a detailed list

CLINICAL FEATURES

Isolated spinal cord dysfunction with no evidence of a compressive lesion:

• time course
  • onset over hours to days
  • progression over days to weeks
• segmental spinal cord dysfunction, commonly thoracic
  • paresthesiae ascending from the feet with or without back pain at or near the level of the myelitis
  • weakness that preferentially affects the flexors of the legs and the extensors of the arms (pyramidal distribution of weakness)
  • sphincter dysfunction
  • autonomic dysfunction is common: bowel and bladder dysfunction, temperature dysregulation, or labile blood pressure
  • can have total motor and sensory loss below the level
• spinal shock (implies poor prognosis)

Click here for diagnostic criteria

• look for evidence of underlying cause (e.g. SLE)

DIFFERENTIAL DIAGNOSIS

Must rule out:

• Extrinsic compression of the cord
• neoplastic disease
• vascular spinal disease
• radiation-induced changes of the cord

INVESTIGATIONS

• MRI spine
  — gadolinium enhancement of the cord (may be absent)
  — rule out compressive or reversible lesion
  —  always image the level of the clinical signs and symptoms and the spinal cord superior to that level (lesions often manifest as the level below)
• MRI brain
  — should be normal unless MS or coexistent pathology
• CSF analysis
  — pleocytosis and/or an elevated IgG index (may be absent)
  — viral PCR
  — cultures
• Septic screen
  — rule out infection, e.g. meningoencephalitis
• Autoimmune screen

MANAGEMENT

Resuscitation

• if level high may need intubation

Specific therapy

• early neurologist and neurosurgical opinion
• high dose steroids (standard of care, not based on RCTs)
  • IV methylprednisolone
• consider plasmapheresis
  — typical dosing is 1.5 plasma volumes in five treatments over 10 days
  — best when initiated within the first two weeks after the onset of symptoms (clinical improvement in up to 43% of patients up to 2 months)
• case reports of:
  —  IV immunoglobulin (ADEM)
  — cyclophosphamide (idiopathic transverse myelitis and SLE)
  — rituximab (NMO)
  — azathioprine (NMO)

Supportive care and monitoring

• including IDC and VTE prophylaxis

PROGNOSIS

Overall prognosis

• one-third of patients recover with little to no lasting effects
• one-third retain a moderate degree of residual disability
• one third remain severely disabled

Poor prognostic indicators

• rapid progression of symptoms
• back pain
• spinal shock
• absent central conduction on somatosensory evoked potential testing

Associated with recovery

• early plasmapheresis

Progression and risk of relapse also depends on the underlying cause

References and Links

Journal articles

• West TW. Transverse myelitis–a review of the presentation, diagnosis, and initial management. Discov Med. 2013 Oct;16(88):167-77. PMID: 24099672.

FOAM and web resources

• Radiopaedia — Transverse myelitis