Transverse myelitis

Transvere myelitis = TM

Differential diagnosis

  • Compressive myelopathy: needs to be excluded by MRI
  • ADEM: acute disseminated encephalomyelitis
    • Acute demyelination involving brain and spinal cord
  • MS: multiple sclerosis, separated by time and space
  • NMO: neuromyelitis optica
    • usually involves 3 or more vertebral segments
    • Optic neuritis
    • AQP4 Ab positive
  • Poliomyelitis, post polio syndrome
  • Spinal cord infarct
  • Systemic autoimmune disorders:
    • Sarcoidosis
    • Sjogren’s syndrome
    • SLE: lupus
    • Less common:
      • Ankylosing spondylitis
      • Antiphospholipid Ab syndrome
      • Behcet disease
      • MCTD: mixed connective tissue disease
      • Systemic sclerosis
  • Paraneoplastic syndrome

General

  • Rare disease
  • Bimodal: more common in10-19 years old, 30-39 years old
  • Subacute onset: average 2 days
  • Idiopathic: about 60%
  • Post infectious: such as enterovirus, varicella zoster, herpes virus, Listeria, hiv
  • MRI: hyperintense signal, no compressive lesion, may show contrast enhancement
  • CSF: abnormal in half of the patients:
    • pleocytosis, some lymphocytes, usually < 100/microL
    • protein usually around 75
    • elevated IgG index
    • Oligoclonal band usually neg. (unlike MS over 85% positive)

Subtypes

  • Acute partial TM, risk of MS 60-90%
  • Acute complete TM, risk of MS 5-10%
  • longitudinally extensive, extends 3 or more vertebral segment
  • Acute flaccid myelitis, symptoms of lower motor neuron involvement

Treatment

  • Acute TM
    • IV solumedrol 1 gram qd for 5 days
    • Plasma exchange: 5 treatments every other day
    • Aggressive cases: single dose of iv cyclophosphamide 800 to 1200 mg/m2
  • Recurrent idiopathy TM: about 25 to 33 %
    • Chronic immunomodulatory therapy:
      • Mycophenolate 3 to 3 gram qd or
      • iv rituximab 1000 mg every 6 months

Further reading