Transverse myelitis

Definition

• Heterogeneous condition characterized by inflammation of the spinal cord

Etiology

• CNS disorders
  • Multiple sclerosis
  • Neuromyelitis optica
  • Acute disseminated encephalomyelitis (ADEM)
  • Myelin oligodendrocyte glyocprotein antibody-associated disorder
• Systemic inflammatory disorders
  • Sarcoidosis
  • Sjögren syndrome
  • Systemic lupus erythematosus
  • Others: ankylosing spondylitis, antiphospholipid antibody syndrome, Behçet disease, mixed connective tissue disease, rheumatoid arthritis, and systemic sclerosis
• Infectious
  • Enteroviruses, especially D68 and EV71
  • West Nile virus
  • Herpesviridae, including HSV, VZV, EBV, CMV, and HHV-6
  • HIV
  • HTLV-1
  • Zika virus
  • Lyme disease
  • Mycoplasma species
  • Treponema pallidum
• Paraneoplastic, most often secondary to small cell lung cancer
  • Anti-Hu disease
  • Anti-CRMP5 disease
• Idiopathic

Clinical Manifestations

• Presentation is either acute or subacute, usually starting with sensory changes, weakness, or pain
• Characterized by both motor and sensory deficits, with specific deficits depending on the spinal level of the lesion

Investigations

• MRI spine with gadolinium, showing characteristic T2 hyperintensity without evidence of cord compression
• CSF showing pleocytosis

Diagnosis

• Sensorimotor and autonomic spinal cord dysfunction
  • Generally bilateral but may be asymmetric
• Clearly defined sensory level
• Bilateral signs and symptoms
• Progression peaking between 4h and 21d after onset
• Demonstration of spinal cord inflammation
  • Cerebrospinal fluid with pleocytosis or increased IGG
  • MRI with gadolinium
• Exclusion of compressive, postradiation, neoplastic, and vascular causes

Management

• Determine cause
• Consider high-dose steroids 1g/d x3-5 days
• Thromboembolism prophylaxis
• Symptom management for pain and spasticity
  • Muscle relaxants
• Rehab (PT/OT)
• Follow-up investigations
  • Repeat MRI