Transverse Myelitis

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DESCRIPTION

  • Transverse myelitis (TM) is an acquired, immune-mediated, inflammatory process of the spinal cord.
  • Symptom onset is acute to subacute with weakness, sensory disturbance (may have sensory level), and/or autonomic dysfunction. Syndrome may be idiopathic or related to an underlying inflammatory disorder (e.g., multiple sclerosis [MS] or neuromyelitis optica spectrum disorder [NMOSD]). In addition, it may be monophasic or polyphasic.

EPIDEMIOLOGY

  • Incidence: estimated 1 to 8 per million cases per year or 1,400 new cases (all ages) in the United States
  • Most common in ages <5 years and >10 years but can occur from infancy to adulthood
  • Prevalence: estimated 34,000 people with chronic morbidity from TM in the United States
  • No difference in ethnicity and sex prevalence with idiopathic TM
  • Female predominance in TM related to an underlying disease such as MS or NMOSD

ETIOLOGY

  • Unknown, although molecular mimicry may play a role, leading to immune response against central nervous system (CNS) proteins with cross-reactivity to infectious antigens
  • Other theories include direct microbial infection of the spinal cord and superantigen-mediated disease.

RISK FACTORS

  • Prodromal infections in up to 66% of cases
  • Recent vaccination (30 days) in up to 28% of cases (although most data suggest no link between TM and vaccination)
  • 1/3 may be due to an underlying disease (secondary TM).

GENERAL PREVENTION

  • Avoid unnecessary exposure to communicable disease and obtain routine immunizations to protect against preventable disease.
  • Because some autoimmune disorders are associated with vitamin D deficiency, supplementation may help prevent CNS inflammatory conditions in high-risk patients.

PATHOPHYSIOLOGY

  • Varied due to the diverse spectrum of disease from idiopathic to disease-associated TM
  • Histopathology may show perivascular infiltration by monocytes and lymphocytes, demyelination, and axon loss.

COMMONLY ASSOCIATED CONDITIONS

  • Predominantly idiopathic due to postinfectious autoimmune process
  • Infectious etiologies include but are not limited to West Nile virus, human T-lymphotropic virus (HTLV)-1, HIV, Zika virus, influenza, enteroviruses, herpes family viruses, mycoplasma, syphilis, Lyme disease, and SARS-CoV-2.
  • Can be a manifestation of a multifocal CNS disease (MS, NMOSD, acute disseminated encephalomyelitis [ADEM], or paraneoplastic syndrome)
  • TM can be associated with systemic inflammatory diseases such as sarcoidosis, Sjögren syndrome, systemic lupus erythematosus, Behçet disease, antiphospholipid syndrome, scleroderma, ankylosing spondylitis, and mixed connective tissue disease.

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