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An acute or subacute inflammatory lesion of the spinal cord manifesting with the appearance of new autonomic, motor, and sensory symptoms. The “transverse” descriptor is a reference to the classic symptom presentation with an identifiable sensory level traversing the midline. Transverse myelitis (TM) is associated with cerebrospinal fluid (CSF) or radiographic abnormalities consistent with an inflammatory spinal cord lesion. TM is usually monophasic but can be a manifestation of a chronic disease.
- Incidence: Estimated 1–8/million cases per year in the United States (20% children) or approximately 300 affected children annually. There are two incidence distributions in children—a peak between 0 and 2 years of age and another broader distribution from 5 to 17 years of age.
- Prevalence: estimated 34,000 in the United States with disabilities due to TM
- The female-to-male ratio is approximately 1:1 for <10 years, with a female predominance observed after age 10 years (2.6:1) in a North American cohort.
- Fever, findings consistent with infection, or vaccinations in the preceding weeks are reported in the majority (>50%) of children with TM, but specific infections are rarely diagnosed.
- Mild trauma and obesity are associated with an increased risk of TM.
Unknown. Several infectious and autoimmune conditions have been associated with TM without a clear unifying immunopathogenesis.
Commonly Associated Conditions
- TM in children is predominantly idiopathic.
- TM can be a manifestation of the acquired demyelinating syndromes, for example, acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), or neuromyelitis optica (NMO).
- The enteroviruses (coxsackie A7, 9, 23, and B strains) and other viruses (dengue fever, hepatitis, human herpes, influenza, polio, and West Nile virus) have been reported in association with acute TM.
- TM can occur during acute mycoplasma, spirochete, or parasitic infections.
- TM has been reported in combination with Guillain-Barré syndrome (GBS).
- TM can occur as part of a systemic autoimmune inflammatory disease (mixed connective tissue disease, systemic sclerosis, systemic lupus erythematosus, sarcoidosis, Sjögren syndrome, Behçet disease, juvenile rheumatoid arthritis, autoimmune thyroid disease, or antiphospholipid syndrome).
- Metabolic deficiencies and mitochondrial disease can cause TM-like presentations.