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Transverse myelitis

Chitra Krishnan, MHS
Benjamin Greenberg, MD, MHS
Section Editor
Francisco González-Scarano, MD
Deputy Editor
John F Dashe, MD, PhD


Acute transverse myelitis (TM) is a rare acquired neuro-immune spinal cord disorder that can present with the rapid onset of weakness, sensory alterations, and bowel or bladder dysfunction. TM can occur as an independent entity, usually as a postinfectious complication, but TM also exists on a continuum of neuro-inflammatory disorders that includes acute disseminated encephalomyelitis, multiple sclerosis, and neuromyelitis optica. The clinical features, diagnostic work-up, and acute and chronic therapies differ between these forms of TM. It is important in the evaluation of patients with acute myelopathies to exclude compressive and noninflammatory causes of myelopathy as well as to distinguish various types of TM, since the prognosis, risk of recurrence, and treatment options may differ among these distinct entities.

This topic will review transverse myelitis. Related conditions are discussed elsewhere. (See "Acute disseminated encephalomyelitis in children: Pathogenesis, clinical features, and diagnosis" and "Acute disseminated encephalomyelitis in adults" and "Pathogenesis, clinical features, and diagnosis of pediatric multiple sclerosis" and "Clinical features of multiple sclerosis in adults" and "Clinically isolated syndromes suggestive of multiple sclerosis" and "Neuromyelitis optica spectrum disorders" and "Disorders affecting the spinal cord".)


The immunopathogenesis of TM is varied and reflects the rather diverse spectrum of this disease from idiopathic to disease-associated myelitis (see 'Associated conditions' below). There is evidence of perivascular infiltration by monocytes and lymphocytes in the lesion [1]. Axonal degeneration is also reported [1]. This pathologic heterogeneity and the involvement of both gray and white matter suggest that TM is not a pure demyelinating disorder but rather a mixed inflammatory disorder that affects neurons, axons, and oligodendrocytes and myelin. Postvaccination TM has been reported [2,3], and autopsy reports have described lymphocytic infiltration with demyelination and axonal loss [4]. Although these case reports describe TM occurring after vaccination, causation has not been established based on the timing and sequence of events alone. In a US database with 64 million vaccine doses administered among children and adults from 2007 through 2012, there were only seven subjects with TM who were vaccinated during the primary exposure interval of 5 to 28 days prior to TM onset [5]. Comparing each TM case with all matched subjects in the exposure interval who received the same vaccination, there was no association of TM with prior vaccination.

In 30 to 60 percent of the idiopathic TM cases, there is an antecedent respiratory, gastrointestinal, or systemic illness [6-12]. In parainfectious TM, the injury may be associated with direct microbial infection of the central nervous system, or with the systemic response to infection by a variety of agents such as varicella zoster virus, herpes virus, and Listeria monocytogenes. Molecular mimicry and super antigen-mediated disease have also been described as potential mechanisms of autoimmunity [4]. Molecular mimicry in TM was postulated to be the cause of injury following infection with Enterobius vermicularis (pinworm) in a patient who had elevated titers of cross-reacting antibodies [13]. Microbial super antigens such as staphylococcal enterotoxins A through I, toxic shock syndrome toxin-1, and streptococcus pyogenes exotoxin, have also been purported to stimulate the immune system and are known to be capable of activating T-lymphocytes without costimulatory molecules [4,14-17], thereby triggering autoimmune disease by activating auto-reactive T cell clones [18,19].

The diverse pathology of disease-associated TM is evident from studies showing that lupus-associated TM could be associated with central nervous system vasculitis or thrombotic infarction of the spinal cord [4,20-24]. Other studies have also described the role of autoantibodies in patients with neuromyelitis optica and recurrent TM [25-28]. Autoantibodies have been implicated in activating other components of the immune system by crossing the blood-brain barrier. The high prevalence of various autoantibodies seen in such patients suggests polyclonal derangement of the immune system. It may also be that some autoantibodies initiate a direct and selective injury of neurons that express antigens that cross-react with antibodies directed against infectious pathogens [4].

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Literature review current through: Nov 2017. | This topic last updated: Nov 17, 2016.
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