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Chapter 18. Transverse Myelitis

Mark P. Gorman; Scott L. Pomeroy

Acute transverse myelopathy is a clinical syndrome consisting of progressive symptoms and signs reflecting sensory, motor, or autonomic dysfunction attributable to the spinal cord. This syndrome can be caused by a heterogeneous group of disorders, including acute transverse myelitis (ATM). Definitions of ATM have varied significantly in the literature.1,2 To address this nonuniformity, the criteria proposed by the Transverse Myelitis Consortium Working Group3 (Table 18–1) should be used to establish the diagnosis and guide the differential diagnosis and workup. The diagnosis of ATM can be further refined by determining whether there is partial or complete involvement of the spinal cord in the axial plane. Complete ATM is characterized by moderate to severe symmetric symptoms, while partial ATM is marked by milder, asymmetric symptoms.4

Table 18–1. Transverse Myelitis Consortium Working Group Diagnostic Criteria
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Table 18–1. Transverse Myelitis Consortium Working Group Diagnostic Criteria

Inclusion Criteria

Exclusion Criteria

  • Development of sensory, motor, or autonomic dysfunction attributable to the spinal cord
  • Bilateral signs and/or symptoms (though not necessarily symmetric)
  • Clearly defined sensory level
  • Exclusion of extra-axial compressive etiology by neuroimaging (MRI or myelography; CT of spine not adequate)
  • Inflammation within the spinal cord demonstrated by CSF pleocytosis or elevated IgG index or gadolinium enhancement. If none of the inflammatory criteria is met at symptom onset, repeat MRI and lumbar puncture evaluation between 2 and 7 d following symptom onset meet criteria
  • Progression to nadir between 4 h and 21 d following the onset of symptoms (if patient awakens with symptoms, symptoms must become more pronounced from point of awakening)
  • History of previous radiation to the spine within the last 10 y
  • Clear arterial distribution clinical deficit consistent with thrombosis of the anterior spinal artery
  • Abnormal flow voids on the surface of the spinal cord consistent with AVM
  • Serologic or clinical evidence of connective tissue disease (sarcoidosis, Behçet's disease, Sjögren's syndrome, SLE, mixed connective tissue disorder, etc.)*
  • CNS manifestations of syphilis, Lyme disease, HIV, HTLV-1, Mycoplasma, other viral infection (e.g., HSV-1, HSV-2, VZV, EBV, CMV, HHV-6, enteroviruses)*
  • Brain MRI abnormalities suggestive of MS*
  • History of clinically apparent optic neuritis*

*Do not exclude disease-associated ATM.

AVM, arteriovenous malformation; SLE, systemic lupus erythematosus; HTLV-1, human T-cell lymphotropic virus-1; HSV, herpes simplex virus; VZV, varicella-zoster virus; EBV, Epstein–Barr virus; CMV, cytomegalovirus; HHV, human herpes virus.

ATM can be associated with more diffuse central nervous system (CNS) demyelinating disorders, systemic autoimmune disorders, or specific associated infections. Idiopathic ATM is associated with a nonspecific preceding infection or no apparent cause and constitutes the most common subgroup of pediatric ATM.

Idiopathic ATM afflicts approximately 1.34 persons per million.5 In the pediatric age group, patients present at a mean age of 8 years with an equal gender ratio.1,2,4,6–9 Approximately 280 cases of ATM occur in pediatric patients in the United States per year....

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