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Chapter 21: Transverse Myelitis

Mark P. Gorman

DEFINITIONS AND EPIDEMIOLOGY

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. Definitions of acute transverse myelitis (ATM) vary significantly in the literature.1,2 To address this nonuniformity, the criteria proposed by the Transverse Myelitis Consortium Working Group3 (Table 21-1) 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 acute transverse myelitis is characterized by moderate to severe symmetric symptoms, whereas partial acute transverse myelitis is marked by milder, asymmetric symptoms.4 A distinct but overlapping condition, acute flaccid myelitis (AFM), came to the fore after an outbreak of cases in the United States in 2014. This diagnosis is appropriate for patients with acute onset of focal limb weakness and a spinal cord lesion largely restricted to gray matter on magnetic resonance imaging (MRI).5

TABLE 21-1Transverse Myelitis Consortium Working Group Diagnostic Criteria
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TABLE 21-1Transverse Myelitis Consortium Working Group Diagnostic Criteria
Inclusion Criteria Exclusion Criteria

Development of sensory, motor, or autonomic dysfunction attributable to spinal cord

Bilateral signs and/or symptoms (although 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 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 2–7 days following symptom onset to meet criteria

Progression to nadir 4 hours–21 days following onset of symptoms (if patient awakens with symptoms, symptoms must become more pronounced from point of awakening)

History of previous radiation to spine within last 10 years

Clear arterial distribution clinical deficit consistent with thrombosis of anterior spinal artery

Abnormal flow voids on surface of spinal cord consistent with AVM

Serologic or clinical evidence of connective tissue disease (sarcoidosis, Behçet’s disease, Sjögren 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 multiple sclerosis*

History of clinically apparent optic neuritis*

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

*Does not exclude disease-associated acute transverse myelitis.

Acute transverse myelitis can be associated with more diffuse central nervous system (CNS) demyelinating disorders, systemic autoimmune disorders, or specific associated infections. Idiopathic acute transverse myelitis is associated with a nonspecific preceding ...

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