Transverse myelitis diagnostic study of choice

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Fahimeh Shojaei, M.D., Julinka Auta Fernandes

Overview

There is no single gold-standard or independently confirmatory diagnostic study for transverse myelitis. The diagnosis is based on a compatible acute or subacute spinal cord syndrome, exclusion of spinal cord compression, and objective evidence of spinal cord inflammation on magnetic resonance imaging (MRI) or cerebrospinal fluid (CSF) examination.

MRI of the spinal cord is the diagnostic imaging study of choice. It should be performed urgently to exclude an extra-axial compressive lesion and to detect and characterize an intrinsic spinal cord abnormality.

Diagnostic Study of Choice

Study of choice

There is no single diagnostic study that independently confirms transverse myelitis. However, urgent MRI of the spinal cord is the diagnostic imaging study of choice in a patient presenting with an acute or subacute myelopathic syndrome.

• MRI of the clinically relevant spinal region should be performed as soon as possible.
• For initial imaging of acute myelopathy, MRI without intravenous contrast and MRI without and with intravenous gadolinium-based contrast material are both considered appropriate. When inflammatory myelitis is suspected and a gadolinium-based contrast agent can be administered safely, precontrast and postcontrast imaging helps demonstrate active enhancement. Imaging should not be delayed when contrast is contraindicated.
• Imaging should include sagittal and axial acquisitions.
• Imaging of the entire spinal cord, including the conus medullaris, should be considered when the neurological level is uncertain, symptoms involve multiple levels, or neuromyelitis optica spectrum disorder (NMOSD) or myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is suspected.
• Brain MRI should be obtained during the etiologic evaluation to identify abnormalities supporting multiple sclerosis or another inflammatory demyelinating disorder.
• When NMOSD or MOGAD is suspected, contemporary European expert consensus supports MRI of the brain, optic nerves, and spinal cord, including the cauda equina nerve roots, without and after gadolinium administration at initial diagnosis.

The principal purposes of spinal MRI are to:

• Exclude spinal cord compression and other structural emergencies.
• Detect an intramedullary spinal cord lesion.
• Determine the craniocaudal extent and axial distribution of the lesion.
• Identify spinal cord swelling and gadolinium enhancement.
• Evaluate for vascular, neoplastic, infectious, and other non-inflammatory mimics.
• Identify imaging patterns that may guide testing for an underlying inflammatory demyelinating disorder.

If MRI cannot be performed, computed tomography (CT) myelography may be considered to evaluate for spinal canal compression. Routine CT of the spine is not an adequate substitute for MRI when assessing intrinsic spinal cord inflammation.

The comparison of various diagnostic studies for transverse myelitis

Universal sensitivity and specificity estimates are not available because transverse myelitis is a heterogeneous clinical syndrome rather than a single etiologic disease. Diagnostic performance varies according to disease cause, disease timing, MRI technique, and whether contrast is administered. MRI, CSF examination, and disease-specific antibody testing are complementary rather than competing diagnostic studies.

Diagnostic study	Sensitivity	Specificity	Principal role
Spinal MRI	No universal validated estimate	No universal validated estimate	Imaging study of choice; excludes compression and detects and characterizes an intrinsic spinal cord lesion.
CSF examination	No universal validated estimate	No universal validated estimate	Demonstrates inflammation and helps investigate infectious and inflammatory causes.
Brain MRI	Not applicable as an isolated test for transverse myelitis	Not applicable as an isolated test for transverse myelitis	Identifies accompanying lesions that may support multiple sclerosis or another demyelinating disorder.
Serum aquaporin-4 immunoglobulin G (AQP4-IgG) and myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG) testing	Assay- and disease-dependent	Assay- and disease-dependent	Determines whether myelitis may be associated with NMOSD or MOGAD; these tests do not independently diagnose transverse myelitis.
CT myelography	No universal validated estimate	No universal validated estimate	Alternative for excluding compression when MRI cannot be performed; limited for evaluating intrinsic spinal cord inflammation.

No diagnostic study should be preferred solely on the basis of a single sensitivity or specificity value.

Diagnostic results

No individual MRI finding is confirmatory of transverse myelitis. Findings that support inflammatory myelitis include:

• An intramedullary T2-weighted hyperintense spinal cord lesion corresponding to the clinical syndrome.
• Focal or longitudinally extensive spinal cord involvement.
• Spinal cord swelling during the acute phase.
• Intramedullary gadolinium enhancement.
• An axial and longitudinal lesion distribution compatible with an inflammatory demyelinating disorder.

Lesion length or enhancement pattern alone should not be used to assign an etiology. Longitudinally extensive lesions may occur in NMOSD and MOGAD but can also occur with infectious, vascular, systemic autoimmune, metabolic, and neoplastic disorders.

A normal initial spinal MRI does not completely exclude inflammatory myelitis, particularly when imaging is performed early. If clinical suspicion remains high, repeat MRI should be considered. Under the Transverse Myelitis Consortium Working Group criteria, when objective evidence of inflammation is absent at presentation, repeat MRI and lumbar puncture should be performed within 2–7 days.

Sequence of Diagnostic Studies

The investigations should generally be performed in the following order:

1. Urgent spinal MRI to exclude an extra-axial compressive lesion and identify an intrinsic spinal cord abnormality.
2. CSF examination after a compressive lesion has been excluded, to evaluate for pleocytosis, an elevated immunoglobulin G index, intrathecal immunoglobulin synthesis, and relevant infectious causes.
3. Brain MRI to evaluate for accompanying abnormalities suggestive of multiple sclerosis or another inflammatory demyelinating disorder.
4. Targeted etiologic investigations, including serum AQP4-IgG and MOG-IgG testing when clinically indicated.
5. Repeat spinal MRI and CSF examination when the initial evaluation is nondiagnostic but clinical suspicion for inflammatory myelitis remains high.

Name of Diagnostic Criteria

The principal published transverse-myelitis-specific criteria are the Transverse Myelitis Consortium Working Group proposed diagnostic criteria for idiopathic acute transverse myelitis. All inclusion criteria and none of the exclusion criteria should be present for classification as idiopathic acute transverse myelitis.

Transverse Myelitis Consortium Working Group criteria for idiopathic acute transverse myelitis
Adapted from the Transverse Myelitis Consortium Working Group, 2002.

Inclusion criteria

Exclusion criteria for idiopathic acute transverse myelitis

Development of sensory, motor, or autonomic dysfunction attributable to the spinal cord Bilateral signs or symptoms, although they do not need to be symmetric A clearly defined sensory level Exclusion of an extra-axial compressive cause by neuroimaging Objective spinal cord inflammation demonstrated by CSF pleocytosis, an elevated CSF immunoglobulin G (IgG) index, or gadolinium enhancement Progression to the clinical nadir between 4 hours and 21 days after symptom onset

History of spinal radiation during the preceding 10 years A clear arterial-distribution deficit consistent with anterior spinal artery thrombosis Abnormal flow voids on the spinal cord surface consistent with an arteriovenous malformation Serologic or clinical evidence of a systemic connective-tissue or inflammatory disease Central nervous system manifestations of an infectious disease Brain MRI abnormalities suggestive of multiple sclerosis History of clinically apparent optic neuritis

If objective inflammation is not demonstrated at symptom onset, the Consortium criteria recommend repeating MRI and lumbar puncture within 2–7 days.

The exclusion criteria above apply to classification as idiopathic acute transverse myelitis. Identification of infection, systemic autoimmune disease, multiple sclerosis, NMOSD, MOGAD, or another defined cause may instead indicate disease-associated transverse myelitis and does not necessarily exclude the presence of myelitis.

The 2002 criteria predate contemporary AQP4-IgG, MOG-IgG, NMOSD, MOGAD, and multiple-sclerosis criteria. They should therefore be used as a clinical and research framework rather than as an independently confirmatory diagnostic test.