AAN Summary of Evidence-based Guideline for CLINICIANS

CLINICAL EVALUATION AND TREATMENT

OF TRANSVERSE MYELITIS

This is a summary of the American Academy of Neurology (AAN) guideline update regarding treatment of transverse myelitis (TM).
Please refer to the full guideline at www.aan.com for more information, including definitions of the classifications of
evidence and recommendations.

For patients with myelopathy, which demographic, clinical, and laboratory features are useful to distinguish TM
from other causes of acute and subacute noncompressive myelopathy?
Demographic features
Weak evidence Age and gender may be considered useful to determine etiology in a patient presenting with TM syndrome,
with spinal infarcts seen more often in older patients and more female than male patients having TM due
to multiple sclerosis (MS) (Level C).
Clinical features
Insufficient There is insufficient evidence to determine whether clinical features of the myelopathy are associated
evidence with myelitis vs. other myelopathies (Level U).
Laboratory features
Weak evidence For patients with subacute myelopathies, an elevated CSF leukocyte count (greater than 10 cells/mm3)
may be considered useful in identifying patients with inflammatory myelopathies (including TM) as opposed
to those with spinal cord infarcts (Level C†).

For patients with myelopathy, which demographic, clinical, radiographic, and laboratory features are useful
to determine the cause of the myelitis?
Demographic features
Insufficient Due to considerable overlap between groups, patient demographic characteristics are not definitive
evidence in establishing the cause of myelopathy (Level U).
There is insufficient evidence to support or refute the usefulness of ethnicity to determine the cause
of a subacute myelopathy (Level U).
Clinical features
Weak evidence In patients with suspected TM, distinction between acute complete TM (ACTM) or acute partial TM (APTM)
may be considered useful to determine the etiology of TM (Level C).
Radiographic features
Weak evidence Brain MRI characteristics consistent with those of MS may be considered useful to predict conversion to MS
after a first episode of partial TM (Level C).
Longer spinal lesions extending over more than 3 vertebral segments may be considered useful in
determining NMO vs. MS (Level C).
Laboratory features
Moderate Neuromyelitis optica immunoglobulin G (NMO-IgG) antibodies should be considered useful to determine
evidence the cause of TM in patients presenting with clinical features of ACTM (Level B).
Weak evidence CSF analysis for OCBs may be considered in determining MS vs. other causes of TM, specifically for the
diagnosis of MS vs. NMO, spinal cord infarct, vasculitis, and parainfectious and idiopathic TM (Level C).
Analysis of CSF for pleocytosis may be considered in distinguishing NMO from MS and MS from all other
causes of TM (Level C†).
 For patients with myelopathy, which demographic, clinical, radiographic, and laboratory features are useful to
identify patients at increased risk for recurrence?
Demographic features
Insufficient There is insufficient evidence to determine whether demographic features are associated with relapsing
evidence TM (Level U).
Clinical features
Weak evidence In patients with suspected TM, distinction between ACTM or APTM may be considered useful to determine
the risk for relapse (more common in APTM) (Level C).
Radiographic features
Weak evidence Longer lesions on spinal MRI may be considered useful in predicting a higher risk of developing NMO
and therefore a higher risk of recurrent TM (Level C†).
Laboratory features
Moderate The presence of NMO-IgG antibodies (aquaporin-4–specific antibodies) should be considered useful
evidence in determining an increased risk of TM recurrence (Level B).

For patients with TM, which therapies alleviate acute attacks?

Plasma exchange
Weak evidence Plasma exchange may be considered in patients with TM who fail to improve after corticosteroid treatment
(Level C).
Rituximab
Weak evidence Rituximab may be considered in patients with TM due to NMO to decrease the number of relapses (Level C).
Other agents
Insufficient There is insufficient evidence to support or refute the efficacy of other therapies (Level U).
evidence
Clinical context Despite the absence of evidence, administration of high-dose IV methylprednisolone (1 g daily for 3 to 7 days)
is typically the first treatment offered to hasten recovery, reduce disease activity, and restore neurologic function.

For patients with TM, which therapies prevent future attacks?

Insufficient There is insufficient evidence regarding the use of other immunosuppressive strategies to reduce
evidence the risk of future TM attacks (Level U).

Policy Implications
TM is relatively uncommon, making reliable data very difficult and expensive to collect. However, the devastating effects of TM make
urgent treatment critical to try to minimize disability. In the likely absence of existing policy for treatment of TM from either government
programs or private insurers, medical review staff should allow for requests for individual consideration with each case [claim] with
reference to published articles or other resources—even if those resources are judged as “lower class” evidence—and further dialogue
in case additional data are needed. A good source on treatment protocols are those used in MS centers.

Based on an AAN guideline endorsed by the Consortium of MS Centers

† Recommendation not formally made in published guideline

This is an educational service of the American Academy of Neurology. It is designed to provide members with evidence-based guideline recommendations to assist
the decision making in patient care. It is based on an assessment of current scientific and clinical information and is not intended to exclude any reasonable alternative
methodologies. The AAN recognizes that specific patient care decisions are the prerogative of the patient and the physician caring for the patient, and are based on the
circumstances involved. Physicians are encouraged to carefully review the full AAN guidelines so they understand all recommendations associated with care of these patients.

©2011 American Academy of Neurology

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