Table E-1 Proposed Criteria for Transverse Myelitis

Table e-1 Proposed criteria for transverse myelitis

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Consortium Working Group criteria for idiopathic

acute transverse myelitis (6)

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Sensory, motor, or autonomic

dysfunction attributable to the spinal cord

Bilateral signs and/or symptoms (not necessarily symmetric),

clearly defined sensory level

Exclusion of other forms of demyelinating disease (e.g.,

no cerebral lesions suggestive of MS, consider: infectious

and autoimmunity serum tests, evoked potentials, etc.)

Spinal cord inflammation demonstrated by CSF pleocytosis or

elevated IgG index or gadolinium-enhanced MRI

Progression to nadir between 4 h and 21 days

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Table e-2 Association between Demographic Variables and Cause of Myelopathy

Author/Year / Design / Data collection / Sample Size / Predictor Variables / Causes of Myelopathy / Method of outcome assessment / Measure of Association
(CI by Wald method for gender association)
Jeffrey9(1993) / CS
Class III / Retrospective / 33 / Age
Gender / Infarct
ATM
MS / NI / No significant gender difference in idiopathic TM
CI:0.54–0.97)
Age : Difference in means only
(SCI = 52 / others = 33.9) insufficient data for t test
deSeze8
(2001) / CS
Class III / Retrospective / 79 / Age
Gender / Infarct /
Other diagnosis / NI / Female predominate in MS (CI:0.51–0.81) and in myelitis overall (CI:0.51–0.77), but not in idiopathic TM (CI: 0.23–0.61)
………………….
Age : Difference in means only
(SCI = 67 / others = 38.3), insufficient data for t test)
Scott (1998)13 / CS
Class III / retrospective / 36 / Age
Gender / MS
ATM / NI / Female predominant in
MS CI 0.63–0.98 (by Wald method) CI 0.50–0.80 for whole group. CI 0.30–0.70 for idiopathic TM.
……………………………….
Age: NS differences (t test)
Bakshi (1998)14 / CS
Class III / retrospective / 22 / Age
Gender / MS
ATM / No gender predominance (CI:0.19–0.81)
For MS, ATM, or whole group.
……………………………….
Age: NS differences (t test)

CS: Cohort Study. CC: case control study. R: Retrospective data collection. P: Prospective data collection. M: Masked. I: Independent. NI: Not independent. CI: Confidence interval. NS: Nonsignificant.

Table e-3 Proposed diagnostic criteria for neuromyelitis optica (NMO)

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Definite NMO

Optic neuritis

Acute myelitis

At least two of three supportive criteria

Contiguous spinal cord MRI lesion extending over ≥3 vertebral

segments

Brain MRI not meeting diagnostic criteria for multiple sclerosis
NMO-IgG seropositive status

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/ Cases n tested(n positive) / Controls n tested(n positive) / Methods / Diagnostic properties
Reference / # tested / NMO / OSMS / Inaugural/ Limited forms of NMO / MS or other IIDD / OND or AID / Healthy / Detection Assay / Gold Standard comparison / Comparison / Sensit. / Specif. / LR+ / LR-
Lennon et al, Lancet 200431 / 124 / 45 (33) / 8 RON (2); / 22 MS w/ ON or TM; (2) / 56 (0) / None / Indirect IF(mouse tissue) / Wingerchuk et al.1999 / NMO vs MS / 73% / 91% / 8.07 / 0.29
27 Relapsing TM (14) / 19MS w/o ON or TM (0) / NMO/limited forms vs MS / 61% / 91% / 6.73 / 0.42
Nakashima et al., JNNP 200621 / 35 / 19 (12) / None / 13CMS (2); 3 Spinal cord MS (0) / None / None / Indirect IF(mouse tissue) / Wingerchuk et al.1999 / NMO vs MS / 63% / 85% / 4.11 / 0.44
Scott et al., Arch Neurol. 200620 / 32 / 4 (3) / None / 6 MS (0); 22 TM w/o LETM (1) / None / none / Indirect IF(mouse tissue) / Wingerchuk et al.1999 / NMO vs Controls / 75% / 95% / 16.5 / 0.26
Tanaka et al., Mult Scler. 200732 / 91 / 26 (16) / None / 6 OSMS w/o (0) LETM / 28(0) / 10 (0) / Indirect IF(AQP4 transfected cells) / Not mentioned / NMO vs MS / 62% / 100% / Inf. / 0.38
21 MS (0)
Takahashi et al., Brain 200733 / 148 / 22 (20) / 13 (11) / 53 MS (0) / 50 (0) / none / Indirect IF(AQP4 transfected cells) / Wingerchuk et al.2006 / NMO vs MS / 91% / 100% / Inf. / 0.09
10 CIS (0) / NMO/limited forms vs MS / 85% / 100% / Inf. / 0.15
Matsuoka et al., Brain 200722 / 200 / 48 (13) / 11 Brainstem -spinal MS (0); / 54 MS (3) / 52 (0) / 35 (0) / Indirect IF(AQP4 transfected cells) / Kira et al, 1996 (OSMS) / OSMS vs. CMS / 56% / 94% / 10 / 0.47
4 TM (1)
Paul et al., PLoS Med. 200734 / 334 / 37 (21) / 6 LETM (6) / 144 MS (4) / 73 (1) / 29 (0) / RIPA / Wingerchuk et al.1999 / NMO vs. MS / 57% / 98% / 12.49 / 0.45
NMO+LETM vs. MS / 63% / 98% / 13.81 / 0.39
Saiz et al., J Neurol. 200735 / 161 / 16 (10) / 7 LETM (3) / 115 MS (0) / None / None / Indirect Immunohistochemistry (rat brain) / Wingerchuk et al.1999; Wingerchuk et. al 2006 / NMO vs. MS / 63% / 100% / Inf. / 0.38
7 RON (1) / 4 TM w/o LETM (0) / NMO/limited forms vs MS / 45% / 100% / Inf. / 0.55
Tanaka et al., JNNP 200736 / 128 / 45 (25) / None / 64 MS (0) / None / None / Indirect IF(AQP4 transfected cells) / Cases were MS w/LETM / MS w/LETM vs MS w/o LETM / 56% / 100% / Inf. / 0.44
Banwell et al., Neurology 200737 / 87 / 17 (8) / 13 ON (1); / 41MS (0) / None / None / Indirect IF (mouse tissue) / Wingerchuk et al. 1999 / NMO vs. MS / 47% / 100% / Inf. / 0.53
10 LETM (1) / 3 TM w/o LETM (0)
3 ADEM (0) / NMO/limited forms vs MS / 24% / 100% / Inf. / 0.76
Marignier et al, MultScler. 200838 / 135 / 26(14) / 13 LETM (7) / 52 MS (5) / 43 (0) / None / Indirect IF (mouse tissue) / Wingerchuk et al. 1999 / NMO vs MS / 54% / 90% / 5.6 / 0.51
21 ON (4) / 8 TM w/o LETM (0) / NMO/limited forms vs MS / 45% / 95% / 4.64 / 0.61

OSN= opticospinal MS, RON=relapsing optic neuritis, LETM=longitudinally extensive transverse myelitis, ADEM=acute disseminated encephalomyelitis, IF=immunofluorescence

Table e-5 Additional Studies of Interest

Disease processes to be ruled out / Example of studies to consider
Viral myelitis / Virus-specific PCRs
Connective tissue disease, vasculitis / ANA, cANCA, pANCA, anticardiolipin
antibodies
Dural AVM / Spinal angiogram
Sarcoidosis, lymphoma (usually slower onset) / ACE, chest CT, sedimentation rate, CSF, MRI evidence of chronic meningitis/multifocal enhancing lesions
Paraneoplasia / Paraneoplastic autoantibody panel, including CRMP

PCR=polymerase chain reaction, ANA=antinuclear antibodies, cANCA= antineutrophil cytoplasmic antibodies, pANCA = protoplasmic-staining antineutrophil antibodies, ACE= angiotensin-converting enzyme, CT= computed tomography, CRMP = collapsin Miscellaneous studies: Evoked potentials, STIR MRI spinal, serial cerebral MRIs