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ACUTE

TRANSVERSE
MYELITIS
Elinore Kaufman MS III
Patient JK: History
… JK is a 46 yo RH woman with a history of
diabetes, hypertension and disc disease who
presented with a 4d history of neck pain and
progressive weakness.
Course of illness:
† 5d PTA: slight sore throat
† 4d PTA: awoke with sore, stiff neck, relieved
with ibuprofen.
† 3d PTA: difficulty holding pen to sign name
† 2d PTA: difficulty walking; dragging right foot.
Patient JK: Physical Exam
… Vital signs: T: 98.7 P: 86 R: 18 BP: 136/70 SaO2: 98% RA
… General remarkable only for 2cm papular rash on L upper chest.
… Neurological examination:
† Mental Status: intact
† Cranial Nerves II-XII: intact
† Motor: Normal bulk and tone in upper and lower extremities. No tremor or asterixis.
† Strength:

Delt Bic Tric WE WF FE FF IP Qu HS TA Gas EHL

L 5 5 5 5 5 5 5 5 5 4+ 5 5 5
R 4+ 4 4+ 4+ 5 4 4+ 4+ 5 4+ 4+ 5 4

† Reflexes: Left: intact. Right: inverted biceps. Babinski: mute


† Sensation: Light touch intact throughout. Pinprick diminished in R C5 distribution and L leg.
† Coordination: No evidence of resting or intention tremor or dysmetria, allowing for weakness.
† Gait: Using walker with good initiation and right steppage gait
Now we will examine imaging
techniques for the spinal cord
and spinal anatomy.
Imaging Myelopathy
… Plain films
† Stability after trauma
† Osteophytic narrowing of spinal canal
… CT: better assessment in trauma
… Spine MRI: MRI: study of choice for cord and soft tissues, as well as for
surgical planning
† Gadolinium contrast, especially if AVM is in the differential
† High sensitivity
… CT myelogram if MRI is unavailable or contraindicated
† Invasive
† Identifies only large masses
† Does not distinguish between cystic and solid lesions
… Radionucleide bone scan can be useful in cases of tumor and infection
… Brain MRI
† Associated demyelinating lesions

(Seidenwurm 2008)
Companion Patient 1: Normal
Cervical Spinal MRI
Cerebellum

Brain Stem

Disc

Spinal Cord
Nerve Root
CSF

Spinous process

Vertebral Body

Sagittal and axial views of cervical spinal cord on T2-weighted MRI (PACS BIDMC
).
Now we will return to our patient
JK to discuss her imaging
findings and diagnosis.
Patient JK: Sagittal MRI of Cervical Spine

Sagittal T2-weighted image Sagittal T1-weighted image


showing disc bulge and after IV gadolinium
hyperintense lesion
within spinal cord. administration showing ring
(PACS BIDMC) enhancing lesion. (PACS BIDMC)
Patient JK: Enhancing lesion on axial
T1-weighted Image with Contrast

NORMAL

Enhancing lesion seen on axial T1 image,


post-contrast (PACS BIDMC)
Work-up of Acute Myelopathy

Patient JK
Adapted from Jacob 2008
Differential Diagnosis of Acute Myelopathy

Noninflammatory Inflammatory

… Compression … Demyelinating disease


† Osteophyte † Multiple sclerosis (MS)
† Disc † Neuromyelitis optica (NMO)
† Metastasis † Acute disseminated encephalomyelitis
† Trauma (ADEM)
† Idiopathic acute transverse myelitis (IATM)

… Tumor … Infection
† Viruses: coxsackie, mumps, varicella, CMV

… Paraneoplastic † Tuberculosis

syndromes † Mycoplasma

… Inflammatory disoders
† Systemic lupus erythematosus
† Neurosarcoidosis
Jacob 2008, Seidenwurm 2009
Imaging Findings in ATM
Condition MRI Spinal Cord MRI Brain
MS Oval‐shaped lesion(s) < 2 spinal cord  White matter lesions: 
segments, usually peripheral location.   periventricular, juxtacortical, 
Hyperintense on T2 with  infratentorial lesions. 
homogeneous or ring enhancement. Dawson’s Fingers.
NMO lesion >3 segments;  cord swelling and  Optic neuritis. 
gadolinium enhancement acutely. Cerebral lesions in 60%.
ADEM lesion >3 segments;  cord swelling and  Large, confluent white matter 
gadolinium enhancement acutely. lesions.
IATM Lesion of variable length and shape,  No brain lesions.
>50% of cross‐sectional area on axial 
scan.  Hyperintense on T2‐weighted  
images, variable hypointensity on T1, 
patchy enhancement and swelling. 

Jacob 2008, O’Riordan 1996, Scotti 2001, Tartaglino 1996


Idiopathic Acute Transverse Myelitis
… Incidence: 1-4 per million per year
… Diagnosis
† Myelopathic symptoms
„ 50% lose all leg movements
„ Nearly 100% have bladder dysfunction
„ 80-94% of patients sensory dysfunction
† MRI evidence of noncompressive myelopathy
† Inflammation: CSF pleocytosis, elevated CSF IgG index, or gadolinium
enhancement
„ if no evidence but strong suspicion, repeat MRI/LP in 2-7 d
† Exclude: cord radiation, thromboembolic disease, AVF, connective tissue
disease, infection, MS/ADEM, malignancy
… Pathology: edema, necrosis, demyelination, white>gray matter
changes (Misra 1996)
… Prognosis
† 1/3 recover without sequelae
† 1/3 have moderate, permanent disability
† 1/3 have severe disability (Transverse Myelitis Consortium Working Group 2002)
Radiologic Findings in IATM
… Of patients with ATM and normal brain MRI, none with
normal CSF progressed to MS.
† 53% of those with CSF oligoclonal bands or elevated IgG
index developed MS. (Perumal 2008)
… Spinal cord atrophy may be evident on follow-up MRI.
(Shen)
… Fractional anisotropy
† Shows water diffusion in the extracellular space
along the axon fibers
† Higher sensitivity than T2 for white matter damage:
can identify lesions in normal-appearing cord
† Decreased values may demonstrate increased
extracellular space due to demyelination, and may
predict greater wallerian degeneration and worse
prognosis. (Renoux 2006, Lee 2008)
Now we will examine other
causes of acute transverse
myelitis.
Companion Patient 2:
Transverse Myelitis in MS

Axial T2-weighted images from MS patient


(Jacob 2008)
… MRI Spine
† Lesions <2 segments
† No cord swelling
Sagittal T2-weighted image from † Peripheral lesions (Jacob 2008)
MS patient (Scotti 2001) † Differential: 1-3% of lupus patients
develop ATM with similar findings
to MS. (Scotti 2001)
Risk of Developing MS after
ATM
… MR Brain
† >= 2 lesions: 88% develop MS. (Jacob 2008)
† No brain lesions: 10-33% develop MS.
… Mean time to MS: 16m, no new cases >24m.
… Next evidence of MS is more likely to be clinical than MRI
based (serial MRI does not speed diagnosis
… African American ATM patients are more likely to develop MS
than Caucasian patients: (35% vs. 26%).
† African Americans develop MS more quickly, in 9.6 months vs.
20.3 for Caucasions.
† African Americans experience greater disability from this disease
despite earlier use of disease-modifying therapy.
(Perumal 2008).
Companion Patient 3: Dural
arteriovenous fistula on MRI

Longitudinal hyperintense lesion on T2; contrast enhancement of dilated blood vessels: AVF
(Jacob 2008)

… AVMs and dural arteriovenous fistulae can either bleed or cause increased
venous pressure and progressive myelopathy; rarely acute onset.
… Anterior spinal artery occlusion: atherosclerosis or complication of aortic surgery:
peak within 4h (Jacob 2008)
… Vasculitis can cause cord swelling followed by cord atrophy. (Scotti 2001)
Companion Patients 4 & 5: Spinal
Cord Tumor on MRI
Sagittal T2 image of lesion initially Sagittal T2 image of lesion initially
thought to be tumor, ultimately thought to be inflammatory, ultimately
determined to have an inflammatory determined to be a tumor. (Jacob
cause. (Brinar 2006) 2006)
Companion Patient RB: History
and Physical Exam
… RB is a 32yo LH woman who presented with
severe headache and worsening neurological
symptoms:
Course of illness:
† 3 wks of severe R-sided retro-orbital headache
† 2 wk of numbness in both legs up to the navel
† 1 wk of R eye blurry vision
† 3d of urinary retention
† 1d of bowel incontinence

… Physical exam confirmed severe vision loss,


decreased pinprick sensation to the T8 level, and
upper motor neuron weakness in the legs.
Companion Patient RB: Spinal lesions and
leptomeningeal enhancement on MRI

Post-contrast T1-weighted image showing three enhancing lesions


and linear leptomeningeal enhancement. (PACS BIDMC)
Companion Patient RB: Clinical
Course
… She was treated with acyclovir (possible CNS HSV),
ciprofloxacin (E. Coli UTI) and methylprednisolone.

… Her vision improved, and she was discharged

… 1 wk later, she returned with fever and severe L-sided


retro-orbital headache. Her other symptoms were stable,
but her vision was now extremely blurry in her left eye.

… She was readmitted and received plasmapheresis with


slight improvement, but her symptoms worsened again
after discharge.
Companion Patient RB: Diagnostic
Work-Up
… Brain MRI showed only the optic neuritis.
… CSF showed pleocytosis and elevated protein but no oligoclonal
bands.
… Extensive CSF and serum studies showed no evidence of CNS
infection, collagen vascular disease or malignancy.
… Preliminary diagnosis: Neuromyelitis Optica
† Atypical features
„ Spinal cord lesion length <3 segments
„ Presence of leptomeningeal enhancement more typically associated
with lymphoma, sarcoidosis or Lyme disease
„ Absence of NMO antibody in serum and CSF.
… Chest CT showed no evidence of sarcoidosis or lymphoma
… PET scan was performed to rule out lymphoma and showed no
lymphadenopathy, bone lesions, or other abnormal uptake.
… Multiple sclerosis
Summary
… Acute myelopathy is most often compressive but
otherwise may be infectious, inflammatory or
idiopathic
… MRI is the imaging modality of choice for the spinal
cord, although CT myelography may be used if
needed.
… Imaging locates the lesion and can provide clues to
the diagnosis but often leaves a wide differential.
† MS: other CNS lesions
† NMO: lesion >3 segments
† Idiopathic ATM: diagnosis of exclusion
Acknowledgements
Many thanks to:
… Dr. Daniel Cohen, Dr. Jed Barasch, Dr.

Jennifer Avallone and Dr. Courtney McIlduff of


the BIDMC Neurology service
… Maria Levantakis

… Dr. Gillian Leiberman


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