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Spine - Myelopathy
Majda Thurnher and Robin Smithuis
Department of Radiology of the Medical University of Vienna, Austria and Rijnland hospital in Leiderdorp, the Netherlands

Introduction

Differential diagnosis

Systematic approach

Short or long segment involvement

Transverse involvement

Multiple Sclerosis

Neuromyelitis Optica

ADEM

Transverse Myelitis (TM)

Diseases associated with Transverse Myelitis


Spinal cord tumor
Astrocytoma
Vascular

Arterial infarction

Vasculitis

Spinal AVF

Cord compression
Publicationdate October 3, 2012
In this article we will focus on spinal cord diseases that are characterised by high signal
within the cord on T2WI.
The most common causes are inflammatory and demyelinating disorders like Multiple
Sclerosis, Neuromyelitis Optica, Acute Disseminating Encephalomyelitis and Transverse
myelitis.
We will discuss the differential diagnosis including tumors, inflammatory and vascular
disorders.
Introduction
Spinal cord abnormalities. Common causes in white and uncommon causes in yellow.

Differential diagnosis

1. Demyelinating diseases
The first question is
usually is it MS?
Does it look like spinal
cord MS and does the
brain look like MS.
If the patient has
myelopathy and optic
neuritis then also think
of the possibility of
Neuromyelitis Optica
(NMO) and do the
NMO-IgG test.
If both halves of the cord
are involved than think
of Transverse Myelitis
(TM) which is not a
specific diagnosis, but

merely a reaction of the


cord to various
autoimmune and
infectious stimuli.
2. Tumor
The major differential of
demyelinating diseases is
an astrocytoma,
especially if there is
swelling and some
enhancement of the cord
and when the symptoms
are more slowly
progressive.
The other common
spinal cord tumors like
ependymoma and
hemangioblastoma do
not cause differential
diagnostic problems,
because in most cases
they just look like a
tumor.
Metastases to the cord
are very uncommon.
3. Vascular
Acute ischemia is
typically seen as a
complication of aortic
aneurysm surgery or
catheterisation.
Cord ischemia due to
venous hypertension or
arterial steal can be seen
in vascular
malformations like AVfistula.
So always look for
abnormal vessels around
the cord.
4. Infammatory
Vasculitis
5. Infection
Infection rarely involves
the spinal cord.

If we exclude myelopathy due to cord compression as seen in trauma, degeneration and


metastatic disease, which is usually not a diagnostic dilemma, then the most common
diseases of the spinal cord are demyelinating diseases.
MS is by far the most common demyelinating disease.
The diagnosis can only be made if there is dissemination over time and space.
Many patients who are diagnosed as having acute disseminating encephalomyelitis (ADEM)
or Transverse Myelitis, may have recurrent disease and later turn out to have MS (red arrow).
Systematic approach

Whenever there is an abnormality in the spinal cord, we need a systematic approach to


analyse the findings.
Clinical findings can be helpful but can be quite similar in most spinal cord disorders.
On MR look for the following:

Short or Long segment?


In MS there is typically a
short segment involved,
i.e. less than 2 segments.
In other diseases like
Transverse Myelitis,
NMO and ischemia there
is usually a long segment
involved.

How much of the cord is


involved on tranverse
images?
Partial involvement is
typically seen in MS.
Complete involvement
includes both halves of
the cord and is typically
seen in TM and NMO.

Location of the
involvement on
transverse images?
Use high resolution
transverse images to
detect the location within
the cord. Is it posterior
like in MS, vitamin B12
deficiensy, lateral like in
MS or anterior like in
arterial infarction.

Is the cord swollen?


In TM and tumor the
cord is swollen, while in
MS and ADEM the cord
is not swollen or less
swollen then you would
expect for the size of the
lesion.

Is there enhancement?
Many diseases show
some enhancement, but
the most important thing
is that astrocytoma has to
be included in the
differential diagnosis.

Short or long segment involvement

1. Short segment
involvement
o common in:

MS

o uncommon in:

Transvers
e myelitis
- partial
form

2. Long segment
involvement
o common in:

Transvers
e myelitis
complete
form

Neuromy
elitis
Optica

o uncommon in:

MS

Transverse involvement

Transverse images are very helpful in the differential diagnosis.


You need high resolution images.
Look for how much is involved (both halves or not), which part is involved and what is the
form of the involvement.

MS typically is
triangular in shape and
mostly located dorsally
or laterally.
However MS can look
like anything and may
uncommonly involve the
whole transverse
diameter or only the
anterior part.

Ischemia as a result of
arterial infarction is
typically located in the
anterior parts, but may
involve the entire
transverse diameter.

Transverse myelitis and


Neuromyelitis optica
typically involve the
whole cord.

Brain abnormalities
In many cases of myelopathy there will also be brain abnormalities and these can be a
diagnostic clue to the diagnosis.
Multiple Sclerosis
MS: short segment focal wedge-shaped involvement of the posterior column of the spinal cord with typical periventricular WM-lesions.

key facts:

MS is an immunemediated inflammatory
demyelinating disease of

the brain and the spinal


cord.

Multiple lesions
disseminated over time
and space.

Brain lesions are


typically in
periventricular,
subcortical and
cerebellar white matter
and also in brainstem
and corpus callosum.

CSF: monoclonal bands.

MS is the most common demyelinating disease and there is overlap between these diseases.
NMO was first thought to be a form of MS, but is now considered to be a distinct form.
ADEM can relapse and progress to MS.
The partial form of transverse myelitis
Here we have images of a typical case.
Many times the clinical history is very helpful like in this case.
This 24-year old patient had visual disturbances on one eye followed by weakness and
sensory disturbances of the lower and upper extremities a couple of years later.
Now she presents with sensory disturbances of both lower extremities.
So we already think MS.
In the cord there are some well-defined lesions, but also some ill-defined foggy lesions.
The transverse image shows the dorsal location and the typical triangular shape.
Continue with the contrast-enhanced images

On the contrast-enhanced images there is no enhancement.


Active MS-lesions in the spine may enhance, but it is not that common as we see in active
lesions in the brain.
Whenever spinal lesions are encountered, it can be helpful to image the brain aswell.
Now sometimes the patient is only sceduled for MRI of the spine and you don't have time to
do a full brain examinations.
In those cases consider to do only a sagittal FLAIR.
Continue with the images of the brain.
The MRI of the brain shows periventricular lesions and a lesion in the corpus callosum.
These locations are very specific for MS.

MS with non-specific findings in the spinal cord.

In another patient there are non-specific lesions in the cord.


Based on the examination of the spine alone, we have a broad differential diagnosis.
However when we examine the brain, it becomes obvious that we are dealing with MS.
Continue with the images of the brain.
Typical MS in the brain

In this case the findings in the brain are very helpful.


The location of the lesions is very typical: pons, periventricular and subcortical.
MS

Now what can we expect in the spinal cord of patients with MS.
It is like in the brain.
Frequently the lesions are focal like we see on the left image.
Less commonly there are diffuse abnormalities and then we have a tough differential
diagnosis which will include TM and NMO.
In long standing cases atrophy will be seen.
One third of MS patients will have spinal symptoms.
One third of patients have isolated spinal MS without any findings in the brain.
However pathologic studies have shown that 95% of MS patients have spinal cord lesions,
whether they have spinal symptoms or not.
MS in the cord

On transverse images MS lesions typically have a round or triangular shape and are located
posteriorly or laterally.

So can we exclude MS if a lesion is located anteriorly?


Unfortunately not.
MS is the great mimicker and can also be located anteriorly like in this patient who has a
lesion in the typical location (blue arrow) but also a lesion ventrally in the cord (red arrow).
This is uncommon, but you can not exclude MS.
When MS lesions are active, they can enhance, but enhancement is not as common as in the
brain.
The enhancement patterns are non-specific.
You can see ring enhancement, intense and less-intense enhancement.
The less intense or vague enhancement is the most common pattern.
Acute exacerbation of cerebral and spinal MS

These are images of a patient with longstanding MS and acute exacerbation.


There is enhancement in the active lesions.
Continue with the images of the cervical spine.

Acute exacerbation of cerebral and spinal MS

Also in the spinal cord there are multiple lesions.


On the transverse image a typical triangular shaped dorsal lesion is seen.
Continue with the contrast-enhanced images.
Acute exacerbation of cerebral and spinal MS

Also in the spine there are multiple enhancing MS-lesions.


Diffuse abnormalities in MS. Courtesy of Sundgren Pia, Lund, SE

MS usually presents with focal abnormalities.


Diffuse abnormalities that can look like transverse myelitis or extensive astrocytoma are
sometimes seen.
This pattern is more common in primary progressive and secondary progressive MS.
Atrophy in longstanding MS

Some say that spinal cord atrophy is specific for primary progressive MS (PPMS).
The atrophy correlates very well with the clinical disability.
It is more prominent in the upper part of the spinal cord.
Duration of the disease is the most important determinant of cord atrophy.

Neuromyelitis Optica
NMO presenting with neuritis optica (arrows). The brain is normal.Courtesy Andrea Rossi

key facts:

Neuromyelitis Optica
(NMO) is an
autoimmune
demyelinating disease
induced by a specific
auto-antibody, the NMOIgG.

NMO preferentially
affects the optic nerve
and spinal cord.

Brain lesions do occur


and often are distinct
from those seen in MS.

Demyelination of the
spinal cord looks like
transverse myelitis, i.e.

often extensive over 4 -7


vertebral segments and
the full transverse
diameter.

NMO IgG is a specific


biomarker for NMO.

Female:male = 9:1

Also called Devic


disease

On the left images of a child who presented with unilateral neuritis optica.
Images of the brain were otherwise normal.
Continue with MRI of the spine.
Patients who have one episode of optic neuritis or myelitis and who test positive for NMOIgG are at high risk for developing the full spectrum of NMO.

One month later this child presented with acute transverse myelopathy, i.e. bilateral
symptoms.
The images show abnormal signal in the spinal cord with swelling and some enhancement.
An astrocytoma could very well present with these images, but given the history of an optic
neuritis and the acute myelopathy, we do not think of a tumor.
This proved to be NMO and the Ig-test for NMO was positive.
In the original description of Devic's disease the optic neuritis and the myelopathy were
simultaneously, but now we know that this is not always the case.
Periventricular lesions in NMO around fourth and third ventricle.

Brain lesions in NMO


Previously it was thought that in NMO the brain was spared, but now we know, that brain
lesions do occur.
They are often distinct from those seen in MS.
In Asia 60-80% of patients with NMO have brain abnormalities.
In Europe only 25-40%.
The location of the brain lesions in NMO is only around the ventricles.
Periventricular lesions in NMO around third and lateral ventricles.

The reason why these brain lesions are located around the ventricles is the following:
The NMO IgG auto-antibodies are directed against Aquaporin-4 water-channels.

So just like sodium- and potassium channels in the cells, there are also water-channels.
The highest concentration of these Aquaporin-4 water-channels is seen around the ventricles.
The images show abnormal signal around the third and frontal horns of the lateral ventricles.
Neuromyelitis optica with callosal lesions. Courtesy Dr Nakamura.

Things become even more complicated, since it is also possible to have large lesions in the
corpus callosum of patients with NMO as was described by Nakamura (6).
So in any CNS disease with optic nerve and spinal cord involvement it is goos to do the test
for NMO-IgG.
ADEM
ADEM

key facts:

Acute disseminated
encephalomyelitis
(ADEM) is an
inflammatory
demyelinating disease of
the CNS after viral
infection or vaccination.

In 75% of patients there


is a clear infectious event
or vaccination (1-4
weeks)

Typically monophasic in
90%.

Multiphasic illness in
10%. In these cases
ADEM behaves like MS
and cannot be
differentiated from MS.

Mostly seen in young


children.

In 50% of ADEM
patients the anti-MOG
IgG test is positive and
supports the diagnosis.
This is antibodyreactivity against Myelin

Oligodendrocyte
Glycoprotein (MOG).

On the left images of a teenage child with a typical history:

Three weeks after


respiratory infection
sudden onset of
neurological symptoms.

Dysarthria, dysphagia,
tetraplegia.
Eye movement
disturbance and
impairment in
consciousness.

This clinical history is typical for ADEM.


Usually the brain is also involved.
30% of cases has spinal involvement.
The imaging findings in this case are also typical.
There is swelling and cord involvement like in TM and no enhancement.
Continue with the images of the brain.
ADEM

First look at the images of the brain and decide what is different from MS-lesions.
What is typical for ADEM and uncommon for MS is:

Massive involvement of
the pons.

Involvement of the basal


ganglia.

ADEM - follow up

The follow up MR shows that the cord has returned to normal again.
On the left another case of ADEM.
ADEM is typically seen in children.
Again there is diffuse involvement of the spinal cord without enhancement and there is
involvement of the brain.

Typical ADEM. Courtesy of Andrea Rossi, Genova, IT

Another case of ADEM.


Notice the typical involvement of the pons and basal ganglia.
Continue with follow up scan.
Courtesy of Andrea Rossi, Genova, IT

On follow up scan almost complete normalisation.


Transverse Myelitis (TM)
key facts:

Focal inflammatory
disorder of the spinal
cord resulting in motor,
sensory and autonomic
dysfunction.

Imaging findings:

o More than 2/3 of


the cross
sectional area is
involved.
o Focal
enlargement.
o T2WI
hyperintensity

o Enhancement + /
-.

Two forms of TM:


o Acute partial
transverse
myelitis - APTM
Lesions
extending less
than two
Segments.
These patients
are at risk of
developing MS.

o Acute complete
transverse
myelitis - ACTM
Lesions
extending more
than two
Segments .

The sagittal image shows a large segment of hyperintensity on T2WI.


The transverse image shows that most of the cord is involved.
ATM on T2WI, CE-T1WI and STIR.

These images are of a 31 year old male with headache, voiding disturbances, urinary
retention, sensory level C3.
The CSF analysis revealed 400/3 cells (meaning no infection) and a slightly higher protein
level.
The images show a long segment myelopathy with full transverse involvement.
There is no swelling and no enhancement.
It does not look like MS or tumor, so we are thinking ATM - acute transverse myelitis.
Diseases associated with Transverse Myelitis

Transverse myelitis may occur in isolation or in the setting of another illness.


When it occurs without apparent underlying cause, it is referred to as idiopathic.
Idiopathic transverse myelitis is assumed to be the result of abnormal activation of the
immune system against the spinal cord.
The table provides a list of illnesses associated with TM (7).
Patients with an acute short segment TM (or APTM) are at risk of developing MS if there is
one of the following:

partial TM, i.e. short


segment TM.

Family history of MS.


Brain lesions on MR.

Oligoclonoal bands in
CSF.

In children with TM the expression is usually more severe.


60-90% are unable to move their legs. However the outcome is usually better than in adults
and in 30-50% there is complete recovery.

Typical for TM is that on the initial MR the abnormalities are usually extensive and less or
completely resolved on follow up scans.
Longitudinal case series of TM reveal that approximately 1/3 of patients recover with little to
no sequelae, 1/3 are left with moderate degree of permanent disability, and 1/3 have severe
disabilities.
Here images of a typical case of TM.
There is multisegment high signal on STIR and T2WI with some swelling.
Most of the cord in the transverse diameter is involved.
There is no enhancement, which is usually the case in TM.
Sometimes there is some patchy enhancement.
Astrocytoma simulating Transverse myelitis

When there is enhancement, it can be difficult to differentiate TM from an astrocytoma.

On the left images of a 60 year old male with an astrocytoma.


He presented with pain in the thoracic region and sensory disturbances in the left lower
extremity followed by left hemiparesis.
There is multisegment high signal on T2WI with some swelling, just like we have seen in
cases of TM.
On CE-T1WI there is a region of enhancement.
The region of enhancement is more tumor-like, but the differentiation is difficult.
Spinal cord tumor

Spinal astrocytoma

Astrocytoma

As we have just seen, the major differential of the spinal cord diseases that we have discussed
so far is an astrocytoma.
Astrocytoma is a diffusely infiltrating tumor, that is not mass-like.
Usually there is some patchy enhancement.
On the left an astrocytoma in a 66 year old patient who presented with progressive sensory
complaints.
Biopsy revealed astrocytoma.
Continue with the follow up.
Spinal astrocytoma - follow up

The patient was not operable and a follow up scan shows progressive disease.
Multiple ependymomas

The other two common spinal cord tumors are ependymoma and hemangioblastoma and they
just look like a tumor.

They present as enhancing masses and will not cause a differential problem.
All other cord tumors are uncommon.
The images are of a patient with neurofibromatosis who has multiple ependymomas.
They present as multiple enhancing masses.
Vascular
Arterial infarction

Spinal cord ischemia is typically seen as a complication of aortic aneurysm surgery or


stenting.
The images are of a patient who developed a paraparesis after stenting of an aortic aneurysm.
Notice the high signal ventrally in the chord, which is typical for arterial infarction.
On transverse images a typical snake-eye appearance can be seen.
DWI in spinal chord ischemia

Aortic aneurysm stenting is the most common cause of spinal chord infarction.
The diffusion images show restricted diffusion.

Vasculitis

The images are of a child with headache, fever, hyperalgesia and numbness of the left side of
the body.
There is also abducens nerve palsy.
The images are non-specific with multiple focal lesions and probably the first choise would
be MS.
The differential diagnosis would include inflammation, infection and metastases.
In such cases always perform a transverse image of the spine to look for the exact location
and perform a MRI of the brain.
Continue.

The lesions are located dorsally and one of the lesions is enhancing.
Now if this was infection or metastases it would be strange that not all lesions enhance.
MS is still on our list.
Continue with the MR of the brain.

Idiopathic Vasculitis

On the CE-T1WI only one lesion shows enhancement.


The location of the lesions and the enhancement could very well fit to the diagnosis of MS,
but this proved to be vasculitis.

Vasculitis can be idiopathic, but is also seen in SLE, Sj?gren and Behcet.
Normally you think of vasculitis as a disease of the vessels in the brain, but all vasculitis can

be seen in the spine as well.


It produces MS-like images.
Spinal AVF

The most common vascular malformation of the spinal cord is the dural AV-fistula.
It consist of an abnormal connection between the artery and the veins , which can lead to
increased venous pressure and predisposes the cord to ischemia and less commonly to
hemorrhage.
AVF's are mostly seen in the elderly population and are believed to be the result of trauma.
An accurate diagnosis is important because these lesions may represent a reversible cause of
myelopathy.
Notice the high signal in the lower thoracic cord and the surrounding dilated vessels on the
T2WI.
On the enhanced T1WI there is subtle enhancement.
Another case with myelopathy and dilated veins as a result of an AVF.

Another patient with myelopathy and dilated vessels surrounding the cord.
Notice the hypointense areas on the T2WI which represents hemorrhage.
Another AVF with myelopathy and dilated vessels.
Cord compression
Although beyond the scope of this article, the most common cause of myelopathy is cord
compression as seen in trauma, metastatic disease and epidural hemorrhage.
This patient has a fracture with posterior displacement.
There is myelopathy due to traumatic cord compression.

Another case of cord compression in a patient who was treated with anticoagulantia.
There is myelopathy as a result of compression by a dorsally located epidural hemorrhage.
The most common cause of cord compression is metastatic disease.
Notice the abnormal signal in the vertebral body as a result of a metastasis which extends into
the vertebral canal.
1. by Kerr et al
Neurology. 2002 Aug 27;59(4):499-505
2. Imaging in acute disseminated encephalomyelitis

by Andrea Rossi
Neuroimaging Clinics of North America, Volume 18, Issue 1, Pages 149-161
3. An Approach to the Diagnosis of Acute Transverse Myelitis (PDF)
by Anu Jacob, M.D., and Brian G. Weinshenker, M.D., F.R.C.P.
4. Devic's Disease (Neuromyelitis optica)(NMO) Notes
5. The spectrum of neuromyelitis optica
by Dean M Wingerchuk, Vanda A Lennon, Claudia F Lucchinetti, Sean J Pittock,
Brian G Weinshenker
The Lancet Neurology, 2007, 6:805-15
6. Occurrence of acute large and edematous callosal lesions in neuromyelitis optica.
by Nakamura M, Misu T, Fujihara K, Miyazawa I, Nakashima I, Takahashi T,
Watanabe S, Itoyama Y.
Mult Scler. 2009 Jun;15(6):695-700
7. Transverse Myelitis: Symptoms, Causes and Diagnosis
by Joanne Lynn, M.D.

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