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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
Transverse involvement
Multiple Sclerosis
Neuromyelitis Optica
ADEM
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
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 there enhancement?
Many diseases show
some enhancement, but
the most important thing
is that astrocytoma has to
be included in the
differential diagnosis.
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
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.
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
Multiple lesions
disseminated over time
and space.
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
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.
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.
Demyelination of the
spinal cord looks like
transverse myelitis, i.e.
Female:male = 9:1
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.
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.
Typically monophasic in
90%.
Multiphasic illness in
10%. In these cases
ADEM behaves like MS
and cannot be
differentiated from MS.
In 50% of ADEM
patients the anti-MOG
IgG test is positive and
supports the diagnosis.
This is antibodyreactivity against Myelin
Oligodendrocyte
Glycoprotein (MOG).
Dysarthria, dysphagia,
tetraplegia.
Eye movement
disturbance and
impairment in
consciousness.
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.
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.
Focal inflammatory
disorder of the spinal
cord resulting in motor,
sensory and autonomic
dysfunction.
Imaging findings:
o Enhancement + /
-.
o Acute complete
transverse
myelitis - ACTM
Lesions
extending more
than two
Segments .
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
Oligoclonoal bands in
CSF.
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
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
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
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
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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