Transverse myelitis (in latin nomenclature: myelitis transversa)
is a neurological disorder caused by an inflammatory process of
the spinal cord, and can cause axonal demyelination. The name is
derived from Greek myelós referring to the "spinal cord", and the
suffix -itis, which denotes inflammation. Transverse implies that
the inflammation is across the thickness of the spinal cord.
Presentation
This demyelination arises idiopathically following infections or
vaccination, or due to multiple sclerosis. One major theory posits
that immune-mediated inflammation is present as the result of
exposure to a viral antigen.
The lesions are inflammatory, and involve the spinal cord
typically on both sides. With acute transverse myelitis, the onset
is sudden and progresses rapidly in hours and days. The lesions
can be present anywhere in the spinal cord, though it is usually
restricted to only a small portion.
Causes In some cases, the disease is presumed to be caused by
viral infections such as cytomegalovirus (CMV) and has also been
associated with spinal cord injuries, immune reactions,
schistosomiasis and insufficient blood flow through spinal cord
vessels. Acute myelitis accounts for 4 to 5 percent of all cases
of neuroborreliosis.
A major differentiation or distinction to be made is a similar
condition due to compression of the spinal cord in the spinal
canal, due to disease of the surrounding vertebral column. An
urgent MRI is thus indicated.
Another possible cause is dissection of the Aorta, extending into
one or more of the spinal arteries.
TM can be a rare complication following cat scratch disease.
Prognosis
Recovery from transverse myelitis usually begins between weeks 2
and 12 following onset and may continue for up to 2 years in some
patients. Some patients show no signs of recovery ever. However,
if treated early, some patients experience complete or near
complete recovery.
Treatment is usually symptomatic only, corticosteroids being used
with limited success.
Symptoms and signs
Symptoms include weakness and numbness of the limbs as well as
motor, sensory, and sphincter deficits. Severe back pain may occur
in some patients at the onset of the disease. The symptoms and
signs depend upon the level of the spinal cord involved and the
extent of the involvement of the various long tracts. In some
cases, there is almost total paralysis and sensory loss below the
level of the lesion. In other cases, such loss is only partial.
If the upper cervical cord is involved, all four limbs may be
involved and there is risk of respiratory paralysis.
Lesions of the lower cervical (C5-T1) region will cause a
combination of upper and lower motor neuron signs in the upper
limbs, and exclusively upper motor neuron signs in the lower
limbs.
A lesion of the thoracic spinal cord (T1-12) will produce upper
motor neuron signs in the lower limbs, presenting as a spastic
paraplegia.
A lesion of the lower part of the spinal cord (L1-S5) often
produces a combination of
upper and lower motor neuron signs in the lower limbs.
The degree and type of sensory loss will depend upon the extent of
the involvement of the various sensory tracts, but there is often
a "sensory level" (at the sensory segmental level of the spinal
cord below which sensation to pin or light touch is impaired).
This has proven to be a reasonably reliable sign of the level of
the lesion. Bladder paralysis often occurs and urinary retention
is an early manifestation. Considerable pain often occurs in the
back, extending laterally to involve the sensory distribution of
the diseased spinal segments so-called "radicular pain." Thus, a
lesion at the T8 level will produce pain radiating from the spine
laterally along the lower costal margins. These signs and symptoms
may progress to severe weakness within hours. (Because of the
acuteness of this lesion, signs of spinal shock may be evident, in
which the lower limbs will be flaccid and areflexic, rather than
spastic and hyperreflexic as they should be in upper motor neuron
paralysis.
Some patients have also described the feeling of their abdominal
area being in a binder.
However, within several days, this spinal shock will disappear and
signs of spasticity will become evident
Differential diagnosis
The three main conditions to be considered in the differential
diagnosis are: acute spinal cord trauma, acute compressive lesions
of the spinal cord such as epidural metastatic tumour, and
infarction of the spinal cord, usually due to insufficiency of the
anterior spinal artery. Lyme disease serology is indicated in
patients with transverse myelitis keeping in mind that
dissociation in Lyme antibody titers between the blood and the CSF
is possible.
From the symptoms and signs, it may be very difficult to
distinguish acute transverse myelitis from these conditions and it
is almost invariably necessary to perform an emergency magnetic
resonance imaging (MRI) scan or computerised tomographic (CT)
myelogram. Before doing this, routine x-rays are taken of the
entire spine, mainly to detect signs of metastatic disease of the
vertebrae, that would imply direct extension into the epidural
space and compression of the spinal cord. Often, such bony lesions
are absent and it is only the MRI or CT that discloses the
presence or absence of a compressive lesion.
A family physician seeing such a patient for the first time should
immediately arrange transfer to the care of a neurologist or
neurosurgeon who can urgently investigate the patient in hospital.
Before arranging this transfer, the physician should be certain
that respiration is not affected, particularly in high spinal cord
lesions. If there is any evidence of this, methods of respiratory
assistance must be on hand before and during the transfer
procedure. The patient should also be catheterized to test for
and, if necessary, drain an over-distended bladder. A lumbar
puncture can be performed after the MRI or at the time of CT
myelography. Steroids are often given in high dose at the onset,
in hope that the degree of inflammation and swelling of the cord
will be lessened, but whether this is truly effective is still
debated.
Unfortunately, the prognosis for significant recovery from acute
transverse myelitis is poor in approximately 80% of the cases;
that is, significant long-term disabilities will remain.
Approximately 5% of these patients will, in later months or years,
show lesions in other parts of the central nervous system,
indicating, in retrospect, that this was a first attack of
multiple sclerosis.
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