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An MRI showing a transverse myelitis lesion (the lesion is the lighter, oval shape at center-right), this MRI was taken 3 months after patient recovered
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Transverse myelitis (Latin: myelitis transversa) is a neurological condition in which the spinal cord is inflamed. The inflammation damages nerve fibers, and causes them to lose their myelin coating leading to decreased electrical conductivity in the nervous system. The disorder's name is derived from Greek myelós referring to the "spinal cord", and the suffix -itis, which denotes inflammation. Transverse implies that the inflammation extends across the entire width of the spinal cord. Partial transverse myelitis and partial myelitis are terms used to define inflammation of the spinal cord that affects part of the width of the spinal cord.
Signs and symptoms
There are disturbances in sensory nerves and motor nerves and dysfunction of the autonomic nervous system at the level of the lesion or below. Therefore, the signs and symptoms depend on the area of spine involved:
- Cervical: If the upper cervical cord is involved, all four limbs may be involved and there is risk of respiratory paralysis (cervical nerve segments C3, 4, 5 innervate the abdominal diaphragm). 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. Cervical lesions account for about 20% of cases.
- Thoracic: A lesion of the thoracic spinal cord (T1–12) will produce upper motor neuron signs in the lower limbs, presenting as a spastic diplegia. This is the most common location of the lesion, and therefore most individuals will have weakness of the lower limbs.
- Lumbar: 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. Lumbar lesions account for about 10% of cases.
Symptoms include weakness and numbness of the limbs, deficits in sensation and motor skills, dysfunctional sphincter activities, and dysfunction of the autonomic nervous system. Symptoms typically develop over the course of hours or days and may progress after weeks. Sensory symptoms of transverse myelitis may include a sensation of pins and needles traveling up from the feet. Back pain can occur at the level of the inflamed segment of the spinal cord. 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 pain or light touch is impaired). Motor weakness occurs due to involvement of the pyramidal tracts and mainly affects the muscles that flex the legs and extend the arms. Involvement of the autonomic nervous system is common and frequently leads to impaired function of the bladder and bowel and can also lead to episodes of high blood pressure. Bladder paralysis often occurs and urinary retention may be an early manifestation.
Transverse myelitis is a heterogeneous condition, that is, there are several identified causes. Sometimes the term Transverse myelitis spectrum disorders is used.It is rare for the condition to have no identifiable cause. When it appears along neuromyelitis optica, it is considered to be caused by NMO-IgG autoimmunity, and when appears in multiple sclerosis cases, it is considered to be produced by the same underlying condition that produces the lesions. 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.
Transverse myelitis is associated with:
- Bacterial Infections - Mycoplasma pneumoniae, Lyme disease, syphilis (tabes dorsalis), tuberculosis
- Viral Infections - herpes simplex, herpes zoster, cytomegalovirus, Epstein-Barr virus, enteroviruses (poliomyelitis, Coxsackie virus, echovirus), human T-cell leukemia virus, HIV/AIDS, influenza, rabies
- Vaccinations. Rarely, transverse myelitis has been reported to develop several days to a few months after vaccination. Between 1970 and 2009 there were 37 reported cases worldwide of transverse myelitis developing soon after vaccination (e.g. against hepatitis B, measles-mumps-rubella, diphtheria-tetanus-pertussis and others).
- Multiple sclerosis
- Paraneoplastic syndromes
- Vascular - thrombosis of spinal arteries, vasculitis secondary to heroin abuse, spinal arteriovenous malformations
This demyelination arises idiopathically following infections 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 diarrhea-causing bacteria Campylobacter jejuni is also a reported cause of transverse myelitis.
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 they are usually restricted to only a small portion.
Longitudinally extensive transverse myelitis
A proposed special clinical presentation is the "longitudinally extensive transverse myelitis" (LETM) which is defined as a TM with a spinal cord lesion that extends over 3 or more vertebral segments. The causes of LETM are also heterogeneous and the presence of MOG auto-antibodies has been proposed as a biomarker for discrimination.
The symptoms considered in the differential diagnosis are acute spinal cord trauma, acute compressive lesions of the spinal cord such as epidural metastatic tumor, and infarction of the spinal cord, usually due to insufficiency of the anterior spinal artery. Also nucleus pulposus embolism.
In 2002 the Transverse Myelitis Consortium Working Group proposed the following diagnostic criteria:
- Inclusion criteria
- Motor, sensory or autonomic dysfunction attributable to spinal cord
- Signs and symptoms on both sides of the body (not necessarily symmetrical)
- Clearly defined sensory level
- Signs of inflammation (pleocytosis of the cerebrospinal fluid, or elevated immunoglobulin G, or evidence of inflammation on gadolinium-enhanced MRI)
- Peak of the condition occurs 4 hours to 21 days after onset
- Exclusion criteria
- Irradiation of the spine (e.g., radiotherapy) in the last 10 years
- Evidence of thrombosis of the anterior spinal artery
- Evidence of extra-axial compression on neuroimaging
- Evidence of arteriovenous malformation (abnormal flow voids on surface of spine)
- Evidence of connective tissue disease, e.g. sarcoidosis, Behçet's disease, Sjögren's syndrome, systemic lupus erythematosus or mixed connective tissue disease
- Evidence of optic neuritis (diagnostic of neuromyelitis optica
- Evidence of infection (syphilis, Lyme disease, Human immunodeficiency virus, Human T-lymphotropic virus 1, mycoplasma, Herpes simplex virus, Varicella-zoster virus, Epstein-Barr virus, cytomegalovirus, Human herpesvirus 6 or enteroviruses)
- Evidence of multiple sclerosis (abnormalities detected on MRI and presence of oligoclonal antibodies in CSF)
From the signs and symptoms 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 computerized 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.
Typically individuals who develop transverse myelitis are transferred to a neurologist or neurosurgeon who can urgently investigate the patient in hospital. Before arranging this transfer, the physician should be certain that breathing 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 doses when symptoms begin with the hope that the degree of inflammation and swelling of the spinal cord will be lessened, but whether this is truly effective is still debated.
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.
Recovery from transverse myelitis is variable between individuals and also depends on the underlying cause. Some patients begin to recover between weeks 2 and 12 following onset and may continue to improve for up to 2 years. Other patients may never show signs of recovery. However, if treated early, some patients experience complete or near complete recovery. Treatment options also vary according to the underlying cause and one treatment option in some cases is plasmapheresis.
The prognosis for significant recovery from acute transverse myelitis is poor in approximately 66% of the cases; that is, significant long-term disability will remain. Approximately 2% 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.
According to at least one source the incidence of transverse myelitis is 4.6 in 1 million, and further, affects men and women equally. Transverse myelitis can occur at any age (particularly age 10, age 20, and after age 40).[clarification needed]
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