Idiopathic Transverse Myelitis – Transverse Myelitis in Multiple Sclerosis and Neuromyelitis Optica – Prognosis – Diagnosis – Treatment
Transverse myelitis is a descriptive term, which refers to the fact of inflammation of a cross-section of the spinal cord. Possible causes of inflammation are infections and variety of autoimmune conditions.
Irrelevantly of the cause, transverse myelitis presents with similar symptoms. It is virtually impossible to diagnose the cause of this condition without an array of diagnostic tests.
Treatment and prognosis of transverse myelitis largely depend on the cause. Transverse myelitis affects both children and adults.
Symptoms of Transverse Myelitis
The most common initial complaint is weakness in the legs, which may have gradual onset or may develop very rapidly. Sensation of tightness or numbness in the torso is not uncommon.
Bowel and urination control problems are almost inevitable (90%). Arms become involved in 40%. Majority will experience numbness and tingling in the affected parts of the body.
In idiopathic transverse myelitis 80% of lesion is in the thoracic spinal cord. The rest 20% are equally shared by cervical and lumbar parts of the cord. “Idiopathic” means that the cause of transverse myelitis is not known.
In 50% of cases myelitis follows some fever, respiratory infection or vaccination. In almost half of transverse myelitis cases an extensive work up reveals no particular cause myelitis or any other disease. This is called Idiopathic Transverse Myelitis.
The most common identifiable causes are multiple sclerosis and neuromyelitis optica.
Diagnostic Testing in Transverse Myelitis
Whole Spine MRI with contrast is required to assess the extent of spinal cord involvement and to rule out potential structural lesions as a cause of spinal cord symptoms. Transverse myelitis may be mimicked by spinal cord stroke, tumor, epidural abscess/hematoma, disc herniation, or AVM.
Brain MRI with contrast is needed to rule out similar lesions in the brain. Presence of brain lesions makes multiple sclerosis a likely diagnosis.
Lumbar puncture is expected to demonstrate elevated protein level and cell count in the cerebrospinal fluid (CSF). CSF is also tested for potential infection (PCR, cultures, serology); Paraneoplastic antibodies panel; oligoclonal bands and IgG index.
Blood test for autoimmune disorders, Aquaporin-4 antibody, paraneoplastic syndromes.
Paraneoplastic disorders are autoimmune conditions caused by immune response to some cancers, leukemia, and lymphoma.
Specific Causes of Transverse Myelitis
Viral (cytomegalovirus, human T-cell lymphotropic virus I) or Mycoplasma infections and lupus may be responsible for transverse myelitis.
Transverse myelitis may be initially confused with Guillain-Barre syndrome, but detailed neurological examination usually enables to differentiate the two. Besides, Guillain-Barre syndrome doesn’t normally cause incontinence.
Treatment and prognosis of these conditions are not discussed in this article. Below are the most common syndromes of transverse myelitis.
Idiopathic Transverse Myelitis
Clinical picture of transverse myelitis was described above. “Idiopathic” refers only to the cause of myelitis. Symptoms typically involve both sides of the body but not necessarily symmetrically.
Neurological examination usually allows detection of specific level of the spinal cord lesion based on the pattern of sensation loss.
Spine MRI detects no structural lesions potentially responsible for spinal cord compression.
There is a typical MRI picture of spinal cord lesion with contrast enhancement. This finding may be delayed for up to a week.
Spinal tap reveals elevated protein, IgG index, and cell count (may be delayed).
It might take from 4 hours to 3 weeks for transverse myelitis symptoms to peak.
In children, contrast enhancement may involve the whole spinal cord. Spinal cord swelling may be present in severe cases.
Treatment of noninfectious transverse myelitis is high dose steroids (1 gram IV) for 3 to five days. Lack of response to corticosteroids requires five to seven plasma exchange treatments.
Idiopathic transverse myelitis is a monophasic illness. Which means that it won’t reoccur. The residual damage to the spinal cord may be substantial, leading to long term disability.
The outcome depends on severity of the spinal cord tissues damage and the timing of treatment. Even in cases with good recovery, improvement of bowel and bladder control is delayed for a few months. Lesions of the uppermost part of the spinal cord carry the worst prognosis.
Prognosis in children is more favorable. Children treated with steroids have a full or decent recovery in 80% of cases. Only 20% will have significant long term disability. Lack of steroid treatment decreases favorable outcome down to 60%.
Transverse Myelitis in Multiple Sclerosis and NMO
Demyelinating spinal cord lesions often produce Lhermite sign. This is an electrical sensation in the back or in the limbs provoked by neck flexion.
The same holds true for painful muscle spasms in the limbs or the trunk. Spasms are usually precipitated by limb movements.
Presence of these symptoms is highly suggestive of either MS or neuromyelitis optica.
Mild or localized weakness and sensation loss associated with patchy involvement of the periphery of the spinal cord on the MRI are suggestive for MS. The same applies to presence lesions on the Brain MRI.
Long, centrally located spinal cord lesions in association with optic neuritis are likely to be caused by neuromyelitis optica.
Presence of oligoclonal bands in the cerebrospinal fluid is typical for MS.
Positive blood test for AQP-4 antibody makes it diagnostic for neuromyelitis optica.
Diagnostic criteria, treatment, and prognosis of neuromyelitis optica and multiple sclerosis are discussed elsewhere.
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