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Acute Transverse Myelitis
Acute transverse myelitis is acute inflammation of gray and white matter in one or more adjacent spinal cord segments, usually thoracic. Causes include multiple sclerosis, neuromyelitis optica, infections, autoimmune or postinfectious inflammation, vasculitis, and certain drugs. Symptoms include bilateral motor, sensory, and sphincter deficits below the level of the lesion. Diagnosis is usually by MRI, CSF analysis, and blood tests. IV corticosteroids and plasma exchange may be helpful early. Otherwise, treatment is with supportive measures and correction of any causes.
Acute transverse myelitis is most commonly due to multiple sclerosis but can occur with vasculitis, mycoplasmal infections, Lyme disease, syphilis, TB, or viral meningoencephalitis or in patients taking amphetamines, IV heroin, or antiparasitic or antifungal drugs. Transverse myelitis occurs with optic neuritis in neuromyelitis optica (Devic disease), once considered a variant of multiple sclerosis but now considered a distinct disorder. The mechanism of transverse myelitis is often unknown, but some cases follow viral infection or vaccination, suggesting an autoimmune reaction. Inflammation tends to involve the spinal cord diffusely at one or more levels, affecting all spinal cord functions.
Pain in the neck, back, or head may occur. A bandlike tightness around the chest or abdomen, weakness, tingling, numbness of the feet and legs, and difficulty voiding develop over hours to a few days. Deficits may progress over several more days to a complete transverse sensorimotor myelopathy, causing paraplegia, loss of sensation below the lesion, urinary retention, and fecal incontinence. Occasionally, position and vibration sensation are spared, at least initially. The syndrome occasionally recurs in patients with multiple sclerosis, SLE, or antiphospholipid syndrome.
Diagnosis is suggested by transverse sensorimotor myelopathy with segmental deficits. Guillain-Barré syndrome (see Guillain-Barré Syndrome (GBS)) can be distinguished because it does not localize to a specific spinal segment. Diagnosis requires MRI and CSF analysis. MRI typically shows cord swelling if transverse myelitis is present and can help exclude other treatable causes of spinal cord dysfunction (eg, spinal cord compression). CSF usually contains monocytes, protein content is slightly increased, and IgG index is elevated (normal, ≤ 0.85).
A test for a marker for neuromyelitis optica IgG (NMO-IgG)—an autoantibody that targets the astrocyte water channel protein aquaporin-4—is highly specific and helps distinguish neuromyelitis optica from multiple sclerosis.
Tests for treatable causes should include chest x-ray; PPD; serologic tests for mycoplasma, Lyme disease, and HIV; vitamin B 12 , folate, zinc, and copper levels; ESR; antinuclear antibodies; and CSF and blood Venereal Disease Research Laboratory (VDRL) tests. History may suggest a drug as a cause.
Brain MRI is done; multiple sclerosis develops in 50% of patients who have multiple periventricular T2 bright lesions and in 5% who do not have them.
Generally, the more rapid the progression is, the worse the prognosis. Pain suggests more intense inflammation. About one third of patients recover, one third retain some weakness and urinary urgency, and one third are bedbound and incontinent. Multiple sclerosis eventually develops in about 10 to 20% of the patients in whom the cause is initially unknown.
Treatment is directed at the cause or associated disorder but is otherwise supportive. In idiopathic cases, high-dose corticosteroids are often given and sometimes followed by plasma exchange because the cause may be autoimmune. Efficacy of such a regimen is uncertain.
Autoimmune and demyelinating disorders, infections, and drugs can inflame tissues in spinal cord segments, causing transverse myelitis, which may progress to complete transverse sensorimotor myelopathy.
Do MRI of the spinal cord, CSF analysis, testing for neuromyelitis optica IgG, and other tests for treatable causes (eg, infections, nutritional deficiencies).
Treat the cause if identified, and, if no cause is evident, consider corticosteroids and plasma exchange.
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