Acute transverse myelitis (ATM) is acute inflammation of gray and white matter across both sides of one or more adjacent spinal cord segments.
Damage will affect function at that segment and segments below it. The cause of ATM is unknown, but most evidence supports an autoimmune process.
Approximately one third of patients with ATM report a febrile illness (flu-like illness with fever) in close temporal relationship to the onset
of neurologic symptoms. Transverse myelitis may be a relatively uncommon manifestation of several autoimmune diseases including systemic lupus
erythematosus, Sjogren's syndrome, and sarcoidosis. Myelitis has rarely been reported secondary to vaccinations. The spinal cord often may be the
site of involvement of the first attack of multiple sclerosis. The symptoms and signs of ATM depend upon the level of the spinal cord involved and
the extent of the involvement of the various long tracts. Symptoms include bilateral weakness, tingling, numbness of the feet and legs, and difficulty
voiding that may develop over hours to a few days. The arms are involved in a minority of cases. 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.
Almost all patients will develop leg weakness of varying degrees of severity. Sensation is diminished below the level of spinal cord involvement
in the majority of patients. Because of the acuteness of the process, 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. However, within several days, this spinal shock
will disappear and signs of spasticity will become evident. Pain in the neck, back, or head, and/or pain in the distribution of a single spinal nerve
may occur. Guillain-Barré syndrome can be distinguished because it does not localize to a specific spinal segment.
Incidence is estimated at approximately 0.1 to 0.5 per 100,000 persons with peaks between 10 and 19 years and 30 and 39 years of age.
Diagnosis often requires MRI, CSF analysis, and/or blood tests. The first concern of the physician who evaluates a patient with complaints and examination
suggestive of a spinal cord disorder is to rule out a mass-occupying lesion that might be compressing the spinal cord. Potential lesions include tumor,
herniated disc, stenosis, and abscess. Therefore, an MRI of the appropriate levels of the cord should be obtained urgently to evaluate for spinal cord compression.
MRI examination of the brain is also commonly ordered to rule out acute disseminated encephalomyelitis or Multiple Sclerosis, both of which can present with
ATM-like features. In ATM, the MRI typically shows inflammatory lesion(s) within the cord and/or cord swelling. Tests for treatable causes as directed by the
H&P may include chest x-ray; PPD; a comprehensive metabolic screen, HbA1c, serologic tests for mycoplasma, Lyme disease, HIV, Herpes simplex virus-2,
Cytomegalovirus, Epstein-Barr virus, Varicella-zoster virus, Human herpes viruses 6 and 7; vitamin B12 and folate levels; ESR; tests for SLE and Sjogren's
syndrome, Hgb A1c, thyroid profile, urine heavy metals, and CSF and blood for syphilis. A specific antibody marker for neuromyelitis optica (NMO-IgG), which
distinguishes neuromyelitis optica from multiple sclerosis, is available. Cerebral spinal fluid usually contains monocytes, protein content is slightly increased,
and IgG index is elevated (normal, ≤ 0.85). CSF cultures, immunoglobulin level, and protein electrophoresis may also be indicated.
Generally, the more rapid the progression is, the worse the prognosis. About 1/3 of patients recover, 1/3 retain some weakness and urinary urgency,
and 1/3 are bedridden and incontinent. Multiple sclerosis eventually develops in about 10 to 20% of the patients. Recovery generally begins within 1 to 3
months and may continue for up to 2 years. Most patients with ATM show good to fair recovery. While a small percentage of patients may suffer a recurrence,
other diagnoses such as MS, SLE, or antiphospholipid syndrome should be sought should symptoms recur.
Treatment is directed at the cause or associated disorder, but is otherwise symptomatic and supportive. High-dose intravenous methylprednisolone
and/or immunoglobulin, sometimes followed by plasma exchange have been used because the cause may be autoimmune. However, efficacy of such a regimen
is uncertain. Following initial therapy, the most critical part of the treatment for this disorder consists of keeping the patient's body functioning
while awaiting either complete or partial recovery of the nervous system.
A 19 y/o white male healthy basic trainee received multiple routine vaccinations. He recalled no preceding illness and no immediate adverse local or
systemic reactions. He denied fever, chills, myalgia, rash, pulmonary or GI symptoms, back trauma, or known exposure to infectious disease.
Approximately four weeks following his vaccinations, the patient noted the onset of mid-back pain and parethesia of his toes bilaterally.
Examination at the time showed no motor dysfunction. Straight leg raises and DTR were normal and symmetrical. Over the next day, his parethesia w/o
numbness progressed to involve his lower legs and thighs. Walking became difficult with perceived weakness in his knees. Within two days he was
unable to move his legs. Evaluation noted UE strength to be 5/5, while RLE strength was 2- proximally and 2/5 distally and 3- on the left.
There was decreased sensation to all modalities in the legs. Reflexes were 2+ in the UEs and 3+ in the LEs. Creatine Kinase was elevated at 565 (57-374).
CSF: Glu 69, Protein 44, (15-45), Myelin Basic Protein 353 (0.07-4.1). There was also documented mild bowel and bladder dysfunction. MRI of brain was normal,
but C-Spine showed an abnormal signal from C7 to at least T2.
Transverse Myelitis, National Institute for Neurologic Disorders and Stroke (NIH)
Jacob A, Weinshenker B, An Approach to the Diagnosis of Acute Transverse Myelitis, Semin Neurol 2008;28:105-120
Acute Transverse Myelitis, Merck Manuals Online Library