Transverse Myelitis – Causes, Complications and Treatment.

Definition

Transverse myelitis (TM) is a neurological condition that happens when both sides of the same section of the spinal cord become inflamed. This inflammation can damage myelin, the fatty substance that covers your nerves. Loss of myelin often leads to spinal cord scarring that blocks nerve impulses and results in physical problems.

Transverse myelitis is a relatively rare disease. It occurs most often in children ages 10 to 19 and in adults ages 30 to 39, but it can happen at any age.

Epidemiology

The epidemiology of TM depends on its underlying cause. The annual incidence of idiopathic complete TM is uncertain, but conservative estimates based on studies in US, European, and Israeli populations range from 1 to 4 cases per million, with a bimodal distribution of age presentation, peaking at ages 10 to 19 and 30 to 39 years. A population-based study estimated a mean annual incidence rate of 0.82 per 100,000 person-years, or 1.2 per 100,000 person-years for adults. Acute partial TM commonly heralds or accompanies multiple sclerosis, which affects up to 0.3% of persons of Northern European ancestry. The pattern of longitudinally extensive TM is commonly associated with neuromyelitis optica spectrum disorders, which may account for approximately 1% of all CNS demyelinating disease in North American and European populations, a somewhat greater proportion of African-Americans and Hispanics, up to 30% in the Japanese population, and virtually all cases in ethnic or racial groups in which typical multiple sclerosis is virtually absent, such as aboriginal peoples of North America and black Africans. Onset may occur at any age. Multiple sclerosis-associated cases typically occur between ages 20 and 50 years (median about 29 years) whereas the median onset age for neuromyelitis optica-related cases is about a decade later. There is a female predominance in all subtypes. These syndromes also affect children, although the incidence is unknown. The treatment of TM in children is usually the same as in adults. The epidemiology of other infectious and inflammatory myelopathies varies depending on the underlying etiology.

Causes of transverse myelitis

Experts don’t know the exact cause of transverse myelitis. The inflammation that leads to transverse myelitis can result as a side effect of a number of other conditions, including:

• Lyme disease
• Syphilis
• Measles
• Viral infections
• Bacterial infections

Some people may also get transverse myelitis as a result of spinal injuries, spinal defects, or vascular diseases like atherosclerosis, all of which can reduce the amount of oxygen in spinal cord tissue.

If parts of the spinal cord don’t have enough oxygen, nerve cells often start to die. The dying tissue can cause the inflammation that leads to transverse myelitis.

Transverse myelitis can also be a warning sign of multiple sclerosis, but this is rare. Since some people with transverse myelitis have autoimmune diseases such as lupus, some doctors believe that transverse myelitis may also be an autoimmune disease. Finally, some cancers can trigger an immune response that leads to transverse myelitis.

Symptoms of transverse myelitis

Signs and symptoms of transverse myelitis usually develop over a few hours to a few days and may sometimes progress gradually over several weeks. Transverse myelitis usually affects both sides of the body below the affected area of the spinal cord, but sometimes there are symptoms on just one side of the body.

Typical signs and symptoms include:

Pain: Transverse myelitis pain may begin suddenly in your lower back. Sharp pain may shoot down your legs or arms or around your chest or abdomen. Pain symptoms vary based on on the part of your spinal cord that’s affected.

Abnormal sensations: Some people with transverse myelitis report sensations of numbness, tingling, coldness or burning. Some are especially sensitive to the light touch of clothing or to extreme heat or cold. You may feel as if something is tightly wrapping the skin of your chest, abdomen or legs.

Weakness in your arms or legs: Some people notice that they’re stumbling or dragging one foot, or heaviness in the legs. Others may develop severe weakness or even total paralysis.

Bladder and bowel problems: This may include needing to urinate more frequently, urinary incontinence, difficulty urinating and constipation.

Complications

People with transverse myelitis usually experience only one episode. However, complications often linger, including the following:

Pain, one of the most common debilitating long-term complications of the disorder.

Stiffness, tightness or painful spasms in your muscles (muscle spasticity). This is most common in the buttocks and legs.

Partial or total paralysis of your arms, legs or both. This may persist after the first symptoms.

Sexual dysfunction, a common complication of transverse myelitis. Men may experience difficulty achieving an erection or reaching orgasm. Women may have difficulty reaching orgasm.

Depression or anxiety, which is common in those with long-term complications because of the significant changes in lifestyle, the stress of chronic pain or disability, and the impact of sexual dysfunction on relationships

Diagnosis and test

• If you suspect that you have transverse myelitis, your healthcare provider will likely review your medical history and do a complete physical exam. Because transverse myelitis can be caused by a number of treatable conditions, your provider may want to test for some of those diseases. One test you might need is a CT or MRI scan.
• Another common test is myelography, a procedure in which a needle is used to inject a special dye into your spinal area. A technician will then use a real time X-ray called fluoroscopy to get pictures of your spinal cord.
• Blood tests and a lumbar puncture (spinal tap) may also be done to rule out other causes for transverse myelitis.

How transverse myelitis is treated?

There is no cure for transverse myelitis at this time and no medications have been specifically approved to treat it. The standard of care for transverse myelitis includes:

Intravenous (IV into the vein) corticosteroids such as methylprednisone or dexamethasone may be given to help decrease the swelling in the spinal cord.  In some cases, prednisone, taken by mouth, is used for a period of time after the IV therapy is completed.

Plasma exchange (PLEX) may be ordered if no improvement occurs with corticosteroid therapy. Plasma exchange involves removing blood from the body through a needle and tubing. Through a series of steps, the plasma (the liquid part of the blood) is separated from blood cells and then replaced with an artificial plasma substitute; the plasma substitute and blood cells are combined and returned to the body through an intravenous line. The procedure lasts several hours and may be repeated multiple times over a number of days.

Other therapies

Additional therapies focus on long-term recovery and care:

Physical therapy: This helps improve strength and coordination. Your physical therapist can teach you how to use any needed assistive devices, such as a wheelchair, canes or braces.

Occupational therapy: This helps people with transverse myelitis learn new ways of performing day-to-day activities, such as bathing, preparing a meal and housecleaning.

Psychotherapy: A psychotherapist can use talk therapy to treat anxiety, depression, sexual dysfunction, and other emotional or behavioral issues from coping with transverse myelitis.

Corticosteroid medications

Physicians often prescribe a high dose of intravenous steroids immediately following the onset of symptoms or diagnosis. These medications can include those such as methylprednisolone or dexamethasone.

Pain management

One of the first symptoms reported by patients is pain. The initial pain management generally includes non-steroidal anti-inflammatory drugs. Non-steroidal anti-inflammatory drugs can include:

• Aspirin
• Ibuprofen (e.g. Advil, Motrin)
• Naproxen (e.g. Aleve, Naprosyn)

These drugs are helpful in relieving pain, and reducing inflammation.

Patients can also be prescribed pain medication for long-term treatment, after the initial treatment is complete.

Prevention and home remedies

Some lifestyle strategies that can help you manage complications of transverse myelitis include the following:

Preventing bowel problems

If you have bowel problems caused by transverse myelitis, eat a fiber-rich diet and drink plenty of fluids to help prevent constipation.

Maintaining strong bones

People with transverse myelitis are at an increased risk of developing osteoporosis because of limited activity. Talk to your doctor about calcium and vitamin D supplements to improve bone health. Weight-bearing exercises, if possible, will also strengthen bones.

Keeping an exercise routine

Stiff or tight muscles are a common complication of transverse myelitis and may limit your ability to get around easily. Increase your flexibility by following a stretching routine suggested by your physical therapist. A therapist-designed strengthening program can target your weaker muscles to help improve your mobility and balance.