Transverse Myelitis (TM) and Multiple Sclerosis

Transverse myelitis (TM) is a neurological disorder caused by inflammation (swelling) across both sides of one segment of the spinal cord. Inflammation can damage or destroy myelin, the fatty protective substance that covers nerve fibers. This damage causes scars that interrupt the communication between the nerves in the spinal cord and the rest of the body.

Most people affected by TM will have only one attack. A small percentage may have more than one. This is more likely if they have multiple sclerosis, neuromyelitis optica spectrum disorder (NMOSD) or myelin oligodendrocyte glycoprotein antibody disease (MOGAD). TM can appear as the first symptom in conditions such as MS or NMOSD. Some people recover with minor or no problems. Others may have lasting symptoms that impact their quality of life. Physical rehabilitation is recommended to help people with TM recover.

Pain is often the first symptom of TM, generally beginning as a lower back pain. The location of the spinal cord where the damage occurs determines which parts of the body are affected. Damage at one area will affect function at that area and the areas below it.

Other early symptoms can include:

• Sensitivity to touch — clothing or a light touch with a finger causes significant discomfort or pain
• Weakness in the arms or legs
• Sensory symptoms such as numbness, tingling, burning, tickling, or prickling in the legs, feet or toes
• Bowel and bladder problems — may include an increased frequency of the urge to urinate or have bowel movements, loss of bowel or bladder control, difficulty urinating, constipation or the feeling of incomplete bowel evacuation
• Heightened sensitivity to changes in temperature or to extreme heat or cold
• Fatigue

TM symptoms can develop over hours to several days or more gradually over a period of 1 to 4 weeks.

Researchers have not determined the exact cause of the damage to the spinal cord, but believe it is due to an autoimmune reaction to a viral or bacterial infection. In autoimmune diseases, the immune system mistakenly attacks the body’s own tissue, causing inflammation (swelling) that results in damage to the myelin.

These infections can be viral or bacterial and include:

• Viral infections such as herpes simplex, cytomegalovirus, Epstein-Barr, influenza, echovirus, HIV, hepatitis A, rubella, chicken pox and shingles

• Bacterial infections such as skin infections, middle-ear infections and bacterial pneumonia

Some cancers may also trigger an abnormal immune response that leads to TM.

In some people, transverse myelitis is the first symptom of an immune-mediated disease such as MS or NMOSD. Many individuals who have TM do not go on to develop MS.

The process of diagnosis includes:

• A complete medical history, including any history of recent viral, bacterial, fungal or parasitic infections or cancer
• A thorough neurological examination
• Magnetic resonance imaging (MRI)
• Blood tests to identify other possible causes of the symptoms such as systemic lupus erythematosus (lupus), HIV infection, or vitamin B12 deficiency, and to check for antibodies (proteins in the blood) associated with TM, including anti-AQP4 and anti-MOG antibodies
• Lumbar puncture (spinal tap) to examine the fluid that surrounds the brain and spinal cord for white blood cells (indicating a possible infection) and to look for signs of diseases such as MS
• Spinal angiography, which involves injecting dye through a catheter to assess the blood vessels of the spinal cord for problems

If testing and examination do not suggest a specific cause for the condition, the diagnosis is presumed to be idiopathic transverse myelitis. This is the case with many people diagnosed with TM.

TM occurs in adults and children of all races.

• Those assigned female at birth have a higher risk of TM than those assigned male.
• No genetic pattern is known. TM is not related to family history.
• The highest number of new cases in a given year occurs between ages 10 and 19, and between ages 30 and 39.
• The symptoms of TM can appear suddenly, developing over hours or several days. They can also develop more gradually over a period of 1 to 4 weeks.

It is estimated that about 1,400 new cases of TM are diagnosed each year in the United States. About 33,000 Americans have some type of disability resulting from TM.

The annual incidence of TM ranges from 1.34 to 4.60 cases per million. The incidence increases to 24.6 cases per million if acquired demyelinating diseases like MS and NMOSD are included.

TM is caused by several different conditions, and so treatments to prevent it may be found when reviewing treatments for MS, NMOSD and MOGAD. Treatment of TM includes:

• Intravenous (IV) (infusion through the vein) high-dose steroids are given to reduce the inflammation 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) for severe attacks or if no improvement occurs with corticosteroids.
  • The goal of PLEX is to lower the level of anti-AQP4 antibodies in the blood.
  • PLEX 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 replaced with an artificial plasma substitute. The plasma substitute and blood cells are combined and then returned to the body intravenously.
  • The procedure lasts several hours and may be repeated multiple times over a number of days.

After TM occurs, long-term rehabilitation may help prevent or reduce disability. Even in patients with severe weakness, rehabilitation can help restore function and quality of life.

Some people affected by TM recover with minor or no problems. Others suffer permanent impairments that affect their ability to perform ordinary tasks of daily living. Permanent neurological deficits could include severe weakness, spasticity (painful muscle stiffness or contractions), paralysis, incontinence and chronic pain. Aggressive physical therapy/rehabilitation is indicated after an episode of TM to aid with recovery.

Several organizations offer resources and information for people who live with TM and their families:

• The Siegel Rare Neuroimmune Association (SRNA) is a nonprofit organization serving people with ADEM, MOGAD, NMOSD, optic neuritis and TM. They assist individuals living with these diseases and their families, maintain a clinical care network and fund research.
• The Christopher & Dana Reeve Paralysis Resource Center (PRC) works to cure spinal cord injury by funding innovative research and to improve the quality of life for people living with paralysis through grants, information and advocacy. They also offer peer mentoring, local resources and Quality of Life grants.

In addition to these organizations, which serve people living with TM, the National Institute of Neurological Disorders and Stroke conducts extensive research on demyelinating disorders such as TM.

Reviewed by Elias Sotirchos, MD, and Shuvro Roy, MD, April 2023.