Transverse myelitis (TM) is a rare neurological syndrome characterized by inflammation of the spinal cord, resulting in damage to nerve cells. TM can be caused by infections, immune system disorders, and inflammatory disorders as well as other conditions that may damage or destroy myelin, a fatty tissue that protects nerve fivers.
According to the National Organization for Rare Disorders (NORD), there are approximately 1,400 new cases of transverse myelitis each year. Anyone can develop TM and it does not appear to be related to genetics or family history. Treatment is aimed at decreasing the inflammatory response in order to minimize neurological deficits and at preventing recurrences in patients with multiple sclerosis and neuromyelitis optica.
Myelin wraps around nerve fibers in the same way insulation covers electrical wires. When myelin tissue becomes damaged, the nerves underneath do not work properly, causing the deficits commonly seen in TM.
When both sides of the spinal cord are damaged, myelitis is called transverse myelitis.
Transverse myelitis is either acute or subacute. Acute means it develops suddenly over hours to several days, and subacute means the condition develops over a period of one week to four weeks.
TM is known for having four classic features. These are:
- Weakness in the arms and legs: TM causes leg weakness that comes on very quickly and progresses. If the upper part of the spinal cord is affected, weakness in the hands may be experienced. Some people may develop paraparesis (partial paralysis of legs), which eventually progresses to paraplegia (complete paralysis of the legs) if the condition is not quickly treated.
- Pain: Pain associated with TM starts with low back pain and progresses into sharp, shooting pains into the legs and around the torso.
- Sensory alternations: TM can cause paresthesia—abnormal sensations of burning, pricking, numbness, tingling, burning, or coldness—in the arms, legs, torso, and genital area. A person may feel shooting pains in the neck when bending forward that resolves when the neck is back to its normal position. This is a sign called Lhermitte’s phenomenon.
- Bowel and/or bladder dysfunction: TM also causes increased frequency or urge to urinate, incontinence (loss of bladder control), problems with voiding, and constipation.
Additional symptoms that have been reported with TM include:
- Muscle spasms
- A general feeling of discomfort
- Loss of appetite
- Breathing difficulties
- Sexual dysfunction
The symptoms experienced are dependent on the part of the spinal cord that is damaged. Damage in one part will affect function at that level and below.
Sometimes a healthcare provider can pinpoint the cause of TM in a patient. When the cause is unknown, the condition is idiopathic. Having a cause is important because it will assist in treatment.
The most common causes of transverse myelitis are:
Immune System Disorders
Immune system disorders can play a part in causing damage to the spinal cord. Researchers believe a number of immune system disorders are associated with TM, including:
- Multiple sclerosis (MS): MS is an autoimmune disease that causes lesions on the brain, spinal cord, and/or the optic nerve. Partial myelitis that affects only one section of the spinal cord is often seen in people with MS.
- Neuromyelitis optica (NMO): Neuromyelitis optica is an autoimmune disease that primarily affects the optic nerves and spinal cord. NMO is known to cause paralysis of the spinal cord. Most patients with this disease have positive antibodies to an important protein called aquaporine-4 that is responsible for carrying water through the cell membrane in nerve cells.
- Post-infections or post-vaccine TM: In these cases, a person’s immune system mistakenly attacks normal spinal cord tissue while responding to an infection or vaccine.
- Paraneoplastic TM: This is an abnormal immune response to an underlying cancer that causes damage to the central nervous system, which includes the spinal cord.
- Anti-MOG: This is a recently discovered antibody that can affect not only the spinal cord but also the optic nerves.
Numerous infections, some common, some rare, have been associated with TM.
- Viral infection: Viruses associated with transverse myelitis include the varicella-zoster (chickenpox and shingles virus), herpes simplex virus, cytomegalovirus, Epstein-Barr, West Nile, Zika, echovirus, influenza, HIV, hepatitis B, and rubella.
- Bacterial infections: The bacteria that cause tuberculosis, syphilis, Lyme disease, and mycoplasma, among others, have been associated with TM.
- Fungal infections: Fungal infections of the spinal cord—such as aspergillus—have been linked to TM.
- Parasites: Several different kinds of parasite-type infections, including toxoplasmosis, schistosomiasis, and cysticercosis have been associated with the development of TM.
Any inflammatory condition affecting the spinal cord, including rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and mixed connective tissue disease can cause ongoing inflammation that contributes to the development of TM.
In some cases, TM is one of the first symptoms that someone has an autoimmune or immune system disease such as multiple sclerosis or neuromyelitis optica.
Your healthcare provider can make a diagnosis of transverse myelitis based on symptoms and medical history, a neurological examination, and various other testing methods.
The first step is to rule out any spinal cord causes that require emergency care, such as trauma or a tumor that is putting pressure on the spinal cord. Other problems that need to be ruled out are herniated and slipped discs, spinal stenosis (a condition in which the spinal cord canal has narrowed), vitamin deficiencies, blood vessel problems, and abscesses.
Tests your healthcare provider may perform to determine a cause or to rule out certain disorders include:
- Bloodwork: Blood tests for TM may look for specific antibodies, especially those related to neuromyelitis optica. People who have specific antibodies have an increased risk for multiple attacks of TM and will ongoing require treatment to prevent future attacks. Other blood work may identify infections that contribute to TM or rule out other causes.
- Magnetic resonance imaging (MRI): MRI imaging can detect inflammation or other abnormalities affecting the spinal cord.
- Lumbar puncture: Also called a spinal tap, a lumbar puncture involves using a needle to draw a small amount of cerebrospinal fluid (CSF), the protective fluid surrounding the spinal cord and brain. In TM, CSF may show abnormally high levels of white blood cells, increased protein, and the presence of oligoclonal bands (commonly seen in MS). CSF is also tested to look for infections and some types of cancer.
If none of the testing methods suggest a specific cause of TM, a person likely has idiopathic transverse myelitis. In some instances, MRI and spinal taps may show normal results and your healthcare provider will want to retest after a few days.
Transverse myelitis is a rare condition, which means there isn’t much information or research on the best treatment methods, but there are a number of options.
Medications that may be used to treat TM include:
- Drugs to treat an infection (if that is felt to be the cause)
- Drugs to treat symptoms such as pain, bladder urgency, and muscle spasms
- Medications (or procedures) to reduce spinal cord inflammation, such as steroid and plasma exchange
In many cases, hospitalization is required for the initial attack of TM due to its severity and the fact that it affects the spinal cord. At this time, your healthcare provider will have you on intravenous (IV) methylprednisolone, as this is the most effective treatment. The drug is given over a period of days and tapered off slowly.
The goal of steroid treatment is to reduce inflammation and swelling and to speed up recovery. The side effects of methylprednisolone may include:
- Increased blood sugar
- Low potassium levels
- Sleep disturbances
- Mood changes, including anxiety and depression
- Weight gain
- Flushed cheeks or facial swelling
- Metallic taste
When IV steroids don’t work, TM is treated using plasma exchange (PLEX). PLEX is used in moderate to severe cases and has been helpful to people with autoimmune and other inflammatory disorders.
It is uncertain how PLEX works, but it is believed it removes inflammatory antibodies from the blood. It is helpful to people who show active inflammation on MRIs.
For people who do not respond to steroids or PLEX, and in whom active inflammation is the cause of spinal cord problems, other forms of immune-based treatment may help. These are immunosuppressant drugs—medications that inhibit or prevent the activity of the immune system. This may include a cancer drug called cyclophosphamide that works by suppressing the immune system.
People who have idiopathic TM may have recurrences of the condition. In this case, TM might be the result of another disorder, and ongoing treatment that suppresses the immune system may help.
A Word From Get Meds Info
Many people with transverse myelitis will recover with minor or no permanent problems. Others may suffer permanent impairments that affect daily living. Impairments may include severe weakness, muscle stiffness and spasms, paralysis, incontinence, or chronic neuropathic pain. Aggressive treatment and physical therapy have been shown to improve outcomes.
Most people who develop this condition only have one episode of TM. If you have an underlying condition that has resulted in TM, your practitioner will prescribe preventative therapies to reduce the chance of a recurrence.
Healthcare providers don’t know why some are affected by TM more than others. What they do know is that the faster the symptoms appear, the poorer a person’s prognosis will be. Therefore, early diagnosis and treatment can help to improve a person’s outlook.
Call your practitioner right away or go to your local emergency room if you think you are experiencing signs and symptoms of transverse myelitis. A number of neurological conditions can cause similar symptoms and could require a medical or surgical emergency.