An Overview of Transverse Myelitis

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Transverse myelitis (TM) is a rare neurological syndrome that causes inflammation on both sides of the spinal cord, resulting from damage to nerve cells. TM is also an autoimmune disorder, which means the immune system attacks its own healthy tissues because it mistakes them for unhealthy ones. The tissue that gets attacked in this case is the myelin, a fatty tissue that protects nerve fibers.

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 while it is an autoimmune disease, it does not appear to be related to genetics or family history. There is no cure for the condition and treatment is aimed at prevention of recurrences and minimizing neurological deficits.

Symptoms

Myelin wraps around nerve fibers in the same way insulation covers electrical wires. When myelin tissue becomes damaged, the nerves underneath might become inflamed, infected, and/or scarred, a state called myelitis.

Myelitis makes it harder for the nerves to send signals to other parts of the body. A person may also experience pain, weakness, and paralysis.

When the nerves on both sides of the spinal cord are damaged, myelitis is called transverse myelitis.

Transverse myelitis is either acute or subacute. Acute means that 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 also 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 legs, torso, and genital area. It can also cause sensory loss (loss of smell, taste, touch, sight, or hearing). 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 state 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
    • Headache
    • Fever
    • Loss of appetite
    • Breathing difficulties
    • Sexual dysfunction
    • Depression
    • Anxiety

    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.

    For many people, myelin damage affects the upper back, which mean problems in the legs, bowel, and bladder, because all these parts need signals from the lower parts of the spinal cord.

    Causes

    An exact cause of TM and damage to myelin fibers is unknown. Sometimes, doctors can pinpoint a cause, and other times it is considered idiopathic which means the cause is not known. Having a cause is important because it will assist in treatment.

    Certain Antibodies

    The discovery of two antibodies (proteins)—aquaporin-4 and anti-myelin oligodendrocyte—might point to a definite cause in some people with TM. However, the exact process is unknown.

    Antibodies are proteins produced by your immune system that bind to bacteria, viruses, and other foreign substances to keep them from harming your body. Aquaporin-4 is a key protein responsible for carrying water through neural cells—cells that transmit electrical and chemical signals. The myelin oligodendrocyte glycoproteins sit on the outer layers of myelin.

    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 that sends information from the eye to the brain. 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 primary affects the optic nerves and spinal cord. NMO is known for causing severe paralysis and numbness of the spinal cord.
    • Viral infections: Viruses associated with transverse myelitis include the varicella-zoster (chickenpox and shingles virus), echovirus, influenza, HIV, hepatitis A, and rubella.
    • Bacterial infections: Bacterial skin infections, middle ear infections, and bacterial pneumonia have all been associated with transverse myelitis.
    • Fungal infections: Fungal infections of the spinal cord—such as aspergillus—have been linked to TM.
    • Parasites: Several different kinds of parasite-type infections­, like toxoplasmosis, have been associated with the development of TM.
    • Inflammatory disorders: 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.
    • Vascular disorders: Different types of vascular conditions, such as arteriovenous malformation, can contribute 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 is the case with multiple sclerosis or neuromyelitis optica.

    Diagnosis

    Your doctor can make a diagnosis of transverse myelitis based on your symptoms and medical history, a neurological examination, and various other testing methods.

    Your doctor will first want 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 where the spinal cord canal has narrowed), vitamin deficiencies, blood vessel problems, and abscesses.

    Tests that your doctor 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 look for inflammation or other abnormalities affecting the spinal cord or blood vessels and nerves of 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. With TM, CSF may show abnormally high levels of white blood cells and immune system proteins that promote inflammation. 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 abnormal results and your doctor will want to retest after a few days.

    Treatment

    Transverse myelitis is a very rare condition, which means there isn’t much information or research on the best treatment methods.

    Steroid Treatment

    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 doctor will have you on intravenous (IV) methylprednisolone (prednisone), 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 prednisone 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

    Plasma Exchange

    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 disorders and other inflammatory system 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.

    Other Treatments

    For people who do not respond to steroids or PLEX, and where 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 Verywell 

    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 doctor will prescribe preventative therapies to reduce the chance of a recurrence.

    Doctors 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 doctor 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.

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    Article Sources

    1. National Organization for Rare Disorders (NORD). Transverse Myelitis. Updated 2018.


    2. National Institute of Neurological Disorders and Stroke. Transverse Myelitis Fact Sheet. Updated August 13, 2019.


    3. Sechi E, Shosha E, Williams JP, et al. Aquaporin-4 and MOG autoantibody discovery in idiopathic transverse myelitis epidemiology. Neurology. Jul 2019;93(4):e414-e420. doi:10.1212/WNL.0000000000007828


    4. Cleveland Clinic. Transverse Myelitis. Updated January 28, 2019.


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