Transverse myelitis

Transverse myelitis is a neurological disorder caused by inflammation of the spinal cord, the part of the central nervous system that sends impulses from the brain to nerves in the body. The spinal cord also carries sensory information back to the brain.

Myelitis refers to inflammation of the spinal cord. It can damage the insulating material, called myelin, that covers nerve cell fibers. Transverse refers to the pattern of changes in sensation—there is often a band-like sensation across the trunk of the body, with sensory changes below that area.

The segment of the spinal cord at which the damage occurs determines which parts of the body are affected. Damage at one segment will affect function at that level and below. In people with transverse myelitis, myelin damage most often occurs in nerves in the upper back.

Although some people recover from transverse myelitis with minor or no residual problems, the healing process may take months to years. Most people with transverse myelitis have at least partial recovery, with most recovery taking place within the first three months after the attack. Other people may have permanent impairments that affect their ability to perform ordinary tasks of daily living. Some people will have only one episode of transverse myelitis, but others may have a recurrence, especially if an underlying illness caused the disorder.

Transverse myelitis may be either acute (developing over hours to several days) or subacute (usually developing over one to four weeks).

Four classic features of transverse myelitis are:

1. Weakness of the legs and arms—People with transverse myelitis may have weakness in the legs that progresses rapidly. If the myelitis affects the upper spinal cord, it affects the arms as well. People may develop paraparesis (partial paralysis of the legs) that may progress to paraplegia (complete paralysis of the legs), requiring the person to use a wheelchair.
   
2. Pain—Initial symptoms usually include lower back pain or sharp, shooting sensations that radiate down the legs or arms or around the torso.
   
3. Sensory alterations—Transverse myelitis can cause paresthesias (abnormal sensations such as burning, tickling, pricking, numbness, coldness, or tingling) in the legs, and sensory loss. Abnormal sensations in the torso and genital region are common.
   
4. Bowel and bladder dysfunction—Common symptoms include an increased frequency or urge to use the toilet, incontinence, and constipation.

Many people also report having muscle spasms, a general feeling of discomfort, headache, fever, and loss of appetite, while some people experience respiratory problems. Other symptoms may include sexual dysfunction, depression or anxiety caused by lifestyle changes, stress, and chronic pain.

Who is more likely to get transverse myelitis?

Transverse myelitis can affect people of any age, gender, or race. It does not appear to be genetic or to run in families. The disorder occurs most frequently in people who are either between ages 10 and 19 years old or between 30 and 39 years old.

The following conditions appear to cause transverse myelitis:

• Immune system disorders appear to play an important role in causing damage to the spinal cord. Such disorders are:
  
  • Multiple sclerosis (SMS), a disorder in which immune system cells that normally protect us from viruses, bacteria, and unhealthy cells mistakenly attack the protective coating of myelin in the brain, optic nerves, and spinal cord
    
  • Aquaporin-4 autoantibody associated neuromyelitis optica, a disorder that affects the eye nerves and spinal cord. Aquaporin-4 is a channel on the cell membrane that lets water enter the cell and helps maintain the chemical balance so that the central nervous system will work correctly. An antibody is a protein that binds to foreign substances that can attack the host organism.
    
  • Post-infectious or post-vaccine autoimmune phenomenon, in which the body's immune system mistakenly attacks the body's own tissue while responding to the infection or, less commonly, a vaccine
    
  • An abnormal immune response to an underlying cancer that damages the nervous system
    
  • Other antibody-mediated conditions that are still being discovered.
    
• Viral infections including herpes viruses such as varicella zoster (the virus that causes chickenpox and shingles), herpes simplex, cytomegalovirus, and Epstein-Barr; flaviviruses such as West Nile and Zika; influenza, echovirus, hepatitis B, mumps, measles, and rubella. It is often difficult to know whether direct viral infection or a post-infectious response causes the transverse myelitis.
  
• Bacterial infections such as syphilis, tuberculosis, actinomyces, pertussis, tetanus, diphtheria, and Lyme disease. Bacterial skin infections, middle-ear infections, campylobacter jejuni gastroenteritis, and mycoplasma bacterial pneumonia have also been associated with transverse myelitis.
  
• Fungal infections in the spinal cord, including aspergillus, blastomyces, coccidioides, and cryptococcus.
• Parasites, including toxoplasmosis, cysticercosis, shistosomiasis, and angtiostrongyloides.
  
• Other inflammatory disorders that can affect the spinal cord, such as sarcoidosis, systemic lupus erythematosus, Sjogren's syndrome, mixed connective tissue disease, scleroderma, and Bechet's syndrome.
  
• Vascular disorders such as arteriovenous malformation, dural arterial-venous fistula, intra-spinal cavernous malformations, or disk embolism.

The exact cause of transverse myelitis and extensive damage to the bundles of nerve fibers of the spinal cord is unknown in many cases. When doctors cannot identify a cause for the disorder, they refer to it as idiopathic, which means the cause is unknown.

In some people, transverse myelitis is the first symptom of an autoimmune or immune-mediated disease such as multiple sclerosis or neuromyelitis optica.

“Partial” myelitis, which affects only a portion of the spinal cord cross-section, is more common with multiple sclerosis.

“Complete” myelitis, where there is severe paralysis and numbness on both sides of the spinal cord, is more likely to be a symptom of neuromyelitis optica. Myelitis attacks with neuromyelitis optica spectrum disorder (NMOSD) tend to be more severe than attacks caused by multiple sclerosis, and people are less likely to have a complete recovery.

How is transverse myelitis diagnosed and treated?

Diagnosing transverse myelitis. Physicians diagnose transverse myelitis by taking a medical history and performing a thorough neurological examination. These tests can indicate a diagnosis of transverse myelitis and rule out or evaluate underlying causes:

• Magnetic resonance imaging (MRI) produces a cross-sectional view or three-dimensional image of tissues, including the brain and spinal cord. A spinal MRI will almost always confirm the presence of a damaged area (also called a lesion), within the spinal cord, while brain MRI may provide clues to other underlying causes, especially MS.
  
• Computed tomography (CT) is a type of multi-dimensional x-ray that may be used to detect inflammation in the spine.
  
• Blood tests may be used to identify or rule out various disorders, including HIV infection and vitamin B12 deficiency. Blood is tested for the presence of autoantibodies (anti- aquaporin-4, anti-myelin oligodendrocyte) and antibodies associated with cancer (paraneoplastic antibodies). The presence of autoantibodies (proteins produced by cells of the immune system) is linked to autoimmune disorders and point to a definite cause of transverse myelitis.
  
• Lumbar puncture and spinal fluid analysis (also called a spinal tap) can identify more protein than usual in some people with transverse myelitis and an increased number of white blood cells (leukocytes) that help the body fight infections.

Treating transverse myelitis. There is no cure for transverse myelitis, but there are treatments to prevent or minimize permanent neurological problems.

Treatments are designed to address infections that may cause the disorder, reduce spinal cord inflammation, and manage and reduce symptoms.

These are some of the most common initial treatments for transverse myelitis:

• Intravenous corticosteroid drugs may decrease swelling and inflammation in the spine and reduce immune system activity. Such drugs may include methylprednisolone or dexamethasone. These medications may also be given to reduce subsequent attacks of transverse myelitis in people with underlying disorders.
  
• Plasma exchange therapy (plasmapheresis) may be used for people who don't respond well to intravenous steroids. Plasmapheresis is a procedure that reduces immune system activity by removing plasma (the fluid in which blood cells and antibodies are suspended) and replacing it with special fluids, thus removing the antibodies and other proteins thought to be causing the inflammatory reaction.
  
• Intravenous immunoglobulin (IVIG) is a treatment that can help to reset the immune system. IVIG is a highly concentrated injection of antibodies pooled from many healthy donors. It can bind to the antibodies that may cause transverse myelitis and remove them from circulation.
  
• Pain medicines to reduce muscle pain include acetaminophen, ibuprofen, and naproxen. Nerve pain may be treated with certain antidepressant drugs (such as duloxetine), muscle relaxants (such as baclofen, tizanidine, or cyclobenzaprine), and anticonvulsant drugs (such as gabapentin or pregabalin).
  
• Antiviral medications may help people who have a viral infection of the spinal cord.
  
• Medications can also treat other symptoms and complications, including incontinence, painful muscle contractions called tonic spasms, stiffness, sexual dysfunction, and depression.

Following initial therapy, it is critical to keep the person's body functioning during the recovery period. In rare cases when breathing is significantly affected, the person may be placed on a respirator.

Multiple sclerosis and neuromyelitis optica typically require long-term treatment to modify the immune system response. Treatment of MS with immumodulatory or immunosuppressant medications such as alemtuzumab, dimethyl fumarate, fingolimod, glatiramer acetate, interferon-beta, natalizumab, or teriflunomide may be needed.

Immunosuppressant treatments are used for neuromyelitis optica spectrum disorder and recurrent episodes of transverse myelitis that are not caused by multiple sclerosis. They are aimed at preventing future myelitis attacks (or immune attacks on other parts of the body) and they may include steroid-sparing drugs such as mycophenolate mofetil, azathioprine, and rituximab.

Rehabilitation and long-term therapy

Many forms of long-term rehabilitation are available for people who have disabilities resulting from transverse myelitis. Strength and functioning may improve with rehabilitation services, even years after the initial episode.

Although rehabilitation cannot reverse the physical damage resulting from transverse myelitis, it can help people, even those with severe paralysis, become as functionally independent as possible and attain the best possible quality of life.

People with lasting or permanent neurological defects from transverse myelitis typically see a range of rehabilitation specialists, which may include physiatrists, physical therapists, occupational therapists, vocational therapists, and mental health care professionals.

• Physical therapy can help retain muscle strength and flexibility, improve coordination, reduce spasticity, regain greater control over bladder and bowel function, and increase joint movement. People are also taught to use assistive devices such as wheelchairs, canes, or braces.
  
• Occupational therapy teaches people new ways to maintain or rebuild their independence by participating in meaningful, self-directed, everyday tasks such as bathing, dressing, preparing meals, and house cleaning.
  
• Vocational therapy helps people develop and promote work skills, identify potential employers, and assist in job searches. Vocational therapists act as mediators between employees and employers to secure reasonable workplace accommodations.
  
• Psychotherapy for people living with permanent damage includes strategies and tools to deal with stress and a wide range of emotions and behaviors.

What are the latest updates on transverse myelitis?

NINDS funding supports researchers who work to understand how the immune system destroys or attacks the nerve-insulating substance called myelin in autoimmune diseases or disorders. Other research focuses on strategies to repair demyelinated spinal cords, including approaches that use cell transplantation. This research may lead to a greater understanding of the mechanisms that damage myelin. It may ultimately provide a means to prevent and improve treatments for transverse myelitis.

• Glial cell studies—Glia, or neuroglia, are non-neuronal cells (cells that do not generate electrical impulses) in the nervous system that form myelin and provide support and protection for neurons. Oligodendrocyte progenitor cells (OPCs) are stem cells that generate oligodendrocytes, a type of glial cell that produces myelin. NINDS-funded scientists are studying mechanisms in cells that control the generation and maturation of OPCs and possible ways to increase oligodendrocyte proliferation and remyelination after spinal cord injury.
  • Astrocytes are another type of glial cell. The aquaporin-4 IgG antibody binds to astrocytes, which has led to an increased interest in its role in transverse myelitis of neuromyelitis optica spectrum disorder (NMOSD). The antibody appears to cause myelitis in NMOSD by activating other parts of the immune system, resulting in injury to the spinal cord. Many researchers are trying to understand how astrocytes affect autoimmune diseases.
    
• Genetic studies—NINDS-funded scientists are studying a gene called Brg1 (Brahma-related) gene that appears to be involved in oligodendrocyte myelination in order to learn how it controls central nervous system myelination and remyelination. The long-term goal of this research is to develop drugs that control the activity ofBrg1 and other genes in ways that promote myelination and remyelination.
  
• Animal models—Some researchers use animal models of spinal cord injury to learn ways to replace or regenerate spinal cord nerve cells. The goals of these studies are to find ways to restore function in people who have paralysis.
  
• Neuroimaging with MRI—Research funded by NINDS aims to develop and use new MRI techniques to study the relationship between problems in the spinal cord and neurological dysfunction in MS. This new approach may assess changes in lesions and myelin in MS and possibly transverse myelitis. Other researchers plan to develop MRI methodologies that can identify the extent of injury to the spinal cord and monitor recovery after injury. These techniques may allow earlier detection of transverse myelitis and other neurological disorders such as MS.
  
• Brain-machine interfaces and prosthetic devices—Scientists are developing brain-machine interfaces and neural prostheses to help people with spinal cord damage regain functions by bypassing the injured area. These sophisticated electrical and mechanical devices connect with the nervous system to supplement or replace lost motor and sensory function.

More information on transverse myelitis and spinal cord injury research is available through the NIH RePORTER, a searchable database of current and previously funded research, as well as research results and publications.

How can I or my loved one help improve care for people with transverse myelitis?

Consider participating in a clinical trial so clinicians and scientists can learn more about transverse myelitis. Clinical research uses human volunteers to help researchers learn more about a disorder and perhaps find better ways to safely detect, treat, or prevent disease.

All types of volunteers are needed—those who are healthy or may have an illness or disease—of all different ages, sexes, races, and ethnicities to ensure that study results apply to as many people as possible, and that treatments will be safe and effective for everyone who will use them.

For information about participating in clinical research visit NIH Clinical Research Trials and You. Learn about clinical trials currently looking for people with transverse myelitis at Clinicaltrials.gov.

Where can I find more information about transverse myelitis?

Information may be available from the following resources:

Christopher and Dana Reeve Foundation
Phone: 800-225-0292

MedlinePlus

National Organization for Rare Disorders (NORD)
Phone: 617-249-7300 or 800-999-6673

Siegel Rare Neuroimmune Association
Phone: 855-380-3330

The Guthy-Jackson Charitable Foundation
Phone: 310- 620-3074

Content source: https://www.ninds.nih.gov/health-information/disorders/transverse-myelitis Accessed July 17, 2023.

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