Acute drug-induced movement disorders
Jun. 26, 2021
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What is transverse myelitis?
Transverse myelitis is an inflammation of the spinal cord, a major part of the central nervous system. The spinal cord carries nerve signals to and from the brain through nerves that extend from each side of the spinal cord and connect to nerves elsewhere in the body. The term myelitis refers to inflammation of the spinal cord; 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.
Causes of transverse myelitis include infections, immune system disorders, and other disorders that may damage or destroy myelin, the fatty white insulating substance that covers nerve cell fibers. Inflammation within the spinal cord interrupts communications between nerve fibers in the spinal cord and the rest of the body, affecting sensation and nerve signaling below the injury. Symptoms include pain, sensory problems, weakness in the legs and possibly the arms, and bladder and bowel problems. The symptoms may develop suddenly (over a period of hours) or over days or weeks.
Transverse myelitis can affect people of any age, gender, or race. It does not appear to be genetic or run in families. A peak in incidence rates (the number of new cases per year) appears to occur between 10 and 19 years and 30 and 39 years. It is estimated that about 1,400 new cases of transverse myelitis are diagnosed each year in the United States.
Although some people recover from transverse myelitis with minor or no residual problems, the healing process may take months to years. Others may suffer permanent impairments that affect their ability to perform ordinary tasks of daily living. Some individuals will have only one episode of transverse myelitis; other individuals may have a recurrence, especially if an underlying illness caused the disorder.
There is no cure for transverse myelitis. Treatments to prevent or minimize permanent neurological deficits include corticosteroid and other medications that suppress the immune system, plasmapheresis (removal of proteins from the blood), or antiviral medications.
What causes transverse myelitis?
The exact cause of transverse myelitis and extensive damage to nerve fibers of the spinal cord is unknown in many cases. Cases in which a cause cannot be identified are called idiopathic. However, looking for a cause is important, as some will change treatment decisions.
The discovery of circulating antibodies to the proteins aquaporin-4 and anti-myelin oligodendrocyte point to a definite cause in some individuals with transverse myelitis. Antibodies are proteins produced by cells of the immune system that bind to bacteria, viruses, and foreign chemicals to prevent them from harming the body. In autoimmune disorders, antibodies incorrectly bind to normal body proteins. Aquaporin-4 is a key protein that carries water through the cell membrane of neural cells. The myelin oligodendrocyte glycoprotein sits on the outer layer of myelin.
A number of conditions appear to cause transverse myelitis, including:
In some people, transverse myelitis represents the first symptom of an autoimmune or immune-mediated disease such as multiple sclerosis or neuromyelitis optica. (Multiple sclerosis, or MS, is disease that causes distinctive lesions, or plaques, that primarily affect parts of the brain, spinal cord, and optic nerve—the nerve that carries information from the eye to the brain. Neuromyelitis optica, or NMO, is an autoimmune disease of the central nervous system that predominantly affects the optic nerves and spinal cord.) ”Partial” myelitis—affecting only a portion of the cord cross-section—is more characteristic of multiple sclerosis. Neuromyelitis optica is much more likely as an underlying condition when the myelitis is “complete” (causing severe paralysis and numbness on both sides of the spinal cord).
What are the symptoms of transverse myelitis?
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:
Many individuals also report experiencing 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 and depression and anxiety caused by lifestyle changes, stress, and chronic pain.
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 individuals with transverse myelitis, myelin damage most often occurs in nerves in the upper back, causing problems with leg movement and bowel and bladder control, which require signals from the lower segments of the spinal cord.
How is transverse myelitis diagnosed?
Physicians diagnose transverse myelitis by taking a medical history and performing a thorough neurological examination. The first step in evaluating a spinal cord condition is to rule out causes that require emergency intervention, such as trauma or a mass putting pressure on the cord. Other problems to rule out include herniated or slipped discs, stenosis (narrowing of the canal that holds the spinal cord), abscesses, abnormal collections of blood vessels, and vitamin deficiencies. Tests that can indicate a diagnosis of transverse myelitis and rule out or evaluate underlying causes include:
If none of these tests suggests a specific cause, the person is presumed to have idiopathic transverse myelitis. In occasional cases, initial testing using MRI and lumbar puncture may show normal results but may need to be repeated in 5-7 days.
How is transverse myelitis treated?
Treatments are designed to address infections that may cause the disorder, reduce spinal cord inflammation, and manage and alleviate symptoms.
Initial treatments and management of the complications of transverse myelitis
Following initial therapy, it is critical part to keep the person’s body functioning while hoping for either complete or partial spontaneous recovery of the nervous system. This may require placing the person on a respirator in the uncommon scenario where breathing is significantly affected. Treatment is most often given in a hospital or in a rehabilitation facility where a specialized medical team can prevent or treat problems that afflict paralyzed individuals.
Prevention of future transverse myelitis episodes. Most transverse myelitis only occurs once (called monophasic). In some cases chronic (long-term) treatment with medications to modify the immune system response is needed. Examples of underlying disorders that may require long-term treatment include multiple sclerosis and neuromyelitis optica. Treatment of MS with immumodulatory or immunosuppressant medications may be considered when it is the cause of myelitis. These medications include alemtuzumab, dimethyl fumarate, fingomilod, glatiramer acetate, interferon-beta, natalizumab, and teriflunomide, among others.
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 attacks at other sites) and include steroid-sparing drugs such as mycophenolate mofetil, azathioprine, and rituximab.
Rehabilitative and long-term therapy. Many forms of long-term rehabilitative therapy are available for people who have disabilities resulting from transverse myelitis. Strength and functioning may improve with rehabilitative services, even years after the initial episode. Rehabilitative therapy teaches people strategies for carrying out activities in new ways in order to overcome, circumvent, or compensate for permanent disabilities. 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.
Common neurological deficits resulting from transverse myelitis include severe weakness, spasticity, or paralysis; incontinence, and chronic pain. In some cases these may be permanent. Such deficits can substantially interfere with a person’s ability to carry out everyday activities such as bathing, dressing, and performing household tasks. Individuals with lasting neurological defects from transverse myelitis typically consult with a range of rehabilitation specialists, who may include physiatrists (physicians specializing in physical medicine and rehabilitation), physical therapists, occupational therapists, vocational therapists, and mental health care professionals.
What is the prognosis?
Most people with transverse myelitis have at least partial recovery, with most recovery taking place within the first 3 months after the attack. For some people, recovery may continue for up to 2 years (and in some cases, longer). However, if there is no improvement within the first 3 to 6 months, complete recovery is unlikely (although partial recovery can still occur and still requires rehabilitation). Aggressive acute treatment and physical therapy have been shown to improve outcomes. Some individuals are left with moderate disability (such as trouble walking, nerve sensitivity, and bladder and bowel problems) while others may have permanent weakness, spasticity, and other complications. Myelitis attacks with neuromyelitis optica spectrum disorder (NMOSD) tend to be more severe and are associated with less recovery than attacks with multiple sclerosis. Research has shown that a rapid onset of symptoms generally results in poorer recovery.
Many people with transverse myelitis experience only one episode although recurrent or relapsing transverse myelitis does sometimes occur, particularly when an underlying cause (such as MS or NMOSD) can be found. Some people recover completely and then experience a relapse. Others begin to recover and then suffer worsening of symptoms before recovery continues. In all cases of transverse myelitis, physicians will evaluate possible underlying causes such as MS, NMOSD, or sarcoidosis, since most people with these underlying conditions can experience a relapse or worsen when acute treatment is discontinued. These individuals should be treated with preventative care to reduce the chance of future relapses.
What research is being done?
The mission of the National Institute of Neurological Disorders and Stroke (NINDS) is to seek fundamental knowledge about the brain and nervous system and to use that knowledge to reduce the burden of neurological disease. The NINDS is a component of the National Institutes of Health, the leading supporter of biomedical research in the world.
NINDS researchers are working to better understand the process by which the immune system destroys or attacks the nerve-insulating substance called myelin in autoimmune diseases or disorders. Other work focuses on strategies to repair demyelinated spinal cords, including approaches using cell transplantation. This research may lead to a greater understanding of the mechanisms responsible for damaging myelin and may ultimately provide a means to prevent and treat transverse myelitis.
Specialized cells called oligodendrocytes lay down multiple layers of myelin around axons, the long “wires” that connect brain cells. Adult brains contain oligodendrocyte progenitor cells (OPCs), which are stem cells that generate myelin-producing cells. OCPs are found to multiply in the brains of individuals with multiple sclerosis as if to respond to myelin damage, but for unknown reasons they are not effective in restoring white matter. NINDS-funded scientists are studying cellular mechanisms that control the generation and maturation of OPCs to allow remyelination, which could be an effective therapy for transverse myelitis and spinal cord injury. Other NIND-funded investigators are focusing on mechanisms and interventions designed to increase oligodendrocyte proliferation and remyelination after spinal cord injury.
NINDS-funded scientists hope to develop a better understanding of the molecular control of central nervous system myelination and remyelination by studying the Brg1 (Brahma-related gene) that appears to be involved in oligodendrocyte myelination. The long-term objective of this research is to develop compounds that modulate the activity of Brg1 and other genes to promote myelination and remyelination.
The discovery of aquaporin-4 IgG, an antibody that binds to a type of protein called a water channel that is found on the surface of specialized central nervous system cells called astrocytes, has led to increased interest in their role in transverse myelitis of neuromyelitis optica spectrum disorder (NMOSD). The aquaporin-4 IgG antibody appears to cause myelitis in NMOSD by binding to the water channel and activating other components of the immune system, resulting in injury to the spinal cord. Many studies are trying to better understand the role of astrocytes in the autoimmune diseases of the central nervous system. Multiple studies are looking at ways to target different components of the immune system known to be involved in NMOSD to allow more directly targeted treatment of this disease.
The NINDS also funds research using animal models of spinal cord injury aimed at replacing or regenerating spinal cord nerve cells. The ultimate goals of these studies are to develop interventions for regeneration or remyelination of spared nerve fibers in humans and to restore function to paralyzed individuals. Scientists are also developing brain-machine interfaces and neural prostheses to help people with spinal cord damage regain functions by bypassing the injury site. These sophisticated electrical and mechanical devices connect with the nervous system to supplement or replace lost motor and sensory function.
Research funded by NINDS aims to develop and implement new MRI techniques to quantitatively assess the relationship between spinal cord pathology and neurological dysfunction in MS. This new approach may assess changes in lesions and otherwise normal appearing myelin, as well as address the neurological dysfunction in MS and possibly other myelin disorders such as transverse myelitis.
Inflammatory or degenerative diseases of the brain and spinal cord may be related to problems with a person’s immune system. An NINDS-funded study comparing clinical MRI and lumbar puncture of healthy individuals to those with symptoms of immune-related central nervous system damage hopes to identify processes or mechanisms to inhibit or minimize spinal tissue damage and enhance recovery.
Other NIH-funded researchers plan to develop MRI methodologies to non-invasively detect and characterize networks to identify the extent of injury to the spinal cord and to monitor the progression of recovery after injury. These techniques may aid in earlier detection of disorders such as transverse myelitis and MS.
Where can I get more information?
For more information on neurological disorders or research programs funded by the National Institute of Neurological Disorders and Stroke, contact the Institute's Brain Resources and Information Network (BRAIN) at:
P.O. Box 5801
Bethesda, MD 20824
More information on transverse myelitis and spinal cord injury research supported by the NINDS and other NIH components is available through the NIH RePORTER (http://projectreporter.nih.gov), a searchable database of current and previously funded research, as well as research results and publications.
Information also is available from the following organizations:
Transverse Myelitis Association
1787 Sutter Parkway
Powell, OH 43605-4884
Cody Unser First Sep Foundation
P.O. Box 56696
Albuquerque, NM 87187
Christopher and Dana Reeve Foundation
636 Morris Turnpike, Suite 3A
Short Hills, NU 07078
The Guthy-Jackson Charitable Foundation
10525 Vista Sorrento Parkway, Suite 210
San Diego, CA 92121
National Organization for Rare Disorders (NORD)
55 Kenosia Avenue
Danbury, CT 06810
National Library of Medicine
8600 Rockville Pike
Bethesda, MD 20894
"Transverse Myelitis Fact Sheet", NINDS, Publication date $pubdate. NIH Publication No. 17-4841.
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
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