Behavioral & Cognitive Disorders
Theory of mind
Dec. 14, 2022
At vero eos et accusamus et iusto odio dignissimos ducimus qui blanditiis praesentium voluptatum deleniti atque corrupti quos dolores et quas.
What is syringomyelia?
Syringomyelia is a disorder in which a fluid-filled cyst (called a syrinx) forms within the spinal cord. This syrinx can get bigger and elongate over time, damaging the spinal cord and compressing and injuring the nerve fibers that carry information to the brain and from the brain to the rest of the body.
A watery liquid known as cerebrospinal fluid (CSF) normally surrounds and protects the brain and spinal cord. CSF also fills connected cavities within the center of the brain called the ventricles which continue to a small central canal that runs the length of the spinal cord. When a person has syringomyelia this fluid collects within the tissue of the spinal cord, expands the central canal and forms a syrinx. Generally, a syrinx develops when the normal flow of CSF fluid around the spinal cord or lower brain stem is disturbed. When syrinxes affect the brain stem, the condition is called syringobulbia.
What causes syringomyelia?
Syringomyelia may have several possible causes but most cases are associated with Chiari malformation—an abnormal condition in which brain tissue extends through the hole at the bottom of the skull (foramen magnum) and into the spinal canal, and obstructs the flow of CSF. Syringomyelia may also be caused by spinal cord injuries, spinal cord tumors, and damage caused by inflammation in around the spinal cord. In some cases, the cause is unknown (idiopathic).
What are the forms of syringomyelia?
There are two major forms of syringomyelia.
What are symptoms of syringomyelia?
Symptoms of damage to the spinal cord vary among individuals according to where the syrinx forms, how large it is, and how long it extends. Symptoms develop slowly over time and may occur on one or both sides of the body. Sometimes coughing and straining can trigger symptoms, but they do not cause syringomyelia. Symptoms may include:
How is syringomyelia diagnosed?
To diagnose syringomyelia, a physician will review the person's medical history and perform a physical exam focusing on neurological function. The physician will also likely order imaging of the spine or brain. Sometimes, syringomyelia may be found during diagnostic imaging for another disorder.
Magnetic resonance imaging (MRI) is the most reliable way to diagnose syringomyelia. Computer-generated radio waves and a powerful magnetic field produce clear images of the brain and spinal cord. Using this test, a physician will be able to determine if there is a syrinx in the spine or another abnormality, such as a tumor.
In some cases a dynamic MRI may be performed to show the flow of fluid around the spinal cord and within the syrinx. Many images are taken in rapid succession to provide such dynamic views. A dye or contrast agent may be injected to enhance the MRI images.
How is syringomyelia treated?
The type of treatment for syringomyelia depends on the severity and progression of an individual’s symptoms.
Monitoring. In the absence of symptoms, syringomyelia is usually not treated. In addition, a physician may recommend not treating the condition in individuals of advanced age or in cases where there is no progression of symptoms. However, people should be carefully monitored by a neurologist or neurosurgeon as symptoms can worsen over time. Individuals may also want to avoid activities that involve straining (e.g., lifting heavy objects, jumping) since these actions can trigger symptoms. People with an associated Chiari malformation are especially apt to experience headache with straining.
Surgery. Surgery is usually recommended for individuals with symptomatic or progressive syringomyelia. There are two general forms of treatment: restoration of normal CSF flow around the spinal cord, and direct drainage of the syrinx. The type of treatment depends on what is causing the symptoms.
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 (NIH), the leading supporter of biomedical research in the world.
Genetics and birth defects. Studies are underway to better understand the role of genetic factors in Chiari I malformation, the most common cause of syringomyelia. Individuals with a Chiari I malformation who also have a family member with either the abnormality or syringomyelia are being studied to identify the location of the gene(s) responsible for the malformation.
In some cases, birth defects may be associated with brain malformations that can cause syringomyelia. Learning how and when these defects occur during fetal development may help scientists develop strategies that can stop the formation of certain birth defects. The use of folic acid dietary supplements during pregnancy, for example, has been found to reduce the risk of birth defects of the brain and spinal cord, including malformations of the skull, brain, and spine.
NINDS scientists are examining individuals who either have syringomyelia or are at risk of developing the disorder. They are investigating the factors that influence its development, progression, and response to treatment by recording more than 5 years of symptoms, muscle strength, overall function, and MRI findings from individuals who receive standard treatment for syringomyelia. Study results may allow scientists to provide more accurate recommendations to future patients with syringomyelia regarding optimal surgical or non-surgical treatments.
Also, NIH-funded scientists are trying to find ways to stop and reverse the cell damage caused by a spinal cord injury to help individuals recover their ability to move their muscles and other functions.
Diagnostic tools. Diagnostic technology is another area for continued research. Ongoing NIH-funded research is aimed at improving diagnostic imaging techniques to better visualize conditions in the spine, including syringomyelia, even before symptoms appear.
In addition, the NINDS is developing common data elements for Chiari Malformation. This will allow researchers to have a common scientific language that will improve data quality and opportunities to compare and combine data at multiple institutions.
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 about research on syringomyelia supported by NINDS and other NIH Institutes and Centers can be found using NIH RePORTER (projectreporter.nih.gov), a searchable database of current and past research projects supported by NIH and other federal agencies. RePORTER also includes links to publications and resources from these projects.
Information also is available from the following organizations:
American Chronic Pain Association
P.O. Box 850
Rocklin, CA 95677-0850
American Syringomyelia & Chiari Alliance Project
P.O. Box 1586
Longview, TX 75606-1586
Chiari and Syringomyelia Foundation
29 Crest Loop
Staten Island, NY 10312
Christopher and Dana Reeve Foundation
636 Morris Turnpike, Suite 3A
Short Hills, NJ 07078
National Organization for Rare Disorders (NORD)
55 Kenosia Avenue
Danbury, CT 06810-1968
Toll-free voicemail: 800-999- 6673
U.S. National Library of Medicine
National Institutes of Health/DHHS
8600 Rockville Pike
Bethesda, MD 20894
"Syringomyelia Fact Sheet", NINDS, Publication date May 2017. NIH Publication No. 17-3780.
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892
NINDS health-related material is provided for information purposes only and does not necessarily represent endorsement by or an official position of the National Institute of Neurological Disorders and Stroke or any other Federal agency. Advice on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined that patient or is familiar with that patient's medical history.
All NINDS-prepared information is in the public domain and may be freely copied.
The information in this document is for general educational purposes only. It is not intended to substitute for personalized professional advice. Although the information was obtained from sources believed to be reliable, MedLink, its representatives, and the providers of the information do not guarantee its accuracy and disclaim responsibility for adverse consequences resulting from its use. For further information, consult a physician and the organization referred to herein.