Syringomyelia Information Page

Syringomyelia Information Page


What research is being done?

Investigators at the National Institute of Neurological Disorders and Stroke (NINDS) are studying how syrinxes first form, as well as the mechanisms of the disorders. NINDS investigators have found that the normal flow of cerebrospinal fluid that occurs with each heartbeat is obstructed in people with syringomyelia. Surgical procedures that relieve this obstruction usually result in the syrinx becoming much smaller in size. Studies are also underway to better understand genetic factors that influence the development of Chiari I malformations and syringomyelia, as well as to identify the location of the gene(s) responsible for the malformation. Researchers hope to better understand the role of birth defects of the skull and brain imalformations in the development of syringomyelia. Diagnostic technology is another area for continued research.  

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 treatment by recording more than 5 years of symptoms, muscle strength, overall function, and magnetic resonance imaging (MRI) scan findings from individuals who receive standard treatment for syringomyelia.  Study results may allow scientists to provide more accurate recommendations to future individuals with syringomyelia regarding optimal surgical or non-surgical treatments.

Information from the National Library of Medicine’s MedlinePlus
Syringomyelia

×
What research is being done?

Investigators at the National Institute of Neurological Disorders and Stroke (NINDS) are studying how syrinxes first form, as well as the mechanisms of the disorders. NINDS investigators have found that the normal flow of cerebrospinal fluid that occurs with each heartbeat is obstructed in people with syringomyelia. Surgical procedures that relieve this obstruction usually result in the syrinx becoming much smaller in size. Studies are also underway to better understand genetic factors that influence the development of Chiari I malformations and syringomyelia, as well as to identify the location of the gene(s) responsible for the malformation. Researchers hope to better understand the role of birth defects of the skull and brain imalformations in the development of syringomyelia. Diagnostic technology is another area for continued research.  

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 treatment by recording more than 5 years of symptoms, muscle strength, overall function, and magnetic resonance imaging (MRI) scan findings from individuals who receive standard treatment for syringomyelia.  Study results may allow scientists to provide more accurate recommendations to future individuals with syringomyelia regarding optimal surgical or non-surgical treatments.

Information from the National Library of Medicine’s MedlinePlus
Syringomyelia

Investigators at the National Institute of Neurological Disorders and Stroke (NINDS) are studying how syrinxes first form, as well as the mechanisms of the disorders. NINDS investigators have found that the normal flow of cerebrospinal fluid that occurs with each heartbeat is obstructed in people with syringomyelia. Surgical procedures that relieve this obstruction usually result in the syrinx becoming much smaller in size. Studies are also underway to better understand genetic factors that influence the development of Chiari I malformations and syringomyelia, as well as to identify the location of the gene(s) responsible for the malformation. Researchers hope to better understand the role of birth defects of the skull and brain imalformations in the development of syringomyelia. Diagnostic technology is another area for continued research.  

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 treatment by recording more than 5 years of symptoms, muscle strength, overall function, and magnetic resonance imaging (MRI) scan findings from individuals who receive standard treatment for syringomyelia.  Study results may allow scientists to provide more accurate recommendations to future individuals with syringomyelia regarding optimal surgical or non-surgical treatments.

Information from the National Library of Medicine’s MedlinePlus
Syringomyelia

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Definition
Definition
Treatment
Treatment
Prognosis
Prognosis
Clinical Trials
Clinical Trials
Organizations
Organizations
Publications
Publications
Definition
Definition

Syringomyelia (sear-IN-go-my-EEL-ya) is a disorder in which a fluid-filled cyst forms within the spinal cord. This cyst, called a syrinx, expands and elongates over time, damaging the spinal cord. Since the spinal cord connects the brain to nerves in the extremities, this damage may cause pain, weakness, and stiffness in the back, shoulders, arms, or legs. Symptoms vary among individuals. Other symptoms may include headaches and a loss of the ability to feel extremes of hot or cold, especially in the hands. Signs of the disorder tend to develop slowly, although sudden onset may occur with coughing or straining. If not treated surgically, syringomyelia often leads to progressive weakness in the arms and legs, loss of hand sensation, and chronic, severe pain. In most cases, the disorder is related to a congenital abnormality of the brain called a Chiari malformation, which causes brain tissue to protrude from its normal location in the back of the head and into the cervical or neck portion of the spinal canal. Syringomyelia may also occur as a complication of trauma, inflammation, spinal cord injury, hemorrhage, spinal cord tumors, or other conditions. Symptoms may appear months or even years after the initial injury, starting with pain, weakness, and sensory impairment originating at the site of trauma. Some cases of syringomyelia are familial, although this is rare.

×
Definition

Syringomyelia (sear-IN-go-my-EEL-ya) is a disorder in which a fluid-filled cyst forms within the spinal cord. This cyst, called a syrinx, expands and elongates over time, damaging the spinal cord. Since the spinal cord connects the brain to nerves in the extremities, this damage may cause pain, weakness, and stiffness in the back, shoulders, arms, or legs. Symptoms vary among individuals. Other symptoms may include headaches and a loss of the ability to feel extremes of hot or cold, especially in the hands. Signs of the disorder tend to develop slowly, although sudden onset may occur with coughing or straining. If not treated surgically, syringomyelia often leads to progressive weakness in the arms and legs, loss of hand sensation, and chronic, severe pain. In most cases, the disorder is related to a congenital abnormality of the brain called a Chiari malformation, which causes brain tissue to protrude from its normal location in the back of the head and into the cervical or neck portion of the spinal canal. Syringomyelia may also occur as a complication of trauma, inflammation, spinal cord injury, hemorrhage, spinal cord tumors, or other conditions. Symptoms may appear months or even years after the initial injury, starting with pain, weakness, and sensory impairment originating at the site of trauma. Some cases of syringomyelia are familial, although this is rare.

Treatment
Treatment

If there are no symptoms, syringomyelia is usually not treated and the person is monitored by a neurologist or neurosurgeon. Surgery is usually recommended for individuals with symptomatic or progressive syringomyelia, with the type of surgery and its location dependent on the type of syrinx. In persons with syringomyelia that is associated with the Chiari malformation, a procedure that removes skulll bone and expands the space around the malformation usually prevents new symptoms from developing and results in the syrinx becoming smaller. In some individuals it may be necessary to drain the syrinx, which can be accomplished using a catheter, drainage tubes, and valves. Syringomyelia can recur after surgery, making additional operations necessary.  Whether treated or not, many individuals with syringomyelia are told to avoid activities that involve straining.

×
Treatment

If there are no symptoms, syringomyelia is usually not treated and the person is monitored by a neurologist or neurosurgeon. Surgery is usually recommended for individuals with symptomatic or progressive syringomyelia, with the type of surgery and its location dependent on the type of syrinx. In persons with syringomyelia that is associated with the Chiari malformation, a procedure that removes skulll bone and expands the space around the malformation usually prevents new symptoms from developing and results in the syrinx becoming smaller. In some individuals it may be necessary to drain the syrinx, which can be accomplished using a catheter, drainage tubes, and valves. Syringomyelia can recur after surgery, making additional operations necessary.  Whether treated or not, many individuals with syringomyelia are told to avoid activities that involve straining.

Definition
Definition

Syringomyelia (sear-IN-go-my-EEL-ya) is a disorder in which a fluid-filled cyst forms within the spinal cord. This cyst, called a syrinx, expands and elongates over time, damaging the spinal cord. Since the spinal cord connects the brain to nerves in the extremities, this damage may cause pain, weakness, and stiffness in the back, shoulders, arms, or legs. Symptoms vary among individuals. Other symptoms may include headaches and a loss of the ability to feel extremes of hot or cold, especially in the hands. Signs of the disorder tend to develop slowly, although sudden onset may occur with coughing or straining. If not treated surgically, syringomyelia often leads to progressive weakness in the arms and legs, loss of hand sensation, and chronic, severe pain. In most cases, the disorder is related to a congenital abnormality of the brain called a Chiari malformation, which causes brain tissue to protrude from its normal location in the back of the head and into the cervical or neck portion of the spinal canal. Syringomyelia may also occur as a complication of trauma, inflammation, spinal cord injury, hemorrhage, spinal cord tumors, or other conditions. Symptoms may appear months or even years after the initial injury, starting with pain, weakness, and sensory impairment originating at the site of trauma. Some cases of syringomyelia are familial, although this is rare.

Treatment
Treatment

If there are no symptoms, syringomyelia is usually not treated and the person is monitored by a neurologist or neurosurgeon. Surgery is usually recommended for individuals with symptomatic or progressive syringomyelia, with the type of surgery and its location dependent on the type of syrinx. In persons with syringomyelia that is associated with the Chiari malformation, a procedure that removes skulll bone and expands the space around the malformation usually prevents new symptoms from developing and results in the syrinx becoming smaller. In some individuals it may be necessary to drain the syrinx, which can be accomplished using a catheter, drainage tubes, and valves. Syringomyelia can recur after surgery, making additional operations necessary.  Whether treated or not, many individuals with syringomyelia are told to avoid activities that involve straining.

Prognosis
Prognosis

Symptoms usually begin in young adulthood, with symptoms of one form usually beginning between the ages of 25 and 40. If not treated surgically (when needed), syringomyelia often leads to progressive weakness in the arms and legs, loss of hand sensation, and chronic, severe pain. Symptoms may worsen with straining or any activity that causes cerebrospinal fluid pressure to fluctuate. Some individuals may have long periods of stability. Surgery results in stabilization or modest improvement in symptoms for most individuals. Delay in treatment may result in irreversible spinal cord injury.

×

Symptoms usually begin in young adulthood, with symptoms of one form usually beginning between the ages of 25 and 40. If not treated surgically (when needed), syringomyelia often leads to progressive weakness in the arms and legs, loss of hand sensation, and chronic, severe pain. Symptoms may worsen with straining or any activity that causes cerebrospinal fluid pressure to fluctuate. Some individuals may have long periods of stability. Surgery results in stabilization or modest improvement in symptoms for most individuals. Delay in treatment may result in irreversible spinal cord injury.

Prognosis
Prognosis

Symptoms usually begin in young adulthood, with symptoms of one form usually beginning between the ages of 25 and 40. If not treated surgically (when needed), syringomyelia often leads to progressive weakness in the arms and legs, loss of hand sensation, and chronic, severe pain. Symptoms may worsen with straining or any activity that causes cerebrospinal fluid pressure to fluctuate. Some individuals may have long periods of stability. Surgery results in stabilization or modest improvement in symptoms for most individuals. Delay in treatment may result in irreversible spinal cord injury.

Definition

Syringomyelia (sear-IN-go-my-EEL-ya) is a disorder in which a fluid-filled cyst forms within the spinal cord. This cyst, called a syrinx, expands and elongates over time, damaging the spinal cord. Since the spinal cord connects the brain to nerves in the extremities, this damage may cause pain, weakness, and stiffness in the back, shoulders, arms, or legs. Symptoms vary among individuals. Other symptoms may include headaches and a loss of the ability to feel extremes of hot or cold, especially in the hands. Signs of the disorder tend to develop slowly, although sudden onset may occur with coughing or straining. If not treated surgically, syringomyelia often leads to progressive weakness in the arms and legs, loss of hand sensation, and chronic, severe pain. In most cases, the disorder is related to a congenital abnormality of the brain called a Chiari malformation, which causes brain tissue to protrude from its normal location in the back of the head and into the cervical or neck portion of the spinal canal. Syringomyelia may also occur as a complication of trauma, inflammation, spinal cord injury, hemorrhage, spinal cord tumors, or other conditions. Symptoms may appear months or even years after the initial injury, starting with pain, weakness, and sensory impairment originating at the site of trauma. Some cases of syringomyelia are familial, although this is rare.

Treatment

If there are no symptoms, syringomyelia is usually not treated and the person is monitored by a neurologist or neurosurgeon. Surgery is usually recommended for individuals with symptomatic or progressive syringomyelia, with the type of surgery and its location dependent on the type of syrinx. In persons with syringomyelia that is associated with the Chiari malformation, a procedure that removes skulll bone and expands the space around the malformation usually prevents new symptoms from developing and results in the syrinx becoming smaller. In some individuals it may be necessary to drain the syrinx, which can be accomplished using a catheter, drainage tubes, and valves. Syringomyelia can recur after surgery, making additional operations necessary.  Whether treated or not, many individuals with syringomyelia are told to avoid activities that involve straining.

Prognosis

Symptoms usually begin in young adulthood, with symptoms of one form usually beginning between the ages of 25 and 40. If not treated surgically (when needed), syringomyelia often leads to progressive weakness in the arms and legs, loss of hand sensation, and chronic, severe pain. Symptoms may worsen with straining or any activity that causes cerebrospinal fluid pressure to fluctuate. Some individuals may have long periods of stability. Surgery results in stabilization or modest improvement in symptoms for most individuals. Delay in treatment may result in irreversible spinal cord injury.

What research is being done?

Investigators at the National Institute of Neurological Disorders and Stroke (NINDS) are studying how syrinxes first form, as well as the mechanisms of the disorders. NINDS investigators have found that the normal flow of cerebrospinal fluid that occurs with each heartbeat is obstructed in people with syringomyelia. Surgical procedures that relieve this obstruction usually result in the syrinx becoming much smaller in size. Studies are also underway to better understand genetic factors that influence the development of Chiari I malformations and syringomyelia, as well as to identify the location of the gene(s) responsible for the malformation. Researchers hope to better understand the role of birth defects of the skull and brain imalformations in the development of syringomyelia. Diagnostic technology is another area for continued research.  

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 treatment by recording more than 5 years of symptoms, muscle strength, overall function, and magnetic resonance imaging (MRI) scan findings from individuals who receive standard treatment for syringomyelia.  Study results may allow scientists to provide more accurate recommendations to future individuals with syringomyelia regarding optimal surgical or non-surgical treatments.

Information from the National Library of Medicine’s MedlinePlus
Syringomyelia

Patient Organizations
American Chronic Pain Association (ACPA)
P.O. Box 850
Rocklin
CA
Rocklin, CA 95677-0850
Tel: 916-632-0922; 800-533-3231
American Syringomyelia & Chiari Alliance Project (ASAP)
P.O. Box 1586
Longview
TX
Longview, TX 75606-1586
Tel: 903-236-7079; 800-ASAP-282 (272-7282)
Chiari & Syringomyelia Foundation
29 Crest Loop
Staten Island
NY
Staten Island, NY 10312
Tel: 718-966-2593
Christopher and Dana Reeve Foundation
636 Morris Turnpike
Suite 3A
Short Hills
NJ
Short Hills, NJ 07078
Tel: 973-379-2690; 800-225-0292
March of Dimes
1275 Mamaroneck Avenue
White Plains
NY
White Plains, NY 10605
Tel: 914-997-4488; 888-MODIMES (663-4637)
Paralyzed Veterans of America (PVA)
801 18th Street, NW
Washington
DC
Washington, DC 20006-3517
Tel: 202-USA-1300 (872-1300); 800-555-9140
Spina Bifida Association
4590 MacArthur Blvd. NW
Suite 250
Washington
DC
Washington, DC 20007-4266
Tel: 202-944-3285; 800-621-3141
United Spinal Association
120-34 Queens Boulevard, #320
Kew Gardens
NY
Kew Gardens, NY 11415
Tel: 718-803-3782; 800-962-9629
Worldwide Syringomyelia & Chiari Task Force
P.O. Box 491975
Lawrenceville
GA
Lawrenceville, GA 30049
Tel: 914-510-2873
Publications

Chiari Malformation (Arnold-Chiari malformation) information page compiled by the National Institute of Neurological Disorders and Stroke (NINDS).

Patient Organizations