Syringomyelia
SyrinxSyringomyelia is a cyst-like collection of cerebrospinal fluid (CSF) that forms in the spinal cord. Over time, it may damage the spinal cord.
Causes
The fluid-filled cyst is called a syrinx. The spinal fluid buildup may be caused by:
- Birth defects (especially Chiari malformation, in which part of the brain pushes down onto the spinal cord at the base of the skull)
- Spinal cord trauma
Spinal cord trauma
Spinal cord trauma is damage to the spinal cord. It may result from direct injury to the cord itself or indirectly from disease of the nearby bones,...
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The fluid-filled cyst usually begins in the neck area. It expands slowly, putting pressure on the spinal cord and slowly causing damage.
Onset of syringomyelia is usually between 25 to 40 years old. Males are more affected than females.
Symptoms
If the condition is due to birth defects, there may be no symptoms until age 30 to 40 years. Symptoms of syringomyelia usually appear slowly and worsen over many years. In the case of trauma, the onset of symptoms may be as early as 2 to 3 months of age. If there are symptoms, they may include:
- Headache
Headache
A headache is pain or discomfort in the head, scalp, or neck. Serious causes of headaches are rare. Most people with headaches can feel much better...
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Scoliosis
Scoliosis is an abnormal curving of the spine. Your spine is your backbone. It runs straight down your back. Everyone's spine naturally curves a b...
Read Article Now Book Mark Article - Loss of muscle mass (wasting, atrophy), often in the arms and hands
Wasting
Muscle atrophy is the wasting (thinning) or loss of muscle tissue.
Read Article Now Book Mark Article - Loss of reflexes in upper limbs
- Increased reflexes in lower limbs
- Spasms or tightness in the leg or hand and arm muscles
- Muscle function loss, loss of ability to use arms or legs
Muscle function loss
Muscle function loss is when a muscle does not work or move normally. The medical term for complete loss of muscle function is paralysis.
Read Article Now Book Mark Article - Numbness that decreases the feeling of pain or temperature; lowers the ability to feel when the skin is being touched; occurs in the neck, shoulders, upper arms, and trunk in a cape-like pattern; and slowly gets worse over time
Numbness
Numbness and tingling are abnormal sensations that can occur anywhere in your body, but they are often felt in your fingers, hands, feet, arms, or le...
Read Article Now Book Mark Article - Pain down the arms, neck, or into the middle back or legs
- Weakness (decreased muscle strength) in the arms or legs
- Painless burn or injury of the hand
- Difficulty walking or toe walking in children
- Uncontrollable movements of the eyes (nystagmus)
Nystagmus
Nystagmus is a term to describe uncontrollable movements of the eyes that may be:Side to side (horizontal nystagmus)Up and down (vertical nystagmus)R...
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Horner syndrome
Horner syndrome is a rare condition that affects the nerves to the eyes and face.
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Exams and Tests
Your health care provider will perform a physical exam and ask about the symptoms, focusing on the nervous system. Tests that may be done include:
- MRI of the head and spine
MRI of the head
A head MRI (magnetic resonance imaging) is an imaging test that uses powerful magnets and radio waves to create pictures of the brain and surrounding...
Read Article Now Book Mark Article - Spinal CT scan with myelogram (may be done when an MRI is not possible)
Spinal CT
A lumbosacral spine CT is a computed tomography scan of the lower spine and surrounding tissues.
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Treatment
There is no known effective treatment for syringomyelia. The goals of treatment are to stop the spinal cord damage from getting worse and to improve function.
Medicines and devices can help with pain.
Surgery may be needed to relieve pressure in the spinal cord. Physical and occupational therapy may be needed to improve muscle function and help maximize independence.
Ventriculoperitoneal shunting or syringosubarachnoid shunting may be needed. This is a procedure in which a catheter (thin, flexible tube) is inserted to drain the fluid buildup.
Ventriculoperitoneal shunting
Ventriculoperitoneal shunting is surgery to treat excess cerebrospinal fluid (CSF) in the cavities (ventricles) of the brain (hydrocephalus)....
Read Article Now Book Mark ArticleOther treatments are available to manage bowel and bladder problems and spasticity.
Outlook (Prognosis)
Without treatment, the disorder may get worse very slowly. Over time, it may cause severe disability.
Surgery usually stops the condition from getting worse. Nervous system function will improve in about 30% of the people who have surgery.
Possible Complications
Without treatment, the condition may lead to:
- Loss of nervous system function
- Permanent disability
Possible complications of surgery include:
- Infection
- Other complications of surgery
When to Contact a Medical Professional
Contact your provider if you have symptoms of syringomyelia.
Prevention
There is no known way to prevent this condition, other than avoiding injuries to the spinal cord. Getting treated right away slows the worsening due to this disorder.
References
Alobeidi F, Thurnher MM, Jäger HR. Non-tumoural spinal cord lesions. In: Adam A, Dixon AK, Gillard JH, Schaefer-Prokop CM, eds. Grainger & Allison's Diagnostic Radiology. 7th ed. Philadelphia, PA: Elsevier; 2021:chap 50.
Benglis DM, Jea A, Vanni S, Shah AH, Green BA. Syringomyelia. In: Garfin SR, Eismont FJ, Bell GR, Fischgrund JS, Bono CM, eds. Rothman-Simeone and Herkowitz's The Spine. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 94.
National Institute of Neurological Disorders and Stroke website. Syringomyelia. www.ninds.nih.gov/health-information/disorders/syringomyelia. Reviewed September 23, 2023. Accessed July 3, 2024.
Roguski M, Groves ML. Adult syringomyelia. In: Winn HR, ed. Youmans and Winn Neurological Surgery. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 334.
Central nervous system - illustration
The central nervous system is made up of the brain and spinal cord. The brain functions to receive nerve impulses from the spinal cord and cranial nerves. The spinal cord contains the nerves that carry messages between the brain and the body. Spinal cord injury can occur when there is damage to the cells within the spinal cord or when the tracts of nerves that run up and down the spinal cord are severed.
Central nervous system
illustration
Central nervous system - illustration
The central nervous system is made up of the brain and spinal cord. The brain functions to receive nerve impulses from the spinal cord and cranial nerves. The spinal cord contains the nerves that carry messages between the brain and the body. Spinal cord injury can occur when there is damage to the cells within the spinal cord or when the tracts of nerves that run up and down the spinal cord are severed.
Central nervous system
illustration
Review Date: 6/13/2024
Reviewed By: Joseph V. Campellone, MD, Department of Neurology, Cooper Medical School at Rowan University, Camden, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.