Most often the cavitation is a cyst, called a syrinx. A syrinx can develop in any location inside or outside the spinal cord.
Circulation of Spinal Cord Fluid is Impaired
Usually, cerebrospinal fluid (CSF) circulates around the spinal cord and brain, providing nutrients, and carrying away waste material from metabolism.
When a syrinx forms, fluid that should be circulating around the cord is forced into the syrinx. It is unable to flow out and circulate as it should. As the syrinx enlarges it damages the cord and can eventually destroy it.
Pressure from the syrinx against the cord can affect nerve function leading to severe pain, spasticity, loss of bladder control and other debilitating symptoms. The degree of pain differs from person to person and may not be related to the size of the syrinx.
Estimates of how many people have SM vary from 20,000 to 300,000 in the United States and fewer than 1 in 2000 in Europe. There is no consensus on the numbers. Because the pain and other symptoms differ widely, a person often endures years of living with debilitating symptoms before a proper diagnosis is made.
Types of Syringomyelia
- Arnold Chiari Malformation - The most common form of syringomyelia. In this congenital disorder, the cerebellum, a part of the brain, is pushed out of its normal position in the back of the head . It presses into the upper spinal cord blocking the normal circulation of CSF causing a syrinx to develop.
- Non communicating syringomyelia - When a cyst forms on the cord itself, from injury, illness or some unknown cause. Cysts may develop in any part of the spinal cord.
- Hydromyelia (HI-DRO-My-EEL-YA) - Also called syringohydromyelia, this is not truly a cyst. In this condition, cerebral spinal fluid is forced into the center of the spinal cord itself. The fluid causes the cord to expand, which can damage and destroy it.
Causes of Syringomyelia
The more common causes of Syringomyelia are:
- Congenital -These include Arnold Chiari Malformation and Hydrocephalus.
- Diseases - These include tumors, spinal meningitis, and inflammatory disorders such as arachnoiditis and transverse myelitis.
- Post Traumatic Spinal Cord Injury - Syringomyelia may form after a spinal cord injury (SCI) Estimates are that 3-4% of people in the US who have traumatic SCI will develop a syrinx, sometimes decades after the accident.
- Epidural injections and Lumbar Punctures - Either of these procedures can cause a syrinx to develop and people who have syringomyelia are advised not to have any of these treatments.
- Nutrition - Disorders due to diet or to inborn errors of metabolism may lead to degeneration of the spinal cord and the development of syringomyelia. B12, is one that has been documented in medical journals but not well known. In some cases, early recognition and treatment led to complete recovery from the spinal cord degeneration.
- Idiopathic - When no cause is found to explain why a syrinx develops it is called idiopathic.
Signs and Symptoms
Pain - The most common symptom of a syrinx is pain. It can be in one area or present as widespread pain throughout the body. The pain may be in the form of a dull ache, an area with a persistent burning sensation, or a sharp, stabbing pain.
Scoliosis and Thoracic Outlet Syndromes - The pain of either of these disorders may also be the primary symptom of syringomyelia.
Other symptoms include:
- Sweating
- Numbness
- Muscle stiffness
- Bowel and bladder disorders
- Spasticity
- Loss of pain and temperature sensation
- Persistent neck, shoulder or spine pain
Treatment for Syringomyelia
The focus of treatment for syringomyelia is conservative. The goal is to maintain as much physical strength and neurological function as is possible. Drugs are often used to manage pain .
Surgical treatment is usually the last option when the symptoms have become extreme and life threatening. Surgery often fails and has many serious risks. Surgical procedures may involve inserting a shunt to drain the syrinx, removing obstructions and decompressing the spinal cord. Shunting has had about a 50% long term failure rate due to shunt failure and the syrinx continuing to expand.
Physical Therapy - Physical therapy can offer pain relief and the training needed to maintain or restore full range of motion and improved mobility, muscle strength and stamina.
Physical therapy may be the major form of treatment as long as the condition can be managed without surgery.
Occupational Therapy - An occupational therapist can assist with any of the issues that come up regarding daily living, bathing, cooking, managing at home. Occupational and physical therapists can work together to develop a program to provide the therapy, and daily living aids needed to maximize the ability to live independently and cope well with this sometimes devastating disorder.
Related Link:Living With a Rare Spinal Cord Disorder
Sources:
American Syringomyelia Alliance Project (ASAP)
Goetz, Lance, MD; Post Traumatic Syringomyeliaemedicine.medscape.com/article/322348-overview; updated: Jan 26, 2007
National Institutes of Health
NINDS
Resources:
American Syringomyelia Alliance Project
Syringomyelia Awareness Network
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