What is Syringomyelia?
Syringomyelia is a disease of the spinal cord in which hollow formations occur in the medulla. These cavities are a fluid-filled, elongated cavity called a syrinx (from the Greek: tube, flute). Within this space, the nerve cells are missing. Therefore, no stimuli, which are otherwise so important, can be transmitted here.
In the area of the cerebral ventricles, the cerebrospinal fluid, also called neural fluid or CSF, is formed. It flows around the brain and the spinal cord, both from the outside and from the inside. A very thin canal runs down the center of the entire spinal cord (central canal), which is filled with the cerebrospinal fluid in the younger years. Later, this canal closes from the bottom (lumbar spine area) to the top (cervical spine area). If blockages or premature closure occur at some points of this canal, the fluid accumulates there and a cavity filled with cerebrospinal fluid (syrinx) can develop in the spinal cord. Most often, the disease occurs in the area of the cervical spine and thoracic spine.
Due to the space filling of the resulting cavity, pressure is exerted on nerve tissue, which leads to the appearance of the first symptoms. In this disease, these may include sensory disturbances, pain, muscle weakness or paralysis. After the first appearance of symptoms, there is usually a slow deterioration of the condition over years to decades. The appearance and intensity of individual symptoms, their increase or decrease, as well as the overall course of syringomyelia, cannot be predicted.
Frequency of syringomyelia
Syringomyelia, the formation of a so-called syrinx, is a rare disorder. About six to nine people out of every 100,000 are affected by this disorder. Although the disease is often congenital, symptoms of syringomyelia usually first appear between the ages of 20 and 40. Congenital syringomyelia has a higher than average incidence in some families. Families with many children are particularly affected.
Women are much less likely to be affected by the disease. Men develop syringomyelia about twice as often.
Different causes for syringomyelia
The causes of syringomyelia are extremely diverse. Basically, a distinction must be made between congenital and acquired syringomyelia. In studies, it is mostly assumed that congenital syringomyelia is responsible for 55 - 70% of all cases and therefore occurs significantly more frequently than acquired syringomyelia.
Congenital Syringomyelia due to Chiari Malformation
The congenital form of syringomyelia is usually due to a malformation of the spine or skull abnormalities. A frequent cause is, for example, the Chiari malformation. In this case, there is a downward displacement of parts of the cerebellum in the direction of the spinal canal. The altered position of the brain parts in relation to the skull bone then causes the free circulation of the cerebrospinal fluid to be impaired.
The most common variant of Chiari malformation is CM 1 (Chiari malformation type 1): In this case, parts of the inferior cerebellum shift at least three millimeters through the occipital hole into the spinal canal.
Causes of acquired forms of syringomyelia
The causes of the acquired form of syringomyelia vary:
For example, an accident can be a determining factor: about five percent of all patients with an injury to the spinal cord due to an accident develop syringomyelia in the following years. The cavity formation of the syrinxes starts after a few months to several years after the accident. Sometimes, however, it is repeated microtrauma that the patient does not even remember, but which can cause adhesion of the fine spinal cord membranes with resulting cerebrospinal fluid circulation disturbance.
Also cause of syringomyelia can be inflammation of the spinal cord. Syringomyelia can also develop after treatment by spinal cord surgery.
Typical symptoms of syringomyelia
As diverse as the causes, the symptoms of this disease itself can be. Syringomyelia causes unpleasant and often very painful symptoms.
Typical symptoms of syringomyelia can be:
- Sharp, burning, or dull pain in the shoulder, head, neck, and arms; migraine-like headache;
- Sensory disturbances such as insensitivity to heat or touch of the limbs, tingling, stinging;
- Sensitivity to touch or depth sensitivity, which indicates what position the body and joints are in, may be disturbed;
- Increased sensitivity of the body to heat or cold;
- Disturbances of the sense of position, unsteadiness of gait, dizziness and coordination disorders, temporary memory disorders;
- Sometimes there is misregulation of blood circulation. The skin then appears bluish and cool, later also doughy swollen;
- Cramps, uncontrolled muscle twitching, paralysis, reduction of muscle mass;
- Very unpleasant for patients are incontinence of bladder and bowels - this can lead to paralysis of the bladder or sphincter muscle;
- Impotence, decreasing libido, sexual dysfunction;
- Slowed wound healing;
- Fatigue states, general lack of strength, tendency to rapid exhaustion;
- Insomnia, depressed mood up to depression.
Diagnosis of syringomyelia by magnetic resonance imaging (MRI)
The cavities (syrinx) can only be localized in a detailed examination. The first step is to take a medical history and perform a thorough neurological examination. During the physical examination, the physician primarily checks reflexes and pays attention to muscle strength.
If these initial steps lead to the suspicion of syringomyelia, the diagnosis is made with the aid of magnetic resonance imaging (MRI). MRI images of the spinal canal and adjacent parts of the brain allow direct visualization of the cavity and its extent. To see the circulation of cerebrospinal fluid, a special MRI scan can be used to show the flow of cerebrospinal fluid as a film. This shows the pulsation of the cerebrospinal fluid in relation to the patient's heartbeat. The exact interpretation of this examination allows in many cases to detect the smallest adhesions with circulatory disturbances of the cerebrospinal fluid.
Special neurosurgical therapies for syringomyelia.
It is always important to give the patient hope, but not false hope. An important component in the therapy is a pain therapy adapted to the needs of the patient. This is complemented by early physiotherapeutic care and physiotherapy. Patient education that includes regular self-examination also makes sense. In this way, injuries that are not painful for some patients due to sensory disturbances can be detected in good time and adequately treated.
In addition to these more general therapeutic measures, there are specific neurosurgical therapies for syringomyelia.
In order to prevent the progression of the deficits, it is necessary to enlarge the space for the spinal cord in an operation (surgery) or to drain the fluid that is in the cavity from there. In this way it is possible to stop the enlargement of the cavity and even partially to achieve its reduction. With the help of microsurgery we get into the cavity and create a connection between the syrinx and the epidural space. This is the space where cerebrospinal fluid flows. This allows the cerebrospinal fluid to flow again. By reducing the pressure, we relieve the spinal cord and in this way relieve the pain. However, in most cases, existing symptoms only partially regress.
If the syringomyelia has arisen after an accident, adhesions of the soft spinal cord membranes are loosened and a dura dilatation plastic is implanted. The nerve fluid can then flow off again without obstruction, and the syrinx collapses.
If the cavity has formed at the base of a tumor, it is often sufficient to remove the tumor in order to achieve an improvement in the symptoms.
Prognosis - how to improve the quality of life
Syringomyelia cannot be prevented. Unfortunately, it is not yet possible to cure syringomyelia. However, the progression of the disease can be stopped or slowed down and severe accompanying symptoms, pain and disorders can be alleviated.
Overall, the course of the disease varies widely. In one-third to one-half of patients, its progresses only slowly or stops completely. In a quarter of patients, there is a progressive deterioration of the condition, which can be slowed only slightly even by surgery. Syringomyelia in particular, which have developed after trauma, tend to deteriorate suddenly and in some cases persistently. For cavities in tumors of the spinal cord, the prognosis depends largely on the underlying disease.
A prognosis for life expectancy in syringomyelia depends largely on the course, as described above. It happens that syringomyelia completely stops its progress. In other cases, relapses are reported.
The quality of life of affected individuals can be significantly improved by timely diagnosis and subsequent surgery at the right time.
Information about the article
The article was last checked and updated on November 14th, 2022.
About the author
Dr. med. Munther Sabarini, MD, is the director and founder of the Avicenna Clinic. As a specialist neurosurgeon, he particularly has expertise in the treatment of spinal disorders. Dr. Munther Sabarini has more than 30 years of professional experience. During this time he treated more than 30,000 patients.
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Sources and further literature
T. Milhorat, A. Capocelli, R. Kotzen, P. Bolognese, I. Heger, J. Cottrell: Intramedullary pressure in syringomyelia: clinical and pathophysiological correlates of syrinx distension. Neurosurgery. Oxford University Press, 1997.
J. Medow, J. Sansone, BJ Iskandar. Syringomyelia and Hydromyelia. Principles and Practice of Pediatric Neurosurgery. Thieme Medical Publishers. 2 Ausgabe, New York, 2008.
N. Di Lorenzo, F. Cacciola. Adult syringomielia. Classification, pathogenesis and therapeutic approaches. Journal of Neurosurgical Sciences. Minerva Medica. Turin, 2005.
U. Batzdorf. Primary spinal syringomyelia. Invited submission from the joint section meeting on disorders of the spine and peripheral nerves. Journal of Neurosurgical Sciences. Minerva Medica. Turin, 2005.
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