Syringomyelia is a disease of the spinal cord. In this condition, cavities (syringes) filled with fluid occur along the spinal cord canal. The cavities displace and crush nerves, which can result in paralysis in addition to sensory disturbances and pain. Syringomyelia is not curable, as it can recur despite treatment.
What is syringomyelia?
Shoulder pain or neck pain are particularly prominent symptoms in syringomyelia. Syringomyelia is a spinal cord disease. The spinal cord is located in a bony canal where it is surrounded by cerebrospinal fluid. If the cerebrospinal fluid cannot circulate freely, the spinal cord fluid becomes congested. Because of the prevailing pressure, the CSF seeks a new path and forms cavities where the fluid accumulates. These cavities can form along the spinal cord as well as in the lower areas of the brain. If the syringes are located in the brain, it is called a syringobulbia. A distinction is made between primary and secondary syringomyelia. Primary syringomyelia is congenital and develops over the years. In the congenital variant, the cavities are filled with cerebrospinal fluid (CSF). In secondary (acquired) syringomyelia, the cavities are also filled with cerebrospinal fluid, but here the protein concentration is significantly increased. The formation of cavities displaces the nerve tissue, which leads to more or less severe neurological deficits, depending on the severity. Sensory disturbances, pain, numbness and paralysis can be the result. If syringomyelia is not adequately treated, a paraplegic syndrome will inevitably develop as it progresses.
Causes
Syringomyelia has several causes, depending on whether it is primary or secondary. In primary (congenital) syringomyelia, there is usually a condition called a Chiari malformation. This is a malformation located in the transitional area of the brain and spinal cord. Here, the cerebellum – also known as the medulla oblongata – is displaced into the spinal canal. The cause is a developmental disorder of the embryonic anlage between the fourth and sixth week of pregnancy. As the cerebellum is displaced, blocking the spinal canal, the circulation of cerebrospinal fluid is also disrupted. Many years may pass before a cavity (syringes) forms over time. Usually, the area between the cervical and thoracic spine is affected. Secondary syringomyelia is usually acquired as a result of an accident or injury. These are usually injuries to the spine involving the spinal cord. Clinical evaluations showed that approximately six percent of these cases develop syringomyelia in the following years. Another cause of syringomyelia is inflammation of the brain or spinal cord skin, also known as spider skin. This inflammation is often the result of meningitis. If the spinal cord skin is inflamed, so-called adhesions with the surrounding tissue can occur, which impairs the flow of cerebrospinal fluid. Tumors in the spinal cord are also a possible cause. These can also disrupt the circulation of the cerebrospinal fluid. However, combinations of the above causes may also occur, especially in acquired syringomyelia.
Symptoms, complaints, and signs
Depending on the location of the cavity formation in the spinal cord, syringomyelia may manifest itself with various symptoms. Severe pain in the head, shoulder, and arm areas, described by affected individuals as sharp, burning, or dull, is typical. Individual skin areas of the extremities may be affected by sensory disturbances, and there is often a pronounced insensitivity to heat – but increased sensitivity to cold or heat is also possible. Occasionally, even light touch is felt as painful, and many patients report tingling or stinging in the extremities. Furthermore, dizziness, coordination disorders and gait unsteadiness may occur, and temporary memory disorders also occur. Other possible signs include muscle cramps, uncontrolled muscle twitching, and paralysis:
Impairment of the bladder or bowel sphincter results in urinary or fecal incontinence. Hearing and vision disorders may develop as a result of the disease, and speech may also be impaired.Sexual dysfunctions are not uncommon: While in women these are mainly noticeable by a decreasing libido, men often experience potency disorders and even impotence. In the further course of the disease, malformations and inflammations may develop in the area of the head and spine. Nonspecific symptoms of syringomyelia include rapid fatigability, general weakness, insomnia, and depressed moods that can develop into depression.
Diagnosis and course
Syringomyelia is diagnosed with the help of imaging techniques such as MRI, CT, and X-ray. With the help of contrast agents, the spinal cord canal can be well visualized so that cavities can be clearly seen. Further MRI examinations can also show the flow of cerebrospinal fluid. Here, even the smallest changes or disturbances can be detected. To exclude an inflammatory cause, a so-called lumbar puncture is usually performed. A sample of cerebrospinal fluid is taken from the lower part of the lumbar spine using a cannula. Before surgery is considered, a so-called myelography is often used. Here, the cerebrospinal fluid space is punctured under X-ray control. After injection of a contrast medium, X-rays are taken to determine how far the spinal canal can expand. The images show whether the cavity is directly connected to the spinal canal and how it is structured. Syringomyelia is a slowly progressive disease. Particularly in the primary (congenital) variant, syringomyelia develops slowly or may stop and regress. In about 20 percent of all affected patients, even surgery cannot stop the syringomyelia. Syringomyelia acquired through an accident shows a strongly degenerative course, i.e. it continuously worsens. The prognosis or further course depends on the cause, especially when tumors are the triggers of syringomyelia. In addition to damage to the nerves, the blood supply to the spinal cord is often affected. Because of this, paraplegia is usually the result. Surgery cannot eliminate syringomyelia, but it can be used to improve the quality of life of the affected person.
Complications
In the worst case, paralysis and various sensory disturbances may occur due to syringomyelia. In this case, these paralyses are irreversible and thus cannot be reversed. Even with treatment, not all symptoms can usually be limited. Patients suffer from severe pain due to the disease. These can occur in the back, neck and arms. Furthermore, the pain spreads to other regions. Due to the sensory disturbances, there may also be restrictions in everyday life. Young people in particular may show spasticity and twitching in the muscles due to syringomyelia. In many cases, they are teased and bullied in the process, especially in kindergarten or school, so that psychological complications or depression can result. This also significantly limits the child’s development. With the help of various therapies, the paralysis can be limited. However, a completely positive course of the disease is rarely achieved. However, life expectancy itself is not negatively affected. If a tumor has formed, it must be removed. The further course of the disease thus depends strongly on the success of this intervention.
When should one go to the doctor?
In syringomyelia, the affected person is dependent on a medical examination and treatment so that the symptoms can be alleviated. In this case, self-healing cannot occur either, so the affected person is always dependent on an examination. The earlier the examination and treatment of syringomyelia is initiated, the better the further course of this disease usually is. Therefore, a doctor should be consulted at the first signs and symptoms. The doctor should be consulted if the affected person suddenly suffers from severe visual complaints. These usually occur without any particular reason and remain permanent. In the worst case, this can lead to complete blindness, although syringomyelia does not always affect both eyes. Not infrequently, pigmentation disorders or other complaints on the skin also indicate the disease and should be examined by a doctor.As a rule, the disease can be treated by a general practitioner or by an ophthalmologist. The life expectancy of the affected person is not limited by syringomyelia.
Treatment and therapy
Treatment of syringomyelia is primarily aimed at relieving symptoms. Because the disease is associated with pain from the onset, adequate pain management should be initiated. Since syringomyelia is a gradual disease, physical and occupational therapy should be performed from the beginning. Here one learns to deal with the disease and the associated neurological deficits and to integrate them into everyday life. In order to stop syringomyelia or to slow down its progression, surgical intervention is the only option. This involves a neurosurgical procedure to insert a so-called shunt (tube) into the cavity so that the fluid can drain away. However, this procedure carries some risks because the shunt must remain in the spinal cord to ensure constant drainage. The shunt itself can also cause syringomyelia because, as a foreign body, it can interfere with CSF circulation. Furthermore, pathogens can enter through the shunt or wound and cause inflammation. Another surgical procedure is FMD (foramen magnum decompression surgery). In this procedure, the opening of the skull is widened toward the spinal cord. In the process, the first two vertebral arches are removed. If a tumor is the cause of syringomyelia, it is surgically removed. If the spinal cord skin is stuck together, the adhesion is surgically released so that the cerebrospinal fluid can flow freely again. However, all treatment options must take into account that syringomyelia is not curable and can always recur.
Prevention
To date, there are no known adequate measures that can help prevent syringomyelia.
Aftercare
Affected individuals have very few or even limited options for follow-up care in most cases of syringomyelia because it is a relatively rare disease. In order to avoid other complications or complaints in the further course, the affected person should in any case consult a doctor at an early stage. A doctor should be contacted at the first signs or symptoms of the disease. In most cases, however, syringomyelia causes various deformities in the child, so that the child is dependent on intensive care in his or her daily life. Usually, syringomyelia cannot be treated during pregnancy, so it is only after the child is born that the various malformations and deformities can be corrected. In many cases, those affected by syringomyelia are also dependent on the help and care of their own family. This especially alleviates or prevents possible depression and other psychological upsets. Even after a successful surgery, regular checkups and examinations by a doctor are very important to monitor the current condition of syringomyelia. Usually, this disease does not reduce the patient’s life expectancy.
What you can do yourself
Syringomyelia is not always immediately recognizable as such in everyday life. Therefore, it is difficult to initiate the right self-help measures. When the typical symptoms appear, at-risk patients should therefore see a doctor at an early stage. Once diagnosed, there are some ways for sufferers to reduce their suffering. While physical exertion and stress exacerbate the characteristic underlying pain, relaxation breaks and sedatives provide improvement. However, conventional drugs as well as neuroanalgesics often help only temporarily. That is why patients should be careful with physical activities and only engage in gentle sports. Adjustment of living and working conditions is inevitable for them. Whether sufferers are on their feet for long periods of time, sitting or lying down, they always need a change of posture. This has an unfavorable effect on everyday activities and severely restricts the persons. A regular daily routine is hardly possible. That is why regular physiotherapy is necessary after rehabilitation. Depending on the course of the disease and the individual situation, the doctor may prescribe psychotherapy.On the one hand, this helps patients to accept the disease, and on the other hand, it provides a stronger self-esteem in everyday life.
