What is syringomyelia?Shoulder pain or neck pain are particularly pronounced symptoms in syringomyelia.
Syringomyelia is a spinal cord disease. The spinal cord is located in a bony canal where it is washed with cerebrospinal fluid. If the cerebrospinal fluid can not circulate freely, the cerebrospinal fluid builds up. Due to the prevailing pressure, the cerebrospinal fluid seeks a new path and forms cavities where the fluid accumulates. These cavities may form along the spinal cord as well as in the lower areas of the brain.
If the syringes in the brain are called a syringobulbia. One distinguishes between a primary and a secondary syringomyelia. The primary syringomyelia is innate and develops over the years. In the congenital variant, the cavities are filled with cerebrospinal fluid (cerebrospinal fluid). In secondary (acquired) syringomyelia, the cavities are also filled with CSF, but here the protein concentration is significantly increased.
Due to the formation of cavities, the nerve tissue is displaced, which leads to more or less severe neurological deficits depending on the severity. Sensory disorders, pain, numbness and paralysis can be the result. If the syringomyelia is not adequately treated, it inevitably leads to a cross-sectional syndrome.
Syringomyelia has several causes, depending on whether it is a primary or secondary variant.
In the case of primary (congenital) syringomyelia, there is usually a so-called Chiari malformation. This is a malformation located in the transitional area of the brain and spinal cord. Here, the cerebellum - also known as medulla oblongata - relocated to the spinal canal. The cause is a developmental disorder of the embryonic plant between the fourth and sixth week of gestation.
Since the cerebellum is displaced and thus blocks the spinal canal, the circulation of the liquor is also disturbed. Many years may pass before a cavity (syringe) forms over time. As a rule, the area between the cervical and thoracic spine is affected.
Secondary syringomyelia is usually acquired as the result of an accident or injury. These are usually injuries to the spine involving the spinal cord. Clinical evaluations showed that about six percent of these cases develop a syringomyelia in the following years.
Another cause of syringomyelia is inflammation of the brain or spinal cord, also referred to as spider skin. This inflammation is often the result of meningitis. If the spinal cord is inflamed, so-called adhesions can occur with the surrounding tissue, which affects the flow of cerebrospinal fluid.
Tumors in the spinal cord are another possible cause. These can also disturb the circulation of the spinal fluid. However, combinations of the named causes may also occur, especially in the acquired syringomyelia.
Symptoms, complaints & signs
Depending on the location of cavitation in the spinal cord, the symptoms of syringomyelia may be different. Typical are severe pain in the head, shoulder and arm area, which are described by the affected as sharp, burning or dull. Individual skin areas of the extremities may be affected by sensory disturbances, there is often a pronounced insensitivity to heat - an increased sensitivity to cold or heat is also possible.
Occasionally, even light touches are perceived as painful, many patients report tingling or pricking in the extremities. Furthermore dizziness, coordination disturbances and gait uncertainties can occur, also temporary memory disturbances occur. Other possible signs include muscle cramps, uncontrolled muscle twitching and paralysis:
Impairment of the sphincter of the bladder or bowel causes urinary or fecal incontinence. Disorders of hearing and vision may develop as a result of the disease, and the ability to speak may also be limited. Sexual dysfunctions are not uncommon: While in women this is mainly due to a decreasing libido noticeable, it comes in men often to impotence to impotence.
In the course of the disease, malformations and inflammations in the area of the head and spine can form. Unspecific symptoms of syringomyelia include rapid fatigue, general weakness, insomnia, and depressive moods that can develop into depression.
Diagnosis & History
The syringomyelia is diagnosed using imaging techniques such. B. MRI, CT and X-ray. With the aid of contrast agents, the spinal canal can be displayed well, so that cavities are clearly visible. Additional MRI examinations can also represent the CSF flow. Here you can find even the smallest changes or disturbances.
To exclude an inflammatory cause, a so-called lumbar puncture is usually performed. This involves taking a sample of the cerebrospinal fluid from the lower part of the lumbar spine using a cannula. Before an operation is considered, a so-called myelography is often used. Here, the CSF space is punctured under X-ray inspection.
After injection of a contrast agent, X-rays are used to determine how far the spinal cord can expand. The images show whether the cavity is directly connected to the spinal canal and how this is constructed.
Syringomyelia is a slowly progressive disease. Especially in the primary (congenital) variant, a syringomyelia develops only slowly or can also come to a standstill and regress. In about 20 percent of all those affected, surgery can not stop the syringomyelia.
The acquired by an accident syringomyelia shows a strong degenerative course, that is, it is deteriorating continuously. The prognosis or further course depends on the cause, especially if tumors are the trigger of syringomyelia. In addition to damage to the nerves, blood supply to the spinal cord is often compromised. Because of this, paraplegia is usually the result. An operation can not eliminate the syringomyelia, but it can increase the quality of life of the person affected.
In the worst case, it may come to paralysis and various emotional disorders due to the syringomyelia. These paralyzes are irreversible and can not be reversed. Also with a treatment usually not all complaints can be limited. Patients suffer from severe illness due to illness.
These can occur in the back, neck and arms. Furthermore, the pain spreads to other regions. Due to the sensory disturbances it can also come to restrictions in everyday life. Especially young people can show spasticity and twitching in the muscles due to syringomyelia.
In many cases, they are teased and bullied, especially in kindergarten or at school, which can lead to psychological complications or depression. At the same time, the child's development is considerably reduced. With the help of various therapies, the paralysis can be limited.
However, there is rarely a completely positive course of disease. However, life expectancy itself is not negatively affected. If a tumor has formed, it must be removed. The further course of the disease depends strongly on the success of this procedure.
When should you go to the doctor?
In the case of syringomyelia, the person is dependent on a medical examination and treatment so that the symptoms can be alleviated. It can not come to a self-healing, so the person concerned is always dependent on an investigation. The sooner the examination and treatment of syringomyelia is initiated, the better the disease usually is. Therefore, a doctor should be consulted at the first signs and complaints.
The doctor should be consulted if the person concerned suddenly suffers from strong visual problems. These usually occur for no particular reason and remain permanent. In the worst case scenario, complete blindness can occur, with both eyes not always having to be affected by syringomyelia. Not infrequently, pigmentation disorders or other complaints on the skin indicate the disease and should be examined by a physician. As a rule, the disease can be treated by a GP or by an ophthalmologist. The life expectancy of the person affected is not limited by the syringomyelia.
Treatment & Therapy
The treatment of a syringomyelia is aimed primarily at alleviating the symptoms. Since the disease is associated with pain from the beginning, adequate pain therapy should be initiated. Since the Syringomyelie is a creeping disease, should be performed from the beginning of physiotherapeutic and occupational therapy. Here you learn to deal with the disease and the associated neurological deficits and integrate them into everyday life.
To arrest or slow down the process of syringomyelia surgery is the only option. In a neurosurgical procedure, a so-called shunt (tube) is introduced into the cavity, so that the fluid can flow away. However, this procedure involves some risks as the shunt must remain in the spinal cord to ensure a constant drainage. The shunt can also trigger a syringomyelia, as it can disturb the CSF circulation as a foreign body. In addition, pathogens can invade the shunt or wound and cause inflammation.
Another surgical procedure is the FMD (Foramen Magnum Decompression Operation). In this procedure, the cranial opening is extended to the spinal cord. The two first vertebral arches are removed. If a tumor is the cause of syringomyelia, it is surgically removed. When bonding the spinal cord skin, the bond is surgically released so that the cerebrospinal fluid can flow freely again.
In all treatment options, however, it must be taken into account that the syringomyelia is not curable and can occur again and again.
To date, no adequate measures are known to help prevent syringomyelia.
Affected in Syringomyelia in most cases, very few or even limited options for follow-up care available because it is a relatively rare disease. So that it does not come to other complications or complaints in the further course, the affected person should definitely go to a doctor early.
A doctor should be contacted at the very first signs or symptoms of the condition. In most cases, however, the syringomyelia causes various malformations in the child, so that the child relies on intensive care in his or her everyday life. As a rule, the syringomyelia can not be treated during pregnancy, so that the various malformations and malformations can be corrected only after the birth of the child.
In many cases, the victims are also dependent on the help and care of their own family through syringomyelia. This will especially alleviate or prevent possible depression and other mental disorders. Even after a successful procedure, regular checks and examinations by a doctor are very important to monitor the current state of syringomyelia. As a rule, this disease does not reduce the life expectancy of the patient.
You can do that yourself
Syringomyelia can not always be recognized as such in everyday life. Therefore, it is difficult to initiate the right self-help measures. Therefore, if the typical symptoms occur, the risk patients should consult a doctor early. After diagnosis, there are some ways for sufferers to alleviate their suffering.
While physical stress and stress intensify the characteristic underlying pain, relaxation pauses and sedative medications provide for an improvement. Conventional drugs and neuroanalgesics, however, often help only temporarily. Therefore, patients should be careful with physical activities and only do gentle sports. The adaptation of living and working conditions is essential for them. Whether those affected are on their feet for a long time, sitting or lying down, they always need a change of posture. This has an unfavorable effect on the everyday activities and severely restricts the persons. A regulated daily routine is hardly possible.
That is why regular physiotherapy is required after rehab. Depending on the course of the disease and the individual situation, the doctor may prescribe psychotherapy. On the one hand, it helps the patient to accept the disease, and on the other, it helps to increase self-esteem in everyday life.