Syringomyelia is a disease of the spinal cord. Cavities (syringes) filled with fluid appear along the spinal canal. Due to the formation of cavities, nerves are displaced and squeezed, which can result in paralysis in addition to sensory disturbances and pain. Syringomyelia cannot be cured because it can recur despite treatment.
What is syringomyelia?
Syringomyelia is a spinal cord disease. The spinal cord is located in a bony canal where it is irrigated with liquor. If the liquor cannot circulate freely, the spinal fluid builds up. Due to the prevailing pressure, the liquor seeks a new path and forms cavities where the liquid accumulates. These cavities can form along the spinal cord as well as in the lower areas of the brain. For what is hypertrophy, please visit gradinmath.com.
If the syringes are in the brain, it is called 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 spinal fluid (liquor). In secondary (acquired) syringomyelia, the cavities are also filled with liquor, although the protein concentration is significantly increased here.
The nerve tissue is displaced by the formation of cavities, which, depending on the severity, leads to more or less severe neurological deficits. Sensory disturbances, pain, numbness and paralysis can be the result. If syringomyelia is not treated adequately, it will inevitably progress to a paraplegic syndrome.
Causes
Syringomyelia has several causes, depending on whether it is a primary or secondary variant.
In the case of primary (congenital) syringomyelia, a so-called Chiari malformation is usually present. This is a malformation located at the junction of the brain and spinal cord. Here, the cerebellum – also known as the medulla oblongata – is relocated into the spinal cord canal. The cause is a developmental disorder of the embryonic system between the fourth and sixth week of pregnancy.
Since the cerebellum is displaced and thus blocks the spinal cord canal, the circulation of the cerebrospinal fluid is also disturbed. Many years can pass before a cavity (syringe) forms over time. The area between the cervical and thoracic spine is usually 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 about six percent of these cases develop syringomyelia in the following years.
Another cause of syringomyelia is inflammation of the lining of the brain or spinal cord, 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 liquor.
Another possible cause is tumors in the spinal cord. These can also disrupt the circulation of spinal fluid. However, combinations of the above causes can also occur, particularly in the case of acquired syringomyelia.
Symptoms, Ailments & Signs
Depending on the location of the cavity in the spinal cord, syringomyelia can present with a variety of symptoms. Typical is severe pain in the head, shoulder and arm area, which is described by those affected as sharp, burning or dull. Individual parts of the skin on the extremities can 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 touches are perceived as painful, and many patients report tingling or stinging in the extremities. Furthermore, dizziness, coordination disorders and unsteady gait can occur, and temporary memory disorders can also occur. Other possible signs include muscle spasms, uncontrolled muscle spasms, and signs of paralysis :
Impairment of the sphincter muscle of the bladder or bowel results in urinary or fecal incontinence. The disease can cause hearing and vision disorders, and the ability to speak can also be restricted. Sexual dysfunctions are not uncommon: While these become noticeable in women, above all through a decreasing libido, men often experience erectile dysfunction up to and including impotence.
As the disease progresses, malformations and inflammation can develop in the area of the head and spine. Non-specific symptoms of syringomyelia are easy fatigability, general weakness, insomnia and depressive moods, which can develop into depression.
Diagnosis & History
Syringomyelia is diagnosed using imaging methods such as B. MRI, CT and X-ray. With the help of contrast media, the spinal cord canal can be visualized so that cavities can be clearly identified. Further nuclear spin examinations can also show the liquor flow. Even the smallest changes or disturbances can be detected here.
In order to rule out an inflammatory cause, a so-called lumbar puncture is usually performed. A sample of the liquor is taken from the lower area of the lumbar spine using a cannula. Before an operation is considered, a so-called myelography is often used. Here, the liquor space is punctured under X-ray control.
After the injection of a contrast medium, X-rays are used 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. Especially in the primary (congenital) variant, syringomyelia develops only slowly or can also come to a standstill and regress. In about 20 percent of all those affected, even surgery cannot stop syringomyelia.
Syringomyelia acquired through an accident shows a strongly degenerative course, which means that it continuously worsens. The prognosis or the further course depends on the cause, especially if tumors are the trigger of syringomyelia. In addition to damaging the nerves, the blood supply to the spinal cord is often compromised. This usually results in paraplegia. Surgery cannot eliminate syringomyelia, but it can improve the patient’s quality of life.
Complications
In the worst case, syringomyelia can lead to paralysis and various sensory disturbances. This paralysis is irreversible and can therefore no longer be reversed. Even with treatment, not all symptoms can usually be reduced. Patients suffer from severe pain as a result of the disease.
These can occur in the back, neck, and arms. The pain continues to spread to other regions. The sensory disturbances can also lead to limitations in everyday life. Young people in particular 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. Child development is also significantly restricted. With the help of various therapies, the paralysis can be limited.
However, a completely positive course of the disease rarely occurs. However, life expectancy itself is not negatively affected. If a tumor has formed, it must be removed. The further course of the disease depends heavily on the success of this intervention.
When should you go to the doctor?
In the case of syringomyelia, the affected person is dependent on medical examination and treatment so that the symptoms can be relieved. Self-healing is also not possible here, so that the affected person always has to be examined. The earlier the examination and treatment of syringomyelia is initiated, the better the further course of this disease is usually. Therefore, a doctor should be consulted at the first signs and symptoms.
The doctor should be consulted if the person concerned suddenly suffers from severe visual problems. These usually occur for no particular reason and are permanent. In the worst case, this can lead to complete blindness, although both eyes do not always have to be affected by syringomyelia. It is not uncommon for pigment disorders or other skin complaints to indicate the disease and should be examined by a doctor. As a rule, the disease can be treated by a general practitioner or an ophthalmologist. The life expectancy of those affected is not limited by syringomyelia.
Treatment & Therapy
Treatment of syringomyelia is primarily aimed at relieving the symptoms. Since the disease is associated with pain from the start, adequate pain therapy should be initiated. Since syringomyelia is an insidious disease, physiotherapeutic and ergotherapeutic measures should be carried out right from the start. Here you learn to deal with the disease and the associated neurological deficits and to integrate them into everyday life.
Surgical intervention is the only way to stop syringomyelia or to slow it down. In a neurosurgical procedure, a so -called shunt (tube) is inserted into the cavity so that the liquid can drain off. However, this procedure carries some risks because the shunt must remain in the spinal cord to ensure constant drainage. The shunt itself can also trigger syringomyelia because, as a foreign body, it can disrupt CSF circulation. Furthermore, pathogens can enter via the shunt or the wound and cause inflammation.
Another surgical procedure is FMD (foramen magnum decompression surgery). During this procedure, the cranial opening is widened towards the spinal cord. The first two vertebral arches are removed. If a tumor is the cause of syringomyelia, it is surgically removed. In the case of adhesions in the spinal cord skin, the adhesion is surgically released so that the liquor can flow freely again.
With all treatment options, however, it must be taken into account that syringomyelia cannot be cured and can occur again and again.
Prevention
To date, no adequate measures are known to help prevent syringomyelia .
Aftercare
In most cases, those affected with syringomyelia have very few or even only limited options for follow-up care, since this is a relatively rare disease. In order to avoid other complications or symptoms in the further course, the person concerned should definitely consult a doctor at an early stage.
A doctor should be contacted as soon as the first signs or symptoms of the disease appear. In most cases, however, syringomyelia leads to various malformations in the child, so that the child is dependent on intensive care in its everyday life. As a rule, syringomyelia cannot be treated during pregnancy, so that the various malformations and deformities can only be corrected after the birth of the child.
In many cases, those affected are also dependent on the help and care of their own family due to syringomyelia. This primarily alleviates or prevents possible depression and other mental upsets. Even after a successful procedure, regular check-ups and examinations by a doctor are very important to monitor the current state of syringomyelia. As a rule, this disease does not reduce the patient’s life expectancy.
You can do that yourself
Syringomyelia is not always immediately recognizable as such in everyday life. It is therefore difficult to initiate the right self-help measures. If the typical symptoms appear, patients at risk should therefore consult a doctor at an early stage. Once diagnosed, there are a few ways people can reduce their suffering.
While physical exertion and stress intensify the characteristic basic pain, relaxation breaks and sedative medication bring about an improvement. However, conventional drugs and neuroanalgesics often only help temporarily. Therefore, patients should be careful with physical activities and only practice gentle sports. It is essential for them to adjust their living and working conditions. Whether those affected are on their feet for a long time, sitting or lying down, they always need to change their posture. This has an unfavorable effect on everyday activities and severely restricts people. A regular daily routine is hardly possible.
Regular physical therapy is therefore required after rehabilitation. Depending on the course of the disease and the individual situation, the doctor can prescribe psychotherapy. On the one hand, this helps the patient to accept the disease and, on the other hand, provides a stronger feeling of self-esteem in everyday life.
