Syringomyelia

Word meaning

"Flute-tube-like cavity formation in the spinal cord"; Syrinx = (Gr.) Flute (Pl .: Syringen); Myelon = (large marrow)

definition

Syringomyelia is a very rare disease of the spinal cord in which cavities form inside the spinal cord over a long period of time. Various triggering factors are discussed. The formation of cavities leads to neurological symptoms, depending on the affected nerve cells and pathways, up to a complete paraplegic syndrome due to the use of space.
A distinction is also made between syringobulbie, a syringomyelia, which is so high in the spinal cord that it also affects the lower parts of the brain. Syringoencephaly, on the other hand, is a disease that is restricted to the brain itself.

Syringobulbie

The syringobulbie is a subform of the syringomyelia. In syringomyelia, the cavity in the spinal cord formed by the outflow disorder is usually at the level of the cervical or thoracic vertebrae. In the case of the syringobulbie, however, this usually extends further up, up to the so-called midbrain (= Mesencephalon), a part of the brain, and can thus even influence the control of basic body functions. This can lead to uncoordinated eye movements, dizziness and paralysis of the muscles of the tongue, the larynx and the palate.

frequency

The Syringomyelia occurs very rarely. In Germany there are 0.5 new cases per 100,000 inhabitants per year.

causes

The reasons for the occurrence of syringomyelia can be divided into congenital and acquired causes. In the case of the congenital form, a developmental disorder is assumed that leads to the formation of cavities, but the exact mechanism of its formation has not yet been clarified. Syringomyelia occurs very rarely.

In Germany there are 0.5 new cases per 100,000 inhabitants per year. the acquired form of syringomyelia leads to a narrowing of the nerve water (Cerebrospinal fluid) filled cavities whose free flow is always restricted and forces the liquor to find other ways: the syrinx is created.

The aforementioned narrowing of the liquor spaces can be traumatic, i.e. through an accident, but also through meningitis, a herniated disc or a mass caused by a tumor. Several caves can also form.

Symptoms

One can have many different complaints Syringomyelia because their location often affects several nerve tracts and cells without these being directly functional, but which can be assigned a specific lesion height by observing their combination. One finds at the Syringomyelia Perceptual disorders such as a decrease in fine or gross sensation, but also Pain and Tingling sensations, decreased or increased temperature sensation, one Disturbance of the sense of position and position of the Joints and Muscles, as well as Muscle paralysis or –Spastiken. Stool and urinary incontinence and sexual dysfunction such as impotence can occur, as can syringomyelia located particularly high in the spinal cord Speech, swallowing and coordination disorders. By impaired control of blood vessels can Circulatory and wound healing disorders occur, as well as rarely described Memory problems or mental abnormalities. While the height of the syringe in the spinal cord has to be reconciled with the symptoms it causes, its size is not necessarily related to the severity of the symptoms.

diagnosis

In order to diagnose syringomyelia, observation of the clinical picture (Combination of the above symptoms), as well as the comparatively slow course of a disease in which many different, affected body systems have to be brought under one roof for the Neurologist the possibility of anatomically delimiting the location of the action. At this point it usually comes to one MRI image of the brain and spinal cord, on which the syringe can usually be seen quickly. In a special MRI image, the Circulation of the nerve water to assess the relevance of the syringe as a hindrance to passage. A nerve fluid withdrawal (CSF puncture) and its examination provides information about acute inflammatory processes. Ultimately, past events that explain syringomyelia can be reconciled with the findings in the anamnesis.

MRI of the spine

To make the diagnosis of syringomyelia, an MRI (magnetic resonance imaging) of the spine should be performed. The cavity is usually located in the spinal cord at the level of the neck or chest area (cervical spine / thoracic spine) and leads to compression of the nerve cords surrounding the cavity. Nevertheless, an MRI of the entire spine should always be carried out for the final clarification.

For more information, see MRI of the spine.

therapy

The focus is on removing an existing and treatable one Cause of syringomyelia. To remove the pressure of the syringe on the nerve tissue is one surgery necessary when the neurological impairment is correspondingly severe or progressing. Here, for example, an attempt is made to create a drainage option for the liquid from the syringe by adding a Shunt (Tube) that drains fluid to another area of ​​the body. However, due to the risk of damage to the nerve tissue immediately surrounding the syringe, this surgical technique is no longer favored. The surgical attempt to loosen adhesions and obstacles to the passage that triggered the syringomyelia is more desirable nowadays, but it also involves the risk of the surgical trauma new bonds entails. Ultimately, pain therapy is often necessary for the nerve pain caused by syringomyelia, as well physical therapyto maintain and train movement and everyday skills. Alternative therapeutic approaches are also helpful, especially when it comes to alleviating symptoms.

Surgery for a syringomyelia

The operation of the syringomyelia is the only so-called curative (=healing) Therapy. This means that it is the only way to truly cure your nerve water drainage disorder, not just combat the symptoms. Nowadays, due to the limitations imposed by surgery, it is usually only performed if symptoms worsen rapidly.
During the operation, a shunt is inserted microsurgically. The shunt is there to remove part of the nerve water (Liquor) from the brain in order to avoid an increase in intracranial pressure. For this purpose, a connection is surgically established between the spaces in the brain filled with nerve water (Ventricle) and, for example, the abdominal cavity. This allows the excess nerve water to run off and is excreted in the stool as normal.
However, this operation is quite extensive and is usually carried out for other diseases, such as hydrocephalus (so-called "water head"). In addition, surveys have shown that many patients without surgery are more satisfied than patients who have had surgery.

Risks of the operation

Since this operation takes place on the patient's brain, it involves many risks. Therefore, it should be considered very carefully whether it is really necessary for it to be carried out. A very feared complication is, for example, an infection of the shunt, since this pathogen can serve as a kind of “road” into the brain. Thus, in the event of an infection, it can quickly spread to the brain and trigger severe symptoms. Furthermore, the drainage of the shunt can result in too low or too high an outflow of nerve fluid.

forecast

The Syringomyelia is chronically progressive, which cannot always be stopped by an operation, but sometimes only slowed down over time. All of the above neurological symptoms are usually irreversible due to the long damage time to the affected nerve cells and the patient has to come to terms with them in everyday life. It can too limp like spastic paralysis, to muscular dystrophy, to posture damage and secondary pain.