Syringomyelia

What is syringomyelia?

The term syringomyelia is used to describe the formation of an elongated cavity within the spinal cord. This cavity is filled with spinal fluid, the so-called cerebrospinal fluid.  

Technically speaking, syringomyelia does not qualify as a disease on its own, but rather as a symptom. Therefore, it must always be caused by an underlying disease that hinders the outflow of cerebrospinal fluid, leading to the formation of this cavity within the spinal cord.  

Syringomyelia affects men more often than women and usually occurs between the ages of 20 and 40. Even though the formation of a fluid-filled cavity can appear anywhere in the spinal cord, it is most frequently found in the sections near the head. This is known as the cervical and thoracic spinal cord.  

How does syringomyelia develop?

Syringomyelia is always the result of a spinal fluid drainage disorder. In turn, this obstruction can have a variety of causes.  

The cerebrospinal fluid performs important functions in the body. It surrounds the brain and protects it from external forces, for example. The spinal cord also has areas in which cerebrospinal fluid flows. One of these is the so-called central canal, situated in the middle of the spinal cord.  

The cerebrospinal fluid chambers of the brain and spinal cord are connected to each other so that there is a constant flow of cerebrospinal fluid. If the outflow of cerebrospinal fluid is disrupted at one point, the respective space becomes congested and dilated. The result is syringomyelia. 

Malformations are a possible cause of a spinal fluid drainage disorder. They can occur in the area of the brain or also in the area of the upper spinal cord. A well-known example of such a malformation in the area of the spinal cord is a condition called spina bifida, colloquially known as "open back". This disease is often associated with syringomyelia.  

Other causes can be tumors or inflammations that impede or obstruct the outflow of cerebrospinal fluid. Less common causes of syringomyelia are accidents or degenerative (wear-related) conditions of the spine such as slipped discs.

What symptoms are typical of syringomyelia?

The symptoms of syringomyelia arise when the cavity within the spinal cord compresses surrounding structures. This often involves nerve tissue of the spinal cord, the function of which is then impaired.  

Many patients develop rather vague symptoms at the beginning of syringomyelia. They include a disturbed sensation of pain and temperature in the shoulders and arms because the corresponding nerve pathways are impaired. This can result not only in increased but also reduced sensation.   

As the condition progresses, the nerves which control the muscles may also be impaired. This can lead to muscle twitching or paralysis, for example. The activation of reflexes can also be impaired.  

Furthermore, vegetative nerve fibers can be impaired in their function due to syringomyelia. They control all involuntary reactions of the body. For instance, this can lead to disorders in sweat production, bladder and bowel activity or erectile dysfunction.  

If syringomyelia also affects cranial nerves, other symptoms may occur such as involuntary eye movements (nystagmus), sensory disturbances in the face or atrophy of the tongue muscles.  

Which options are there for treating syringomyelia?

When choosing an appropriate treatment for syringomyelia, the patient's current symptoms and the long-term course of the underlying disease must be taken into account. As it is usually a chronic disease, a precise and detailed diagnosis is required.  

The primary aim should be to treat the underlying disease so that the normal flow of cerebrospinal fluid can be restored. Syringomyelia often regresses once the drainage disorder has been eliminated.  

Sometimes, depending on the extent of the symptoms, syringomyelia can also be treated conservatively. Patients with only minor neurological impairments can be prescribed painkillers and medication for muscle spasticity, for example. Regular medical check-ups are required.  

Surgical procedures are also necessary in many cases to treat the underlying disease. For example, this includes the correction of malformations or the removal of tumor tissue. These operations always require a cautious approach to avoid subsequent damage such as the development of further outflow obstructions.     

Should the disease progress very quickly or if the syringomyelia has already reached a large scale, action should be taken to drain the spinal fluid quickly. One such measure is the creation of a so-called shunt.  

A shunt acts like a drainage system and allows the cerebrospinal fluid to flow out. Generally, the shunt is implanted in such a way that the fluid can drain into the abdominal cavity via a thin tube (ventriculo-peritoneal shunt).  

Since this procedure often only brings short-term relief and is associated with many side effects, it is only used in a few cases nowadays.

Prognosis and chances of cure

The prognosis of syringomyelia depends on the underlying disease and how advanced the neurological symptoms are. Although in some cases the underlying disease and therefore the syringomyelia cannot be completely cured, the aim of treatment should be to prevent progression.  

In many cases, the affected nerve structures can recover completely if treatment is carried out in time. Patients are then able to live a normal life without any loss of quality of life.  

Once syringomyelia has spread to such an extent that surrounding structures have suffered permanent damage, the prognosis worsens. It is often still possible to prevent the disease from progressing, however permanent nerve damage is to be expected. Symptomatic therapy is then usually provided for the rest of the patient's life.     

Which doctors and clinics specialize in syringomyelia?

Syringomyelia requires careful diagnosis and treatment in order to maintain or improve the quality of life of patients. Specialists in the treatment of syringomyelia are neurologists and neurosurgeons.  

Especially because syringomyelia is a rare disease, it can be a great challenge for those affected to find a competent practitioner. Our mission is therefore to bring patients and medical experts together.  

All of the specialists listed are experts in the field of syringomyelia and have extensive experience in the diagnosis and treatment of this condition. Schedule an initial appointment with one of our experts quickly and easily and convince yourself of their expertise.