What is Syringomyelia?

Syringomyelia is a disease of the spinal cord that causes cavities in the medulla. These cavities are filled with liquid, it is an elongated cavity called Syrinx (from the Greek: pipe, flute). Within this space, the nerve cells are missing. Therefore, here - otherwise so important - stimuli cannot be forwarded.
 
In the area of ​​the ventricular system, there is the brain fluid also called nerve water or cerebrospinal fluid (Liquor). It surrounds the brain and the spinal cord, both from the outside and from the inside. A very thin canal runs in the center of the entire spinal cord (Central Canal), which at a very early age fills with cerebrospinal fluid. Later, this channel closes itself from below (Lumbar through thoracic spine) upwards (to the cervical spine). In the case of blockages or premature closure in some parts of this channel, the fluid accumulates and can lead to the formation of a cerebrospinal fluid-filled cavity (syrinx) in the spinal cord. The disease usually occurs mostly in the area of ​​the cervical and thoracic spine.
 
The mass of the resulting space causes pressure on the nervous tissue and therefore the onset of the first symptoms - for example, sensory disturbances, pain, muscle weakness or paralysis. After the first appearance of symptoms, it usually comes to a slow deterioration of the condition over years or even decades. The occurrence and intensity of individual complaints, their growth or decrease, as well as the entire course of syringomyelia, cannot be predicted.

The congenital syringomyelia usually announces itself between the 20th and 40th year of life

Syringomyelia is a rare disease. About six to nine people out of 100,000 are affected. This  disease is often congenital and its symptoms usually appear at the age of 20 or 40. Congenital syringomyelia prevails in some families, especially big ones. In men, syringomyelia develops about twice as often as in women.

There are different causes for a syringomyelia

Basically, one has to differentiate between a congenital and an acquired syringomyelia.

The innate form of syringomyelia is usually based on malformation which sometimes leads to a downward shift of parts of the cerebellum in the direction of the spinal canal. The altered position of the brain parts in relation to the cranial bone (Chiari Malformation) then causes the impairment of the free circulation of the nerve fluid.

In the case of acquired syringomyelia, causes are different:

About 5% of all patients with spinal cord injury, due to an accident, develop syringomyelia in the following years. Cavities form themselves after a few months and its formation can last several years after the accident. Sometimes they are also repeated micro-traumas that patients do not remember at all. Furthermore, they can cause adhesions of the fine spinal cord membranes with a resulting Cerebral-Spinal Fluid circulation disorder (CSF).

An inflammation, of the spinal cord, can also cause syringomyelia which can develop even after interventions on the spinal cord.

Typical symptoms of syringomyelia

Syringomyelia causes unpleasant and often very painful symptoms. They include:

  • acute, burning or tedious pain in the area of ​​the shoulder, head, neck, and arms as well as headache;
  • sensitivity disorders such as heat insensitivity or touch sensitivity of the limbs, tingling, stinging;
  • touch sensitivity or depth sensitivity, which indicates what position of the body and joints, may be disturbed;
  • disturbed sensitivity to heat or cold;
  • disorders of the sense of position, gait insecurity, dizziness and coordination disorders, temporary memory disorders;
  • sometimes there is a dysregulation of blood circulation. The skin then appears bluish and cool, later also doughy swollen;
  • convulsions, uncontrolled muscle twitching, paralysis, reduction of muscle mass;
  • very uncomfortable for the patients are incontinence of the bladder and intestine - this can lead to paralysis of the bladder or of the sphincter muscle;
  • impotence, diminishing libido, sexual dysfunction;
  • slowed wound healing process;
  • fatigue, general weakness;
  • insomnia, depressed mood that can lead to depression.

Magnetic resonance imaging (MRI) - diagnosis syringomyelia

Only with a detailed investigation, these cavities can be located. On the basis, the step that follows is always the anamnesis and then the neurological clarification. During the physical examination, the specialist checks especially the reflexes and pays attention to muscle power. If these initial steps result in suspected syringomyelia, the diagnosis continues through magnetic resonance imaging (MRI). In the MRI images of the spinal canal and the adjacent parts of the brain, the cavities and their extent can be directly visualized. In order to see the circulation of cerebrospinal fluid, a special MRI scan can be used to see it as a film. The pulsation of cerebral water is displayed as a function of the patient's heartbeat. The exact interpretation of this study allows in many cases to detect the smallest adhesions with circulatory disturbances of cerebrospinal fluid.

Syringomyelia - there are special neurosurgical therapies

Hope vs Illusion: Patients should be reassured giving them hope, but not Illusions. In fact, an important part of the therapy concerns a tailored pain therapy that respects the patient's needs. First of all, physiotherapy takes place but patients should be also trained to do regular self-examinations. This allows specialists to identify and treat certain injuries that are painless in patients suffering from sensory problems.

In addition to these therapies, syringomyelia can also be treated with special neurosurgical therapies:  
To prevent the progression of the disease it is necessary to expand the space of the spinal cord surgically or to drain there the fluid present in the cavities. This can stop the enlargement of the cavity and even, partially, set out a reduction. Using microsurgery, we enter the cavity and create a connection between the syrinx and the epidural space (space where the cerebral-spinal fluid flows) which will allow us to relieve the spinal cord by reducing the pressure.

Existing symptoms improve, in most cases only incompletely.

If the syringomyelia occurred as a consequence of an accident, the adhesions of the soft membrane of the spinal cord are loosened and the plastic of dura extension is sewn. The brain water can then flow freely again and the syrinx collapses.

If the cavity floor has formed a tumor, it is often sufficient to remove the tumor in order to improve the symptoms.

Prognosis - this is how the quality of life can be improved

Syringomyelia can not be prevented and even if it is not possible to cure it, its progression can be stopped or slowed, relieving as a consequence concomitant symptoms. Overall, the course of the disease is very different. In one-third to one-half of patients, it progresses slowly or comes to a complete standstill. In a quarter of patients there is an increasing deterioration of the condition, which can be slowed by surgery only slightly. Especially syringomyelia, which has arisen after a trauma, tends to a sudden and sometimes persistent deterioration. When cavities form in tumors of the spinal cord, the prognosis essentially depends on the underlying disease.
 
The quality of life of those affected can be significantly improved by timely diagnosis and subsequent surgery at the right time.

Dr. med. Munther Sabarini

Author
Dr. med. Munther Sabarini
Specialist in Neurosurgery

Hans-Heinrich Reichelt

Co-author
Hans-Heinrich Reichelt
Chief Editor of Medizin für Menschen

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Since the year 2001, the Avicenna Clinic is based in Berlin. Our doctors have at least 25 years of international experience in their respective fields (neurosurgery, spinal surgery, anesthesia, and orthopedics).

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