Syringomyelia: causes, symptoms and complications

Syringomyelia is a rare disease that affects the spinal cord. This is a cylindrical structure found within the spinal canal. It is responsible for transmitting nerve impulses from the body to the brain and vice versa.

What happens in this disease is that a cyst develops in the spinal cord. This cyst is filled with fluid and can compress neurons, damage nerves, cause pain, weakness or stiffness.

Although it is an unusual entity, its origin is in multiple situations. Further, the treatment is complex. Therefore, in this article we explain everything you need to know about syringomyelia, how it is diagnosed and how it can be treated.

What is syringomyelia?

Syringomyelia, as we have noted in the introduction, is a rare disorder. According to the Spanish Federation of Rare Diseases, its incidence is 0.84 cases per 10,000 people. It usually makes its debut between the ages of 25 and 30 and affects women and men equally.

A fluid-filled cyst forms within the spinal cord. This cyst is called “siringo” (syrinx in English). What forms it is cerebrospinal fluid.

As an article in the National Institute of Neurological Disorders and Stroke, the cerebrospinal fluid is responsible for surrounding and protecting both the brain and the spinal cord. The problem is that, by accumulating to form a cyst, it can press on the nerve fibers that make up the spinal cord.

Syringomyelia usually appears in situations in which the physiological circulation of cerebrospinal fluid is impaired. Normally, it should be continually renewing itself and being reabsorbed in different structures of the central nervous system.

It is important to highlight the difference between syringomyelia and syringobulbia. Both entities have the same pathophysiology, although the location is different. While in the first the cyst is located in the medulla, in the second it is in the brain stem.

The spinal cord is a structure of the central nervous system that runs through the interior of the spinal column.

Causes of this disease

It is not known exactly why syringomyelia occurs. It can be congenital or acquired. The first is one that is present from birth and is usually associated with a pathology called Chiari malformation.

Chiari malformation is also a rare entity. It consists of part of the brain tissue that extends into the spinal canal. There are three types, depending on how much tissue is bulging.

On the other hand, acquired syringomyelia can be due to multiple causes. One of the most prominent is meningitis, which refers to inflammation of the membranes that surround the brain and spinal cord. It can also be due to trauma or bleeding.

There are other congenital conditions that can lead to this condition. For example, the tethered spinal cord. Similarly, any tumor that is located in the spinal cord can cause it.

What symptoms does syringomyelia produce?

Symptoms of syringomyelia usually begin between the ages of 25 and 30. However, this depends on what the underlying cause is. It is usually progressive and progresses slowly.

According to specialists from the Mayo Clinic, sometimes there can be triggers, such as exertion or coughing. One of the most common symptoms is muscle weakness. It can affect the extremities, shoulders, or back.

Sensitivity and reflexes may also be affected. These people usually do not have much notion of pain or temperature. However, others experience severe pain in the neck, arms, and back.

Similarly, there may be stiffness and headache. The spine may even curve abnormally. This is called scoliosis and affects the thoracic part.

Possible complications

As we just saw, the cyst that characterizes syringomyelia can progress very slowly. This sometimes causes many people to not even experience symptoms or they are very mild. However, pressing on the nerves can lead to serious problems.

In some cases paralysis appears. Muscle weakness and stiffness often cause disturbances to perform daily activities, such as walking. Another of the most frequent complications is chronic pain, complex to treat and alleviate.

How is syringomyelia diagnosed?

The diagnosis of syringomyelia can be guided by the symptoms that the patient presents. However, many are discovered accidentally. That is to say, a study is performed for any other pathology and the cyst is observed.

The two most useful tests are MRI and CT. In some cases, a lumbar puncture is also required to obtain cerebrospinal fluid. Thus, other possible causes of the symptomatic picture can be ruled out.

Treatment options

Treatment of syringomyelia will depend on the severity of the disorder. It must be individualized, since if no symptoms are present, no approach may be required. In fact, in these cases, periodic controls and good follow-up are preferred.

However, in those patients who do have symptoms, treatment is usually surgical. The goal is to reduce the cyst and the pressure it exerts on the nerves of the spinal cord. However, the cause must also be borne in mind.

For example, in the case of Chiari malformation, it is usually necessary to intervene this pathology first. This ensures that the cerebrospinal fluid circulates normally again. Similarly, if there is a congenital spinal anomaly, it is also indicated to treat it before.

Spinal cord tumors can be tried to remove. However, we must bear in mind that many times these surgeries carry high risks. They may not be indicated in certain patients. It must be a decision made by experts.

In some cases, a cyst drainage system can be inserted. It consists of inserting a tube directly and connecting it with another area of ​​the body or the outside. This removes accumulated cerebrospinal fluid and prevents syringomyelia from progressing.

Doctors often refer to the drainage system as siringoperitoneal shunt. This is because the tube connects to the peritoneum, which is a part of the abdomen.

The chronic pain associated with syringomyelia affects various aspects of the quality of life of patients.

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Recommendations for those who suffer from syringomyelia

Living with syringomyelia can be very complex. Most people suffer from chronic pain and have a progressive loss of mobility. Therefore, on a psychological level it is a disease that has a profound impact.

Ideally, avoid activities that make symptoms worse. Any movement that tends to strain the spine can exacerbate the pain. Likewise, intense efforts, such as picking up heavy things, also increase stiffness.

It is recommended that patients have professional psychological support. Especially to learn to cope with pain and limitations. In addition, rehabilitation and physiotherapy are also indicated. They can improve symptoms dramatically.

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Syringomyelia is a chronic and hard disease to cope with

This disease affects the spinal cord, which is responsible for transmitting nerve impulses between the body and the brain. Thus, syringomyelia can adversely affect mobility and sensation. In fact, is frequently associated with chronic pain.

It is essential that treatment is individualized. Both the affectation and the symptoms are highly variable, so it must be agreed upon by a multidisciplinary team of experts.