Syringomyelia is characterized by the formation of one or more cavities inside the spinal cord, which can cause severe chronic pain, neurological disorders and motor impairment including loss of autonomy. These cavities called “syrinx” damage the neurological functions of the spinal cord. It is often the result of a Chiari malformation and its prevalence is around 8 in 100 people. (000)

The severity of the symptoms of syringomyelia varies a lot, depending on its underlying cause, the characteristics of the cavity (size, shape, location, progression) and the damage thus caused to the spinal cord. Half of people with syringomyelia have no symptoms or only minor discomfort. When syringomyelia occurs without a known cause, it is benign in two-thirds of cases. (1) The disease develops silently for several years and the first signs occur between the ages of 20 and 40 and can gradually progress to a major handicap and a loss of autonomy of the affected person:

• Progressive weakness in the arms and legs, pain and numbness in the limbs, reduced reflexes, insensitivity to pain and temperature (putting the patient at risk of injury);
• Urinary and / or anal incontinence as well as erectile dysfunction;
• When the cavity is located at the base of the brain, it involves other symptoms such as headache, dizziness, difficulty speaking and swallowing, and sometimes facial paralysis.

Some of these symptoms sometimes lead the doctor’s diagnosis first to multiple sclerosis. Syringomyelia ultimately causes scoliosis in half of those affected.

In about half of cases, syringomyelia originates from a birth defect known as Chiari’s malformation. It is a malformation of part of the nervous system in the cerebellum which, placed abnormally low, disrupts the flow of cerebrospinal fluid around the spinal cord. The fluid then finds its way inside the spinal cord and forms cavities. Cerebrospinal fluid (also called cerebrospinal fluid) protects the central nervous system against shock and supplies brain cells with essential elements.

Syringomyelia can also be secondary, with the syrinx developing on a segment of the spinal cord damaged by trauma, infection (meningitis), hemorrhage, tumor, or arachnoiditis (inflammation of the meninges). Note that we do not always know the cause of the disease, which is then referred to as primary or idiopathic syringomyelia, often benign.

Syringomyelia usually starts around the age of 30 and affects men more often than women. There is also a familial syringomyelia representing 2% of all syringomyelia cases. (1)

Treatment aims to correct the underlying cause, such as the removal of a tumor. The use of surgery stabilizes the progression of symptoms or makes them regress a little, but the risk of relapse is high and may require a second operation. The type of surgery depends on the characteristics of the syrinx and the experience of the neurosurgeon. The operation involves restoring the flow of cerebrospinal fluid.

Some patients experience severe chronic pain which greatly affects the quality of life. They are treated with medication (tricyclic antidepressants, muscle relaxants, anticonvulsants). People with syringomyelia often use a neurostimulation device for pain relief. Electrodes placed on the skin send out small electric shocks that will interrupt the transmission of pain signals.