What is it?
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 up to the 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 about 8 people in 100,000.
What are the symptoms of syringomyelia?
The severity of syringomyelia symptoms varies greatly, 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 20 and 40 years of age and can gradually progress to a major disability and loss of autonomy of the person with the disease:
Progressive weakness in the arms and legs, pain and numbness in the limbs, reduced reflexes, insensitivity to pain and temperature (exposing the patient to the risk of injury); Urinary and/or incontinence as well as erectile disorders; When the cavity is located at the base of the brain, it involves other symptoms such as headaches, dizziness, speech and swallowing disorders, and sometimes facial paralysis. Some of these symptoms may initially lead to the doctor’s diagnosis of multiple sclerosis. Syringomyelitis causes scoliosis in half of those with the disease in the long run.
The origins of the disease
In about half of the cases, syringomyelia is caused by a congenital malformation known as Chiari’s malformation. It is a malformation of a part of the nervous system in the cerebellum that, placed abnormally low, disrupts the flow of cerebrospinal fluid around the spinal cord. The fluid then makes its way inside the spinal cord and forms cavities. Cerebrospinal fluid (also known as cerebrospinal fluid) protects the central nervous system from shock and irrigates brain cells into essential elements.
Syringomyelitis can also be secondary, as the syrinx develops on a segment of the spinal cord damaged by trauma, infection (meningitis), haemorrhage, tumour or arachnoiditis (inflammation of the meninges). It should be noted that the cause of the disease is not always known, which is then referred to as a primitive or idiopathic syringomyelia, often benign.
Syringomyelia usually occurs around the age of 30 and affects men more frequently than women. There is also familial syringomyelia, which represents 2% of all cases of syringomyelia.
Prevention and treatment
The treatment aims to correct the underlying cause, such as the removal of a tumour. Surgery helps stabilize the progression of symptoms or cause them to regress a little, but the risk of relapse is significant and may require a second operation. The type of surgery depends on the characteristics of the syrinx and the neurosurgeon’s experience. The operation involves restoring the flow of cerebrospinal fluid. Some patients experience severe chronic pain that greatly impairs the quality of life. They are treated with medications (tricyclic antidepressants, muscle relaxants, anticonvulsants). People with syringomyelia often use a neurostimulation device to relieve pain. Electrodes placed on the skin send small electric shocks that will interrupt the transmission of pain signals.