SYRINGOMYELIA

Syringomyelia: causes, symptoms, diagnosis, and treatment options

Syringomyelia is a rare but complex condition. In some cases, it does not cause any symptoms and monitoring is sufficient. In others, its symptoms can drastically affect the quality of life and severe complications can occur.[1][2] Learn more about the causes and symptoms of this condition, as well as what diagnosing and treating it entails below!

What is syringomyelia?

Syringomyelia is a rare neurological condition characterized by the formation of a fluid-filled cyst, called a syrinx, within the spinal cord. This cyst can expand over time, putting pressure on the spinal cord and damaging nerve fibers responsible for transmitting information between the brain and the rest of the body.[1]

It usually occurs in the upper portions of the spinal cord, namely in the cervical or thoracic region. The fluid in the formed cavities is cerebrospinal fluid (CSF), which normally surrounds and protects the brain and spinal cord. When the normal flow of this fluid is disrupted, it can accumulate in the spinal cord tissue, expanding the central canal and leading to the formation of the syrinx.[3]

Most people with syringomyelia are diagnosed between the ages of 20 and 50. However, the disease can also affect young children or older adults.[3]

Causes of syringomyelia

There are two main types of syringomyelia: congenital and acquired.

The congenital form of syringomyelia is often associated with Chiari malformation type I, a structural abnormality in which the lower part of the cerebellum extends into the foramen magnum, the opening at the base of the skull through which the spinal cord normally passes. This malformation impedes the normal flow of cerebrospinal fluid, leading to syringomyelia. Another congenital cause of syringomyelia is myelomeningocele, a condition where the spine and spinal canal do not close before birth (a type of neural tube defect).[1][3]

Acquired syringomyelia can have several possible causes. These include spinal cord injuries, spinal cord tumors, inflammation of the arachnoid membrane (arachnoiditis), or meningitis (inflammation of the meninges, the membrane that protects the brain and spinal cord). In some cases, the cause of syringomyelia may remain unknown, in which case we speak of idiopathic syringomyelia.[1][3]

Symptoms of syringomyelia

The symptoms of syringomyelia can vary depending on the exact location of the syrinx in the spinal cord, its size, and the degree of extension. In general, symptoms develop slowly and can affect one or both sides of the body.

Common symptoms of syringomyelia include:

• pain (in some cases chronic);
• stiffness of the back, shoulders, neck, arms, or legs;
• progressive weakness of the arms and legs;
• headaches;
• numbness;
• tingling;
• loss of sensitivity to pain or temperature;
• problems with bowel and bladder control;
• balance problems;
• sexual dysfunction;
• scoliosis (often the only symptom in children).[1]

Symptoms usually appear between the ages of 25 and 40 and can be exacerbated by certain activities (e.g., coughing, jumping, or anything that involves lifting weights or straining the spine).[2]

The diagnosis process of syringomyelia

The diagnosis of syringomyelia is based on a combination of factors. One of these is the evaluation of the patient's symptoms and medical history. If, following anamnesis and physical examination, syringomyelia is suspected, imaging tests will be performed to confirm the diagnosis. MRI is the most common imaging method used to diagnose syringomyelia. With this investigation, doctors can determine if there is a syrinx in the spinal cord or any other anomaly, such as a tumor. CT scans and other investigations may also be used.[2][3]

Treatment options for syringomyelia

Treatment options for syringomyelia are varied, with the main goal being to reduce pain, improve neurological function, and prevent the progression of the disease.[2]

If syringomyelia does not cause symptoms, no treatment may be necessary, and monitoring the condition, including periodic MRI scans and other neurological tests and investigations, may be sufficient.

If there are symptoms that affect the quality of life or if they worsen, surgical treatment of syringomyelia may be recommended. This may involve draining the fluid from the syrinx or correcting congenital malformations that cause the condition.[1][2][3]

For example, if syringomyelia is caused by Chiari malformation, surgery may involve removing a small section of bone from the back of the skull. This surgery can reduce pressure on the brain and spinal cord and restore the regular flow of cerebrospinal fluid.[2]

Additionally, physiotherapy can be a vital component of the treatment plan, offering significant benefits to patients with syringomyelia, including improved mobility and pain reduction.[2]

The prognosis for syringomyelia varies significantly from person to person, depending on the severity of the condition and the promptness of diagnosis and treatment. If you notice any concerning symptoms, consult a doctor for a correct diagnosis and appropriate treatment plan as soon as possible!

References

1. „Syringomyelia”, National Institute of Neurological Disorders and Stroke, 2023, www.ninds.nih.gov/health-information/disorders/syringomyelia. Accessed May 15, 2024.
2. „Syringomyelia”, Mayo Clinic, 2024, www.mayoclinic.org/diseases-conditions/syringomyelia/symptoms-causes/syc-20354771. Accessed May 15, 2024.
3. „Syringomyelia: What It Is, Symptoms, Causes & Treatment”, Cleveland Clinic, 2022, my.clevelandclinic.org/health/diseases/6126-syringomyelia. Accessed May 15, 2024.