Doctronic

Understanding Syringomyelia: Causes, Symptoms, Diagnosis, and Treatment Options

Jerome Albert Ecker | MD

Medically reviewed by Jerome Albert Ecker | MD, Assistant Professor of Medicine, Duke University - Durham, NC on August 19th, 2023.

Key Takeaways

  • Fluid-filled cysts (syrinxes) develop within the spinal cord when cerebrospinal fluid flow becomes blocked, creating cavities that expand over time and compress surrounding nerve tissue.

  • The condition predominantly affects adults aged 20-40, with Chiari malformation Type I being the most common underlying cause, accounting for approximately 60% of cases.

  • Early symptoms include asymmetric loss of pain and temperature sensation in a "cape-like" distribution across shoulders and arms, while touch and position sense remain intact initially.

  • MRI with contrast is the gold standard for diagnosis and monitoring—cyst size and location determine symptom severity, with cervical spine involvement causing the most disability.

  • Posterior fossa decompression surgery has success rates of 70-80% for halting progression when performed early, but existing neurological damage is often permanent.

Syringomyelia is a long-term condition that causes fluid-filled cysts, called syrinx, to form inside the spinal cord. This condition is most common in adults between the ages of 20 and 40, with men being more likely to develop it than women. While some people with syringomyelia may not experience any symptoms, severe cases can lead to chronic pain and other complications that require treatment.

Causes of Syringomyelia

Syringomyelia occurs when the normal flow of cerebrospinal fluid, which protects the spinal cord and brain, is blocked. This blockage can cause the fluid to enter the central canal of the spinal cord, leading to the formation of a cyst. There are two main reasons for this blockage:

  1. Congenital syringomyelia (also called communicating syringomyelia): This type of syringomyelia is caused by a birth defect, such as a Chiari I malformation, which causes the lower part of the brain to push downward into the spinal canal.

  2. Acquired syringomyelia (also called primary spinal or noncommunicating syringomyelia): This type of syringomyelia occurs when a cyst forms in a damaged part of the spinal cord, which can be caused by swelling of the spinal cord covering, meningitis, spinal cord injury, spinal tumor, or tethered spinal cord.

Symptoms of Syringomyelia

The symptoms of syringomyelia depend on the location and size of the cyst. As the cyst grows wider and longer, it can damage the nerves in the center of the spinal cord, leading to chronic pain that is difficult to treat. Some common symptoms of syringomyelia include:

  • Curving of the spine (scoliosis)

  • Changes in or loss of bowel and bladder function

  • Heavy sweating

  • Loss of sensation to hot and cold in the fingers, hands, arms, and upper chest

  • Loss of reflexes

  • Muscle stiffness that may make it difficult to walk

  • Muscle weakness, especially in the arms, hands, and shoulders

  • Numbness, tingling, burning, or piercing pain in the neck, shoulders, arms, and hands

  • Paralysis (in severe cases)

Understanding Syringomyelia: Causes, Symptoms, Diagnosis, and Treatment Options

Diagnosing Syringomyelia

To diagnose syringomyelia, doctors use magnetic resonance imaging (MRI) scans of the brain and spine to look for cysts or other conditions that may be causing the symptoms. Sometimes, syringomyelia is discovered incidentally during an MRI for another health reason. In addition to an MRI, doctors may also use a CT scan of the spine to help diagnose the condition.

Treatment Options for Syringomyelia

The treatment for syringomyelia depends on the presence and severity of symptoms. If there are no symptoms or if the symptoms are mild, treatment may not be necessary. However, regular checkups are important to monitor for cyst growth and changes in symptoms. In cases where symptoms are causing problems, surgery may be necessary to restore the normal flow of cerebrospinal fluid. The type of surgery depends on the underlying cause of the syringomyelia, such as a Chiari I malformation or spinal cord injury.

Posterior Fossa Decompression Surgery

For syringomyelia caused by a Chiari I malformation, a surgical procedure called posterior fossa decompression may be recommended. This surgery involves removing a portion of the bone at the back of the skull and spine to create more space for the brainstem and cerebellum, which can help restore the normal flow of cerebrospinal fluid.

Outlook for Syringomyelia

While surgery can be effective in treating syringomyelia, it does not always completely resolve the condition. In some cases, fluid can build up again, and nerve and spinal cord damage may be permanent. Regular follow-up appointments with a doctor, including MRI scans, are essential to monitor for cyst recurrence and manage any ongoing symptoms. For more information on syringomyelia and its treatment options, visit the National Institute of Neurological Disorders and Stroke website.

The Bottom Line

Early detection and surgical intervention within the first few years of symptom onset offers the best chance of preventing permanent neurological damage from progressive cyst expansion. The key is recognizing the characteristic pattern of losing temperature sensation while maintaining normal touch sensation in the arms and shoulders. If you're experiencing these unusual sensory changes or unexplained arm weakness, Doctronic can help connect you with specialists for proper evaluation.

Related Articles