How Common is Syringomyelia?

Syringomelia is a spinal condition that raises many questions. If you or a loved one are facing this diagnosis, you likely want to find out: how common is syringomyelia?

Syringomyelia disease is considered an uncommon neurological condition. Population studies suggest a prevalence of 8 to 9 people per 100,000, although estimates vary between countries and patient groups. While these figures indicate a low overall occurrence, the condition accounts for a significant portion of complex spinal neurosurgery cases due to the severe impacts that it can have on a person’s quality of life.

In this blog, we will delve deeper into this specific condition, its rarity and address its causes, symptoms, and treatment for syringomyelia. 

What is Syringomyelia?

Syringomyelia (sear-IN-go-my-EEL-ya) is a complex neurological condition affecting the spinal cord. It occurs when a fluid-filled sac, known as a syrinx, develops within the spinal cord. This syrinx contains cerebrospinal fluid (CSF), which typically flows in the brain and spinal cord to cushion and protect the central nervous system.  As the syrinx expands, it presses against and damages the nerve fibres that transmit information between the brain and the body, causing a variety of significant neurological problems.

What Causes Syringomyelia?

When determining what the causes of syringomyelia are, neurosurgeons classify the condition into congenital and acquired types. In both, the core issue is a disruption of normal cerebrospinal fluid (CSF) flow.

The most frequent cause is a Chiari malformation. Here, brain tissue extends into the spinal canal, blocking CSF flow and causing a syrinx to form within the spinal cord.

Syringomyelia can also result from acquired factors, such as:

• Spinal Cord Trauma (even an old one)
• Spine Tumours
• Infections and inflammatory conditions, such as meningitis or arachnoiditis
• Tethered Cord Syndrome

What are the Symptoms of Syringomyelia?

The presence of a syrinx that damages the spinal cord can cause a wide range of neurological deficits. You may wonder what the symptoms of syringomyelia are. It depends on the size of the cyst and its location along the spine, most commonly the neck (cervical) region. Symptoms develop slowly over time, but they can be accelerated by physical exertion or stress.

5 common syringomyelia symptoms include:

1. Chronic pain in the neck, shoulders, arms or back 
2. Sensation loss, which often shows up in the hands and upper body, with a distinct ‘cape-like’ distribution.
3. Progressive motor and muscle weakness, stiffness, or muscle wasting (atrophy) affecting the arms, hands, and legs.
4. Headaches, especially those triggered by coughing, straining or sneezing. Common, particularly when Chiari malformation is the underlying cause.
5. In children, scoliosis may be the only initial sign.

Syringomyelia Treatment and Outlook

A critical question is: Is syringomyelia curable? While there is no procedure to reverse permanent nerve damage, the goal of syringomyelia treatment is to halt the disease’s progression. Mild, non-progressing cases are managed with careful monitoring. 

For progressive symptoms, surgical intervention of the appropriate type is required, depending on the cause.  Chiari Decompression, Syrinx Shunting (tube drainage of the syrinx), and Surgical Obstruction Removal (tumours/scar tissue) are the primary options.

Following surgery, rehabilitation and physical therapy are crucial for long-term function. Effective management of syringomyelia disease requires ongoing follow-up.

Any surgical and invasive procedures carry risks. Outcomes, recovery timeframes, and the potential for symptom improvement differ between individuals, and no specific result can be guaranteed. Decisions regarding investigation and treatment should be made in consultation with a qualified medical specialist who can assess your individual condition and discuss appropriate options, including benefits, risks, and alternatives.

Call Us Now to Discuss Your Syringomyelia Care

When you face a diagnosis like syringomyelia, you need care that is informed, attentive and tailored to you. Our team of experienced neurosurgeons in Australia have subspecialty interests in complex spinal conditions, including Chiari malformation and syringomyelia treatment. Located at Macquarie University Hospital, we use state-of-the-art diagnostic and surgical technology. 

We offer collaborative, multidisciplinary care, ensuring that both surgical and nonsurgical approaches are carefully considered. Book your appointment with us today for an additional clinical opinion, and we’ll organise a time and place that works for you.