Syringomyelia

Overview

Syringomyelia is a rare condition characterized by the formation of a fluid-filled cyst, known as a syrinx, within the spinal cord, which disrupts the normal flow of cerebrospinal fluid (CSF). This disruption can lead to damage to the spinal cord and compression of nerve fibers, resulting in symptoms such as pain, weakness, and stiffness. When the brain stem is affected, it is referred to as syringobulbia.

Typically, syringomyelia affects the upper regions of the spinal cord, including the cervical and thoracic areas. One common cause of syringomyelia is Chiari malformation, where brain tissue protrudes into the spinal canal. Other factors contributing to syringomyelia include spinal cord tumors, injuries, and inflammation around the spinal cord.

Treatment for syringomyelia, especially when symptoms are severe, often involves surgery. However, in cases where syringomyelia is not causing significant issues, regular monitoring may be all that is necessary.

Symptoms

Syringomyelia is a condition characterized by the development of fluid-filled cysts within the spinal cord, with symptoms that generally emerge gradually and may worsen over time. Although some individuals with a small syrinx may not immediately experience symptoms, the severity and nature of these symptoms can vary based on the syrinx’s location, size, and how far it extends along the spine, affecting either one or both sides of the body. Symptoms can range from mild to severe. In cases where syringomyelia is caused by brain tissue protruding into the spinal canal, it is known as a Chiari malformation, with symptoms typically starting between the ages of 25 and 40. It’s also noted that actions like coughing or straining might trigger symptoms but are not causes of the condition.

Common symptoms:

• Muscle weakness and wasting
• Loss of reflexes
• Reduced sensitivity to pain and temperature
• Headaches
• Stiffness in the back, shoulders, arms, and legs
• Pain in the neck, arms, and back
• Scoliosis, which involves a sideways curvature of the spine

Symptoms Specific to Children:

• Scoliosis
• Toe walking

If you experience any of the above symptoms, consult a healthcare professional. For those with a history of spinal cord injury, it is crucial to monitor for symptoms of syringomyelia, as they may develop months to years after the injury. Ensure your healthcare provider is aware of any past spinal injuries.

Causes

Syringomyelia occurs when the fluid that typically envelops and safeguards the brain and spinal cord accumulates inside the spinal cord itself. While the precise reason for this buildup remains elusive, theories propose it may stem from an obstruction or disturbance in the flow of cerebrospinal fluid (CSF).

The causes of syringomyelia can be broadly categorized into two groups: congenital, meaning present at birth, and acquired, which develop later in life. Occasionally, syringomyelia can manifest without a discernible cause, termed idiopathic syringomyelia.

Generally, common causes include:

• Chiari malformation: The majority of cases are linked with Chiari malformation type 1. While this is typically present at birth, symptoms may not appear until adolescence or adulthood, or some individuals may never experience symptoms at all. This disorder causes brain tissue to protrude into the spinal canal and obstructs the normal passage of CSF fluid.
• Open spina bifida: Also known as myelomeningocele, is a birth defect where the backbone and spinal canal fail to close before birth, classified as a type of neural tube defect (NTD).
• Tethered cord syndrome: Commonly linked with conditions like spina bifida and scoliosis, this rare neurological condition involves the spinal cord being attached to the surrounding spine tissues.
• Meningitis: This is an infection of the protective lining around the brain and spinal cord which can cause syringomyelia.
• Spinal cord tumor: This include hemangioblastomas and ependymomas, which can disrupt CSF circulation, leading to syringomyelia.
• Spinal cord injury: This is termed post-traumatic syringomyelia and typically arises months or years after the initial injury due to scar tissue blocking cerebrospinal fluid (CSF) flow.

Risk factor

Most individuals diagnosed with syringomyelia are between the ages of 20 and 50, although the condition can also manifest in young children and older adults.