Conditions Commonly Associated With Syringomyelia

Ehlers-Danlos syndrome is a group of 13 heritable connective tissue disorders caused by genetic changes that affect the body's connective tissue — the material that provides structure and support to skin, joints, blood vessels, and organs. EDS is estimated to affect 1 in 5,000 to 20,000 individuals worldwide, though many experts believe it is significantly underdiagnosed due to the wide variability of its symptoms. The most common type, hypermobile EDS (hEDS), is frequently seen alongside syringomyelia and Chiari malformation.

Why it matters: Weakened connective tissue in EDS can contribute to craniocervical instability — instability at the junction of the skull and cervical spine — which may obstruct normal cerebrospinal fluid (CSF) flow and contribute to the formation or worsening of a syrinx. EDS can also complicate surgical outcomes, increasing the risk of CSF leaks, poor wound healing, and spinal instability following decompression procedures. Recognizing EDS in a syringomyelia patient is essential for building a complete and accurate picture of their condition.

Symptoms may include: joint hypermobility, frequent joint dislocations or subluxations, joint pain, skin that is unusually stretchy, soft, or fragile, easy bruising, chronic fatigue, muscle pain, digestive issues including IBS, brain fog or difficulty concentrating, autonomic dysfunction, and in some cases craniocervical or atlantoaxial instability.

Chiari malformation type I is the condition most commonly associated with syringomyelia. It happens when part of the brain sits lower than usual at the base of the skull and can crowd the opening where CSF normally flows.

Why it matters: When CSF flow is blocked or altered, pressure changes can contribute to the formation or enlargement of a syrinx.

Symptoms may include: headaches, especially with coughing or straining; neck pain; dizziness; balance problems; swallowing issues; numbness; weakness; or sensory changes.

Tethered cord syndrome occurs when the spinal cord is abnormally attached to surrounding tissue and cannot move freely as it should. This abnormal tension can affect spinal cord function and may also be associated with syringomyelia.

Why it matters: Stretching and tension on the spinal cord may affect fluid movement and contribute to neurologic symptoms.

Symptoms may include: back pain, leg pain, weakness, changes in walking, numbness, bowel or bladder problems, and worsening symptoms over time.

Myelomeningocele is a form of spina bifida in which the spine does not fully close before birth. Because it changes the structure of the spinal cord and surrounding tissues, it can be associated with altered CSF flow and syringomyelia.

Why it matters: Congenital differences in the spine and spinal cord can increase the chance of other neurologic conditions occurring together.

Symptoms may include: weakness, sensory loss, orthopedic issues, bowel or bladder dysfunction, and mobility challenges, depending on the level of spinal involvement.

A syrinx can develop months or even years after a traumatic spinal cord injury. This is often called post-traumatic syringomyelia.

Why it matters: Scar tissue and changes in CSF flow after an injury can interfere with normal spinal cord fluid dynamics and lead to progressive symptoms.

Symptoms may include: increasing pain, weakness, numbness, stiffness, muscle spasms, changes in sweating, and new bowel or bladder changes after a prior injury.

Some spinal cord tumors are associated with syringomyelia because they can press on the spinal cord or block the normal flow of CSF.

Why it matters: When a tumor changes the space around the spinal cord, fluid may collect within the cord and contribute to syrinx formation.

Symptoms may include: back or neck pain, progressive weakness, numbness, tingling, walking difficulty, and bowel or bladder symptoms.

Arachnoiditis is inflammation of the arachnoid, one of the membranes surrounding the spinal cord. It may lead to scarring that disrupts normal CSF flow.

Why it matters: Inflammation and scar tissue around the cord can contribute to pain, nerve irritation, and in some cases syringomyelia.

Symptoms may include: burning or stinging nerve pain, tingling, numbness, muscle cramps, weakness, and pain that worsens over time.

Meningitis is inflammation or infection of the protective membranes surrounding the brain and spinal cord. In some cases, the resulting inflammation or scarring can affect CSF flow.

Why it matters: Changes after meningitis may contribute to syrinx formation in a smaller number of patients.

Symptoms may include: fever, severe headache, neck stiffness, sensitivity to light, nausea, confusion, and later neurologic problems depending on the cause and severity.

Some cases of syringomyelia are described as communicating, meaning the syrinx is connected to fluid spaces in the brain. This pattern may occur alongside hydrocephalus, which is a buildup of excess fluid in or around the brain.

Why it matters: These cases highlight how closely the brain, spinal cord, and CSF system are connected.

Symptoms may include: headaches, nausea, vision changes, balance issues, cognitive changes, or symptoms related to pressure and spinal cord involvement.

In some people, no clear underlying cause is found even after MRI and specialist evaluation. This is often called idiopathic syringomyelia, which means the cause is unknown.

Why it matters: Not every syrinx has an obvious explanation, and some people may be monitored over time depending on symptoms and imaging findings.

Symptoms may include: pain, numbness, weakness, sensory loss, stiffness, or sometimes no symptoms at all.

Scoliosis is a sideways curvature of the spine. It is not usually considered a direct cause of syringomyelia, but it is commonly seen as an associated finding or complication, especially in children and adolescents.

Why it matters: In some patients, scoliosis may be one of the first visible signs that leads doctors to look more closely for an underlying neurologic condition.

Symptoms may include: uneven shoulders, rib prominence, back pain, posture changes, or curve progression during growth.

These are not always separate diagnoses, but they are common issues associated with syringomyelia as it affects the spinal cord over time.

Examples include: chronic pain, numbness, reduced sensation to hot and cold, weakness, stiffness, muscle wasting, balance problems, and bowel or bladder dysfunction.

Why it matters: Symptoms can vary a lot depending on where the syrinx is located, how large it is, and whether it is changing over time.