Conditions linked to syringomyelia
Syringomyelia happens when a fluid-filled cavity, called a syrinx, forms inside the spinal cord. It’s often tied to another condition that affects the brain, the spinal cord, or the normal flow of cerebrospinal fluid (CSF).1
No two people share the exact same mix of diagnoses, symptoms, or treatment path. This page walks through the conditions most often seen alongside syringomyelia, so you and your family know what to ask about.
One syrinx, many roots.
Finding the condition behind a syrinx often shapes the whole treatment plan. That’s why the full picture matters.
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~50%
Tied to Chiari type 1
Chiari malformation type 1 is the most common cause, behind roughly 48% to 50% of adult cases and about 43% of cases in children.3
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25–45%
With myelomeningocele
A syrinx shows up on imaging in an estimated 25% to 45% of people born with myelomeningocele, a form of spina bifida.3
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Up to 30%
After spinal cord injury
Imaging studies suggest up to 30% of people with a spinal cord injury may develop a syrinx over decades, though far fewer develop symptoms.4
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25–58%
With tumors inside the cord
A syrinx is found in 25% to 58% of people with intramedullary spinal cord tumors, which grow inside the cord itself.3
It usually comes back to fluid flow
CSF surrounds and cushions the brain and spinal cord. When something gets in the way of how it normally moves, fluid can collect inside the cord and form a syrinx.1 Most associated conditions fall into one of four groups.
Blocked flow at the skull base
Brain tissue or extra fluid crowds the opening where CSF passes between the skull and the spine.
Chiari malformation, hydrocephalus
Differences present from birth
The spine or spinal cord forms differently, which can add tension or change the space around the cord.
Tethered cord, spina bifida
Injury, scarring, or growth
Trauma, inflammation, or a tumor creates scar tissue or a blockage that disrupts fluid around the cord.
Spinal cord injury, arachnoiditis, meningitis, tumors
Often seen alongside
Conditions that commonly show up in the same patients and can shape symptoms, surgery, and recovery.
EDS, POTS, MCAS, scoliosis
What to know about each
Each card covers what the condition is, why it matters for syringomyelia, and common symptoms. Links open example images and trusted references in a new tab.
Blocked flow at the skull base
Chiari malformation type 1
Part of the brain, the cerebellar tonsils, sits lower than usual and extends through the opening at the base of the skull into the spinal canal, crowding the space where CSF normally flows.1
- Why it matters
- When CSF flow is blocked or altered, pressure changes can lead to a syrinx forming or growing. Syringomyelia is diagnosed in about 65% of people with Chiari type 1.2
- Symptoms may include
- Headaches, especially with coughing or straining; neck pain; dizziness; balance problems; trouble swallowing; numbness; weakness; or sensory changes.
Hydrocephalus
Hydrocephalus is a buildup of excess CSF in the brain, with larger than normal fluid spaces called ventricles. It can occur with congenital syringomyelia.1
- Why it matters
- In communicating syringomyelia, the syrinx connects to the fluid spaces of the brain, and this pattern is closely tied to hydrocephalus.2 It shows how tightly the brain, spinal cord, and CSF system are linked.
- Symptoms may include
- Headaches, nausea, vision changes, balance problems, changes in thinking or memory, and symptoms tied to pressure on the spinal cord.
Differences present from birth
Tethered cord syndrome
The spinal cord is abnormally attached to tissue in the lower spine, which limits how freely it can move.1
- Why it matters
- Tension on the cord can affect fluid movement and nerve function. A syrinx is reported in roughly 21% to 67% of people with different forms of spinal dysraphism, the group of birth differences that includes many tethered cords.5
- Symptoms may include
- Back or leg pain, weakness, changes in walking, numbness, bowel or bladder problems, and symptoms that slowly get worse.
Myelomeningocele and spina bifida
Myelomeningocele is a form of spina bifida in which the spine doesn’t fully close before birth, changing the structure of the spinal cord and the tissue around it.
- Why it matters
- A syrinx appears on imaging in an estimated 25% to 45% of people with myelomeningocele, though only a small share develop an active, symptomatic syrinx.3
- Symptoms may include
- Weakness, loss of sensation, orthopedic problems, bowel or bladder changes, and mobility challenges, depending on where the spine is affected.
Injury, scarring, or growth
Spinal cord injury
A syrinx can form months or even decades after a spinal cord injury. The median time to diagnosis is 9 to 15 years.4
- Why it matters
- Scar tissue at the injury site can disrupt CSF flow. New or worsening symptoms years after an injury deserve a fresh look, not an assumption that it’s just the old injury.4
- Symptoms may include
- Increasing pain, weakness, numbness, stiffness, muscle spasms, changes in sweating, and new bowel or bladder changes.
Arachnoiditis
Arachnoiditis is inflammation of the arachnoid, one of the three membranes that surround the spinal cord.1
- Why it matters
- Inflammation can leave scar tissue that disrupts normal CSF flow around the cord, which can contribute to a syrinx.2
- Symptoms may include
- Burning or stinging nerve pain, tingling, numbness, muscle cramps, weakness, and pain that worsens over time.
Meningitis
Meningitis is inflammation of the membranes around the brain and spinal cord, usually caused by an infection.1
- Why it matters
- Inflammation and scarring after meningitis can change CSF flow and lead to a syrinx in a smaller number of people.2
- Symptoms may include
- Fever, severe headache, stiff neck, sensitivity to light, nausea, and confusion. Later nerve problems depend on the cause and severity.
Spinal cord tumors
Some tumors press on the spinal cord or block the flow of CSF. A syrinx is found in 25% to 58% of people with tumors that grow inside the cord.3
- Why it matters
- This is one cause where treatment can make a clear difference. When a tumor is causing the syrinx, removing it almost always eliminates the syrinx.1
- Symptoms may include
- Back or neck pain, progressive weakness, numbness, tingling, trouble walking, and bowel or bladder changes.
Often seen alongside
These conditions don’t usually cause a syrinx on their own, but they’re common in our community and can affect symptoms, surgery, and recovery.
Ehlers-Danlos syndromes (EDS)
EDS is a group of inherited disorders that affect the connective tissue supporting the skin, joints, blood vessels, and organs. The 2017 classification describes 13 types. All types combined affect at least 1 in 5,000 people worldwide, and the hypermobile type alone may affect as many as 1 in 5,000 to 20,000.6
- Why it matters
- Chiari malformation, craniocervical instability, and tethered cord have all been reported alongside EDS.7 Weaker connective tissue can also raise the risk of CSF leaks, slow wound healing, and spinal instability after surgery, so it’s worth making sure your surgeon knows.7,8
- Symptoms may include
- Joint hypermobility, frequent dislocations or partial dislocations, joint pain, soft or stretchy skin, easy bruising, chronic fatigue, digestive problems, brain fog, and autonomic symptoms.
Dysautonomia and POTS
Dysautonomia is an umbrella term for problems with the autonomic nervous system, which runs heart rate, blood pressure, digestion, and temperature. In postural orthostatic tachycardia syndrome (POTS), heart rate rises by at least 30 beats per minute in adults within 10 minutes of standing.9
- Why it matters
- POTS is strongly associated with joint hypermobility disorders, including EDS,9 and was reported in about 71% of people with hypermobile EDS in one large study.8 It can add to neurologic symptoms and make standing, work, and exercise harder.
- Symptoms may include
- Racing heart when standing, lightheadedness or fainting, brain fog, fatigue, headaches, nausea, heavy sweating, and trouble tolerating exercise.
Mast cell activation syndrome (MCAS)
In MCAS, immune cells called mast cells release chemicals like histamine too easily, causing repeated episodes of allergy-like symptoms that can affect several parts of the body at once.10
- Why it matters
- MCAS is often reported alongside hypermobile EDS and POTS. Researchers are still working out how these conditions are linked, but specialists are advised to watch for MCAS when symptoms involve two or more body systems.11 Its symptoms can overlap with, and add to, the pain and fatigue of syringomyelia.
- Symptoms may include
- Flushing, hives, itching, stomach pain, nausea, diarrhea, racing heart, lightheadedness, shortness of breath, brain fog, and headaches. Triggers can include certain foods, temperature changes, stress, and some medications.11
Scoliosis
Scoliosis is a sideways curve of the spine. It isn’t usually a cause of syringomyelia, but it’s a common finding with it, and in children it may be the only sign.1
- Why it matters
- Syringomyelia should be considered in any young person whose scoliosis is getting worse. Finding and treating it early can help reverse the curve.2
- Symptoms may include
- Uneven shoulders, a rib hump, back pain, posture changes, or a curve that progresses during growth.
Common neurologic problems
These aren’t always separate diagnoses. They’re problems that often develop as a syrinx affects the spinal cord over time.1
- Examples include
- Chronic pain, numbness, reduced feeling for hot and cold, weakness, stiffness, muscle wasting, balance problems, and bowel or bladder changes.
- Why it matters
- Symptoms vary a lot depending on where the syrinx sits, how large it is, and whether it’s changing over time.1
Idiopathic syringomyelia
For some people, no clear cause is found even after MRI and a specialist evaluation. This is called idiopathic syringomyelia.1
- Why it matters
- Not every syrinx has an obvious explanation. Depending on symptoms and imaging, some people are monitored over time with follow-up MRIs.2 An unknown cause doesn’t make your symptoms any less real.
- Symptoms may include
- Pain, numbness, weakness, loss of sensation, stiffness, or sometimes no symptoms at all.
See the whole picture.
One diagnosis rarely tells the full story.
Many people in our community live with more than one of these conditions. Each one can be managed by a different specialist, and it’s easy for the connections between them to get lost along the way.
Bring a full list of your diagnoses to every appointment, and ask how they might affect each other, especially before any surgery. If a provider dismisses symptoms that don’t fit neatly into one diagnosis, you deserve a second opinion.
Use our provider directory, our appointment prep guide, and our medical glossary to help.
Questions to ask your doctor
Write these down and bring them with you. They can help connect the dots between specialists.
- What do you think is causing my syrinx?
- Should I be checked for Chiari malformation, tethered cord, or another related condition?
- Could my joint, heart rate, or digestive symptoms point to EDS, POTS, or MCAS?
- How might my other diagnoses affect surgery or recovery?
- Which specialists should be part of my care team?
- How will my care be coordinated between them?
You don’t have to do this alone.
Find a provider who understands these conditions, connect with others through a local chapter, or help fund care packages, patient grants, and the Regenerative Therapy Access Program for people living with syringomyelia and associated conditions.
Disclaimer
The information on this page is for general education only. It is not medical advice and is not a substitute for diagnosis or treatment from a qualified, licensed healthcare professional. I AM HEARD Coalition does not diagnose conditions, recommend or endorse a particular treatment, or guarantee any medical outcome. Links to outside sites are provided for reference only.
Every person’s situation is different. Talk with your own healthcare team about diagnosis, testing, and treatment. If you have sudden or rapidly worsening symptoms, such as new weakness, loss of bladder or bowel control, or trouble breathing or swallowing, seek emergency care right away.
Sources
- National Institute of Neurological Disorders and Stroke. Syringomyelia. ninds.nih.gov
- Shenoy VS, Munakomi S, Sampath R. Syringomyelia. StatPearls. Updated 2024. ncbi.nlm.nih.gov/books/NBK537110
- American Academy of Physical Medicine and Rehabilitation. Pediatric Syringomyelia. PM&R KnowledgeNow. now.aapmr.org
- Posttraumatic Syringomyelia. StatPearls. ncbi.nlm.nih.gov/books/NBK470405
- Syringomyelia in the tethered spinal cords. J Korean Neurosurg Soc. 2020. pmc.ncbi.nlm.nih.gov/articles/PMC7218206
- MedlinePlus Genetics, National Library of Medicine. Ehlers-Danlos syndrome. medlineplus.gov
- Henderson FC Sr, et al. Neurological and spinal manifestations of the Ehlers–Danlos syndromes. Am J Med Genet C. 2017. doi.org/10.1002/ajmg.c.31549
- Severance S, et al. Hypermobile Ehlers-Danlos syndrome and spontaneous CSF leaks: the connective tissue conundrum. Front Neurol. 2024. pmc.ncbi.nlm.nih.gov/articles/PMC11289524
- Johns Hopkins Medicine. Postural Orthostatic Tachycardia Syndrome (POTS). hopkinsmedicine.org
- Cleveland Clinic. Mast Cell Activation Syndrome (MCAS). my.clevelandclinic.org
- American Gastroenterological Association. Clinical Practice Update on GI manifestations and autonomic or immune dysfunction in hypermobile Ehlers-Danlos syndrome: expert review. Clin Gastroenterol Hepatol. 2025. pubmed.ncbi.nlm.nih.gov/40387691