EDS, Tethered Cord Syndrome & Chiari: A Trio of Neurological Mayhem

Ah, the joys of having a complex, multi-systemic condition that most doctors treat like an urban legend. If you’re an Ehlers-Danlos Syndrome (EDS) patient, you already know the routine: persistent symptoms dismissed as anxiety, endless specialist referrals that vanish into the ether, and the creeping suspicion that you know more than your doctor (because you probably do).

Enter Tethered Cord Syndrome (TCS)—yet another poorly understood condition that can quietly dismantle your life while the healthcare system collectively shrugs.

But wait—what happens when you throw Chiari Malformation (CM) into the mix? Spoiler alert: it’s not great.

So, let’s talk about TCS, its sneaky sibling Occult Tethered Cord Syndrome (OTCS), Chiari, and why this trio is a medical nightmare masquerading as "just EDS."

What the Hell is Tethered Cord Syndrome?

Tethered Cord Syndrome is a neurological disorder where the spinal cord becomes abnormally attached—or "tethered"—to surrounding structures. Think of your spinal cord as an elastic band: it should glide up and down freely as you move. Now imagine it stapled to the base of your spine. That’s TCS in a nutshell—restricted movement, resulting in pain, neurological dysfunction, and a parade of baffling symptoms [1].

TCS can be congenital (you're born with it) or acquired through trauma, spinal surgery, or degenerative disease. It’s easier to diagnose in children, as MRIs may show a low-lying conus medullaris (the lowest tip of the spinal cord). But for adults—especially those with EDS—Occult Tethered Cord Syndrome plays ninja, dodging standard imaging and casting doubt on your very sanity.

EDS, Tethered Cord, and Chiari: A Match Made in Medical Hell

For those of us blessed with hypermobile EDS (hEDS), the risk of developing TCS is higher because—shocker—our collagen is faulty. The filum terminale, a ligament anchoring the spinal cord, is made of collagen. When your collagen is more marshmallow than structural support, this ligament becomes inelastic and starts pulling down on your spinal cord like an over-tight violin string [2].

Now, enter Chiari Malformation. This occurs when the cerebellar tonsils (part of the brain) herniate through the foramen magnum (the hole at the base of your skull), compressing the spinal cord and brainstem. Basically, your brain is trying to leave your body—and honestly, can you blame it?

Here’s how they team up to ruin your life:

• Tethered Cord pulls the spinal cord down, creating spinal tension that worsens Chiari symptoms.

• Chiari Malformation blocks cerebrospinal fluid (CSF) flow, exacerbating tethered cord symptoms.

• EDS throws instability into the mix, making both conditions even harder to manage.

The result? A neurological pressure cooker—fun!

Symptoms of Tethered Cord Syndrome (Now with Extra Chiari Chaos!)

Neurological Mayhem

• Lower back and hip pain (often a deep, pulling sensation)

• Leg numbness, weakness, or pain—worse with activity

• Difficulty standing or walking (gravity’s personal vendetta)

• Foot abnormalities (high arches, flat feet, toe-walking)

• Headaches, dizziness, and cognitive fog (Chiari’s love letter)

• Neck pain and pressure, especially when coughing or straining

Bladder & Bowel Bingo

• Urinary retention or incomplete emptying

• Random urgency or incontinence

• Constipation or unpredictable bowel movements

• Painful bladder symptoms mimicking interstitial cystitis

Bonus Features for EDS Patients

• Symptoms worsen after sitting, bending, or prolonged activity

• Pain progresses despite conservative care

• Rapid mobility decline—cue the wheelchair

  
  

Why is Tethered Cord So Hard to Diagnose?

Because the medical system likes checklists and easy answers, and you, my friend, are neither.

Most neurologists were taught that TCS only affects kids or that a normal conus on MRI rules it out. That’s like saying your house can’t be on fire because you can’t see the flames from the front door.

For EDS patients, Occult Tethered Cord Syndrome is especially slippery:

• The conus medullaris may appear “normal” in position

• Standard MRIs don’t assess cord tension during motion

• Symptoms are misattributed to EDS, POTS, anxiety, or “deconditioning” (a fan favorite within the NHS)

  
  

So How Do You Prove It’s Real?

If your symptoms scream TCS or Chiari, you may need:

• Upright or flexion-extension MRI – captures spinal tension in motion

• Cine MRI – evaluates CSF flow blockages

• Urodynamic studies – documents neurogenic bladder issues in black-and-white

A standard MRI alone won’t cut it. You need imaging that respects gravity—and you.

The Risks of Ignoring It

If left untreated, TCS can cause:

• Permanent nerve damage

• Full loss of bladder/bowel control

• Severe mobility impairment

• Worsening Chiari compression

• Chronic, treatment-resistant pain

And no, it’s not just EDS acting up again.

Treatment: What’s the Plan?

1. Surgical Detethering

• Involves cutting the filum terminale to relieve spinal tension

• Can halt progression and relieve symptoms

• EDS patients may require dural grafts or amniotic membrane grafts to reduce re-tethering risk [3]

• Not without risks—but for many, it’s life-changing

  
  

2. Conservative Management

If surgery isn’t an option:

• Physical therapy (EDS-aware!) – focus on stabilization, not stretching

• Pain management – including nerve modulators like gabapentin

• Bladder support – intermittent catheterization or medication

  
  

Final Thoughts: Don’t Let the System Gaslight You

Tethered Cord Syndrome—especially in the context of EDS and Chiari—is one of the most underdiagnosed neurological conditions in medicine today.

So, if your pain is increasing, your legs are giving out, or your bladder is staging a coup, don’t accept “it’s just EDS” as a diagnosis. Fight for advanced imaging. Find a specialist. Keep pushing.

Because no one else is going to do it for you.

And if you were waiting for a sign to advocate harder? This is it.

SOURCES & LINKS:

1. National Organization for Rare Disorders: Tethered Cord Syndrome

2. The Ehlers-Danlos Society: Chiari and Tethered Cord

3. NIH / NCBI Case Studies: Occult Tethered Cord Syndrome Review (2023)