CCI 101

Understanding Craniocervical Instability

and the Road to Recovery

Christopher J. Centeno, M.D.

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Dedication: To Ed Jacobsen, Ph.D. for teaching me that the neck can cause more than neck and
headache pain.

Copyright 2021

The Centeno-Schultz Clinic

Colorado, USA

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 Table of Contents

➢ Forward and History-Page 1

➢ Chapter 1-What is CCI?-Page 6
➢ Chapter 2-Getting to a Diagnosis-Page 8
➢ Chapter 3- Chapter 3-Upper Cervical Anatomy and Basic CCI Concepts-Page 12
➢ Chapter 4-Imaging-Page 25
➢ Chapter 5-Treatment-Page 33
➢ Chapter 6-Is There Another Way? PICL-Page 38
➢ Chapter 7-Surgery-Page 52
➢ Chapter 8- Other Issues Surrounding CCI-Page 67
➢ Chapter 9-Wrapping It All Up-Page 70

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 Forward and History

Way back when, when I was a young doctor with brown hair (it’s grey now) and more enthusiasm than
common sense, I had a patient who was different. She had headaches, but also dizziness and brain fog.
In looking through the chart, I saw an ENG study by a local audiologist Ed Jacobsen, Ph.D., so I called him
and that began an odyssey that ultimately led to a new way to treat CCI.

The audiologist I met that day told me about patients he was seeing for dizziness and imbalance that his
ENG tests told him had neck issues as the cause. I hadn’t really heard about anything like that, but after
some on-line research (back then you had to have software to search the National Library of medicine), I
saw that he was right. This neck and balance connection went way back to the 1920s when two
physicians (Barre and Lieou), one French and one Chinese, independently described the syndrome. At
the time they thought that this collection of symptoms which included headaches, imbalance, brain fog
and other things was caused by damage to the cervical sympathetic chain (posterior cervical
sympathetic syndrome). However, what Barre and Lieou syndrome are now credited for is figuring out
that headaches and other symptoms can come from the neck.

The next big advance in neck and balance came during NASA research in the 1960s as scientists tried to
figure out what damage to the front neck muscles would do to astronauts exposed to levels of
acceleration never before experienced by humans. They began to cut the sternocleidomastoid muscles
of primates and found that the monkeys lost their balance and would bang into walls.

Then in the 1990s, a new procedure to help neck pain was developed called radiofrequency ablation.
The procedure used a radiofrequency probe placed using x-ray guidance to burn the nerves taking pain
from the neck joints and reduce neck pain. However, when the early doctors began to treat the C2-C3
joint in the neck, some patients got permanently dizzy. While later advancements in the technique
solved this issue, the upper neck was now firmly implicated in balance.

After confirming what Dr. Jacobsen had told me I soon began treating the upper necks of these patients
who had whiplash injuries and also had headaches and dizziness. At first with simple muscle trigger
point injections and then with upper neck facet injections into the C2-C3 neck joints. Many of them got
better. Hence, as more physicians and colleagues learned that this was becoming an area of expertise
for our clinic, they referred more of these patients.

However, in this group of patients, we had some people who never got better. When a local
chiropractor, Evan Katz came to Boulder and began using a DMX (Digital Motion X-ray), we both began
to see that these were those patients who had excessive motion due to damaged upper neck ligaments.
Hence, my interest in craniocervical instability (CCI) was born. While more aggressively treating the
posterior ligaments helped a bit, most remained largely untreatable.

What was available to these patients? First, many were often bounced from specialist to specialist
without answers or any diagnosis. Second, almost all of them would flare up in physical therapy, so
many physicians would blame the lack of progress on that patient’s lack of effort. Finally, the few that
got a diagnosis were just beginning to be offered upper neck fusion, a procedure that in the past would
have only been offered to those with neck fractures or severe life-threatening dislocations of the upper
neck bones.

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Then one day in 2013, I began to play with a model we had of the upper neck bones and ligaments. I
literally had it on my desk and would look at it periodically to see if we could access these ligaments.
While some of my spinal interventionalist colleagues thought it might be possible to access the
ligaments that hold the head on (alar and transverse) from the back, after trying this in a few patients I
concluded that it couldn’t be done safely as the spinal cord was in the way. It then dawned on me that
injections from the front might work as there was a tiny bony tunnel between the C1 and C2 vertebrae.

I then spent months reviewing the anatomy of this area to make sure we wouldn’t injure someone and
going back and forth with experts on this issue. I tried my first patients in 2015 and by 2016 had enough
dialed in that we were starting to see amazing results in these formerly untreatable patients. We added
many procedural improvements as the years went on and the rest as they say, is history.

I’ve written this book to help CCI patients. My goal is to go over everything they need to know about all
of their options. So, I’ll cover anatomy, diagnosis, conservative care, surgery, and the procedure we
developed. Why take the time to do this as a busy physician? Because patients who know more, in my
experience, are the ones that can successfully navigate our medical system to get the best possible
results.

However, please realize that in writing this book, I’ve also tried to “thread the needle” between a
patient book and one with enough detail so that patients can give it to their physicians. Why? There is
still a serious and devastating lack of knowledge on CCI and this adversely impacts patients in all sorts of
ways. Hence, oftentimes patients need a resource that they can hand to their doctors so that the
physician can understand what’s wrong with the patient.

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 Chapter 1-What is CCI?

CCI stands for craniocervical instability. This is the area where the head meets the top of the neck also
called the craniocervical junction or CCJ. Instability means that the head or upper neck bones move
around too much due to loose ligaments that hold the head in place. Patients often complain of
headaches, dizziness, visual problems, and many other problems. We’ll get into that all later.

Patients who are ultimately diagnosed with CCI can have a broad swath of disability. I’ve seen everything
from high-functioning patients who can still exercise with symptoms to those who are completely
disabled an must lie down most of the day or wear a cervical collar to function.

What Exactly Is Instability?

All of your joints are built to move within certain defined directions. The two things that make that
happen are ligaments and muscles. Let’s dive in.

Ligaments make sure that your joints can’t move too much. When they get damaged, the joint moves in
ways in which it wasn’t designed. What does this do? It places excessive forces on the joint and bones
and damages them. In the upper neck, many other structures can get harmed. That includes the facet
joints, nerves, cranial nerves, muscles, etc… As the book progresses, we’ll go into all of this anatomy.

CCI Due to Fractures vs. Ligament Laxity

Many surgeons have been taught more about CCI due to upper neck fractures than ligament laxity.
These are classical “hangman” type fractures where the upper neck bones are no longer able to protect
the spinal cord. This is a surgical emergency as unless something is done to stabilize this area surgically,
turning the head could literally cause a spinal cord injury. This type of CCI is VERY RARE.

The type of CCI covered in this book is caused by damaged ligaments and intact bones. This is much
more common, but much more mysterious to classically trained spine surgeons. Hence, specialists in
this more common type of CCI are few and far between.

The Causes of CCI

Here we can break CCI into two camps: traumatic and congenital. Traumatic is caused by some injury
mechanism like a car crash, a manipulation of the head and neck gone bad, or a blow to the head.
Congenital means that the patient has a condition that would predispose them to CCI. The most
common is EDS, or Ehlers Danlos Syndrome where the patient is born with ligaments that are too
stretchy. In that type of CCI, while there can also be an inciting episode, the symptoms can just begin
without an event.

Traumatic CCI

I’ve heard many different causes of traumatic CCI that my patients through the decades have relayed:

• Car crash where the head was turned, hit something (like the back of a pick-up truck window),
or without head trauma
• Forceful manipulation of the head during a therapeutic manipulation
• Something striking the head
• Falling or inadvertently diving on the head

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 • A history of old head and neck trauma when younger

There are many more, but these are the most common ones I hear from patients.

One issue to bring up is that some patients report having CCI after being involved in what are classified
by insurance companies as low damage crashes. How is this possible?

Cars are designed to deform to absorb force and keep it away from the occupants. However, there are
three phases to absorbing forces. The first is, as you see above, when the car is elastic (not much
damage), since the car is not absorbing force, it gets transferred to the occupant. This is where many
patients get injured. Next, in crashes where the car starts to deform, the forces on the occupant goes
down because now the car is now absorbing the brunt of the force. Finally, in crashes where big damage
happens, you get immense forces on the occupant. My friend and college Michael Freeman, a forensic
epidemiologist recently published an excellent paper that quantified why the insurance company
position that patients can’t be injured in a low damage crash is not supported by crash test data (26).

Congenital CCI

When a patient is born with ligaments that are too stretchy, this condition is known as Ehlers Danlos
Syndrome or EDS. In this condition, the body makes collagen with the wrong mix of components. This is
a spectrum disorder from milder ligament laxity to more severe. As patients with more severe ligament
laxity get older, all of the extra wear and tear and accumulated injuries over time begin to cause pain
and disability. One such area where some patients’ manifest problems is the cranio-cervical junction.

Because of the overly stretchy ligaments that hold the head on, EDS patients have a lower threshold for
injury here. So, while all of the above listed traumas can cause CCI, lesser things like banging the head
on a car while getting out can cause it as well. Other times there is no specific trauma that these
patients can point to, the CCI symptoms just begin to manifest.

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 Chapter 2-Getting to a Diagnosis

The diagnostic journey for CCI is often long and arduous. Few patients get to the right specialists quickly
and get a concrete diagnosis within months of the onset of symptoms. In fact, many end up going down
unfruitful and costly rabbit holes as the medical care system focuses on only one symptom and works
that up. So, for example, here are common unfruitful journeys for CCI patients based on their
symptoms:

• Headaches that are caused by the CCI end up getting worked up and treated by a neurologist as
migraines which are a different animal involving blood vessels in the brain.
• Dizziness/Imbalance/Lightheadedness caused by the bad position sense information coming in
from the damaged upper neck is often worked up as a vestibular problem by an ENT doctor with
tests like a VNG.
• Rapid heart rate caused by Vagus nerve irritation is often worked up by a cardiologist with a
Holter or other wearable monitor to rule out supraventricular tachycardia.
• Brain fog or problems concentrating caused by the weird inputs coming in from the damaged
neck is often worked up by a neurologist as a brain injury, stroke, or a seizure disorder.

We use many different things to get to a CCI diagnosis. I’ll cover these here:

• History of Onset-see above in causes of CCI section

• Symptoms
• Response to treatment
• Imaging

The Symptoms of CCI

There has never been a formal published survey of CCI symptoms. Hence, I complied the symptoms of
80 patients who we confirmed as having CCI and who were about to undergo a PICL procedure. This was
the word cloud of what they reported:

The bigger the word, the more often it was reported. Based on this data and decades of experience
treating these patients, this how symptoms break down.

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These are classical symptoms that almost all CCI patients would have like:

• Headaches
• Upper neck pain
• Dizziness/Imbalance/lightheadedness
• Brain fog
• Clicking/popping in the neck (crepitus)

Then there are a collection of symptoms shared by most CCI but not all patients:

• Ringing in ears (tinnitus)

• Shoulder pain
• Rapid heart rate (tachycardia)-POTS and/or increased anxiety
• TMJ
• Memory Loss
• Wandering pain or numbness/tingling in various areas of the body
• Gastric disturbances
• Clicking or popping in the upper neck with movement
• Fatigue
• Nausea

Then there are a collection of symptoms shared by some CCI patients:

• Facial pain
• Tongue symptoms
• Loss of consciousness episodes
• Sweating

Hence, diagnosing CCI by symptoms alone can be very difficult.

A Word of Advice on Symptoms

As you can see, CCI patients can have a large number of symptoms. During this book I’ll go through
various rabbit holes of misdiagnosis to avoid. One tip here is that patients these days often find like
patients on social media and begin self-diagnosing. When they see a doctor, they begin using more
diagnostic terms like “atypical tachycardia” rather than just saying that when my neck is bad my heart
races. This can often confuse the diagnosis for the doctor. Another thing they do is to begin listing every
possible symptom they believe is related to their CCI rather than just the main symptoms they know are
for sure related. This also can make the doctor confused. So when you talk to a doctor about your
problem, keep it focused on what you experience and keep it streamlined and simple.

Response to Treatment

CCI patients have a few classical responses to treatment. One is that active physical therapy focused on
strengthening tends to make them worse. Why? The upper neck ligaments are just too loose. Hence,
when they try to strengthen the neck muscles, the upper neck moves around too much and irritates
joints, nerves, tendons, etc…

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On the other hand, many patients find good temporary relief from upper cervical chiropractic
adjustments (NUCCA or AO chiropractic). They have improved symptoms that can last for hours to
weeks. This can also be highly dependent on finding the right chiropractic expert. Meaning not all upper
cervical chiropractors have success in all CCI patients and some patients find only a handful of expert
chiros who can handle their case.

Some quick definitions here:

• NUCCA (National Upper Cervical Chiropractic Association) Chiropractic-focused on gentle

manual adjustments of the upper neck bones that are guided through precise x-ray
measurements.
• AO (Atlas Orthogonal) Chiropractic-Very similar to NUCCA, but the x-rays are used to program a
vibration device which performs the adjustment.
• Blair Chiropractic-Also focused on gentle manual adjustments of the upper neck as well as
diagnostic imaging where angles are calculated to drive what needs to be adjusted. There is also
a leg length test that’s commonly used to see how the upper neck is impacting the rest of the
body, all the way down where the feet meet the ground.

Other types of treatments where we look for a response are injections. For example, the upper neck
instability can cause the upper neck facet joints to get beat up and hurt. Hence, in many CCI patients,
injecting the C0-C3 facet joints will help their pain. Or for example, occipital nerve blocks which can help
headache pain. The focus here is to determine if there are structures in the upper neck causing pain as
that helps the doctor pinpoint the area of the problem.

Imaging

There are many ways to try to image the problem of CCI. However, before we get into that in chapter 3,
we need to learn a little anatomy.

Examples of How These Pieces Come Together

You now have the diagnostic categories, which to review are:

• History of Onset-see above in causes of CCI section

• Symptoms
• Response to treatment
• Imaging

So now let’s review some examples. While we still have some things to learn as we move forward, this is
just to show you how a CCI diagnosis would be made.

Example 1-A patient was in a car crash where they were rear ended while their head was turned. Now
let’s plus some things in:

• History of Onset-The problem began at the car crash, which was sufficient to cause this kind of
injury.
• Symptoms-Headache, dizziness, brain fog, and upper neck pain. Hence the symptoms match CCI.

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 • Response to treatment-Physical therapy makes the symptoms worse. An upper cervical facet
injection decreased headache pain for 3 weeks. Both of these things fit the profile of how a CCI
patient would respond to treatment.
• Imaging-The patient’s DMX study shows 6 mm of overhang which fits with instability.

All of this shows that we’re 4/4 in determining f this patient has CCI.

Example 2-A patient who has EDS bangs their head on a the roof of a cab while getting out. Here’s the
analysis:

• History of Onset-The patient has EDS, which means that they have a major risk factor for CCI.
The new problems began getting out of the car, which could injure the upper neck ligaments in
an EDS patient who already has stretchy ligaments.
• Symptoms-Headache, neck pain, rapid heart rate, GI problems, and brain fog. Hence, the
symptoms match.
• Response to treatment-Upper cervical chiropractic (NUCCA) helps the symptoms for days at a
time, but then they return. Home strengthening makes the patient worse. Both of these
responses to treatment match what a typical CCI patient would report.
• Imaging-The patient’s flexion-extension MRI shows that their Grabb-Oakes measurement
increase from 9 mm to 12 mm with flexion. This matches CCI.

Again, another 4/4 match.

Example 3-Now we have a patient who has no history of trauma whose symptoms just began without
any warning:

• History of Onset-No trauma, no risk factors for CCI.

• Symptoms-Brain fog, rapid heart rate, GI problems, and jaw pain. This may or may not be a
match.
• Response to treatment-Upper cervical chiropractic (NUCCA) doesn’t help the symptoms.
Strengthening is not a problem, it also doesn’t help. This doesn’t match CCI.
• Imaging-There are no concrete measurements that match CCI other than one measurement
which is borderline.

So we have a 1.5/4 here, so this is less likely to be CCI.

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 Chapter 3-Upper Cervical Anatomy and Basic CCI Concepts

While this next chapter may seem tedious, it’s essential for anyone with CCI to understand how the
basic anatomy works. I’ve purposefully made this chapter a bit more complex that the average patient
may want to know, to again, thread that needle between a book that can help patients versus one that’s
detailed enough to also help their doctors understand CCI. If it gets too intense for you as a patient, then
read as much as you want to know and skip forward to the next chapter.

Bones

The head on the neck is like a bowling ball on a stick, meaning it’s inherently unstable. Hence, we have
two things that evolved to hold it on. Tough ligaments and muscles. In addition, the upper neck bones
are specially adapted to help this effort as well. Let us begin there.

The neck bones are numbered. C0 is the skull and C1 is called the atlas while C2 is called the axis. Both
are ring shaped bones that I have simplified above. The peg of C2 fits into C1 and the skull rests upon C1.
There are also joints connecting the skull to C1 and C1 and C2 (not shown here). These are called facet
joints.

Here’s what the bones really look like:

Now back to our simplified drawings. The peg in C2 (Dens) is there so the head and the atlas can rotate
around it:

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Ligaments

How would we keep these two bones together? That’s where ligaments start factoring in. The big one
here is the transverse ligament (aka the transverse band of the cruciform ligament):

If this transverse ligament is loose, it allows too much movement of C1 on C2 when the head is flexed
forward.

However, how do we keep these two bones firmly attached to the skull? The alar ligament does that job:

This ligament goes from the dens to the skull base, kind of like arms reaching up from the dens to the
skull. Hence, it binds C2 to the skull and indirectly holds C1 in place as well by sandwiching it between C2
and the skull. When this ligament is loose or damaged, it can allow C1 to rotate on C2 because it no
longer applies that sandwich force to help keep C1 in place. In addition, when damaged, it can also allow

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the skull and C1 to slide sideways when the head is side bent. We measure this as C1-C2 “over-hang” on
a DMX study (more on that later).

It should also be note that when viewed above, one band of the alar ligament travels toward the front
as shown here. Remember that this ligament goes the base of the skull. Hence, then the head and neck
bend forward, the weight of the head is partially supported by this ligament.

There’s another part of the alar ligament which extends downward between the alar ligament and C2
which is called the accessory ligament as shown below (in this picture I made the C1 and C2 bones
transparent (except for the dens)):

If we look from the side at the whole neck, we see that there are lots of ligaments in the back or
posterior part:

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These are the nuchal, VDL (Vertebro-dural Ligament), supraspinous, and interspinous. They all help
prevent the vertebrae from moving too far forward and are very active when you look down. The VDL is
special because it connects directly to the dura, which is the covering of the brain and spinal cord.
Hence, it is believed that damage to this ligament may cause headaches.

Finally, there are deeper cervical ligaments to consider, which are shown below:

These include from front to back:

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 • SAAOL-Superficial Anterior Atlantooccipital Ligament (this is the extension of the anterior
longitudinal ligament upwards. This connects the front the cervical vertebral column with the
skull.
• AAOM-Anterior Atlantooccipital membrane-This connects C1 to the skull.
• Apical-This extends from the dens of C2 to the skull.
• Cruciate-This is a cross shaped ligament that has as its horizontal part the transverse ligament
discussed above, but has parts that go up and down as well.
• Tectorial-This is the upward extension of the posterior longitudinal ligament that connect the
back of the vertebral column to the skull.
• PAOM-This is the upward extension of the ligamentum flavum that connects the back part of
the spinal canal to the skull.

The front ligaments help to stabilize your head on your neck when you look up, the middle ones help to
hold your head on, and the back ligaments keep everything stable when you look down.

Facet Joints

Your upper neck (and the rest of it as well), has finger size joints called facets. Their purpose is to help
your neck move normally. When the ligaments are damaged, the joints can sustain too much wear and
tear and begin to get injured.

This is how those joints really look:

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Here’s a simplified diagram:

These upper neck joints are

• C0-C1-This joint allows the nodding movement of the head. If injured or painful, this joint can
cause pain in the back of the head.
• C1-C2-This joint allows for about half of the rotation of your head. This joint can causes pain to
the back of your upper neck where your head and neck meet.
• C2-C3-This joint allows for a little rotation of the head and a little forward bending of the neck. It
causes pain in the upper neck and the back of the head.

These are the referred pain patterns for these joints (24):

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The red area is where pain from the C0-C1 joints is felt. The yellow/green is where pain from the C1-C2
joint localizes. Finally, the blue area is where pain coming from the C2-C3 joint travels.

Hence, these upper neck joints can cause headache. In addition, all of them also provide information to
brain about head, neck, and eye position. This happens through tiny sensors present in the joint
capsules called proprioceptors. Hence, if these joints are injured they can cause dizziness, imbalance, or
visual problems.

C1-C2 and Inherent Instability

The C1-C2 facet joint is responsible for 50% of the total neck rotation. Like all facet joints, it has cartilage
and a joint capsule. What makes this joint very unique is the shape of its internal joint surfaces:

Above is an MRI side view of the C1-C2 facet joint. Its surfaces are shown to the right. Note that they are
both convex, which is unusual in the body. Why? Usually, the joint surfaces fit together like a puzzle
piece with one surface being convex and the other being concave. That makes them inherently stable.
However, the C1-C2 joint is inherently unstable.

What does that mean? If we have a convex/concave joint like the one below and there are no muscles
or ligaments acting on it, it’s not going anywhere. Meaning the joint surfaces stay together. The
opposite is true if the joint surfaces are convex sitting on top of convex:

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So the C1-C2 joint is inherently unstable. This explains why we have an entire field of chiropractic care
devoted to this part of the neck! However, the C0-C1 joint above it is that convex/concave type, so it is
inherently more stable.

In the section above, we discussed how the atlas (C1) was sandwiched between the skull and C2 and
held in just the right amount of tension by the alar ligaments. These ligaments help to maintain the
concave joint surfaces in approximation as you move your neck. However, if this ligament is damaged,
these joint surfaces cause the bones to rotate (25).

Why Inherent C1-C2 Instability Can Wreak Havoc with Patients

Given that the C1-C2 joint is inherently unstable, if it doesn’t have ligaments and muscles to hold it in
place, it tends to move out of place. This results in the C1 bone rotating on the C2. In fact, this is why an
entire type of chiropractic (NUCCA) is devoted to trying to get C1-C2 back in place. So if you have C1-C2
instability due to damaged ligaments (Craniocervical Instability or CCI) it’s easy to see why this joint
would not be “in place”.

What happens when this joint gets out of whack? The capsule is rich in position sensors that help
maintain the position of the body relative to the neck, so your body will feel out of whack as well. In
addition, the joint, when painful, refers its pain to the head. So you can also have headaches and other
symptoms (see below).

In addition, it’s possible for this joint to be out of whack without having damaged ligaments. This is
where a NUCCA chiropractor or an experienced manual physical therapist can make a big difference.
Symptoms here include headaches, dizziness/imbalance, visual disturbances, brain fog, or spasm, or
pain at the back of the head.

Muscles

The muscles in this area all control the fine movements of bowling ball in the stick:

The RCP (Rectus Capitis Posterior) major and minor connect the C2 and C1 muscles to the skull and can
help you look up. The RCP minor is unique in that it connects directly to the covering of the brain and
spinal cord (dura), so it’s implicated in headaches. The obliquus capitis muscles connect C1 to C1 and the
skull and help with bending your head sideways on your neck.

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The deep muscles in the front of the neck look like this:

The longus capitis and colli live just in front of the spinal column and help to flex the neck and maintain
the curve.

As we move outward, we find the strap muscles in front including the SCM:

The SCM is a critical muscle up front as it is the prime mover for rotating the head. This is important for
CCI as C1-C2 is a critical joint that’s responsible for 50% of your ability to rotate.

There are a slew of other muscles like the upper trapezius, levator scapula, and paraspinals muscles that
usually get in on the act in CCI, but we’ll discuss those later.

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Nerves

Pissed off upper neck nerves tend to cause headaches. The nerves that are critical for CCI live in both the
back and front of the neck. Here are the ones in the back of the neck:

Realize that since these upper neck muscles help to hold and stabilize the head, when they spasm and
go into overdrive to help stabile the head on the neck, they can irritate these nerves can cause
headache. There are also nerves that live and exit in front of and behind the SCM:

• TON-Third Occipital Nerve

• GON-Greater Occipital Nerve
• LON-Lesser Occipital Nerve

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The TCN is the transverse cervical nerve (also called the superficial cervical plexus) and the TA is the
temporal-auricular nerve (also called auriculo-temporal nerve). The TCN nerves live behind the SCM
muscles, so they can become irritated as those muscles get tight in C1-C2 instability. They can cause
headaches, as well as jaw, side of head and face pain. Note that the TA nerve also travels right behind
the jaw, so any extra motion or overload of the jaw can cause it to be irritated and this can lead to
headaches at the side of the head. you

Cranial Nerves

The cranial nerves come out the skull and head south, as shown below:

Given that the skull moves too much on the upper neck and these nerves also are close to the atlas bone
which can move around too much as well, these nerves can get irritated. The more common cranial
nerves are:

• CN10-Vagus Nerve-This is literally the brakes on the heart, lungs, and digestive tract. If the nerve
gets irritated the patient can get rapid heart rate, anxiety, and digestive problems.
• CN11-Spinal Accessory Nerve-Controls the upper trapezius and sternocleidomastoid (SCM)
muscles. When it gets irritated these muscles can go into spasm.
• CN12-Cranial Nerve 12-Hypoglossal-Controls the tongue muscles. This can cause spasm in the
tongue area.
• CN V and VII-These both go to the face.

Foramen Magnum

The base of the skull has a big hole in it and the name for that in Latin is “foramen magnum”, which is
what we doctors call it. Realize that this area has quite a bit of room for the spinal cord, which is great as
that means that quite a bit of motion can happen here before there is a spinal cord injury:

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The black arrows one either side of the yellow spinal cord show that room, but also note the yellow line
which is the covering of the spinal cord and brain (the dura) which is closely adhered to ligaments. So if
that area gets irritated due to CCI (too much skull movement the dark blue arrows), then while there is
no spinal cord injury, there can be all sorts of referred pain in all sorts of different places in the body. In
addition, in many CCI patients the dens (here marked C2) can move backwards due to a loose transverse
ligament, which can cause pressure on the dura in the front and possibly the spinal cord (also called
cervical medullary syndrome).

Cervical Medullary Syndrome

As discussed above, when there’s instability, the skull moves too much against the spine and as a result,
the dens can move too far backward (yellow arrowhead) and irritate the top part of the spinal cord or
brainstem (medulla). When that happens, as shown above, that’s called a cervical medullary syndrome.

Also realize that the white stuff you see above on either side of the spinal cord is cerebrospinal fluid
(CSF). This circulates around the brain and acts as the waste removal system for chemicals produced in
the brain. This circulates all the way down to the neck, upper back, and lower back. The flow of that fluid

23
can get obstructed by a cervical medullary syndrome. We’ll go into more detail later on how that’s
measured using specialized MRI.

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 Chapter 4-Imaging

Imaging in medicine means that some technology is used to visualize the deep structures of the body.
This is always an interesting topic with CCI patients. What I mean is that this is such a huge topic that it’s
tough to cover all of it. In the meantime, many CCI patients see surgeons who use some of the metrics
I’ll cover here to determine if they’re surgical candidates, so I want to make sure to cover those
measurements. However, to make this part of the blook less cluttered, I’ll give brief descriptions of the
measurements and then point to a YouTube video or reference that goes into more depth.

Imaging 101

X-ray Exposure

It’s important to understand a bit about common types of imaging discussed here and x-ray exposure.
For example, a simple neck x-ray has about the same about of amount of x-ray exposure as a chest x-ray,
which is about the same amount natural background radiation that most people get in 10 days of
normal living. However, a CT scan exposes you to about 8 years of background radiation exposure! (27)
Hence, we generally DO NOT recommend CT scans to diagnose CCI.

MRI Imaging Pitfalls

Before we get into the topic of imaging and MRIs, it’s critical to point out that a routine cervical MRI has
limitations in CCI patients. Let’s explore that a bit.

An MRI (Magnetic Resonance Imaging) machine is a big magnet. It applies a huge magnetic field which
aligns the small molecules in your body and then removes that magnetic field and lets these flip back to
their normal state. When that happens, these molecules produce a radio signal that is reconstructed by
the computer into an image.

The Good News

Static measurements like Grabb-Oakes and the CXA can be measured on most routine cervical MRIs.
This may help make the diagnosis of CCI. The problem is that, as you’ll see below, movement like flexing
your neck forward and backward usually enhances the information gathered. That kind of motion can
only be picked up on a specialized “Stand-up” MRI unit and not on the standard “lie face up in a tube”
type. More on that below.

The Bad News

What an MRI can detect depends on the specific coil used. This is something placed around the body
part that acts an antenna to pick up the signals coming out of your body that are generated by the big
magnet. There is a different coil used for each body part.

The usual cervical MRI only images from C2-T1 because that’s what the neck coil is optimized to image.
However, the pathology in a CCI patient is above C2. In fact, to get good MRI pictures above C2, a head
coil is often needed. Hence, having your doctor write a script for a routine cervical MRI may not be
helpful in getting to a diagnosis.

Imaging Resolution and Strength

25
It’s also critical to understand that imaging strength is directly related to the resolution or quality of an
image. Hence, whatever type of static MRI you get, you should make sure that you get the best possible
image. In addition, getting imaged on a more or less powerful MRI machine is agnostic to your insurance
benefits. Meaning, getting imaged on a 2 million dollar 3.0 Tesla MRI is covered by insurance just as
much as a getting imaged on an old-school 0.3T open MRI that someone would pay you to haul away for
parts.

MRI imaging strength is measured by a unit called a Tesla (T). That’s for early 20th century electrical
engineering genius Nicolai Tesla and NOT the car company. One Tesla is the magnetic field strength of
the earth. Hence, 0.3T means about 1/3 that amount.

Here is how they rate out:

• Worst quality images-0.3-0.6T

• Good quality images-1.5T
• Best quality images-3.0T

You can find this out by asking the center the “field strength” of the machine. They should report it as
above.

Realize that any open or Stand-up MRI has a lower field strength than most high-quality lying down
MRIs. This is a regrettable trade off. For example, in order to be able to image someone in a machine
that allows for motion, you need more room, and hence the magnetic field will have lower strength to
make that work. However, more information about an instability condition like CCI can be gathered
when patients move. Hence while field strength may be important in a static MRI, we’re never going to
see a Stand-up MRI with 3.0T.

The “Best” Type of Imaging for CCI?

In our clinic, we like using DMX (discussed below), which allows for movement that approximates
reality. Our second choice would be movement-based MRI or a rotational CT scan. Static MRI of the
upper neck can sometimes be helpful and there are times that we can see abnormal measurements on a
routine neck MRI.

However, this preference for DMX is not universal. For example, many neurosurgeons prefer routine
static MRIs where some of the measurements shown below can be measured.

A Gold Standard?

While it’s always great if any diagnosis has a gold standard test to determine if you have or don’t have
the disease, that doesn’t exist in CCI. Hence, different physicians all use different tests to get to a
diagnosis. While that makes it harder for patients, who understandably want certainty, it’s reality.

Types of Imaging

Imaging can be broken down into two main categories: static and dynamic. Static means that the patient
doesn’t move during the imaging. This can be a problem for diagnosing instability as that, by definition,
involves movement. To solve that issue, more recently applied imaging techniques use movement and
this is called dynamic imaging.

26
Before I jump into explanations of all of the different types of measurements and imaging used, realize
that this chapter is yet another example of “threading the needle” between a book that can be read by
patients, but that has enough detail to be handed to a physician that needs to be educated. Hence, it’s
OK if you jump through parts of this chapter that are in too much detail for you.

Static Measurements

Grabb-Oakes

This is the distance between a line drawn from the front of the spinal canal (basion) and the back
inferior corner of the C2 bone and the back of the dens. Given that the C2 bone (axis) is held against the
C1 bone (atlas) by the transverse ligament, a high Grabb-Oakes measurement can be due to a lax
transverse ligament. An abnormal measurement is often considered to be 9 mm or more (2,5). With the
advent of flexion-extension MRI, it’s easier to measure the G-O measurement when the patient moves,
which provides more information. See this video for more on the G-O measurement
[https://youtu.be/4f-Yi9fuKD0].

CXA (Clivo-Axial Angle)

This is the angle between the clivus (the inside front area on the bottom of the skull) and the back of the
dens (C2). The problem being measured here is the skull is falling forward on the upper neck which can
cause irritation of the front of the brainstem and upper spinal cord. This movement is controlled by
strong ligaments in the back of the neck such as the nuchal, supraspinous, and interspinous ligaments.
It’s also controlled by the posterior atlantoaxial membrane (PAOM, a ligament at the back of the spinal
canal) and to a lesser extent the transverse ligament. Abnormal is a bit different when reported by
different authors. Less than 150 degrees (4) was originally reported with others stating that normal is
between 145° to 160° in the neutral position (3). The differences in opinion are likely due to the fact that
things like kyphosis (forward head posture) and male/female sex can change the angle.

27
Power’s Ratio

This is another measurement that determines if the head has moved forward on the upper neck. This
one is more complex, requiring two lines (one between the basion and the back of the spinal canal at C1
and another between the opisthion and the posterior aspect the front of the atlas) and then some
division is applied. If the calculated measurement is less than 1, the ratio is normal, if it’s >1 then it’s
abnormal (2). The Powers Ratio is measuring whether the head is aligned properly on the upper neck
bones. For example, if the number if greater than 1, the head is too far forward on the spine and
multiple ligaments might be injured.

See my video here for more information [https://youtu.be/mQw7Sx5QA2c].

BAI (Basion-Axial Interval)

This is the distance between the basion (front of the skull) to the back of the dens of C2 (axis). The
problem here is that CT scan studies have shown the usual normal of 12mm to be unreliable with

28
normal ranging from 9-26 mm. This can also be measured in flexion and extension where >4mm
difference between the two positions is considered abnormal enough to warrant surgery. The ligament
most responsible for keeping this measurement in check is the transverse ligament. This is again a
measurement that can determine if the head if too far forward on the spine.

BDI (Basion-Dens interval)

This measurement focuses on vertical instability. This would be damage to the ligaments preventing
vertical translation of the head like the PAOM, tectorial membrane, AAOM, apical, and SAAOL. The
normal value is less than 12 mm on x-rays and 8.5 mm on CT scan (2). Like the BAI, it can also be
measured in flexion and extension. The BDI change can also be measured as traction is applied to the
head.

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Chamberlain Line

This is a line drawn from the back of the hard palate of the skull to the back of the hole in the skull
(opisthion). If the dens of C2 is more than 3mm above this line, this indicates cranial settling or that the
skull is too low on the cervical spine. This is also called basilar invagination. Both of these conditions are
more extreme than are usually seen in most CCI patients. Meaning in in purest neurosurgical sense, both
cranial settling and basilar invagination have the dens touching the brain stem.

ADI (Atlantodental Interval)

This is the distance between the front of the C1 (atlas) and the dens of C2. Normal is 2mm (2). If this is
greater than 2mm, the transverse ligament may be injured. Like many of these measurements, the ADI
can be measured in flexion as well to see if it increases, as that stresses the transverse ligament.

30
Upper Cervical MRI

Note that a routine neck MRI rarely shows these upper cervical structures. Much of that has to do with
the wrong type of coil used to collect the information which is designed to an image from the neck and
not the upper neck/head. This means that ligaments like the alar, transverse, and others just don’t show
up on the usual cervical MRI.

However, if a specialized MRI is taken, the upper neck ligaments can be imaged. This requires a head coil
and other specialized image sequences. Once we have those images, you can look at how the ligaments
appear and grade them based on a scale published by Krakenes (6). While we look at this information,
the problem is that it’s not showing whether those ligaments work functionality to stabilize the head on
the neck. That would require movement-based imaging. If you want to learn more, see my video
[https://youtu.be/ZQaztACMzbk].

CSF Flow Imaging

One of the more promising new technologies out there is using a specialized MRI to measure the flow of
cerebral spinal fluid though the upper neck. This is critical because many problems that surround CCI can
restrict the normal flow of the brain’s fluid through this area. The world’s expert in this area is Scott
Rosa, DC of upstate New York. He’s been using this technology in CCI patients for years and can show
improvements of CSF flow through the upper neck before and after specialized chiropractic adjustments
(see http://rosaclinic.com/)

Dynamic Imaging

Flexion-Extension X-rays

These are x-rays where the patient is asked to look down and then an image is taken and then they are
asked to bend their neck back and look up and another picture is taken. The goal is to see if the neck
vertebrae move too much against one another, which they can only do if the ligaments are damaged.
For CCI, the one measurement than can be looked at here, is the ADI when the neck is in flexion. In
addition, many CCI patients have also damaged other neck ligaments in the lower neck that can be
evaluated here.

One of the biggest issues with this type of imaging is that many radiology techs don’t get enough
movement out of the patient to allow proper measurements to made. For these films to be diagnostic,
the patient needs to flex and extend as far as possible, despite the instructions from the tech taking the
picture. Rad techs often tell people to move minimally because it’s easier for the tech, but make sure
you’re a rebel here and get as much motion as possible!

Upright MRI

MRI of the neck is usually performed with the patient lying face up in a tube (supine). Upright MRI
allows a patient to be imaged in a weight bearing position. This places more natural stress on the upper
neck and other ligaments, so it’s possible that measurements that weren’t indicative of CCI lying face up
in the usual MRI scanner could be become positive when imaging the patient this way. Having said that,
normal values obtained while imaging people supine may not apply to upright MRI.

31
Upright MRI also allows for motion. Hence, the patient can do things like bend or extend the neck. This
can give valuable information about whether the ligaments perform normally or allow too much motion.
To learn more, see my video here [https://youtu.be/rpHv0AalJ4Y].

Functional CT (C1-C2 Rotatory CT)

This is a specialized CT scan where the patient rotates their head and the movement between
the skull, C1, and C2 is measured (7). Instability is indicated by rotation of the skull on the atlas
of more than 7 degrees, and of the C1/C2 joint of more than 54 degrees.

DMX (Digital Motion X-ray)

DMX uses a fluoroscope which is real time x-ray imaging and captures video of how the upper neck
bones move. The technology is the only one that allows for natural movement from the patient as all
others are contrived and artificial in some way. DMX allows a patient to move in one direction at various
velocities and accelerations. The patient then must quickly stop and go another direction. This is very
different than a neck flexion-extension x-ray where the patient only moves in one direction maximally
and then a picture is taken.

The measurements that are important for CCI patients here are C1-C2 overhang and ADI. C1-C2
overhang means that the patient bends their head to the side and the doctor looks to see if C1 slips
sideways against C2. If that happens beyond 3mm with also a difference in the distance between the
dens and the atlas, then the alar ligament on the same side may be injured. The ADI can also be
measured. To learn more, see my videos [https://youtu.be/nPBW4Bk-8l4 and
https://youtu.be/6MRnZw2BpV8].

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 Chapter 5-Treatment

Understanding the Ladder of Invasiveness

How do physicians look at medical care for their own families? They use “the ladder”. What’s that?

If you want to keep patients as safe as possible, you only expose them to the least risk needed to get the
job done. For CCI, that system creates a ladder that looks like this:

You’ll notice at the bottom I have listed exercises. Things like physical therapy and alternative healing
techniques like craniosacral would be in this category.

Next up would be manipulation of the upper neck. These are things like NUCCA or AO chiropractic.
These are slightly more invasive than physical therapy.

Then we get into posterior injections (from the back), which could be prolotherapy of the ligaments in
the back, facet injections, epidurals, etc. This is more invasive than manipulation.

Next up are anterior (from the front) injections which includes the PICL procedure. This means injecting
the deep ligaments that hold the head on. Since this is a newer procedure where fewer have been done
than posterior injections, it’s automatically considered higher risk than more commonly performed
posterior injections.

Finally, at the top of the list are spine surgeries like detethering, decompression, and fusion. For
example, a surgical detethering procedure would be more invasive than PICL since it involves open

33
surgery and destroys a part of the spine. Even more invasive would be fusion which is where screws,
rods, or plates are placed to make sure parts of the spine or a joint can never move again.

So how can you use the ladder like I would to protect my wife and kids? Anything lower on the ladder
that applies to that injury or problem that may work needs to be tried first before moving up the ladder.
That way you don’t get exposed to more risk than you need.

Conservative Care

Conservative care for CCI breaks into a couple of common areas:

• Bracing
• Physical therapy
• Chiropractic
• Curve restoration
• Cranio-sacral

Bracing

Neck bracing is an interesting topic in the CCI community. On the one hand, you have patients that find
the right neck brace and swear by it. On the other hand, you have patients who can’t find a brace that
will keep their head/neck in the position where it’s happy with less pain. For those who like braces, this
is a two-edged sword. Why? Because stability requires normal ligaments and muscles. Hence, using a
neck brace for an extended period of time can make the neck muscles weaker, which can make the
instability worse. So if you use a brace, use it only for short periods. For example, some patients will only
use one when travelling or exercising. Some need it more often or they can’t function. Just remember,
like all CCI treatments there are advantages and disadvantages.

Physical Therapy

One of the things that’s almost diagnostic for CCI is that patients do poorly with active physical therapy
that focuses on strengthening. This response can change if the patient has had their upper neck
stabilized through the PICL or a fusion procedure. At that point, especially with the first two treatments,
exercises are encouraged to slowly help the patient build back the upper neck muscles that stabilize the
head on the neck. Of mote, if the patient is fused, normally some muscles are destroyed by the
procedure. More on this later.

However, there are physical therapists with specialized manual knowledge who can help CCI patients.
Meaning that these therapists have years of additional training, so they represent less than 1 in 100 of
all PTs. A helpful guide to finding an upper cervical expert is finding a therapist who knows the upper
neck. Here are some resources:

• IPA Physical Therapy [https://instituteofphysicalart.com/]

• Ola Gimsby Manual Therapy [https://www.olagrimsby.com/]

Other types of PT to consider that don’t focus on the upper neck, but do focus extensively on postural
correction that may help the upper neck include:

• Feldernkrais [https://feldenkrais.com/]

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 • Egoscue [https://www.egoscue.com/]

Chiropractic

While physical therapists have few experts who know how to treat this specialized area of the body,
that’s not true for chiropractors. In particular, there is NUCCA [https://nucca.org/], which is a national
upper cervical chiropractic association that qualifies and trains these providers. Many of my patients
swear by NUCCA chiropractors who are able to help their symptoms. Usually, if they find their way to
me, they will find one who can get temporary relief from hours to days to weeks, but then things come
out of place. On the other hand, for other patients, NUCCA is not helpful.

Another type of upper neck chiropractic is Atlas Orthogonal (AO). This is very similar to NUCCA, but
instead of a hands-on manual adjustment, a specific low force instrument is used to precisely move the
atlas back into place.

How do both of these differ from traditional chiropractic? Many chiropractors will perform “long lever
arm” neck manipulations where they suddenly twist the skull. NUCCA instead uses a precise, non-
invasive spinal adjusting technique. This uses precise and objective x-ray views of the head and neck,
mathematical measurement and analysis to plan the specific low force adjustments used by these
providers.

Curve Restoration

Your neck is born with a natural shallow c-shaped curve. This distributes the forces from your head to
the discs in the front of the neck and the facet joints in the back. In our modern society, looking down at
computers and phones or neck trauma can cause the neck can straighten or develop a reverse curve.
That puts too much force on the discs in front and can cause too much force on the upper neck
ligaments as well. Hence, we’ve seen several CCI patients get improvements with curve restoration.
There is an online credentialing organization where providers can be found [www.idealspine.com]

Curve restoration involves forward traction to increase the curve by pulling the neck forward (not to be
confused with axial traction that pulls your head upwards). Specialized machines are used to do this and
at home devices are also available. It should be noted that only some CCI patients find this helpful
(usually fewer than find NUCCA or AO to be helpful).

Injections

This is a big topic. Before I get too far into it, it’s good to spend a few minutes on how different injection
procedures stack based on complexity:

35
Why? We often see patients who get confused that Level 1 injections as shown have something to do
with Level 5 injections. For example, blindly injecting prolotherapy solution into the back of the neck has
little to do with using endoscopy and fluoroscopy to inject stem cells into the alar and transverse
ligaments (PICL). To use another analogy, the skill level needed to do the level 1 injection can be taught
in a few hours and the skill level needed to perform a level 5 procedure would require many years to
master.

For CCI patients, the different skill levels break down as follows:

Level 1-Posterior prolotherapy. This is injecting the easy to reach ligaments usually with no imaging
guidance (blind or palpation guided). Sometimes the doctor will use a fluoroscope, but not in the same
way a more experienced and highly trained doctor performing higher level injections would use that
machine. The Level 1 doctor is just using that fluoroscope to make sure they have hit certain bone
landmarks, whereas the higher-level physician with more training is using that machine to make sure
that they are in certain joints by injecting radiographic contract. I could teach the first technique in a few
hours, but the second takes months to years to master.

This is not to say that in the right patients with general neck instability, posterior injections are
worthless. They can be very helpful. However, the vast majority of CCI patients that try posterior
injections don’t get relief and functional benefit. This is a concern as MANY CCI patients spend
thousands to tens of thousands of dollars on posterior injections with prolotherapy, platelet-rich
plasma, or bone marrow stem cells that ultimately don’t work. This is despite the promises of a few
practitioners that these procedures will be highly effective.

Why won’t posterior injections help most CCI patients? Because the ligaments that need the most
attention can’t be reached via this approach.

Level 2-Lower neck facet or epidural injections. This is using x-ray or ultrasound guidance to inject the
C2-C7 facets or spinal nerve levels. These procedures can usually be performed by any competent
interventional pain physician.

Level 3-This is a bit more intense and for CCI patients, this might include injecting the C2-C7 neck discs if
needed.

36
Level 4-For CCI patients, a major issue is finding a physician who has the rare experience of injecting the
upper neck facet joints (C0-C2). Why? These are much more technically demanding and thus more rarely
preformed.

There are only a handful of US physicians who have injected these joints more than a few dozen times.
Since repetition in medicine breeds competency, if you’ve only every done something a dozen or two
times, you’re not an expert. Hence, these are physicians who have injected these joints at least 100
times or more. For comparison, our clinic physicians have injected them much more than 1,000 times.

Level 5-Finally, now we’re at the level of a physician who would be capable of performing the PICL
procedure which will be discussed later. This would only be a handful of experienced interventional
spine physicians who would also be willing to take the time to learn how to treat this patient population.
At the time of this writing, the only site that is qualified to perform this procedure is the Centeno-Schultz
clinic in Colorado.

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 Chapter 6-Is There Another Way? PICL

What if, instead of fusing the upper neck with hardware, we could instead prompt the damaged and
loose ligaments to heal? That’s the concept behind the PICL procedure. This acronym stands for
Percutaneous Implantation of the Craniocervical Ligaments.

Who Is a Candidate for this Procedure?

Patients with CCI who continue to have disability despite conservative care and who have been qualified
as discussed in earlier chapters. We tend to use a DMX study the most often to nail the CCI diagnosis
and less often, a movement based MRI.

Procedure Summary

This is a procedure where we use endoscopy and fluoroscopy to precisely guide a needle into the
craniocervical ligaments from the front. We start by imaging the back of the throat using endoscopy and
preparing a sterile field. A special 3D printed mouthpiece is also used to keep the mouth open and the
tongue depressed. A needle is then placed on either side of the uvula and fluoroscopy is then used to
guide the needle into the craniocervical ligaments. Bone marrow concentrate is injected to help
ligaments heal.

What’s Injected?

We use bone marrow concentrate prepared in a cGMP class clean room. This means that we start the
procedure with a bone marrow aspiration. Most patients want to be put asleep with an IV for this part
with some just getting local numbing. The bone marrow aspirate is then processed in our lab to
concentrate the stem cell fractions.

Two Types of PICL Procedure

Under the Atlas

38
There is a small hiatus (opening) between the C1 and C2 bones where a needle can be threaded (yellow
circles). Once through that area, the lower alar, accessory, transverse, and other ligaments can be
reached and injected.

Over the Atlas

The needle can also be directed above the C1 bone to reach the upper part of the alar ligament and the
ligaments that extend upwards from the atlas to the skull (SAAOL, AAOM, apical, cruciate).

Between these two techniques, at the time of this writing, we have more experience with the under the
atlas technique and are gaining experience with the over the atlas. Given that, we often begin the first
PICL with the less invasive under the atlas technique where we have more experience and then possibly
adding the over the atlas at the second PICL procedure visit based on patient response to the first
procedure.

Posterior Injections

39
Nearly all PICL patients also receive posterior injections with bone marrow concentrate as well. This will
be at least the supraspinous, interspinous, nuchal, vertebrodural, and PAOM ligaments.

Many also get upper cervical facet injections using sophisticated fluoroscopic guidance. These are often
the C0-C3 facets as shown above. Some patients also need occipital nerve hydrodissection. This is where
the growth factors from the patient’s own platelets are injected around the injured nerves at the back of
the skull to help them heal.

What to Expect

The Exam

The patient is first seen for a hands-on evaluation in the office. The purpose is largely to define other
areas outside of the PICL that need to be treated. For example, the doctor will try to answer questions
like:

• Localizing which upper neck facet joints are largely involved

• Which occipital nerves may be causing headaches?
• Whether the superficial cervical plexus under the SCM has issues?
• Is the TMJ involved?

In addition, many CCI patients have developed other problems in various body areas. For example, the
shoulder, upper beck, numbness or tingling in the arms or legs, pinched spinal nerves, SI joint instability,
etc… The goal is to see which of these issues are likely to resolve once improved upper neck stability is
achieved and which problems have taken on a life of their own and need separate treatment.

The Bone Marrow Aspiration

A Bone Marrow Aspiration (BMA) is a procedure where the doctor takes the liquid portion of the bone
marrow from the back of the pelvis. The goal is to tap the stem cell rich bone marrow aspirate. Before
we proceed, we’ll review some discussion on the best way to improve your stem cell counts.

40
While nobody is yet 100% sure based on high-quality research, we’ve seen a few things improve stem
cell counts:

• Intermittent fasting prior to a procedure. One good resource to investigate is the Prolon system
(also called a fasting mimicking diet)-see https://prolonfmd.com/
• The Stem Cell Support Formula offered by Regenexx (see https://store.regenexx.com/)
• Reduce your chronic inflammation-Supplements like fish oil and turmeric can be helpful here,
see https://regenexx.com/blog/running-on-empty-fish-oil-and-my-ankle/mat

An IV is started and the patient is taken back to a procedure room, here the doctor will usually use IV
sedation. Hence, it’s critical that the patient not eat anything for 6 hours before or drink anything for 2
hours before the procedure. The doctor will get the patient sleepy or asleep and then numb the back of
the pelvis area. A specialized needle called a trocar is used to access the bone near the PSIS (dimples of
Venus). Bone marrow aspirate is drawn using ultrasound or x-ray guidance and then sent via sterile
transport to the lab. The area is bandaged up and the patient is sent to recovery. This part usually takes
30-40 minutes. Sometimes additional blood will be drawn which will be collected from a vein at this
time. After this is done, this is a good time to hydrate before the PICL procedure, but please don’t eat.

The patient is then free to go home, hang out near the office, or head back to their hotel until the
procedure in the afternoon.

The Processing

Because the PICL is a hyper sterile procedure and to avoid infection in this area, we use a full cGMP class
cleanroom to process cells. This means that this is a specialized space with HEPA filtration and strict
entry and exit criteria.

You may never see this facility when you’re in the office, but you can ask for a tour of it. It’s a 1,200
square foot specialized room in the first floor of the practice (the clinic is on the second floor of the
building). This is where our processing staff works on one patient sample at a time in an ISO 5 biologic
safety cabinet with laminar flow. The bone marrow is centrifuged and then the two stem cell fractions
are combined for later injection. Platelet-rich plasma, platelet lysate, and platelet poor plasma are also
made either from the bone marrow or peripheral blood. All of these are placed in sterile packaging
which is labelled with the patients name and date of birth and sent back up to the clinic where it’s
stored in a specialized 4C medical grade refrigerator until the patient’s procedure.

The PICL Procedure

The patient returns in the afternoon and is first checked into a patient room. An IV is started and then
the patient meets with the nurse anesthetist. Some initial relaxation medications are given and then the
patient must perform a sterile cholorhexadine mouthwash. They are then taken back to the procedure
room where more medications are given once the patient is placed face up on the table.

The anesthetist then matches the right 3D printed mouthpiece for the patient. We have a number of
different designs we’ve designed and used. If this feels uncomfortable, just realize that more medication
can be given IV to help with the anxiety of this part of the procedure.

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Next, the procedure room staff then clean off the front of the mouth area with iodine and drape the
patient as they are getting very sleepy. The doctor usually comes in around this time and then is dressed
by staff in a sterile gown, gloves, mask, and hat.

The doctor then uses endoscopy to visualize the back of the throat while using sterile cotton swabs to
apply iodine several times to define a sterile field. The doctor will also inject a numbing medication at
the back of the throat and then clean again. The injection needles are then placed on either side of the
uvala (blue dots above) using an endoscope and c-arm fluoroscopy and the procedure begins. Usually,
the patient doesn’t remember much after the drapes go on.

After the PICL procedure is complete, the staff will wake the patient up partially so that they can help
get turned face down. The sterile prep will be repeated and the patient will be given more medications.
The doctor will complete the posterior injections and then the patient will be discharged to the recovery
area.

In Recovery/PACU

The patient will wake up in recovery. It’s very common to have pain at the back of the throat and back of
the head. If the patient has a history of nausea, then this can be flared up. Same with other common
symptoms like dizziness, imbalance, etc… In addition, a drug called ketamine may be used during the
anesthesia, so you may want to inform loved ones that the “ketamine stare” may be present and that
you’ll be a bit “off” for a few hours. The patient typically spends 1-2 hours in recovery.

In the Hotel or At Home the First Night

The patient is typically in pain and can have all the symptoms described above. Pain medications and
antibiotics and possibly anti-nausea medications will be provided via script. It’s best to take pain
medications about an hour after returning back to the hotel or home. Realize that we want you to stay
ahead of your pain, so don’t be shy about taking the prescribed pain medication amounts.

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It is very common to be very flared up after the procedure. Hence, this isn’t a cause for panic. Here are
some things that indicate that you may need to call your doctor:

• Disorientation or confusion that isn’t related to the anesthesia or medications

• Fever, chills, sweats
• New neurologic symptoms that you have never experienced before

Post-Procedure

About half of the patients have a “honeymoon” period where they may be in pain, but they feel stable.
Hence, they feel better for the first 1-2 weeks because the swelling causes the loose ligaments to
tighten. Then this goes away as the procedure caused swelling reduces. Some sense of stability and
improvement in symptoms returns once the patient is able to lay down new tissue promoted by the
stem cells in the bone marrow concentrate.

The other half of the patients experience a flare-up with no honeymoon period. They generally have
increased symptoms for 1-3 weeks and then things calm back down to baseline. A handful of patients
can experience longer flare-up periods lasting 1-2 months. An even smaller number of patients may
have flare-ups lasting a few months.

We generally look at months 3 or 4 to see if there has been any benefit from the procedure.
Improvements by or after month 4 are the most common. A few patients might get benefits later than
that time period, but this is more the exception rather than the rule.

Understanding How Ligaments Heal

To get a sense of the phases that patients will cycle through after the PICL procedure, it’s important to
look at how our ligaments heal after a regenerative injection. The first phase is inflammation, which
lasts for 1-2 weeks after the procedure. This is when the body calls in the right cells to initiate a healing
cycle. The next phase is remodeling, when the body is laying down new tissue. This phase can last for 2-8
weeks. Finally, the third phase is maturation, when the newly laid down collagen fibers in the ligament
align to allow the ligament to gain strength. This is beginning 2 months out, with a shorter final healing
period of 3-4 months in younger patients and a longer period of up to 6-9 months in older patients.

The Timing of PICL Procedures

Given that it takes 3-4 months to see results, we usually sequence these procedures every 4-6 months.
Patients who want to wait a full 6 months after the first procedure will get to that place after the flare-
up where they can access if the procedure helped. Some patients who want to compress the time frame
of the whole process by getting procedures done every 3-4 months in rapid fire could stay flared up for
most of the time, hence this if the patient finds themselves flared up for a longer period of time than a
few weeks, it’s generally recommended that they consider waiting until the procedure flare-up ends
before scheduling the next procedure.

On the other end of that spectrum, waiting a year or more between these procedures could be
counterproductive. Meaning that the procedures work by building new tissue. If the rate at which new
tissue is built is exceeded by it’s breakdown through daily wear and tear because of instability, then the
patient may feel better for awhile and then backslide.

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The average number of procedures required is 2-4 with some patients being “one and done” and some
going onto five procedures.

What to Expect-Two Steps Forward and One Back

For some patients, their recovery will be linear, meaning after the flare-up, each procedure will provide
benefit until they eventually achieve maximum healing. For others, it may be two steps forward and one
back. Meaning there may be ups and downs in their recovery journey. For still others, this type of
procedure may not be effective, and they may need to consider fusion.

Results?

At the time of this writing, about 7 in 10 CCI patients can be helped with this approach and avoid fusion.
Here are two PICL cases to conder:

• FW was a physical therapist involved in a very high-speed rear end crash where his seat back
broke. He was eventually diagnosed with CCI after becoming disabled and unable to practice.
His C1-C2 overhang on DMX was severe and he could do very little without severe pain. His
recovery is detailed here [https://youtu.be/9Oo0v9cHdGY]. After several PICL procedures, he
can now do things like ski with his daughter and hike for miles in the Colorado mountains.
• RL is a chiropractor who was in a car crash in Canada. He became completely disabled due to
CCI, having to lie down 23 hours a day and only being able to be vertical for four 15-minute
periods. After several PICL procedures, he was able to reverse that, meaning he can now be up
for the whole day other than four 15-minmute lie down periods.

Strengthening

It’s absolutely critical that you understand that strengthening is a critical part of the PICL procedure.
Why? Because half of stability is ligaments and the other half is muscles. Hence, helping tighten and
repair ligaments is only half of the treatment.

Why Do You Need to Rehab if You Have CCI? What do CCI Exercises Look Like?

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Stability has two major components: ligaments and muscles. Ligaments check joints from moving too
much. Many CCI patients have loose ligaments due to damage or laxity and these allow too much upper
neck joint movement (typically at C1-C2 and/or C0-C1). However, what’s talked about less often is that
the upper neck muscles that stabilize the head on the neck also become small and weak (aka atrophied).
These muscles are shown here. In order to get the most out of the CCI procedure, you have to begin
strengthening these muscles that haven’t worked well in months or years. The main concept is that
you’ll be able to do more as you become more stable.

Another strategy for higher functioning CCI patients is trying this program before the procedure. While
many CCI patients can’t tolerate any physical therapy exercises, some can. However, in this case, be very
cautious and go very slowly. If you find you can’t tolerate these exercises before the procedure, then
don’t despair, most patients can do them once the ligaments are tightened down by the procedure.

Level 0

A critical first step BEFORE you start any rehab program is to determine if you’re ready to start at all.
That’s a big deal for CCI patients, as without some ligament stability this program won’t be effective.
While most patients would start this program several months after the first or second treatment has
begun, some may be able to start early. Either way, you need to answer “No” to all of these questions if
you’re going to begin this program:

• Do simple head movements throw your upper neck “Out” or cause severe symptoms? Y/N
• Can just random light tasks like reaching for something or typing throw your upper neck “Out”
or cause severe symptoms? Y/N
• If you use an upper cervical chiropractor, do your adjustments hold for less than just a few days?
Y/N

If you have any confusion about whether it’s time to start, talk to your doctor. When I refer to being
“out” or a flare-up throughout this document, this means that your symptoms get worse due to the
activity. For most CCI patients that would be headaches, dizziness, visual disturbances, rapid heart
rate/anxiety (vagal nerve related symptoms), or neck pain.

Adjunct Therapies

If you work with an upper cervical chiropractor and this type of treatment helps, then you should
continue adjustments after the PICL procedure and especially during this rehab program. While I have
set this program up as something you can do at home as finding a physical therapist with CCI experience
is difficult, if you have a physical therapist who has helped, then please provide him or her this book so
they can see what we want post-procedure. Also, curve restoration therapies (aka chiropractic
biophysics) may or may not be recommended for you, but can be continued during this program. In
addition, any activities that cause you reliably to “go out” or significantly flare your symptoms should be
avoided. Meaning, no pain no gain does not apply here.

In particular, depending on how much ligament stability you’ve gained through PICL and/or how much
muscle atrophy you have as well as other things (whether irritated nerves or joints are involved), you
may move this program more or less slowly than I have described. That’s OK. If it takes you 2-3 times as
long, that’s fine. Similarly, if you can move through it quickly without flare-ups, then that’s great. Again,

45
LISTEN TO YOUR BODY! In addition, if you can’t do it at all, don’t worry. You may just need more
ligament tightening through another PICL procedure before this program is right for you.

One word of caution. This rehab program is NOT for patients who have already had an upper neck fusion
for CCI. It should only be attempted upon the advice of your treating physician.

Level 0.25 – Getting the Right Equipment for Your Craniocervical Instability Exercises

To do these exercises, you need a LASER headlamp system. There are a few different options from low
budget to medium. This is a LASER pointer that sits on top or on the front of your head while you look at
where the LASER is pointing. You will use this rig to begin to get position sense and strength back in the
upper neck muscles.

Medium Price

The SenMorCor system is a headlamp and wall target system that comes ready to use out of the box. It
looks like this (click on the picture to the left for an Amazon link). You put that black strap device on your
head with the LASER pointing forward. You then place one of the posters on the wall and use your head
movements to hit various targets. The farther you are from the wall, the smaller and more fine-grained
the movements. If you’re closer, then the movements of your head required will be bigger. This rig and
wall posters run just over a hundred USD. More links to buy this system:

• OPTP

• Physiotherapy Room

Lower Price

I found this system on Amazon which is only 20 bucks and looks like a knock off of the SenMoCor device.
Some physical therapists who left reviews said it worked fine. You can also buy a wall target like this one
on Amazon. You can also easily make your own as shown below, which will be required for the lower
levels of this rehab program.

Level 1-Small Clock Number Movements

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Now that you have your LASER pointer headpiece, find a dimly lit room. There are two options for
making your exercise targets. So let’s dig in.

The cheapest way to do this is to get a 36 X 48-inch white poster board (not glossy, but matte) and a
black magic marker. Draw two clock faces as shown to the left (click on the picture to see a bigger
image). This is two concentric clock faces and the larger one should fill the whole width of the poster (36
inches). The smaller clock should be 24 inches wide (diameter). There should be a point in the middle of
the circles. You can use bowls to draw the circles or a pin with string in the middle point and attach that
to the marker while drawing a circle at that set length. Alternatively, I have designed a PDF file that you
can upload to a local or online printing service. The link to the PDF is here
[https://s5q6n6g5.rocketcdn.me/wp-content/uploads/2020/01/CCJ-Instability-Institute-CCI-Exercises-
v2.pdf] . I got it printed for $25 at VistaPrint on a matte 36X48 poster. You can use any other service
including your local print shop like Kinkos or office supply store (Staples and Office Depot have services).
The nice part about uploading the PDF online and picking up the print is that you can save on the $9
shipping fee that Vistaprint charged.

Place the poster on the wall and get a comfortable chair. You will be measuring the distance from the
wall to the headpiece and placing some pieces of tape on the floor to ensure that you know where the
chair should be.

Now that you have the equipment, it’s time to set-up your level 1 exercise area. Place the chair 5 feet
back from the wall (this distance is from your head to the wall). Get yourself aligned horizontally to the
poster (you should be aligned left to right with the center of the poster and looking at it). Put the LASER
device on your head and move the LASER pointer to the center point of the clock faces.

How the device sits on your head and the poster height should be such that when your LASER point hits
the center of the circle, that’s your neutral comfortable head position (head straight without being
turned left or right or up or down). Some CCI patients have slightly off-kilter head positions that feel
more comfortable, but don’t worry about this right now. Hence, you may need to adjust the device on
your head so that the LASER point hits the middle of the clock with your head in that comfortable
position. Make sure your shoulders are back and relaxed and that you’re sitting up straight (no slouching
or keeping your head forward). If this is difficult, then try a small pillow in the small of your back
(lumbar) which will often help keep your shoulders back.

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You should try this program as written with head movements. Again, if you can’t do it without a
significant consistent flare-up, then you likely need additional PICL treatment. However, you could also
try an “eyes” only version of this program to start. This means you just look at these targets while
keeping your LASER point in the middle of the clock face. This will still cause some very minor firing of
the muscles we’re interested in and may help coordinate your eyes to your cervical system before
starting the part of the program where you move your head.

Level 1- CCI Exercise 1

For this exercise, we’ll be using the small clock face inside the bigger one. The first exercise is moving the
LASER point from the center point to the 12 and back (as shown to the left). Do this very slowly. Now
add in the 9, 3, and 6. So from the center to 12, back to center, to 9 and then back to center, then to the
3 and back to center, then to the 6 and back to center.

Again, these should be slow and controlled movements. In particular, as with the entire program, watch
the LASER point and make it move smoothly and in straight lines from target to target. If it shakes back
and forth or doesn’t initially move in a straight line that’s fine. But your goal is smooth movement in
straight lines from point to point.

Start with what you can tolerate up to 10 reps of going to 12, 9, 3, and 6. One rep is defined as 12, 9, 3,
and 6. If this is difficult for you, then you may need to stay at this level for a week or more to get to 10
reps. If it’s easy, then for day 2 or 3 move onto level 2.

This is a good place to realize that you need to listen to your body VERY CAREFULLY with this program. If
you have any flare-up or you feel yourself “go out” then STOP. In that case, perform fewer reps next
time or move back to the easier level or exercise!

Level 1- CCI Exercise 2

For this exercise, start at the center point and then try 11, 1, 7, 5 in the way as described above (center,
number, back to center). This is 10 reps. Initially just perform L1-Ex2 (Level 1, Exercise 2) alone for a few
days. Then integrate L1-Ex1 + L1-Ex2 together (so 20 reps in total). You may want to stay with these first
two exercises for the first week or move on more quickly if this is easy.

Level 1- CCI Exercise 3

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For this exercise, start at the center point and then try 10, 2, 8, 4 in the way as described above (center,
number, back to center). 10 reps.

Initially just perform L1-Ex3 alone but then quickly integrate L1-Ex1 + L1-Ex2 + L1-Ex 3 together (so 30
reps in total).

For week 2 at level 3, up the reps to 15 for all exercises (45 reps total). For week 3, up it to 20 reps for all
exercises (60 reps total). Again, if you can move through these more quickly than that. great. If you need
to go more slowly than described here, that’s fine as well.

Level 2 – Small Clock Circles

Level 2 – Exercise 1

Here, start at the 12 and move the LASER pointer carefully around the clock in a circle all the way
around. Hit every number starting at the 12 and going back to the 12 as shown to the left. On day 1 of
this new level, do this for 10 reps, initially only this exercise (no level 1 exercises). On day 2, do 20 reps.
On day 3 add back in your level 1 exercises in addition to the 20 reps of level 2, exercise 1. Keep with
that program for the rest of that week. The next week add in 20 reps of the going in the opposite
direction, starting at the 12 and going counter-clockwise, for a total of 40 reps of level 2 exercises.

Again, how many exercises from which levels you are able to do here depends on how much you can
tolerate. Higher functioning patients may find that they can do all level 1 plus level 2 exercises easily.
Other patients may need more time to integrate level 1 and two exercises.

Level 3 -The Big Clock

For level 3, you should repeat level 1 and level 2 using the big outer clock face. For lower functioning
patients this may take the whole time periods described for those levels. For higher functioning patients
this may only represent a week.

Level 4 – Upping the Movements by Pulling the Chair Closer

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Now we’re going to make things harder by moving the chair closer to the wall. The distance between
your head and the wall should be 3 feet. For level 4, if this is an easy jump for you, repeat level 3 using
the big clock. If this is a harder jump with more flare-ups, then repeat levels 1 and 2 before going on to
level 3. This level will require more head movement than levels 1-3, so go slowly and listen to your body.

Level 5 – Closer and Closer

For this level, move the chair so that your knees are touching the wall. This should be about 18 inches to
two feet depending on the length of your legs. Again, as above, if this is tough, repeat levels 1 and 2
before going to level 3. If it’s easy, repeat level 3.

Level 6-90 Degree FOV Room Targets

Next, you want to begin hitting random targets around the room. In this case, make a list of the things
that are within 90 degrees of your visual field (see image left). This is 45 degrees to the left and 45
degrees to the right. Make sure some are higher and some are lower and you have an equal number on
both sides. Then write those targets down on a card and have someone randomly read them to you

50
while you very slowly look at them and move your pointer on to them. As you get better at this level,
you can increase the pace and then more quickly go between targets. Again, start slow and slowly
increase your pace.

Try this for a week starting with 20 reps and working up by the end of 1-2 weeks to 40 reps. Again, if you
can do this without flare-ups, then you may want to begin timing yourself to see how long it takes to get
through the list and then trying to reduce that time as the days wear on. If this is difficult, then you may
want to stay at this level longer and slowly up your pace. The goal of this level is to train your muscles to
be able to react to the real world where you’re often forced to look at things in your environment on a
moment’s notice.

Level 7-180 FOV Degree Room Targets

For this level, you increase your targets to 180 degrees (about 90 degrees to each side as shown). Do
this just as described in level 6.

Level 8-Mazes

For this level, you can either make a maze on the other side of your clock poster board or buy one like
the one I have shown here for $25 on Amazon. If you search google for maze patterns, there are also
many you can find. In addition, the SenMorCor system I showed above has a maze poster that’s
included. I’ve also created a maze pattern that you can get printed just like the clock faces above. That
PDF download link is here [link to https://s5q6n6g5.rocketcdn.me/wp-content/uploads/2020/01/CCJ-
Instability-Institute-CCI-Exercises-Maze.pdf].

Here you begin at the beginning of the maze and move your LASER pointer though to the end. Having a
few different ones to work with so you’re thinking and moving your head at the same time is a good
idea. Start with 20 reps at 3 and 5 feet, moving very slowly to start and then gradually increase your
pace. You can also time yourself so that you can improve your times over a few weeks.

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 Chapter 7-Surgery

There are many different types of surgeries performed in CCI patients. The two main types are
decompression and fusion. There are also secondary procedures that some CCI patients get performed
that I’ll also discuss like detethering and styloidectomy. Let’s dig in.

Decompression Procedures for CCI

Posterior Fossa Decompression

This is a procedure where the doctor “decompresses” the back of the skull by removing bone for
patients who have a low-lying cerebellum (Chiari malformation). As shown above, this is carving a hole
in the back of the head.

What Is Chiari Malformation?

Chiari malformation occurs when a small part of the brain pushes down through the hole in the base of
the skull called the foramen magnum. This can cause pressure on the back of the brain (cerebellum)
and/or the brain stem. This can lead to a host of symptoms:

• Neck pain
• Balance problems, dizziness, or vomiting
• Muscle weakness or numbness
• Difficulty swallowing or speaking
• Vomiting
• Ringing or buzzing in the ears (tinnitus)

The biggest concern is that these symptoms overlap with many other problems including CCI. For
example, patients with upper neck facet joint injuries get neck pain, balance issues, and other
symptoms. Patients who have craniocervical instability (CCI) often have all of the above symptoms.

52
Hence, having an MRI suggestive of a Chiari malformation and these symptoms does NOT mean the next
step is decompression surgery.

Types of Chiari Malformation

Where the rubber meets the Chiari road is which type of malformation is present. The least severe is
actually not listed in the above pictures because it’s more controversial, which is often called Chiari 0 or
cerebellar ectopia. Few physicians would argue that if you have a portion of your brain that has
herniated downwards into the spinal canal then you have a very real need for Chiari malformation
treatment through surgical decompression (Chiari 1 or 2 as shown above). However, all too often these
days, patients get operated on for Chiari 0 without a frank lower brain herniation, which we’ll discuss in
detail below.

Chiari 0 or CTE (Cerebellar Ectopia)

First, Chiari 0, or Cerebellar Tonsillar Ectopia (CTE), is when the back of the brain (cerebellum) isn’t really
herniating down through the bottom hole in the skull. Instead, it’s “on the line” of pushing down and
out of the skull or has done so just slightly. All of this is much less severe than Chiari 1, which means that
surgery for Chiari 0 is VERY controversial. For example, we don’t have a huge body of research that
shows that this MRI finding is associated with symptoms (14).

My colleagues and I performed a research study many years ago which looked at how many cerebellar
ectopia or Chiari 0 patients could be found on upright MRI imaging after car crashes (15). We found that
about 1 in 5 patients with chronic pain after a car crash had this issue. The question was whether this
was causing their symptoms, or an upper neck injury, as many of these patients also had evidence of
CCI. Meaning that there was no way to tell whether the ligaments that held their head on or their brain
suspended in the skull had been injured and the Chiari 0 was being caused by that ligament injury.

Diagnosing Chiari 0

This is a bit of a sticky wicket. Meaning that we have no sure-fire way to determine if what we see on
your MRI as Chiari 0 is in fact causing your symptoms. This is a big problem.

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For example, if we suspected you had pain in the right C1-C2 facet joint I could inject numbing medicine
using radiographic contrast confirmation and x-ray guidance to make sure that I was in the joint. If your
pain and symptoms go away, we have our answer, which is that this specific joint is causing your
problems. However, I can’t do that with Chiari as there is no safe way to numb out your Chiari
malformation.

The only way that is used to try to diagnose Chiari is to use invasive traction. This is where the doctor
places screws into the skull and pulls upward, trying to make space for the lower brainstem and to check
if your symptoms go away. The problem? This also takes weight off of the upper neck joints, discs, and
spinal nerves. Hence, the pain could be coming from any one of these areas. Hence, the only real way to
see if Chiari malformation is causing your symptoms is to cut a hole in the back of your head
(decompression) and see if this makes the symptoms better.

Surgery for Chiari 0

Chiari malformation treatment often involves surgery. The goal is to remove bone and “decompress”
the area of the brain with pressure. That means opening the skin, retracting back the muscles, and then
removing the bone shown here in yellow. This is often the back of the skull and the back of the c1
and/or C2 vertebrae (if needed). The skull bone removal procedure is called a posterior fossa
decompression.

Complications from this procedures are substantial (16). They include more than 1 in 10 of the patients
having a dural leak. This means that the doctor inadvertently cuts through the covering of the brain and
spinal cord (the dura) which is the sac containing cerebrospinal fluid in which the brain floats. The dura
is then sewn back up and leaks, often requiring another surgery. Other common complications include
9% who get an infection of the brain (meningitis), infected wounds at 7%, and damaged nerves in 5%.
About 3% of the patients die from the surgery.

What Is the Collateral Damage?

If you survive the surgery, a common issue is the collateral damage. To understand that, we need to
review the critical anatomy in this area. In the image shown here, note that the PAOM is a ligament in

54
the back of the neck that helps to keep the skull stable on the neck as you look down. Then note the
myriad of muscles in the upper neck at the base of the skull. They’re responsible for maintaining your
head stability on your neck. Both this ligament and many of these muscles are destroyed when a
posterior fossa decompression surgery is performed. Why is this a big deal?

Permanent Bobblehead

This ligament and these muscles at the back of the upper neck and skull are critical for stabilizing your
head on your neck. Think of the head as a bowling ball at the end of a stick (your neck). Without an
important piece of duct tape (the PAOM ligament) or the muscles that actively balance the bowling ball
on the stick, you can become a permanent bobblehead. Hence, outside of huge fusion surgery to bolt all
of this together, there is no way to fix the collateral damage done by this aggressive surgical Chiari
malformation treatment.

My Patient

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This is my patient’s MRI. It shows a Chiari 0 or cerebellar ectopia, meaning that she doesn’t have a Chiari
1 with a good chunk of her lower brain having herniated through the bottom of the skull and into the
spinal canal. Instead, the surgeon believed that pressure on the lower part of the brain (the cerebellar
tonsils) was causing her symptoms. She therefore underwent a posterior fossa decompression at great
personal risk. This didn’t help any of her symptoms which primarily include balance problems, neck pain,
and brain fog. She was then later diagnosed with craniocervical instability (CCI) by a neurosurgeon in
Spain who wants to now fuse C0-C2 (the skull to her upper neck bones) and her lower neck to boot. She
wisely said no and began to explore other options.

Fusion

Surgical fusion is the usually the absolute last resort procedure for any patient with a spine problem and
this is doubly true for CCI patients. There are two common types of upper cervical fusion:

• C1-C2 Fusion
• Occipital-cervical Fusion

Let’s focus first on the most common type of fusion surgery applied these days in CCI patients, a C1-C2
fusion.

C1-C2 fusion means using screws, rods, or bone to make sure that the C1-C2 joint doesn’t move. While
the most common reason this rare procedure was performed used to be because of an upper neck
fracture or severe ligament rupture, these days more of these procedures are performed in patients
with ligament laxity leading to upper cervical instability. This is where this review of C1-C2 fusion will
focus.

C1-C2 Screw Fixation or Magerl Technique

The two most common types of C1-C2 fusions that I see in patients I consult on are posterior screw
fixation (Magerl Technique or C1-C1 trans-articular screws). In the C1-C2 screw fixation, a single screw is
placed across the joint as shown here. The positives for this technique are that it disrupts less of the
stabilizing muscles at the back of the upper neck, which are critical for stability. Since the goal of the
procedure is getting the normally mobile C1-C2 joint to grow together and become one solid mass of
bone, when this doesn’t happen, it’s called a non-union.

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Non-unions are a big problem that I see in patients who have had this procedure. They are then left with
a screw through their joint, which destroys it. Without a fusion, that screw moves as they move their
head, which further damages the joint.

Based on the published literature, an even bigger issue seems to a risk of vertebral artery injury (8). This
is the artery that supplies blood to the back of the brain. An injury here requires emergency
vascular/neurosurgery and can be life threatening.

Goel and Harms C1-C2 Fusion Technique

The Goel and Harms technique shown here is more extensive than the C1-C2 screw fixation. Here two
screws are placed into the bone and a rod system is used to hold the C1-C2 joint. Bone chips from the
patient are often placed around the rods to help provide stability as the bone grows. The big downside
with this approach is the extensive muscle damage that’s required to get all of this hardware in place.
Other risks include again, damaging the vertebral artery. In addition, many surgeons will also sacrifice
the C2 nerve, which can lead to chronic headache pain.

Occipital-cervical Fusion

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In this surgery, as shown here, the base of the skull is fused to the upper neck with rods and screws. This
procedure has very high complication rates of about 50% (8). Also, fusing the upper neck to the skull
places tremendous pressure on the remaining neck bones, which is a set-up for adjacent segment
disease. That means that the levels below the fusion wear out due to the extra pressure, often requiring
more surgery.

Fusion Risks

Now that these procedures are being performed for non-life-threatening injuries like ligament laxity, the
risk-benefit ratio is different. For example, when a patient with an upper neck fracture may have died or
become a high quadriplegic if the fracture wasn’t stabilized, the risks allowed for the surgery can be
quite severe and the risk versus benefit equation still makes sense. However, now that these invasive
procedures are being performed for patients with loose upper neck ligaments due to damage or
congenital problems like EDS, the risk-benefit bargain can be a bit off. Let me show you what I mean.

Nonunion

One of the big risks of any fusion surgery is that the joint being fused never actually grows together. For
these procedures, accurate information that applies to adults is hard to find, as many of these surgeries
are performed on children with congenital abnormalities. However, at least one author states that
nonunion rates can be high with these techniques (9). However, there’s nothing like a good case report
to make complications personal. Katie is a twenty-something I treated several years ago. She had a C1-
C2 screw fixation performed for CCI after a DMX showed too much movement at C1-C2 and nothing else
was helping. Regrettably, the joint never fused, leaving her with new strange movements of the C1-C2
joint as it pivoted around the screw going through it. The joint was also damaged by the screw and still
moving, making her headaches worse and not better (pain from the C1-C2 joint refers to the head). She
was also not a good candidate for our PICL injection procedure due to the surgery. Through injections of
PRP into the C1-C2 joint, we were able to get the joint to fuse, but she also had damage to the occipital
nerves and extra force across the C0-C1 joint leading to new pain there. These areas were treated with
platelets and stem cells with some improvement.

Misguided Screws

A big issue we’re seeing in the clinic is the fact that these screws placed into bone are hard to guide.
Hence, they can inadvertently reach places that can damage structures. For example, the screw can hit
the vertebral artery and damage it, end up hitting and damaging nerves, and even destroy the C0-C1
joint. Since these screws are large, what they hit is usually obliterated. The vertebral artery runs through
the neck bones and this upper neck area. It supplies blood to the back of the brain.

The good news is that most people have one on each side, so losing one vertebral artery can often be
compensated for by the other. Realize though, that destroying one of these arteries with a big screw is
still a very bad thing. For example, in an older patient, this could lead to a stroke (blood clot or other
debris floating into the blood supply of the brain). This happens 4-10% of the time with upper cervical
fusion (8,10). At the very least, emergency vascular surgery may be required.

Damage to the upper spinal nerves can also happen (11). The most common nerve injury is to the C2
spinal nerve (3). This supplies the back of the head, so damaging it can lead to chronic headaches. As
discussed above, the C2 spinal nerve is also sometimes sacrificed in the surgery itself. That means it’s

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taken out by the surgeon because it’s in the way of the desired screw placement. This can also lead to
chronic head pain about 1/3 of the time.

The destruction of the C0-C1 joint is also possible and frankly, one of the most common complications I
have seen. Here the screw is placed too high and travels not only through the C1-C2 joint, but also
through the C0-C1 joint, which destroys the joint. This means that the screw causes arthritis in the joint,
which is bone and cartilage damage. Hence, the C0-C1 joint becomes a new source of pain. This joint
also causes headaches at the back of the head.

Complication Case Reports

The first patient is Rosalyn, who was a student at an elite university. She was diagnosed with CCI due to
a car crash and had moderate instability on her DMX and evidence on MRI of alar/transverse ligament
injury. She underwent C1-C2 screw fixation where a screw was placed through the C1-C2 joint to fuse it
and to reduce her instability. Regrettably, on one side that screw was placed too deep and she woke up
from surgery with new pain right at the base of the skull on that side. This worsened over the ensuing
few days and she also reported a “scraping sound” at that spot when she went to look up or down. That
was the screw scarping off some of the cartilage in the C0-C1 joint. She then underwent subsequent
surgeries to remove the screw, but by then she had two problems. The first was severe arthritis in this
joint and disabling new headaches from that damage. The second was that the two surgeries destroyed
most of the critical stabilizing muscles in this area, which only increased her instability. We tried to
perform a PICL injection procedure and injected stem cells into the C0-C1 joint to aide repair, but this
only helped minimally as the damage from the screw was extensive. The good news is that as far as I
know, she was able to get enough relief to finish college, but the bad news is that she continues to have
this disabling pain to this day.

C1-C2 Fusion

The second case is also of a young woman, but her issue was a loose C1-C2 level due to EDS. She had a
modified Goel and Harms technique C1-C2 fusion. On the right, the screw was placed too high into the
C1 bone and ended up inside the C0-C1 joint. She reported immediate pain at that site after surgery.
However, it seems that she wasn’t taken seriously and it took 3 months to get imaging showing the
problem. The screw was eventually backed out, but then the mass of bone placed in this area to
promote fusion fractured, leaving her without any bone stabilization. On a positive note, all of the new
scar formation from the surgery provided some stability, but the recommendation is still to fuse her
from C2 to the skull, which she didn’t want.

Adjacent Segment Disease

Adjacent segment disease (ASD) is the bane of every fusion. ASD happens because all spinal levels are
built to move just a little bit. When that movement is stopped due to fusion, the levels above and below
take too much force and can develop degenerative arthritis and breakdown. Here the C1-C2 joint is
responsible for half of all of the rotation of the head on the neck, so fusing it dramatically increases
force both on the C0-C1 and C2-C3 joints above and below. Meaning that over time, you can expect
these levels to break down in most patients. Far too many of these patients, in my experience, will
eventually require additional surgical fusions above and below.

The Other Side of the Argument

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There are patients who really need these surgeries. They are few and far between, but they are out
there. In fact, I can remember one young woman from more than a decade ago who had life-changing
results. She was completely 100% disabled and had a hard time doing anything but lying down. She had
an upper cervical fusion and was able to begin functioning and even have a child.

Having said that, I’m seeing much higher complications and side effects than have been reported by
surgeons in the literature. Hence, trying less invasive procedures first just makes common sense. Again,
remember the ladder of invasiveness and use it as your guide!

Who might consider fusion?

• There is an active fracture in one of the upper neck bones

• Conservative care and PICL have failed to help
• The patient is not a candidate for PICL, but has diagnosed CCI
• The level of Chiari malformation or a physical obstruction like a pannus leading to cervical
medullary syndrome is severe and leading to direct compression of the spinal cord

The upshot? Upper cervical fusion is not a routine spinal surgery. It’s much higher risk than the average
procedure due to the vertebral artery as well as the other problems I’ve shown. In addition, I’ve seen far
too many misplaced screws. So, while there are a handful of patients that really need this surgery,
please do your homework!

Detethering

This is a surgery to cut the connective tissue that anchors the end of the spinal cord to the spine. It’s
performed for patients who have a tethered cord. Let’s dig in.

What Is a Tethered Spinal Cord?

At its most basic, your spinal cord projects down from your brain and contains the major wiring that tells
your muscles to move and allows you to feel things. It’s connected to the brain and travels through the
spinal bones (vertebrae) and usually ends at the lowest part of the upper back (T11-L1). There are
nerves below that called the cauda equina (horse’s tail). There’s also a piece of connective tissue that

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anchors it from below called the filum terminale (filum). In addition, there are a ton of small ligaments
throughout the cord that help to anchor it all the way down.

When something from below causes the filum to get stuck, like a tumor or scarring from prior low back
surgery, the filum can yank on the cord and pull it down as shown here. When that happens, it’s called a
“tethered cord” (17).

Most of the pressure is on the lower part of the spinal cord with the connective tissue of the cord
diffusing the pressure as you go up. That’s why most of the symptoms are in the low back and legs
(lower spinal cord and nerves). However, another aspect of that diagram is that some believe that a
tethered cord can also pull on the brain and cause it to hang low in the skull base, intersecting with a
disease called “Chiari Malformation”. More on that below.

The most frequent tethered cord patient is usually a kid with Spina Bifida. It can be congenital (the
person is born with it) or acquired later in life. If it’s the later, there is usually something pulling on the
filum terminale or it’s stuck on a physical obstruction or scarred down. That obstruction can be a fatty
tumor, a spinal cord tumor, or a bone spur. In addition, local scarring of the nerve roots after back
surgery (i.e. failed back syndrome) is also a known cause. In rare instances, this problem can also be
seen in scoliosis.

Symptoms

Symptoms include back pain that radiates to the legs, hips, and genital or rectal areas. There can also be
bowel and/or bladder issues. The legs can feel numb or tingly and in some patients get weak or begin to
lose muscle.

Diagnosis

This is where you need to break an adult with a tethered cord into two camps: traditional and non-
traditional. For traditional tethered cord due to masses in the spinal canal or prior back surgery, the
diagnostic criteria is that the end of the spinal cord which is normally located at T12 or L1 is low, below
L2 with a thickened filum. However, for non-traditional where there is no mass or prior back surgery, the
diagnosis can also be made on clinical symptoms, abnormal urodynamics (testing), and a lack of other
things helping the condition.

Urodynamics

One of the items that advocates point to as an indication for the surgery is urodynamics. This is testing
of the bladder performed by a urologist that can identify a “neurogenic bladder”. The concern I have is
that we have seen many patients through the years with sacral nerve irritation with a neurogenic
bladder on urodynamic studies. These are commonly patients with a central L5-S1 disc bulge that
irritates the descending sacral nerves or chronic SI joint syndrome that irritates the sacral nerves. Their
symptoms and urodynamic studies normalize after a simple caudal epidural to reduce this nerve
inflammation. So this usual cause of a neurogenic bladder would need to be ruled out in surgical
detethering candidates before a procedure was performed.

Surgery for Tethered Cord

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The most common surgery for tethered cord involves cutting the anchoring tissue on the bottom called
the filum terminale. This is called “detethering”. Complications include infection, bleeding, and damage
to the spinal cord, which may result in paralysis or loss of bowel or bladder function.

Tethered Cord in Adults

So if a tethered cord is a diagnosis normally found in kids with spina bifida and adults who have rare
spinal tumors or prior back surgery gone bad, how is it that some patients who fit none of those
descriptions are now getting surgery to treat this issue?

The diagram above is how various problems may intersect with tethered cord.

The connection between tethered cord surgeries and adult patients starts with Chiari malformation (18).
This is a problem where the brain is hanging low in the skull and the bottom part (the cerebellum) can
get pushed into the spinal canal. The idea is that the cord and thus the brain is being pulled downward.
This is still pretty controversial, as the biomechanics of the spinal cord don’t necessarily support that this
is possible. Meaning that any downward force from a tight filum would be absorbed by the lower spinal
cord.

Despite this, there are patients (and a handful of neurosurgeons) who believe in the diagram seen here-.
That is that a tethered cord is pulling the brain down, thus leading to Chiari malformation thus these
patients require detethering. That interfaces with EDS (Ehlers Danlos Syndrome) patients, who have
super stretchy ligaments, because it’s believed that the connective tissue that normally anchors the
brain and holds it up is too loose, thus allowing it to hang low in the skull (19). In addition, these same
stretchy ligaments also cause CCI and the extra movement causes irritation of the brain and spinal cord.
Hence, the rationale is that they too may need detethering to reduce the downward pull on the brain.

So how do we get from CCI (craniocervical instability) to detethering? Another way we can get there is if
a surgical fusion tries to correct vertical instability. The idea here is that the CCI causes “cranial settling”
which is that the skull is pushed into the upper neck. The surgeon uses traction during fusion surgery
and thus fuses the patient in a position where the skull is higher than normal. This then stretches the

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spinal cord too much, leading to the need for detethering. This is a VERY controversial topic, so
detethering after surgical fusion is not a common procedure.

Research?

I searched the US National Library of medicine for any research on the following topics:

• Adult Tethered Cord Syndrome-Not much on PubMed using that search, but more on Google
Scholar-A case series that again doesn’t apply to this discussion as the detethering was
performed for traditional causes of tethering like tumors (20). Or an overview paper that again
focuses on kids or adults with spinal masses or prior back surgery (21). Or a theoretical paper on
this actual topic (adults with symptoms without known masses causing tethering) but provides
no higher level clinical data. Another paper describes 24 patients treated over 11 years who
didn’t have masses and had a primary presentation of back pain (22). In reviewing many of these
listed papers, most again focus on patients with traditional causes of a tethered cord and not on
adults with no known risk factors who suddenly develop symptoms.
• Ehlers Danlos Syndrome and detethering – NONE
• Chiari Malformation and detethering – Some, no high-level research, most in kids with spina
bifida or other common causes of tethered cord like tumors.
• Craniocervical instability detethering – 2 hits-one case report on a rare congenital defect and a
surgery planning study for C1 screw placement. Meaning zip on an adult with CCI getting a
detethering procedure.

What Could Go Wrong?

This is the mantra that every patient considering an invasive surgery like cutting the connection of the
spinal cord needs to consider. Almost all of the research on detethering and possible complications is
from studies that reviewed the results in kids with spina bifida. Unfortunately, most of these kids don’t
walk and have severe functional and developmental delays. Meaning that detecting complications in this
group would be very tough. In an adult who walks and talks and otherwise isn’t wheelchair-bound, the
significance of complications that could include never walking again is a much bigger deal. However, we
have no real reports of the complications of detethering in this new group of patients getting the
surgery.

Should You Get Chiari or Detethering Surgery?

These are NOT first-line treatments. Cutting the connection for the spinal cord is a big deal. One of the
big problems that I see is that the filum terminale is there for a reason. It anchors the spinal cord and
nerve roots. Cutting it will permanently impact the biomechanics of how your spinal cord and spinal
nerve roots move. Once that’s done, there is no going back and reconnecting it.

Styloidectomy-Eagle Syndrome Surgery

We’ve been noticing more CCI patients who get surgery for Eagle syndrome. What is that and why? Let’s
dive in.

What Is Eagle Syndrome?

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Eagle syndrome was first described in 1949. It can happen when a bone at the base of the skull (the
styloid process) grows too long and impinges on important nerves and/or blood vessels. The symptoms
include throat pain (which can range from dull and nagging to severe) or trouble swallowing. There can
also be neck and/or facial pain. This can also be felt in the jaw or ear. Other symptoms can also include
tinnitus or a ringing sound in the ear or increased symptoms with head movements, such as turning the
head or chewing.

This problem impacts women more than men. This is a RARE condition with an incidence of 4-8 per
10,000 people.

Anatomy

The styloid process is the key piece of anatomy to understand Eagle syndrome. This is a tooth-like
projection from the side at the bottom of the skull just in front of and down from the ear (shown in
yellow here). It connects to the stylohyoid muscle (red) which connects to the hyoid bone (blue). The
styloid process also has muscles that go from there to the tongue (styloglossus) and back of the throat
(stylopharyngeus).

There is also a ligament connecting the styloid process to the hyoid bone called the stylohyoid ligament
and one that goes to the jaw bone (stylomandibular ligament). Confused yet? You should be, this is a
complex area. But here’s the summary so far – this styloid process is connected to lots of critical stuff!

What else is in this vicinity? There are vessels and nerves in this neighborhood as well. The internal
jugular vein, internal carotid artery, and glossopharyngeal nerve (CN IX), vagus nerve (CN X), and
accessory nerve (CN XI) run inside of the styloid process. The occipital artery, hypoglossal nerve (CN XII),
(CN VII) run to its outside. The trigeminal nerve (CN V) is also in this general vicinity.

Why the Symptoms?

The idea here is that the elongated bone aggravates nerves in the area. Here’s what advocates for this
diagnosis believe happens:

• Tongue spasm and throat pain-irritation of the glossopharyngeal nerve which supplies the
tongue and the back of the throat and the hypoglossal nerve which moves the tongue.

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 • Neck pain-irritation of the accessory nerve which supplies the trapezius muscle
• Facial Pain-irritation of a part of the facial nerve or the trigeminal nerve

How is This Diagnosis Made?

The first thing that’s usually noted is a long styloid process on x-ray. A normal one is about an inch long
(25mm). A styloid process that’s longer than 30mm is considered abnormal. However, this is where you
as a patient need to be VERY CAREFUL! Why? 4-7% of everyone walking around out there have a long
styloid process, Only 4% of patients with elongation of the styloid process show symptoms!

Isn’t there a highly accurate test that doctors perform to tell if I’m one of those 4% of patients with a
long styloid bone that is causing my symptoms? NO. Most patients are operated on based on the x-ray
(or CT scan) and the symptoms.

There is an ultrasound or x-ray guided diagnostic numbing injection that can be performed, but nobody
is sure if that’s a 100% accurate way to chose patients who will respond to surgery. However, you should
consider getting this done by THE RARE QUALIFIED EXPERT who understands how to perform this
injection. If it’s a positive block, your pain and symptoms should go away for several hours.

Why Is My Styloid Bone Long?

Nobody is 100% sure why this happens. However, looking at the diagrams above, note that the styloid
process is attached to the jaw through ligaments and muscles. Hence, chronic TMJ or neck issues
causing too much force on the TMJ can pull on the bone causing it to get beat up and lengthen. This
wear and tear can also cause the normal pliable ligament to turn to bone (ossify).

Do Other Things Cause These Symptoms?

YES. Many other conditions cause these same symptoms. Meaning that this 4% of patients with a long
styloid bone and symptoms may actually have something else causing their pain. While I know that this
may be a disconcerting message to hear when you believe you have finally found the cause of your
symptoms, it’s a very critical one to absorb.

One of the biggest overlaps we see is Craniocervical Instability (CCI). All of these same cranial nerves can
get irritated not by a long styloid bone, but by loose ligaments that hold the head causing too much
movement of the skull on the spine. This can cause irritation of these nerves where they exit the skull.
Hence operating on the neck and taking out a piece of this bone will do nothing to relieve these
symptoms.

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We have also been tracking a number of patients who got this diagnosis and ended up with surgery.
Why? As a clinic, we have seen a rash of patients getting their styloid bones removed who are no better
or worse after the procedure.

What Else Could Be Causing My Symptoms?

Many things cause neck pain that are MUCH more common than Eagle syndrome, including damaged
neck joints, pinched neck nerves, loose ligaments, irritated muscles, etc… Other things cause facial pain
such as TMJ syndrome and trigeminal neuralgia. Other things cause throat pain including local lesions in
the throat.

Does Surgery Work?

The real answer is we really don’t know for sure, as there are NO gold-standard randomized controlled
trials comparing surgery to no surgery. However, there are some lower-level studies that suggest that
surgery may help. We do know that the surgery, because of all of the nerves and vessels near this area
has potential complications. These include:

• A localized infection requiring IV antibiotics and/or additional surgery

• Trigeminal neuralgia (damage to this nerve that causes more face pain)
• Vascular injury leading to severe bleeding and requiring microsurgical repair of the vasculature
• Facial paralysis due to injury of the facial nerve

Are There Other Ways to Get Me Out of Pain?

USUALLY. Meaning in our experience, most patients who have been told they need surgery for Eagle
Syndrome actually have other causes of their symptoms. Many different neck treatments can be applied
to help these symptoms if the actual cause is a neck problem in the spine and not the long styloid
process. Again, realize that 96% of patients with a long styloid process do not have Eagle syndrome.

A Surgeon Told Me that I NEEDED to Have this Removed

This messaging of severe problems like a stroke or other issue that could happen if the styloid bone is
not cut out are surgical sales techniques. While for some patients with severe disease there may be
additional risks, in our clinical experience, for the vast majority of patients who are told they need
surgery, the risk of removing the bone is greater than the risk of stroke or puncture of vessels due to the
elongated bone.

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 Chapter 8-Other Issues Surrounding CCI

There are a number of medical conditions that intersect CCI. What I mean is that either CCI patients
tend to get these diagnoses as well or that people prone to CCI can have these issues. I’ve already
mentioned a few under the surgical section and we’ll also get into some others here:

• EDS
• Chiari malformation
• Tethered cord
• Eagle syndrome
• Dural leaks
• Chronic fatigue syndrome (CFS or ME-CFS)
• Mast Cell Activation Syndrome (MCAS)
• Postural Orthostatic Tachycardia Syndrome)

The hard part here is that many of the symptoms of these medical conditions overlap with CCI
symptoms. For example, in the diagram below I list some of the common symptoms of CCI and Chiari:

As you can see, there are shared symptoms between these two diagnoses. A careful review of the
symptoms would tend to push someone toward one or the other. In addition, the diagnosis of CCI could
be bolstered by imaging. However, one of the tests to diagnose Chiari malformation is invasive traction.
This is where screws are placed into the skull and the doctor pulls traction to clear space for the Chiari
malformation and asks the patient if certain symptoms have improved. However, realize that if the
headache pain was caused by an upper neck facet joint injury, which is common in CCI patients, since
we’re taking weight off that painful joint, the headache would also be relived having nothing to do with
the Chiari malformation. Hence, it’s important to be very careful about figuring out what’s wrong by
looking at only symptoms.

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Chronic Fatigue Syndrome

ME-CFS or myalgic encephalomyelitis/chronic fatigue syndrome is a condition where the patient has
extreme fatigue. Rest usually doesn’t help, and physical activity makes it worse. They also suffer from
disability in that common tasks like taking a shower or cooking dinner become difficult.

There is a huge cross-over between ME-CFS and musculoskeletal and spine conditions. Meaning that it’s
not uncommon for us to see chronic pain patients with neck or back issues who also carry an ME-CFS
diagnosis. Since nobody knows what causes ME-CFS and there is no diagnostic test, the diagnosis is
made based on symptoms of fatigue, which is also a common side effect of having chronic pain.

Many of our CCI patients also carry an ME/CFS diagnosis. The presumption is that their ME/CFS
symptoms are caused by or made worse by the CCI.

MCAS/POTS

Mast cell activation syndrome (MCAS) and Postural Orthostatic Tachycardia Syndrome are overlapping
diagnosis with CCI, mostly with patients who have EDS (23). MCAS involves over-reactions of the
immune system and unexplained allergic reactions and POTS is a fast heart rate when changing positions
such as getting up from a chair or bed. Both of these diagnoses are quite common in our CCI patients
with EDS.

Dural Leaks

Most patients are unaware that there’s fluid around their brain, spinal cord, and nerve roots that
literally floats their whole neurologic system. They also don’t realize that the system designed to hold it
all can spring a leak and that when it does, all heck can break loose. While we’ve treated many patients
with dural leaks through the years, one in particular stands out as a happenstance of being in the right
place at the right time—a 16-year-old named Harry. His case is a great way to shed some light on dural
leak treatment.

What Is the Dura?

Your nervous system is made up of your brain, the spinal cord, and the spinal nerves that leave the
spine. There are also peripheral nerves that go to and from various parts of the body and a few other
parts and pieces, but our focus today is on the central nervous system. To protect these fragile
structures, your brain and spinal cord live inside strong bones (the skull and vertebrae).

Your body has a problem, and its solution dictates why dural leaks happen. The nerves and brain are
sensitive structures that can be easily damaged. In addition, if you put any pressure on the nerves, they
will fire off, giving a false signal, like a wire that’s short-circuiting. To get around this, nature has
enclosed all of the nerves into a sheath called the dura. It’s kind of like a specialized water balloon that
allows your brain, spinal cord, and nerves to float in liquid (called cerebrospinal fluid, or CSF). This
provides excellent protection as well as shock absorption.

What Is a Dural Leak?

As you might imagine, at the end of the day, no system of containment is perfect. If you place enough
pressure on even the world’s best water balloon it can pop or spring a leak. In addition, if you poke a
hole in it or if it was manufactured with a weak spot, the same thing can happen. The body is the same.

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The containment system of the CSF, called the dura, can also spring a leak. When it does, the fluid can
leak out and the brain, spinal cord, and nerves can lose their containment system.

What’s unique about your personal water-balloon nerve-flotation system is that it’s constantly topping
off the system. To do this, your body makes CSF at a certain rate. If you spring a small leak, the body can
handle that by simply ramping up the production of the fluid. However, if the volume per unit time of
the leak exceeds the body’s ability to replace the fluid, then you get “low in the tank,” causing your brain
to literally bang into the skull—talk about a headache!

What Are Dural Leak Symptoms?

Imagine an ice cream headache on steroids and multiply that by 20—that’s what many patients with
dural leaks experience. In addition, they can have a bevy of other nerve-type complaints because their
spinal cord and nerves are being stretched. This can include confusion, disorientation, numbness or
tingling, or pain referring to strange places. Given the vague nature of the complaints, it’s not hard to
see why these patients get lost in the medical care system. However there is one symptom that’s pretty
specific to dural leak patients. They’re better when lying flat, and it all gets worse when they’re upright.

What Causes Dural Leaks?

The biggest cause is iatrogenic, which is a fancy medical way of saying that we doctors are the culprit.
Some happen as a result of epidural injections that inadvertently puncture the dura. Some happen
because of spinal taps, where the goal is to puncture the dura. Some can also occur in car crashes where
sudden pressure builds up in the CSF, causing a dural blowout. Finally some just happen (spontaneous),
likely due to a pre-existing defect in the dura (a weak spot).

What Is a Dural Leak Treatment?

As silly as it sounds, the treatment for dural leak is as simple as “patch the hole”! Sometimes patients
get offered a surgical repair, but that can be difficult if there are many holes to patch. In addition,
finding where the leak is occurring in the first place can be maddeningly difficult as few tests exist to
accurately show where the leak is located.

The old reliable dural leak treatment is called a blood patch. This is what it sounds like—injecting the
patient’s whole blood into the area around the dura (epidural) and allowing this to coagulate to seal the
hole. The advantage of a blood patch is that you can inject several epidural sites and cover a whole area
at a time, like the lumbar (low back) spine. If that doesn’t work, you can march up to the lower thoracic,
then upper thoracic, and then cervical. By process of elimination, you can thus find the region of the
leak.

Some chronic dural leakers are also injected with fibrin glue, which is a newer dural leak treatment. This
is the same stuff used to close skin wounds in the emergency room. It can be easily injected, and then it
sets up with a rubber-cement-like consistency. We’ve heard mixed results from our dural leaker patients
as some report that it can cause scarring of the dura, and at least one poor woman got hepatitis from a
poorly screened human donor of the fibrin.

We’ve been blessed in this area with a natural dural leak treatment for many of these patients—platelet
lysate (PL). We routinely inject epidural growth factors isolated from blood platelets to help patients
with things like herniated discs. However, that same injectate turns out to be a great dural leak

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treatment as it can be easily polymerized to form what looks like a blood clot by injecting a little
activation agent right after the PL. It also has a natural growth-factor cocktail to assist in healing the hole
in the dura.

Chapter 9-Wrapping It All Up

Thanks for taking the time to dig into CCI. My goal here was to create a book that was simple enough to
read that allowed patients to learn but also had enough information that physicians would learn new
things as well. If you find any typos or feel that there are things missing that really need to be in the
book, send me an email at [email protected]. While I want to keep this book short
enough to be a quick read, if 3-4 patients all request a section that’s missing, I’ll likely add that topic.

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