Ask the Expert: Craniocervical Instability with Dr. Fraser C. Henderson
VIRTUAL MEETING - JUNE 18, 2020
The craniocervical junction is the area at the back of the skull which houses the brainstem, the cerebellum and the top of the spinal column.
Abnormalities in this region can cause a collection of symptoms that are common in
Chiari malformation, craniocervical instability,
Ehlers-Danlos syndrome and related disorders. These symptoms also can overlap with those of other conditions like tethered cord syndrome or mast cell activation disorder.
In this Ask the Expert,
Dr. Fraser Henderson answers questions about craniocervical instability, Chiari malformation, tethered cord and Ehlers-Danlos syndrome from the perspective of a neurosurgeon.
All questions were sent in by real people impacted by Chiari malformation,
syringomyelia and related disorders.
This presentation was given on Thursday, June 18, 2020 and was presented entirely online. (2020)
Note from Bobby Jones CSF: We want to take a moment to sincerely thank all those who donated in support of this virtual presentation. No one at the organization was expecting that much generosity during such a difficult time for us all. From the bottom of the hearts of our boards, staff and volunteers, we want to thank you for your donations. Your kindness continues to motivate us and makes us all the more certain that this work is valuable and helping the people who need it. Thank you.
Full transcript:
[Kaitlyn] Okay. There we go. Hello, welcome everyone.
Thank you all for coming tonight. I know tonight we have Dr. Fraser Henderson with us, so I'm just gonna briefly introduce him and then we can get started.
Dr. Henderson received his Bachelors and Medical Degree from the University of Virginia at Charlottesville. After graduation, Dr. Henderson served for the multi-national peace-keeping force in Beirut, Lebanon. He earned the Navy Commendation Medal for his service. He then returned to the U.S. to complete his residency at MUSC before going back to active duty as commander at the National Navy—Naval Medical Center in Bethesda where he was the brigade neurosurgeon.
He then completed his fellowship in cranial-- craniospinal surgery at the National Hospital
for Neurology and Neurosurgery in London, England. And he became the Director of Neurosurgery of the spine and craniocervical junction at Georgetown.
He currently serves as the Director of Neurosurgery at Doctors Community Hospital and serves patients at Metropolitan Neurosurgery Group in Silver Spring, Maryland. He's invented numerous devices related to the brainstem and spinal cord and he's authored-- I checked the last video-- over 90 peer reviewed articles and books. And as part of a mission to continue education about craniocervical disorders and Ehlers-Danlos syndrome, different spinal abnormalities, he's given over 190 invited presentations.
And in his spare time, when he's done doing all of that [laughs] he serves on our SEA Board,
our medical board.
And he's arranged countless in-person and now two virtual presentations to educate health care providers, patients and their families. So, we are really, really grateful that Dr. Henderson
could be with us today and with that, I'm gonna see if Dr. Henderson wants to say anything?
[Dr. Henderson] Nope! All ready to go!
[Kaitlyn] Cool. Alrighty then. So, let's get started.
I'm gonna start with some symptomatic questions. There were two questions kind of related to vertigo.
Do you know what percent of Chiari patients have vertigo spells as a number one issue? And is vertigo more typical of craniocervical instability? Or, is it typical of Chiari?
[Dr. Henderson] Yeah, I think that vertigo would be more typical of craniocervical instability. I would say dizziness is extremely common with Chiari and craniocervical instability.
Vertigo is specifically a type of dizziness where the room is spinning around the patient. So, it's a more specific type of dizziness. And I'd say it's a little bit more common with craniocervical instability.
[Kaitlyn] Do you know if there’s a percentage of Chiari patients who typically have vertigo? Or, is it just prohibitively small?
[Dr. Henderson] No, I-- I'd say-- it's a common-- Milhorat's paper mentions that number and it's been a while since I've read it, but I think it's in the order of about 35%.
[Kaitlyn] And we can try and locate that also and share it with the group, too. What are some symptoms of occult tethered cord in Ehlers-Danlos patients?
[Dr. Henderson] Occult tethered cord. Alright. I actually like the term "tethered cord syndrome" as opposed to "occult tethered cord". Tethered cord syndrome refers to the symptoms and signs that arise from pulling down of the spinal cord by a thickened remnant of what's called a "filum terminale".
When a baby is-- in the third trimester, the distal third of the spinal cord atrophies and forms a tissue called the filum terminale, which is like a little fishing line from the end of the spinal cord to the base of the spine.
And normally, this is very floppy, but in some people, the filum can be thickened, become inflamed, and pull downward on the spinal cord. And that probably causes injury to the lower spinal cord. It's generally assumed that pulling causes injury.
So the patients have low back pain, leg pain, numbness and weakness, sensory loss in the lumbar and sacral dermatomes, and urinary problems, like urgency, frequency, hesitancy, incomplete emptying and incontinence.
Almost all patients with tethered cord have urinary-- severe urinary symptoms which have to be confirmed with the urodynamics, which is electrical and pressure-testing of the bladder to confirm the presence of neurogenic bladder.
In addition, about 10% of patients have rectal incontinence. About half of the patients have some degree of headache, due to the tethering. In my experience, some even have increased "dysphagia", difficulty swallowing. And we know, in children, that tethered cord can result in delayed speech. And children also have developed scoliosis. Many of them may have toe-walking, where they walk on their toes. Or, they have-- their legs may be turned inward, or one leg may be slightly atrophic. Very often, there are neurocutaneous markers on the back, like a hairy patch or a dimple.
Radiologically, you might see a number of findings including scoliosis. You may see the spinal cord pulled downward. Usually, the spinal cord ends at the L1 level. But in tethered cord syndrome, it may be pulled all the way down to the sacrum.
It might be a thickened filum-- that little band that I talked about. Or it may be filled with fat, and that shows up on MRI. Sometimes, we see a small syrinx, a small amount of fluid in the spinal cord in the thoracic or lumbar spine.
And we often see "spina bifida occulta", which is a-- incomplete lamina, backbone, at the S1 level. [clears throat]
So, they're the radiological findings. And when people say "occult tethered cord" , they're just saying that the spinal cord is ending what is generally a normal level. But, it's well-established in the literature going back to the 90s, somewhere between eighteen to 28% of those studies back in the 90s had-- patients had tethered cord syndrome, even though the spinal cord ended at a normal level.
[Kaitlyn] Sticking with some symptomatic questions... One came up in the chat: is "strabismus", so being cross-eyed, related to Chiari? And can it be made worse from tethered cord, or something else being present?
[Dr. Henderson] Uh, yes, you can get strabismus from Chiari malformation. Could it be worse with tethered cord? I've not-- I've never read that, but I'm sure there are occasions where. tethering can exert an influence all the way up-- but I would-- that's not something that I would really... that is to say.
[Kaitlyn] Yeah. There are a handful of questions-- I'm gonna try and do this in a flow that makes sense from symptoms, diagnosis, and all that.
So, there's a question here: are clinical symptoms, or provocations-- are there any clinical symptoms or provocation tests that a PT-- it would be safe for them to administer in clinic for specifically hypermobil-- blah-- oh, my-- hypermobile EDS.
[Dr. Henderson] Okay, in order to diagnose Ehlers-Danlos syndrome?
[Kaitlyn] Yes.
[Dr. Henderson] Okay. Yes.
In fact, I think that physical therapists could be really central to the diagnosis of EDS.
And they-- they're really a great part of the medical providers. And this is right down their alley. So, number one: patients with EDS have to have joint pain. So, they have, you know, knees, elbows, big joints, not the little ones. The little joints tend to be things like rheumatoid arthritis. The hands, the feet. That's rheumatoid arthritis or lupus. But in EDS, it's the big joints. So, you have: elbows, shoulders, hips and knees, generally. Maybe ankles. And so they have joint pain. Several joints more than-- more than a few months.
Secondly, they are um, very hypermobile. This may not be evident: the patients tend to be quite athletic. Most of the patients I've seen were very athletic, at least in their early adolescence. You can apply the test of hypermobility. And that's very simple.
So, one of them is bending the thumb back to the forearm. Now, I can't bend my thumb more than that far but if I could bend it back to touch my forearm, that would be very hypermobile. Another test is bending the little finger. Now, I can bend my little finger back that far, if I could bend it back parallel to my forearm, then that would be-- that would be one point there. One point for each thumb going back. If I bend my elbow, but if you have EDS, then it goes backward a little bit. And that's 15 degrees more than normal. And the same with the knees. So, patients bend the knees backward and introduce a little reverse bend. A point for each of those. And then if the patient can stand with his or her feet together and place both palms on the floor, that's one point. For adults, you need six points. Or, if you used to be able to put your palms to the floor, and you're over age 40, then you can have five points. With a history of being able to have 6 points.
If you have six points or more, then you're in the race for hypermobility spectrum disorder. That could be anything from benign hypermobility which is very common. It's probably, at least, four percent of the population. This is benign hypermobility. And that's probably one end of the spectrum.
If you have that, plus the joint pain, and you have skin changes in a normal person-- if you pinch the skin, you can pinch it up maybe, you know, maybe a centimeter, but with EDS, you can pull up the skin and it'll tent from the metacarpal of the well, the knuckles here, back to the wrist. You can tent the skin from the knuckles to the wrist. They also have very soft skin. They have Piezogenic papules, little bumps on the ankles when the patient stands up. And unexplained straie-- stretch marks.
So, if they have all of those and a couple of other things then they have EDS. By the 2017 criteria. If there's a family member with EDS, then that's you're 1/3 of the way there.
There are a few other things like a high-arched palate. The palate is normally flat. If it's shaped like a turret, inside, that is one of the findings. And there are a few more, but I don't want to.. okay.
[Kaitlyn] [laughs] No, that's great! Those are really practical, too. So, that's very helpful.
[Dr. Henderson] Any physical therapist can look up the criteria and make the diagnosis. You know?
[Kaitlyn] Yup!
[Dr. Henderson] It's a-- I get annoyed when people say "It's too difficult to make the diagnosis." You know, because it's not that.
[Kaitlyn] I'm gonna shift a little bit. There are two kind of, definitional questions that people were curious about. One of them is: would you be able to define and explain what a "pre-syrinx" is?
[Dr. Henderson] Um, well. It's sort of a nebulous concept in that sometimes on an MRI you can see softening, or edema in the spinal cord. And that, in another month or six months, that could turn into a syrinx. A little fluid filled cavity.
[coughs] But I don't think that's been firmly established, you know? I think that's-- a number of us feel that's what it is. And I think that makes logical sense. There's an injury-- an ongoing injury to the spinal cord and that manifests as tissue injury and then later the cells die, leaving a cavity, which fills with fluid.
[coughs] Sorry. COVID virus...
[Kaitlyn] Oh, God. Don't even say that. You good? Hydrate, hydrate!
There was another question about-- I guess a good thing to start with is what exactly is a "pseudomeningocele"? And do they always require surgery is the point of the question.
[Dr. Henderson] Okay. So, a [coughs] Sorry.
Pseudomening-- A "meningocele" is a collection of spinal fluid covered by the arachnoid, which develops outside of the spine. That's a meningocele.
They're usually after... some sort of injury. There may be an out-pouching or there may be a tear in the dura. And the arachnoid can pouch outward and expand and that would eventually become a meningocele.
Now, if you have surgery and you get a CSF leak, the CSF can leak into the space around the spine and eventually be covered by a "pseudocapsule", or normal tissue that just envelops that CSF. That's a "pseudomeningocele". It's not an extrusion of meninges, it's essentially a CSF leak.
Now, if you leave a CSF leak long enough, provided the tissues over it are sealed, the meningocele will reach a pressure that's equal to the pressure on the inside of the spinal canal and it won't leak out anymore. It'll achieve an equilibrium; it won't keep growing. And so, many of these will eventually stabilize and maybe, gradually get smaller with time. However, many of these meningoceles can be quite painful. And eventually disabling to the patient. Some of them can break out and leak CSF and then cause a meningitis. And so, it's my posture to... if I see a pseudomeningocele and unless there's some reason not to, I'll go in, find the leak, fix it, or take some measure to get rid of the pseudomeningocele and take care of the problem.
[Kaitlyn] Um, a question came up... There's been talk recently about.. Oh man, I always say the ME part very wrong, but ME/CFS, so chronic fatigue syndrome, um, that it's been-- it might be caused by craniocervical instability. Have you seen some similar conversations about it possibly causing-- uh, sorry-- tethered cord possibly causing CFS-like syndromes?
[Dr. Henderson] Well, so ME is meningoencephalitis (myalgic encephalomyelitis), and I think that's-- My personal feeling is that may be a misnomer because that implies inflammation of the meninges, like a true meningitis. And, in fact, usually on an MRI, you won't see an inflammation of the meninges and it's not-- you usually don't see a big inflammation; there may be a few white cells there, but it's not a true inflammatory condition.
That said, there's always a full spectrum so on the severe side, there's some-- real infections where you will see meningoencephalitis. You know, like Lyme disease can cause that, for instance... But I think that a lot of patients who are diagnosed with ME/CSF-- oh, CFS-- chronic fatigue syndrome-- actually have craniocervical instability and Chiari malformation. The only way to differentiate or to know would be to do all of the dynamic imaging and a very thorough exam and history and so on.
Could tethered cord syndrome cause chronic fatigue syndrome? I wouldn't say it would cause it, but it could certainly exacerbate the whole problem. Fatigue can be multifactorial. Many—hundreds of things could cause fatigue. And tethered cord can cause severe headaches, and tiredness, and weakness of the lower extremities. Things that make you depressed. When I get depressed, it makes me very fatigued, you know?
[Kaitlyn] Mhm.
[Dr. Henderson] I mean, In fact, whenever I hit a medical problem and I don't know how to deal with it, immediately-- it immediately makes me tired, you know?
[Kaitlyn] So, it could make everything a little bit more complicated, if anything. There are a couple of questions about this that have come up in the questions that were submitted, and a couple of people have been asking it in the chat. It boils down to: what are the imaging tests that help to accurately identify craniocervical instability. For instance, types of MRI, some types of CTs or-- someone specifically asked about DMX, so-- I don't know if you can speak to those...
[Dr. Henderson] Right. Well, for craniocervical instability, the most useful test is a dynamic MRI. The dynamic flexion/extension, upright MRI. And what this involves is the patient sitting up and then flexing the head forward as far as he can, and then flexing it backward. And a guy Harris, back in 1994, showed that In normal people, the basion, the base of the skull, the base of the clivus, never moves more than 10 millimeters from the odontoid. 12 milli-- it never moves more than 12 millimeters on an X-ray. And X-ray is a slightly enlarged view, so 12 millimeters on an X-ray, means 10 millimeters on a CT scan.
We adopted, with the Chiari & Syringomyelia Foundation in 2013, we adopted a standard that the skull base would never move more than 12 millimeters from the odontoid, the tip of the odontoid-- the spine. If it did, that represented craniocervical instability, which means the ligaments are too lax to hold the head in the right position and that-- then the head is sliding, the cranium is sliding back and forward on the spine and that's causing traction to the lower cranial nerve roots and traction on the brainstem and that causes headache and can cause... It causes headache and all the neurological symptoms of the cervical medullary syndrome.
That's the definitive way of defining craniocervical instability. Now, if you can't have an upright MRI, then you can do a supine MRI lying down, and put a pillow under the head to flex the neck forward. And then put a pillow under the shoulders and allow the head to extend backward and get a supine flexion/extension MRI. And that would get you in the ballpark, you know, eighty percent of the time.
Now, if you can't do that, then you can do the same same thing with a CT scan. A "cat scan". Pillow under the head for flexion, pillow under the shoulders for extension. And then do the same measurements. And the head, the cranium, should be pivoting over one point, over the odontoid. It should not be sliding back and forward. So, by measuring the basion-axis interval (BAI) which is the distance from the base of the skull, the basion, to the line drawn behind the odontoid. That's the basion-axis interval, or Harris measurement and that measurement should not be changing more than one or two millimeters. If you have craniocervical instability, it may be varying up to 10 millimeters.
So, that's that. Now, it's very difficult to make the diagnosis with X-ray, and it's um,a nd for the same reasons, it's difficult with "DMX", or "digital dynamic fluoroscopy". Because there's so much-- there's such a great thickness of bone, that you're not seeing one point of bone, you're seeing the full width of the bone and you lose the accuracy and it makes it much more difficult to you know, for me anyway, to make the diagnosis of craniocervical instability.
[Kaitlyn] Um, let's kind of stay in radiology for a second. There's a very specific question that I have a feeling came from and engineer or a radiologist. So, I'm going to ask it almost verbatim. CSF flow abnormalities on axial PC MRI cine are seen in symptomatic Chiari patients-- Chiari 1 patients-- that are certainly the result of elevated pressure. Elevated CSF pressure, to be specific. Although it appears there has never been a direct and invasive measurement of CSF pressure in mild traumatic brain injury patients, it's likely that some degree of elevated pressure might be present. Would a similar non-invasive diagnostic for mild traumatic brain injury be plausible, and perhaps supercede the comple-- or be complementary with the Glasgow coma scale? What do you think?
[laughs]
[Dr. Henderson] There-- so, my 200 page response... [laughter] Okay well, let me pick at a few sides of that question.
If you have a head injury, typically, there's a little bit of bleeding.
And a little bit of blood can actually-- is known to
increase cerebrospinal fluid
pressure
and can cause
acute hydrocephalus,
or can cause
normal pressure hydrocephalus
which is a
a mild increase in pressure
that occurs in older people.
It has significant effects.
It is known
that if you have a head injury,
that the cerebellar tonsils
very frequently descend.
So, they did a study
in Europe.
Hundreds of patients.
And they found that
after trauma,
the number of
the number of low lying
cerebellar tonsils,
or Chiaris,
were five times higher
than the average.
And so,
they made the
logical conclusion
that if you have a head injury,
that very often,
that can cause a
Chiari malformation.
There are probably
a hundred reasons
or causes for a Chiari
to develop.
Some are genetic,
and some relate to
shape and size
of the bone In childhood and babies.
Some are--
if you have a brain tumor
up here,
that can cause
a Chiari malformation.
There are many,
many causes.
And head injury
is certainly one.
Moreover, you are more likely
to pick up
a low lying cerebellar tonsil
with the flexion/extension MRI
than with a [normal] supine MRI.
So that said, the author of that question talked about the the flow characteristics of CSF. And that flow is mostly--
Changes in flow
is mostly the result of
obstruction of CSF.
If there's a reduction in
space
then that can cause
an increased speed of
CSF flow through there.
Obviously-- if it's completely obstructed, then there's no flow.
People like Raymond Damadian,
who invented the MRI,
have demonstrated that
the flow characteristics
when you're sitting upright
are quite different
to when you're lying down.
I think most neurosurgeons they look for flow or
the potential for normal flow,
but they don't
hang their hat on--
they don't base their decision
to operate on that.
It's just
one more thing to look at.
Like these measurements
that I've talked about.
They're just something
to look at,
to add to,
or take away from
your decision
to operate, or not.
[Kaitlyn] We're gonna do one more question about
radiology.
Do you know
if any doctors,
especially now,
given everything going
electronic with COVID;
are doctors willing to read
those extension and flexion
dynamic MRIs remotely?
And if so, how do you get
that kind of testing read?
[Dr. Henderson] Yeah, I--
I think everyone who does
craniocervical work.
You know, I have two
colleagues
and we all read them remotely.
We do it in conjunction
with the--
You have to look at it
in conjunction
with the whole history.
You can have wildly
abnormal measurements,
and the patient
may not need to have
surgery at all, when you put it all together.
And I know Dr. Bolognese,
and I think everyone
who's doing craniocervical work
reads these things remotely,
even in Europe.
Yep.
[Kaitlyn] Okay, that's great!
That kinda leads into
getting into the surgical questions
we have
because
the first one that I can kind of see
flowing well is:
what are the criteria
for surgical intervention for
say, a teenager,
or an adult with Chiari,
craniocervical instability
and EDS?
Either a decompression, alone
or a decompression/fusion?
[Dr. Henderson] Alright, well.
If they have a Chiari, if it's a small Chiari,
usually they just need
a wide, but a relatively
small decompression--
a foramen magnum
decompression.
If it's a big Chiari,
then they need--
they usually need
a bony decompression.
And usually, opening
up the dura and putting in
a patch to expand the dural space and
allow normal CSF flow,
and take the pressure
there has to be enough space
so the cerebellum--
the cerebellar tonsil is not
pushing on the brainstem.
Now,
if that--
if you're doing a big enough
decompression, with a big dural patch,
there's a significant risk
of a meningocele forming
because you've got
several inches of
of closure
and if you leak just
one drop of spinal fluid
every hour,
in a week you will
have a meningocele.
And if you get a CSF meningocele,
that can
be a major impairment
to a fusion.
The decision to deal with
the Chiari is based upon
the significant headache
attributable to the Chiari,
and the presence of
some neurological findings,
like lower cranial nerve findings,
abnormal gait,
maybe some spasticity.
Those would be the criteria
for doing the Chiari surgery.
Now,
most of the patients
I take care of
have craniocervical instability
as the major problem,
with or without a Chiari.
And so, if they don't
have a Chiari,
then I don't do a decompression.
If they have a small Chiari,
I do a small decompression,
and then a fusion stabilization.
And if they have a big Chiari
then I'll take care of
the Chiari first
and then come back
and do the fusion stabilization.
Now, the indications
for a craniocervical fusion
would be a significant headache--
usually a 7 or above.
Occasionally, I'll do some
someone with a headache
less bad if they
clearly—Well, occasionally, I will take
lesser headaches.
And the presence
of a cervical medullary syndrome.
These are all the symptoms
attributable to
pathology of the
craniocervical junction.
That would be
the headache,
neck pain,
nausea,
dizziness, maybe vertigo,
visual changes,
diplopia, blurred vision,
altered hearing,
tinnitus, ringing in the ears,
choking,
dysarthria, difficulty speaking,
dysphagia, difficulty swallowing,
odynophagia, painful swallowing
altered sleep architecture,
sleep apnea,
abnormal breathing,
weakness and sensory loss.
Oh, and dysautonomia,
which is dysfunction of
the autonomic nervous system,
which are all the
everything that happens
in your body
that you don't have to think about.
It just happens automatically.
Then, on top of the symptoms,
they have to have
demonstrable neurological
findings.
So they should have
numbness,
almost always weakness.
there's usually hyperreflexia,
or spasticity.
Instability of gait;
they may be wobbly.
A positive Romberg,
where they close their
eyes and lose
their balance.
And a number
of other neurological findings.
I want to see,
clear neurological findings
to back up symptoms.
And
they have to have tried
made a reasonable go
at non-operative treatment.
So, physical therapy,
neck brace,
medications,
isometric exercises,
avoidance of those things
that make their
craniocervical instability worse.
Usually
I see patients after they've
been doing this for a year,
or sometimes, ten years. You know?
Before they get to me.
And so,
almost every patients has
already run the gamut
of the non-operative treatment.
But when there's clearly
no logical alternative,
then surgery works well.
But it's not
to be embarked upon
until you've done the
"easy"-- safe, easy things.
[Kaitlyn] You had mentioned
a little bit there that
there's some risk of
CSF leak
if that's not sealed up well.
How--
oh, I lost the question--
[laughs]
How often do you find
CSF leak in patients with CCI,
especially if they're hypermobile?
[Dr. Henderson] I um, you know,
I don't know how many
CCI cases I've done,
but it must be quite a number.
And
I don't remember a--
once in a while if we're
doing a craniocervical
fusion stabilization
and a small Chiari,
there may be a tiny hole
in the dura
and we'll sew it up.
But I don't remember
there being a meningocele
with a craniocervical fusion.
It's pretty uncommon.
But if you were to do
a big Chiari,
then it would be
much more likely. Yeah.
[Kaitlyn] Okay. There's a follow up question. And I think you covered
this last week.
Or, whenever that was.
Is there a particular type
of neck brace that
you recommend?
Does it it need to be
fitted to the individual?
And why a three-week
neck bracing, rather than traction?
[Dr. Henderson] Well,
I think...
Well, first, the brace
I recommend is the
Vista. Vista brace.
And it has a little
clicker on the front,
and it allows you to
adjust the height.
And so,
and the Velcro--
you can tighten up
the vest
to fit a small neck,
or you can
you know,
loosen the Velcro
and accommodate a big neck.
So, I've found that the one brace
appears to fit about
eighty-five percent of patients.
But if the patients have
a lot of temporo-mandibular
joint problems,
then the brace--
the Vista brace--
by pushing upward on the
jaw, on the mandible,
it can exacerbate the TMJ
and cause severe headache.
And for those patients,
a Miami J,
which is more on the side,
is more comfortable.
So, that's the brace that I prefer.
And then the other part
of your question?
[Kaitlyn] Um, why
three-week neck bracing
rather than traction?
[Dr. Henderson] Well,
it's a little hard to walk around.
When I recommend bracing,
I tell people:
put on the brace for two
or three weeks
and wear it night and day.
Take it off for meals,
but otherwise wear it.
But the neck muscles would atrophy
if you don't--
if that patient did not
do isometric exercises.
They must do isometrics,
build up their neck muscles
while they're in the neck brace.
The neck braces allows
for the neck to gradually
get realigned
and settle down
and some of the inflammation
and pain to go away.
Most patients get much better
in the neck brace.
Patients would all feel better
with traction,
but it's a little bit hard
to have someone
walk around behind you
holding your head up
twenty-four hours a day.
We don't--
We used to do a lot
more traction in neurosurgery but
I think, you know, with the hypermobility
disorders,
one of the problems
is that the ligaments are
too stretchy.
So, they feel good when you
stretch them out,
but they don't--
really, we want unstretchy!
We want more stability in the ligaments.
And so,
if patients feel better in traction,
that's maybe one indicator
that they might do better
with fusion stabilization.
But,
when we do the fusion stabilization,
we're not really fusing them
in a state of traction,
we're fusing them in the ideal
state of alignment.
Without stretching things.
So, I'm not opposed to the concept of it,
but it's
in my view,
it's a little impractical.
[Kaitlyn] Kind of
bouncing off that:
there are some questions..
What is the natural history
of CCI
and atlanto-axial instability
without a fusion?
So, the question really is:
is fusion inevitable?
Um, and what are the
non-operative treatments
or therapeutic options?
Especially for pain?
[Dr. Henderson] Well,
Clair Francomano
and Peter Rowe
my colleagues and I
are actually beginning
a study right now to compare
non-operatives with operatives.
But it's still a retrospective.
It's really hard to do a prospective
study because
if someone really needs the surgery,
it would be very cruel to say,
"I'm sorry, I know your pain
is an 8 or 9 out of 10,
and you feel like you're going to
stop breathing every other night
and every week you feel like
you're having a mild stroke and you can't work and you can't do anything but we're keeping you in Group B, you're the experimental control group. And in a year or two, we'll get to you." You know? I mean, that'd be a little cruel. So, it's really hard to to do a prospective study.
But most of the patients I see
they weren't diagnosed yesterday--
I mean, they may have been
diagnosed a few months ago,
but they've been dealing
with this problem for ten--
or, you know, somewhere between
one and ten years already.
So, I think that
the very--
My guess I that
say 100 people with severe
craniocervical instability
who might need surgery,
let's say who met the
criteria for surgery:
if you didn't operate on them,
then maybe over time,
ten percent would
recover...
And I'm taking about
over a few years;
maybe 10% might recover
without us to do something useful.
And I would think the other
ninety percent would all be
on disability and live in misery
for the rest of their lives.
That would be my expectation
based on
the 2,000 odd patients
I've looked at.
[Kaitlyn] Okay.
What are the non-surgical treatments to kind of get you until you get to that state where you need a fusion? So, physical therapy? There's actually a question that came up about cell regrowth, like stem cell therapies and stuff like that. I wonder if you have any thoughts?
[Dr. Henderson] Well,
the basic things are: first,
physical therapy.
Not range-of-motion; we don't want to increase
range-of-motion, we want
physical therapy to
stabilize the spine
from the lumbo-sacral junction up.
Your posture begins
down in the base of your spine.
And it begins by
pulling in your core muscles,
you know, in the abdomen.
And then it proceeds up
the thoracic spine
and up to the cervical spine.
So,
only a physical therapist
knows how to--
or maybe some
physiatrists and
some osteopaths--
know how to correct the spine
from the base, up.
Secondly,
they need to strengthen the spine.
Build out the muscles
around the spine.
Thirdly, they need to
avoid all those activities
which are damaging
to the ligaments
in the neck.
Like any contact sports.
Anything from rollercoasters
to bumpy boat rides
or bumpy car rides.
You know?
Or, even getting bumped into
on a dance floor.
Or sitting in front of
a computer with the neck
flexed or extended.
Or, looking at the cell phone
too much.
Or, playing games-- computer games.
These are all things
that are very
deleterious to the spine.
So,
the patient needs to adopt
a perfect posture.
And then,
these patients have
premature degeneration
of all the discs in the neck.
And so, it's not just
the craniocervical junction that's---
it's also the levels below.
But most of the mobility is at
C1,2 and cranium,C1.
They're the hardest hit levels.
So, they need to then
begin to
keep their neck
in a good position
at all times.
And not be bobbing their head around
and twisting and turning
and looking down at a cell phone.
They need to stay in perfect posture
while they're awake,
sitting, studying, driving,
sleeping.
You know, sleeping on their side
with a pillow at their shoulders.
Then they need to pay attention
to good nutrition.
Strengthen their bones and ligaments.
Maybe Vitamin D.
Maybe Calcitonin.
Calcium citrate.
A modicum of exercise every day
to maintain muscle tone and posture
and help the tissues.
The isometric exercises
as I have mentioned.
Now, there are
new things like "PRP".
There's a guy in Colorado
who injects
the ligaments through
the back of the mouth
and that can thicken the ligaments
and toughen them up.
I'd be very afraid of an infection,
but I presume he doesn't
have many infections
or he wouldn't still be doing it.
Every neurosurgeon
has tremendous respect for
the craniocervical junction.
And so,
sticking needles in that part
of the anatomy
we think would be pretty dangerous
because the vertebral artery
can have unusual
loops and whatnot.
But those are the main
things we do
to--
for every patient prior to
considering surgery.
Surgery is the last option
when everything else fails.
And, in general, if a patient can still be working and doing the things he has to do, then he should not have surgery. It's when when they 're clearly going downhill and my goal is to get people back to the workplace, not on chronic disability, but back to work. And we accomplish that in about-- two thirds of patients. Back to school, back to work. Whichever.
[Kaitlyn] Mhm.
Kind of to that point:
we had a question submitted prior
and there was just a follow-up.
Do you have any recommendations on
how to basically,
do those day-to-day activities
like you're saying, without doing too much
flexion or extension on your head.
So, while you're sleeping, is there a particular pillow that would be soft enough, but hard enough and maybe another way to prevent you know,
with the typing. All that.
[Dr. Henderson] Well,
the MyPillow things--
they really work. MyPillow.
[Kaitlyn] Oh, wow! Okay.
[Dr. Henderson] And
this is not a paid advertisement.
[laughs]
The um,
and getting in the habit.
You can get in the habit
and discipline yourself
to sleep correctly at night.
On your side.
And to turn every few hours.
So, that's something you can
form the habit of sleeping correctly.
You can form the habit of standing and sitting. And don't turn your head. You look at Grace Kelly. Princess Grace. Perfect posture, all the time. You look at Melania Trump. Never see her slump, or even turning her head. She's perfectly-- perfect posture all the time. Even if she's standing there reviewing the troops for an hour and a half. And the queen is not bad, either. Queen Elizabeth.
Alright, so. Discipline. And no foolishness of activities that you might get injured.
[Kaitlyn] Okay, so... these are kind of CCI-specific.
In a patient with EDS and CCI or AAI there was bilateral compression of both internal jugular veins. And they developed collateral veins-- um-- and has developed collateral veins, as well. Does that kind of patient require craniocervical fusion? Or, are those collaterals going to be surgically an issue?
[Dr. Henderson] I'm very careful
to pay attention to
the venous anatomy when I operate.
We try to spare-- to preserve all the veins because the blood flow out of the brain flows--
half of it goes out the jugular veins
and half goes through all the other veins.
The veins around the spine.
And so,
if you go in and you
trash all the veins
in surgery then
actually, probably,
that's one way you
can get more headache.
That's not been demonstrated.
That's conjecture on my part.
I think we used to think of
all the blood flow
coming out of the brain
through the jugular veins,
but actually,
blood flow comes out
the jugular veins at night
when you're lying supine.
And during the day,
more of the blood flow
comes down through the spine
and the other veins.
And I think the reason for this
might be that jugular veins
can be quite large.
And if they were just open,
if they were just wide open,
then blood flow would
drain too quickly from the brain
and that could cause problems.
I suspect that there has to be a little bit of resistance to preserve a normal pressure in the brain and not have and not have this... If every time you stand up, and your jugulars were wide open, then you would overdrain and you might get a headache. A spinal headache. So, when we stand fortunately, more blood goes through the non-jugulars.
When we do angiograms, CT-angiograms, a lot of the time, we find that one jugular or even both-- they look compressed. When you send them for an angiogram, you're not looking at a snapshot, you're looking at the flow over a few minutes. Actually, the flow is much more normal. So the CT-angiograms and the MRVs can be misleading in that respect.
People who have CT-angiograms
for whatever reason--
a third of patients have--
show that compression.
But only a third of those
have symptomatic--
are believed to be
symptomatic due
to that compression.
So, you have to be very…
Looking at the veins
is a little bit of
a rabbit hole.
You know?
Because it's--
it can be a little misleading.
Now, that said,
in the brain,
I've had many patients
who presented with
acute deterioration
and then we found
blood clots
in the major draining veins
of the brain,
the so-called
draining "sinuses",
the transverse sinus,
the superior sagittal sinus,
the straight sinus,
and so on.
They're blood clots
and they can cause
acute, you know,
alteration of behavior,
memory. Or, a patient
can become comatose.
And if you had significant and permanent stenosis of the jugular veins,
that could also cause
major problems in the brain.
And it could cause
increase in
venous pressure in the brain. And that increased
venous pressure
will then cause
intracranial
increased pressure--
otherwise known as
intracranial hypertension,
or pseudotumor cerebri,
where it seems like
there's a tumor in the brain
because there's pressure.
However,
if you have
jugular venous compression,
blood will always find
another way of getting out
and over time,
the patient will develop collaterals.
So, all the other veins
in the head will increase
and blood will drain out of those
over time.
And the jugular veins will
become less important.
And there's a
time thing here. It's a very-- it's a very complicated--
[Kaitlyn] Yeah [laughs] Definitely. Vascular neurosurgery is kinda crazy. So, I'll go back to fusion for a second. There have been a couple of questions that came in about tethered cord and fusion. So, if someone needs-- oh, I'm sorry!
Decompression/fusion or tethered cord and fusion.
This kinda came up last time as well.
Is there an appropriate
order in which those things happen?
One of these patients had their
tethered cord released
and realized that the neck pain--
I'm sorry the neck instability
got worse, subjectively.
So, is there a specific order
in which this should really happen?
[Dr. Henderson] Well.
A general rule for neurosurgeons is
you start at the top
and go down.
So,
all things being equal,
you do the craniocervical
instability, first.
And that's my preferred strategy.
And because, once in a while,
the tethered cord symptoms
will get better
once you fix the neck.
Now,
in some patients,
the craniocervical instability
is moderate,
but their tethered cord symptoms
are really severe.
And in those patients,
it would seem logical to do
the tethered cord, first.
And in some cases,
they may say, "Hey, my headache actually
got better after
we untethered the cord."
And so, even though they may
have some craniocervical instability,
we might hold off on that operation.
So,
you do the worst problem, first.
But other things--
all things being equal,
you do the neck, first,
and then the tethered cord.
[Kaitlyn] And so,
in the case of a decompression,
that's higher,
so you would do that first?
Or, no?
[Dr. Henderson] Right. Yes. Well, a decompression
of the Chiari, you mean?
[Kaitlyn] Sorry! Yeah, yeah.
[Dr. Henderson] Yeah, right.
[Kaitlyn] Um, there's a question
kind of specific about
duraplasty
and what kind of tissue you use
to do that duraplasty
and why you came to that decision?
[Dr. Henderson] Okay, well.
You can get
fascia from the back of the skull
and you have to do
a big, ugly incision
and, you know, shave
an extra four inches of hair.
Um,
and I don't think anyone
would like that.
Or, you can get the fascia
from the thigh.
The fascia lata.
And that works very well.
People don't seem
to have problems with
the thigh incision.
And that fascia
is more difficult to use,
to sew in,
but it does--
it grows into the dura
really well.
And that probably gets
the best seal.
Or, you can use
a bovine dura,
pericardium-- the heart [from a cow].
And um,
and that's much easier
to handle.
It's much easier to handle.
Much easier to sew.
You don't have to worry
about the thigh incision.
But, sometimes
the ethylene glycol
that's used to preserve
the dura can cause inflammation.
I operated on a princess
of a Middle Eastern country once
and, of course,
she's the one who had
a chemical reaction to the--
And so, her strategy
was tongue-in-cheek.
When I told her this
was a chemical reaction
to the ethylene glycol
or to the bovine dura,
the next day I came in
and she's saying, "I love cows,
I love cows, I love cows!"
[laughter]
[Kaitlyn] Just reminding herself!
[Dr. Henderson] And then, there's this so called [synthetic] dura, but...
I think the best is the fascia lata
from the thigh,
but the--
the bovine
works very well, too.
And that's probably
a little bit more standard.
[Kaitlyn] Um,
specific to Chiari--
Chiari 1--
so, say that's all
the patient would have
they have occasional headache
and they're not sure
if the symptoms are
necessarily
Chiari symptoms.
Is surgery done
in a case like that?
Or, only when it's impairing
quality-of-life?
Or, should
would someone have
a decompressive surgery
especially on a child
or an adult-- I guess, either--
um,
sort of prophylactically
just in case the symptoms
get worse?
Or, do you just watch and observe?
[Dr. Henderson] Yeah,
I don't...
we don't generally do
neurosurgery prophylactically.
We don't do it
in anticipation of possible problems.
Number one.
Number two:
there are so many causes
of headaches.
Like, if you have EDS,
you can have a headache
from mast cell activation syndrome,
from temporomandibular joint
problems,
from instability at C2,3
or C1,2
or C2,3
or C3,4
or even C4,5.
Tethered cord syndrome.
Headaches from TIAs.
A hole in the heart.
From abnormal vasculature
From...
[Kaitlyn] The list goes on.
[Dr. Henderson] Yeah. There's a legion of things.
If you're going to operate
for Chiari,
there ought to be
typical symptoms
and a headache that's significant.
You know,
at least a 6 out of 10,
preferably 7 or more
out of ten.
And it's interfering with
what they need to do.
If they can just take
an ibuprofen and that's it,
then probably not worth
having the surgery.
But if they can't continue
the work that they have to do, then it’s really interfering with the
progress of their life.
And if they have both
the symptoms and some neurological findings, congruent findings, then that's--- We always want to see the pain-- pain and disability.
Symptoms, findings,
congruent radiological findings,
if they all go together,
then that's when we operate.
[Kaitlyn] Okay.
This is more of a
spinal question.
Is it common for scoliosis
to start developing
later in the teenage years,
even if it was never
present before?
[Dr. Henderson] Right, yeah.
Well, tethered cord can cause scoliosis to develop
in teenage years,
or even later.
Could you excuse me for one second?
[Kaitlyn] No problem! We'll wait a minute.
[silence]
And just so everyone knows,
there are a
ton of questions
and I'm trying to get
to all of them.
I really do promise!
[laughs]
I will say--
some of these questions,
Dr. Henderson does go over
in his most recent presentation--
the Q&A--
so I recommend that you--
if you're bored after this,
you can go on to our YouTube.
It's there.
And you can take a look at that
presentation, as well.
[mumbling]
Ooh! The YouTube channel name!
That, I can do.
So, you go to YouTube.com, slash...
it's "CSFinfo2007".
So, I'll put it in the chat.
https://youtube.com/csfinfo2007
So, that's our YouTube channel...
And we have all of our videos,
going back to,
I think,
2009
on there.
So, there's literally,
I want to say days
worth of videos to watch
if you're bored
and, still, some of us are
quarantined.
So, if you're looking
for something to do.
You can definitely go in there
and take a look.
Hello, Dr. Henderson!
Alright, um.. Oh!
This is a good question:
Do you think that a
tethered cord release
following a decompression
and duraplasty
would have a positive effect
on a very large syrinx
that continued to persist
after that decompression?
[Dr. Henderson] No.
[Kaitlyn] No? [laughs] Okay.
[Dr. Henderson] I think it can affect syrinx in the lower thoracic spine, but persistence of a syrinx in the cervical spine, to me, and to others like [inaudible] suggests ongoing instability or basilar-- at the craniocervical junction,
or basilar invagination,
or a
a severe kyphosis
of the brainstem.
[Kaitlyn] Okay.
This is an interesting question, as well.
Can numbing in the legs
be caused by a Tarlov cyst?
The patient isn't able
to find a physician
that's comfortable to them,
do you have any recommendations?
[Dr. Henderson] Tarlov cysts usually cause pain in-- it's a little bit more dermatomal.
Although, it's quite possible
that, over time,
the autonomic nerves
can become involved
and the pain may be
non-dermatomal.
But usually, a Tarlov cyst,
if it's on the S2 nerve,
then it will be pain
in the back of the thigh.
There might be pain on the
front of the leg.
Secondly, Tarlov cyst
is almost always-- they're best lying down
and worst sitting up.
When you're sitting up,
there's a higher--
a greater
column of pressure,
water pressure,
in the spinal column
pushing down,
increasing the pressure
on the Tarlov cyst,
causing more pain.
So, that's...
They're very
specific.
And
Tarlov cysts usually
cause urinary symptoms
or often cause
urinary symptoms,
although, I'm not sure
exactly why.
And um,
but they do not cause
weakness.
Of course, they may
cause numbness.
The lower sacral dermatomes,
especially S2,3,4.
But they do not cause weakness.
[Kaitlyn] Okay. There's an interesting question. This is an older patient,
about sixty-six.
They had a really rough time,
earlier on with Chiari,
scoliosis, syringomyelia.
Um,
she really has, like,
a comment and was curious
to hear your thoughts
on it.
She-- I said she like
I know it's a woman,
I have no idea--
"I went vegan a few years ago,
and the pain seems to have
disappeared."
And then recently,
they had undergone
more facet locking--
joint locking situations
and had some steroid injections
but she was--
she or he--
was wondering if
they should be seeing
a physiatrist or,
what do you think
in that kind of situation?
And if the--
diet had changed
some pain
symptomatology?
[Dr. Henderson] Okay,
I'm a little
confused here.
So,
They've got a syringomyelia?
[Kaitlyn] Chiari, scoliosis,
syringomyelia,
they'd undergone shunting and
a lot of different surgeries.
What-- the patient wound up
doing some walking,
and doing some exercise,
went vegan-- changed the diet.
Is there anything else, I guess,
that you would recommend
to fix some
more joint locking situations
that are going on?
[Dr. Henderson] Yeah, well..
See, here..
A lot of the things you mention
occur with Ehlers-Danlos syndrome.
Either they're comorbid conditions
that are happening
at the same time...
If you have--
[pause]
mast cell activation syndrome
that can be affecting
all of your joints.
Bladder function,
brain fog,
pain level,
and everything.
And so,
altering the diet
and activities
may be improving
those comorbid conditions.
They're the real Joker in the pack.
You have to identify
all of the problems
and not simply assume
that the problem is due to
the Chiari, or the syrinx,
or craniocervical instability.
You have to look
for all the other issues.
The nonsurgical treatments
are really important.
You know, diet…
Sometimes people
have allergic
symptoms
to the air,
to the water.
Mold in the house.
These are gonna have a huge, huge impact on the patient.
That's one good thing
about osteopathic doctors.
They're little more thoughtful
about all the other things, whereas medical doctors
are thinking about
you know,
what drug can I give this patient
to get them out of my office? [laughs]
Or, what test can I order
so they'll leave
and get the test
and leave me alone, at least,
for a moment?
Whereas osteopaths,
you know,
they think about these things.
[Kaitlyn] I'm gonna try and take two more.
So, one of them is:
are there any suggestions
that you might have,
surgically or non-surgically,
in treating
a teenager that has
some dystonia localized
in the neck,
with head and shoulder tremor?
The patient also has Chiari
and is hypermobile.
And unstable in some cases.
[Dr. Henderson] Yeah, um,
yeah, these...
tremors are--
that's kind of a very
non-specific term.
And I saw
someone today remotely.
She had--
if she just touched her leg,
it would start tremoring.
And that was,
I think,
spasticity.
You know, increased tone.
That poor girl
walked into see
doctors in Florida
and said, "Hey, I think I've got
craniocervical instability
and Ehlers-Danlos syndrome."
And they told her to go
see a psychiatrist. You know? And that's exactly what she had.
And she had all the neurological
findings, and everything
to go with it.
Dystonia--
dystonia is another very
nonspecific term
and there are a hundred
causes of dystonia.
But,
instability at the craniocervical--
you know, instability
in the neck--
or something that's irritating
the nerves there
of the neck
or the brainstem
can, I think,
cause dystonia.
So,
the dystonia
could be coming
from up in the brain.
It could be a seizure.
Or, it could be some
irritation of the nerve
at the cervical level.
And so,
I'd be inclined to
treat that with a--
see if a neck brace helps.
And [medications like some benzodiazepines. And some]
anti-pollenergic drugs.
There's a whole host of them [that may] treat dystonias.
But they can be
quite difficult to deal with.
And you know,
there's something called
"torticollis",
which is
where the neck is literally--
the muscles become
contracted and
and the neck is completely
bent over.
And these can be very difficult
to deal with.
So, they're tough,
tough problems.
[Kaitlyn] I--
we're getting so close
to it being very late.
So, I'm just going to ask
one last question.
I know we had extras,
but I promise next time!
So, I think it's a good one
to end on, though
because it's really about
thinking about how we look
at this systemically.
[Dr. Henderson] I don't want any political questions!
[Kaitlyn] [laughs] It's only a tiny political.
[Dr. Henderson] No systemic-- [laughs]
[Kaitlyn] How do we best approach
our local health providers
who are not up-to-date
on certain-- Chiari, CCI, or
EDS situations?
And really, why is this information
not making it to certain neurologists?
Because they're really--
and GPs, really--
they're the first
line of defense
for these problems.
So, how do we get out there more?
[Dr. Henderson] Um,
I won't say
ignorance, arrogance, stupidity.
[laughs]
Um, the
But, I mean...
You know,
we didn't learn a lot about it
in medical school.
Probably the
[clears throat]
the best thing is to--
you don't want to offend
your medical provider--
But
if you can come in
and say, "Hey, I did a little
research and I have to say,
this fits my symptoms
exactly, and I brought you
an article on this."
And if they're a good doctor,
they'll say, "Oh, okay,
I'll read that."
And they'll be interested
in learning about it.
But,
if you approach them well,
and they're not willing to
learn something about it,
and they just--
then, find another doctor.
I think doctors
have this sense
that they learned everything
in medical school
and there's nothing
much more to learn.
[Kaitlyn] Yeah, I think
it's the cognitive dissonance
that-- how much we really
don't know about the body.
Especially the brain
and the central nervous system.
I think it's just
they don't want to deal.
[Dr. Henderson] Yeah, I mean...
the reality is,
most doctors their knowledge
is... two questions deep.
Say, "Well, why is this?"
They'll give you an answer.
Say, "why is that?"
They might give you an answer.
Then, “and why is that?"
And you've already hit
the depth of their knowledge.
[Kaitlyn] Yeah.
[Dr. Henderson] But um,
something like Ehlers-Danlos
syndrome--
I didn't diagnose my first patient
until 2010.
And someone in my office--
one of the nurses in my office.
I know, in retrospect,
I've been dealing with
these patients at Georgetown
and I was intrigued by them,
but I didn't know--
I couldn't put it all together.
But in this case,
I had a nurse who was
a champion swimmer.
And she came to me
with a back problem.
And while I was examining her,
you know, for the back,
I noticed a bruise on her thigh
and I said, "What's that from?"
She said, "Oh, I bruise easily."
Then I noticed a scar
on her leg.
And she said, "Oh, well
I had a knee problem."
And I thought,
Why would a champion swimmer have a knee problem?
You know?
It must be a joint thing...
And then I thought,
joint, bruising...
and I thought,
This sounds like one of those connective tissue disorders.
And I thought--
"Ehlers- something or other"
And I looked it up.
And then I went
and I went to meet
Clair Francomano
who is an expert on this
and she said, "Hey, can I
send you patients?"
And I said sure!
So, that's the first time
I'd made a diagnosis.
And it was just remembering
a five-minute portion
of the lecture,
you know, 25 years before.
[Kaitlyn] Yeah.
[Dr. Henderson] And so,
most doctors
what they see is
more circumscribed
than what I see.
And they're less willing
to expand their
expand their portfolio...
[Kaitlyn] Well,
that story is super inspiring,
I think,
to everybody on the phone
because--
all it took was one patient
and look where you've come--
what is this?
What year is it?
2020-- Ten years later!
[laughs]
So, all it takes
is one patient to come in and say, "I need help."
[Dr. Henderson] Yeah.
[Kaitlyn] Alright! Well, that was great!
[Dr. Henderson] Kaitlyn, thank you for organizing all of this. And, Dorothy.
You all--
you all have done
so much for the knowledge factor.
You all are the hinges
of this whole thing.
We would still be
in the dark ages,
if you will.
And you too, Cathy!
[Kaitlyn] You too!
[Dr. Henderson] Alrighty. Thank you. Alright. Bye bye, everybody.
Revised: 7/2020