Dr. Deborah Friedman—Clinical mimics: Post-Traumatic Headache, NDPH, and Occipital Neuralgia
Dr. Deborah Friedman—Clinical mimics: Post-Traumatic Headache, NDPH, and Occipital Neuralgia
Dr. Deborah Friedman, Neuro-Ophthalmologist and Headache Medicine Specialist, presented this talk on “Clinical mimics: Post-Traumatic Headache, NDPH, and Occipital Neuralgia” at the 2023 Cedars-Sinai Intracranial Hypotension Conference on July 8, 2023. The conference was hosted by Cedars-Sinai with generous support from the Spinal CSF Leak Foundation in Kohala Coast, Hawaii. For a full transcript of the video, visit: https://spinalcsfleak.org/2023-intrac…
Content
9.54 -> Thank you Chris for that very generous
introduction and thank you, Dr.
12.81 -> Schievink and crew for
inviting me to speak.
15.639 -> It's wonderful to be here in person.
18.61 -> I think you will all agree with that.
21.95 -> My only relevant disclosure is that I'm
on the medical advisory board—and for some
27.439 -> reason I didn't touch anything and it's
moving— for the Spinal CSF Leak Foundation
32.76 -> and I got some grant support,
which I'll be talking to you
35.52 -> about tomorrow from them.
37.59 -> Okay.
37.865 -> So I was asked to talk about CSF mimics.
40.495 -> There are a few that are on the title of
the talk that was sent out to you, and
43.585 -> I'll cover them, but then I'll get to the
ones that I really think are the mimics.
47.345 -> So the first one that I was asked to
talk about was post-traumatic headache.
50.895 -> And I'm not so sure that the
mimic is post-traumatic headache.
54.655 -> I think it's more of like
post-concussive syndrome.
57.695 -> But post-traumatic
headache is pretty common.
60.375 -> And it's divided into three categories,
either traumatic brain injury
64.225 -> whiplash injury, or craniotomy.
67.335 -> The presence of headache is inversely
correlated to the amount of the trauma.
72.645 -> So most people with craniotomies don't
get headache, but a lot of people who
76.395 -> have whiplash injuries do get headache.
78.935 -> The relationship and the
timing of the headache to the
81.395 -> trauma is very controversial.
83.525 -> So the ICHD criteria specifies that it has
to occur within seven days of the injury.
89.595 -> But then there are others who think
that this is a little too restrictive.
94.54 -> And maybe we should even allow up to
three months after the injury called
98.57 -> delayed post-traumatic headache.
100.59 -> It specified that if the person has
a pre-existing headache disorder,
103.935 -> this headache is different.
105.41 -> There's no defined headache phenotype
and no defined headache frequency.
111.045 -> So in theory, somebody could walk
in with a headache that occurs
113.655 -> once every two or three weeks and
say, This is from the trauma which
117.77 -> opens a whole other set of doors.
119.815 -> About 30 to 60% of people have persistent
headaches after a traumatic brain injury.
124.795 -> It's considered acute if it
resolves in three months.
127.29 -> Persistent if it goes on for
longer than three months, which
130.465 -> occurs in up to half of the cases.
132.915 -> And when you look at the literature
in this, it's primarily based
135.945 -> on case studies because most
people never seek medical care.
139.495 -> So these are the official
criteria from the ICHD about
142.74 -> acute headache attribute to TBI.
145.265 -> If it you're thinking post-, again,
persistent is just longer than three
148.594 -> months, but it's the same headache.
150.844 -> So the ICDH3, you have to keep
in mind, defines the headache.
156.064 -> It doesn't — they were not really meant to
define the condition causing the headache.
160.974 -> So you have to have trauma.
162.684 -> The injury has to be to the head and
or the person regained consciousness
168.144 -> following the injury to the head.
169.694 -> Or the medication that was
impairing their ability to report
174.734 -> the headache has to be present.
176.544 -> One of the three as far as the onset,
and then either one of the following, the
180.834 -> headache resolved within three months,
if it's acute or, more than three months,
184.294 -> if it's persistent or you're seeing the
patient hasn't been three months, but
189.175 -> their headache is still persistent.
190.965 -> And then again, every single
secondary headache disorder in the
194.595 -> international criteria for headache
diagnosis is a get out clause.
199.265 -> Not accounted by any other diagnosis.
202.115 -> Okay.
203.165 -> Then they have a headache that's
attributed to mild TBI to the head.
207.344 -> And it's basically just the inverse.
209.614 -> The headache should not have occurred
after some major head injury causing.
214.494 -> a low Glasgow coma scale or
prolonged loss of consciousness.
219.094 -> It has to be associated
with one of the following.
221.559 -> In number two, you can see confusion,
disorientation, impaired consciousness,
225.494 -> or anterograde or retrograde amnesia.
228.874 -> And then here's where it gets
tricky for us, two or more of the
231.879 -> following, suggestive of a mild TBI,
nausea, vomiting, dizziness, vertigo
238.499 -> imbalance, trouble with memory.
240.48 -> Okay, so this is where it starts to
sound like it could be a CSF leak
244.899 -> which we know can start after trauma.
247.029 -> Okay.
247.479 -> What about post-concussion syndrome?
249.979 -> This is most likely to occur in females.
253.494 -> People who have either a personal
history or a family history of
257.424 -> headache or migraine, a history
of prior concussions and a history
261.534 -> of mood disorders, and this is
substantiated throughout the literature.
265.344 -> The phenotype of post-concussion headache
sounds most like migraine by far and away.
270.594 -> Next up is tension type headache.
272.854 -> Then occasionally we'll see someone that
has cluster headache after a concussion.
277.614 -> It's usually a bilateral headache
and it can be moderate to severe
281.334 -> in intensity, and it's treated just
like headache with either acute
285.534 -> or preventive treatment as needed.
288.004 -> But the other things that come along
with it, again, are the things that
291.124 -> make us start wondering, is this a
leak or is this from a concussion?
295.274 -> So cognitive difficulties
are pretty common.
297.754 -> Trouble sleeping, cervical injury, people
start talking about their neck pain.
302.314 -> Maybe that's a leak.
303.554 -> Vestibular and ocular motor problems.
306.134 -> Now, we don't see ocular motor
problems too often a leak, but vertigo
309.494 -> and dizziness are common after TBI.
312.194 -> And then the psychological ramifications
of which the things that predict the
317.544 -> post-concussive syndrome at three
months are a pre-injury, psychiatric
322.044 -> history of either depression or
anxiety, acute post-traumatic stress
326.159 -> that's manifested within five days of
the injury, other life stressors, and
330.719 -> pain, resilience, and coping styles.
333.599 -> Then we get to whiplash.
335.219 -> Now whiplash is a big mess.
337.209 -> So whiplash, acceleration, deceleration
head movement with flexion and extension
342.339 -> of the neck, it is very common,
much more common than CSF leaks.
347.479 -> And following whiplash injury, 60% of
people report headache within a week.
352.719 -> 23% at three months, and still
pretty stable, 30% at six months.
357.619 -> You can see on the graph down there,
the green is neck pain and the kind
361.314 -> of burnt orangy color is headache.
364.159 -> Going from seven days to
12 months after injury.
367.639 -> So this kind of plateaus out after time.
370.489 -> Most common causes being rear-ended
in a motor vehicle accident.
374.029 -> It is the number one reason for
personal injury, compensation claims and
378.679 -> malingering after a traffic accident.
381.059 -> So it really is a big mess in a
meta-analysis again of data that are not
386.349 -> all that great, neck pain occurred in
about 84% of people within seven days.
392.184 -> Declined to about 40% after a year.
395.004 -> And then headache again went to from
about 60% to roughly 20% or so over time.
402.664 -> So the literature's really
weak about whiplash.
405.039 -> There's not a lot you can say.
407.404 -> Headache is more prevalent with
those who had pre-existing headache.
410.599 -> Then there's this classification
of the grade of whiplash and which
415.359 -> is used by people like in rehab
medicine who take care of these folks.
419.709 -> So what is it?
420.579 -> I'm gonna end every of the
individual diagnosis with
423.399 -> like, how do we sort this out?
425.169 -> Okay.
425.979 -> History of head or neck trauma.
427.439 -> To have a TBI, you have to have
head and neck trauma, right?
430.359 -> CSF leak.
431.269 -> We sometimes see that too.
433.009 -> History of surgery.
434.879 -> TBI can occur after having brain
surgery if they had an open fracture.
439.819 -> CSF leak can happen after a spine surgery.
443.169 -> The headache is usually
migraine, like with a TBI.
445.989 -> It can sound like anything in a CSF leak.
449.569 -> Neck pain, common in both vestibular
symptoms, common in both, but
452.989 -> cochlear symptoms I think are
pretty well isolated to A CSF leak.
457.969 -> Cognitive dysfunction common in both.
460.314 -> Sleep difficulties also
common but different.
463.254 -> So TBI, it's usually insomnia
and with leak it's usually
468.025 -> nocturnal awakening from pain,
470.034 -> if they get that.
471.244 -> Mood and behavior, that
doesn't really help us.
473.759 -> And the MRI of the brain, it
should be normal in a mild TBI.
477.94 -> And hopefully it'll help us out
and be abnormal in the CSF leak.
481.909 -> New daily persistent headache is
considered a primary headache disorder.
485.729 -> It arises outta the blue.
487.319 -> There's no other cause for it.
489.329 -> The ICHD require that the onset be
distinct and clearly remembered.
494.589 -> And within 24 hours of that onset of
headache, it's persistent and unremitting.
500.14 -> Has to last for at least three months.
501.789 -> And again, no other cause.
503.845 -> It usually resembles either
migraine or tension type headache.
507.875 -> And there are two types.
508.805 -> One is self-limiting
and one is refractory.
511.175 -> So SIH is actually considered
a cause, a secondary cause, of
515.8 -> new daily persistent headache.
517.935 -> And whenever we see somebody in headache
medicine who has a new daily persistent
521.54 -> headache, The first thing we do is we
start looking for a secondary cause.
526.365 -> So how do we tell 'em apart?
528.355 -> NDPH has to have a clearly recalled onset.
531.585 -> Many people with CSF leaks also will
recall exactly when it started.
535.85 -> Thunderclap onset is possible in
either, orthostatic headache is pretty
540.295 -> much only CSF leak, and all those
other symptoms are only CSF leak.
544.615 -> So NDPH is just, it's just a headache.
547.475 -> Not other neurologic symptoms.
550.155 -> I don't think occipital neuralgia
is all that difficult to
552.865 -> distinguish from a CSF leak.
554.515 -> Maybe your experience is different
but occipital neuralgia, in order
558.155 -> to make a diagnosis you're gonna
have to have paroxysmal attacks,
562.305 -> lasting seconds to minutes.
563.995 -> CSF leak headaches really don't do that.
566.48 -> Severe intensity shooting, stabbing sharp.
569.55 -> And then both of the following, either
it's uncomfortable or it's painful
573.005 -> to touch the area in the back of
the head in the distribution of one
576.94 -> of the nerves involved, or there's
tenderness over the affected branches.
582.18 -> Or there are trigger points at the
emergent emergence of the greater
585.85 -> occipital nerve or at the C2 distribution.
589.23 -> The pain is eased temporarily by a
local anesthetic block and again, not
593.82 -> accounted for by any other diagnosis.
596.4 -> And I see actually this diagnosis
I think overdiagnosed in
600.46 -> general, in the headache world.
602.09 -> People with migraine and other kinds
of headaches, they have tenderness.
604.88 -> You press on their head, it hurts.
606.67 -> You press on their great
occipital nerve, it hurts.
609.21 -> And that doesn't mean they
have great occipital neuralgia.
611.82 -> The phenotype of the headache has to fit.
614.57 -> The pain can sometimes extend to the
front of the head on the ipsilateral side.
618.96 -> And you have to distinguish it
from pain that's arising from
622.38 -> the upper cervical region.
624.31 -> So again, overdiagnosed in the headache
world, there are a lot of things that
627.975 -> respond to anesthetic blockade, which
are not occipital neuralgia, including
632.78 -> migraine cluster cervicogenic headache,
and even post-dural puncture headache.
637.995 -> So which one is it?
640.005 -> Occipital pain?
640.935 -> Yes, it happens in both,
but the characteristic of
644.305 -> the pain is very different.
645.985 -> So in occipital neuralgia, we're
looking for brief paroxysmal attacks.
650.64 -> In CSF leak, we're looking
for orthostatic headache.
654.17 -> In occipital neuralgia, it's
usually stabbing shooting.
657.96 -> That's not very common in A CSF leak.
660.3 -> I won't say it never happens, but
I don't think it's very common.
663.72 -> Anesthetic relief is a hallmark
of occipital neuralgia, but it
668.24 -> can also happen with a CSF leak.
670.53 -> And again, other neurologic symptoms,
which I think is really gonna
673.71 -> help you and the cervical imaging
really doesn't help you at all, but
677.31 -> the brain imaging hopefully will.
679.35 -> All right, now let's count down to the
things that are really problematic for us.
684.13 -> And the first, which was
mentioned briefly by Dr.
686.62 -> Wang is POTS.
688.5 -> And this is not so easy.
691.04 -> So POTS, it's a cardio autonomic disorder.
693.77 -> There's no apparent underlying disease.
696.45 -> And it affects one to
2% of the population.
699.03 -> And we usually, it's young
white women that have joint
702.79 -> hypermobility syndromes, including
Ehlers-Danlos, including Marfans.
706.88 -> It's typically associated with
gastrointestinal disorders like
710.74 -> irritable bowel syndrome, and then
other comorbidities like mast cell
714.82 -> activation syndrome and migraine.
718.145 -> So in order to diagnose POTS, you need
to have either a 30 beat per minute
725.6 -> increase in heart rate in adults, a 40
beat per minute heart rate increase in
730.73 -> children, or an absolute heart rate of
at least 120 beats per minute, within
736.94 -> 10 minutes of standing— not twenty; ten.
741.01 -> But you have to exclude secondary causes.
743.2 -> And here's where things get messy.
745.005 -> So the secondary causes include
medications, including a lot of
748.455 -> medications that we use to treat headache.
751.075 -> Tricyclic antidepressants, for
example, beta blockers, for example.
754.845 -> Deconditioning.
756.445 -> And there was a whole s surge of
POTS that occurred during COVID.
760.785 -> And it's questionable whether that
also was related to deconditioning,
764.505 -> dehydration, hyperthyroidism, anemia.
767.535 -> You have to rule out
orthostatic hypotension as
770.215 -> well as cardiac arrhythmias.
772.345 -> So the headache in POTS is, it's common.
775.845 -> At least a third of people
have headache who have POTS.
779.52 -> But you can see the confidence
interval spans the whole gamut
783.02 -> between zero and a hundred, almost so
between two and 70% in the literature.
787.84 -> Orthostatic headache, again, has a very
wide range depending on the report.
794.57 -> So anywhere between two and about 60%
of people have orthostatic headache, but
800.21 -> most people with POTS have non orthostatic
headache and it's usually migraine.
804.95 -> There was a nice case control study
that looked at nine people with
809.12 -> confirmed SIH and 48 people with POTS.
812.44 -> And they found that the things that
were more common with SIH were a
815.95 -> shorter disease duration; presence of
orthostatic headache, which you could
820.35 -> see is highly statistically significant;
as well as neck stiffness, also highly
825.48 -> statistically significant; an older age.
828.97 -> The characteristics that were more common
in people who had POTS were syncope.
834.62 -> And I think syncope really helps you.
836.33 -> Most people with SIH are not passing
out, worsening with menses and the
841.11 -> presence of other myofascial pain.
843.72 -> So next stiffness, as it
turns out, was only present in
846.81 -> those nine people who had SIH.
849.42 -> Now granted this is a small n, but many of
the symptoms were present in both groups.
854.56 -> So there's different kinds of POTS, and
I don't pretend to be an expert in POTS.
859.28 -> But there's hyper adrenergic POTS,
there's neuropathic POTS, hypovolemic
863.12 -> POTS, and then adolescents get
completely different symptoms.
866.47 -> But you can see neuropathic POTS is the
most common type, and you can see that the
871.58 -> major symptoms of neuropathic POTS, some
of them might sound like a leak, right?
877.08 -> Dizziness.
878.54 -> I just feel a little weak, can't
sleep, but then anhidrosis, feet
883.56 -> are turning blue when I stand up.
885.12 -> That doesn't happen during a leak.
886.85 -> Hyper adrenergic POTS, sometimes
our patients not only complain of
890.06 -> headache, but they complain of feeling
tremulous, even if you can't see it.
894.33 -> So there is some overlap in the
symptoms, but it is complicated.
898.815 -> And I think it's really hard to
distinguish these things clinically.
902.475 -> The orthostatic vital signs and
the tilt table test should be done
907.185 -> if you're considering a diagnosis
of POTS, but they're not always
911.055 -> a hundred percent conclusive.
913.175 -> So again, studies have been done
with both people who have POTS
917.86 -> and people who have CSF leaks,
and they did all these autonomic
921.75 -> tests, including tilt table tests.
924.06 -> And first of all, in patients with
POTS, they found that not all of
927.9 -> the patients who complained of
orthostatic headache in real life
931.555 -> developed an orthostatic
headache during their tilt
934.015 -> table test, and then vice versa.
936.865 -> The presence of headache during a
tilt table test didn't correlate
940.465 -> with an orthostatic headache
during regular activities.
943.705 -> Okay.
944.545 -> That's straightforward.
945.565 -> And then SIH patients can also have an
increase in their blood pressure with
951.715 -> head up tilt and increase in heart
rate and heart rate variability with
956.785 -> headache, with upright tilt, and with deep
breathing, identical to people with POTS.
962.495 -> So it's not really a diagnostic test
that separates the two disorders.
968.355 -> And then to complicate the issue even
more, people with POTS, just like people
973.055 -> with SIH, they learn pretty quickly that
they feel better when they're lying down.
976.835 -> And their heart rate's back to normal.
979.605 -> So they get deconditioned.
981.815 -> SIH patients they do
the same thing, right?
984.835 -> And so people with SIH can develop POTS.
989.31 -> So how do you figure this out?
991.2 -> Not so easy.
992.25 -> So I just tried to diagram it a little.
994.33 -> So spinal fluid leak can lead
to physical deconditioning.
998.38 -> Physical deconditioning can lead to POTS.
1001.83 -> POTS can also lead to
physical deconditioning.
1004.83 -> And then you have joint
hypermobility, which can occur
1007.41 -> in either one of the disorders.
1009.33 -> So.
1010.83 -> It's a mess.
1011.85 -> Okay, so what do they have?
1013.77 -> I don't know.
1014.8 -> POTS, CSF leak, or both?
1016.36 -> Okay.
1017.17 -> So headache type.
1019.12 -> Headache type in POTS is usually migraine.
1022.78 -> It's usually either in the front
of the head or the whole head.
1026.63 -> And.
1027.96 -> Who knows how many have
orthostatic headache.
1030.48 -> Everything in POTS almost is orthostatic.
1033.21 -> It all gets worse when they stand up.
1035.29 -> Spinal fluid leak: the headache
can sound like anything.
1038.08 -> It's usually posterior, involves the
neck, but there's no specific location.
1042.88 -> People can even get facial pain.
1044.84 -> Over 90% have an orthostatic
headache at some point with CSF leak.
1049.79 -> Joint hypermobility doesn't
help you: present in both.
1053.48 -> Eighth nerve involvement.
1055.22 -> Similar to the TBI headache.
1058.01 -> People with POTS, they usually
describe lightheadedness.
1062.195 -> They usually don't describe
vertigo and imbalance.
1065.115 -> People with a leak will describe
vertigo, imbalance, and again, those
1068.745 -> cochlear symptoms, syncope, common with
POTS; not so common with a CSF leak.
1075.365 -> Tremulousness, again, common with
POTS, not so much with a CSF leak.
1079.895 -> Abnormal tilt table.
1081.275 -> You have to have an abnormal tilt table
test to have a diagnosis of POTS, but
1085.625 -> it's still possible to have an abnormal
tilt table test and have a CSF leak—maybe
1090.005 -> 'cause those people also have POTS.
1091.715 -> I don't know.
1092.835 -> And this is like one of those things
where I'm like, Please God, make
1096.425 -> the MRI be abnormal, the brain.
1099.045 -> Because otherwise it can
be really hard to tell.
1101.765 -> Next, the one that I think gets
confusing is a Chiari malformation.
1105.675 -> A lot of people in this room are
experts in Chiari malformation.
1109.285 -> They Often are associated with
connective tissue disorders and
1113.245 -> atlantoaxial instability.
1115.115 -> I went online to look on for a picture
of a Chiari malformation, and most
1118.835 -> pictures online now are copyrighted.
1120.965 -> So I ran across this one and I
actually, unfortunately know the
1124.175 -> person who submitted this was submitted
online as a Chiari one malformation.
1128.635 -> I'm like I don't think so.
1129.81 -> Okay.
1130.65 -> But this I think is really
a Chiari malformation.
1133.17 -> The other person has a CSF leak.
1135.72 -> Okay.
1136.29 -> So different types of
Chiari malformations.
1139.38 -> Basically the cerebellar is herniating
down through the foramen magnum.
1143.99 -> If it's a primary malformation, there are
different types of Chiari malformations.
1149.31 -> The one that's usually mistaken for a
CSF leak is Chiari one, and, which can
1154.44 -> occur with or without a syrinx and with
or without obstructive hydrocephalus.
1159.3 -> Secondary causes of this Chiari—which
is not really a Chiari, it's
1163.235 -> tonsillor descent or brain sag
—include intracranial hypotension
1167.1 -> or increased intracranial pressure.
1169.54 -> So people who have idiopathic intracranial
hypertension can have tonsillor descent as
1174.915 -> well as other abnormalities that you see.
1177.475 -> And the official definition is at least
five millimeters of tonsillor ectopia.
1182.57 -> So symptoms of Chiari one, they
arise either from obstruction of CSF
1188.485 -> or from compression of the brainstem
or compression of the cerebellum.
1192.605 -> And it involves the lower cranial
nerves and often downbeat nystagmus.
1197.235 -> And they can also get spinal
cord symptoms and signs related
1201.01 -> to a syrinx, if that's present.
1203.21 -> In the in children, they most likely have
brainstem dysfunction, that if they're
1207.66 -> very young children may just be manifested
as sleep apnea or trouble with feeding and
1213.06 -> headache, which again may be manifested
as like crying and irritability.
1217.78 -> Adults, the pain is usually
it's in the back of the head
1220.45 -> and it's worse with Valsalva.
1221.92 -> That's one of the hallmarks
of the headache from a Chiari.
1225.26 -> Is it orthostatic intolerance?
1226.82 -> Can you have that with a Chiari one?
1228.6 -> Basically, yeah, you can.
1230.31 -> So that kind of messes up the pot too.
1232.81 -> You can have coexisting POTS, you
can have coexisting connective tissue
1236.35 -> disorders with Chiari one as well.
1239.06 -> So you may see all of those
symptoms that we saw in the
1242.57 -> patients who report them from POTS.
1245.33 -> There's not a lot written about that.
1247.37 -> So lastly, how do you tell the difference?
1250.33 -> Chiari one.
1251.4 -> Yes, the headache's
occipital where it has to be.
1254.32 -> But it may be, as with a CSF
leak, coexisting POTS may
1258.58 -> happen with a Chiari one.
1259.99 -> It's not really well reported,
but it's in the literature.
1262.9 -> It's pretty common I think with
patients who have CSF leaks, neck
1267.505 -> pain in both syncope with Chiari one.
1270.365 -> Dizziness in both, but again, Chiari
one is usually lightheadedness.
1273.695 -> And we've already been through what
happens with the CSF leak and this
1277.365 -> time the MRI is definitely gonna
help you the, so you can look for
1280.815 -> all those abnormalities on the brain.
1283.26 -> Hopefully your radiologist will read the
image correctly and realize that it's a
1287.91 -> Chiari one or a CSF leak, but not both.
1290.97 -> So thank you very much for your attention.
Source: https://www.youtube.com/watch?v=jBKRKisGmUg