Dr. Deborah Friedman at the 2023 Cedars-Sinai Intracranial Hypotension Conference
Dr. Deborah Friedman, Neuro-Ophthalmologist and Headache Medicine Specialist, presented this talk on “Clinical Mimics of SIH: 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.
Slides from the talk
View a PDF of Dr. Friedman’s slides here.
Transcript
Dr. Deborah Friedman on Clinical Mimics of Spontaneous Intracranial Hypotension:
[00:00:10] Thank you Chris for that very generous introduction and thank you, Dr. Schievink and crew, for inviting me to speak. It’s wonderful to be here in person. I think you will all agree with that. My only relevant disclosure is that I’m on the medical advisory board—and for some reason I didn’t touch anything and it’s moving— for the Spinal CSF Leak Foundation
[00:00:42] and I got some grant support, which I’ll be talking to you about tomorrow from them. Okay. So I was asked to talk about CSF mimics. There are a few that are on the title of the talk that was sent out to you, and I’ll cover them, but then I’ll get to the ones that I really think are the mimics. So the first one that I was asked to talk about was post-traumatic headache.
[00:01:00] And I’m not so sure that the mimic is post-traumatic headache. I think it’s more of like post-concussive syndrome. But post-traumatic headache is pretty common. And it’s divided into three categories, either traumatic brain injury whiplash injury, or craniotomy. The presence of headache is inversely correlated to the amount of the trauma.
[00:01:22] So most people with craniotomies don’t get headache, but a lot of people who have whiplash injuries do get headache. The relationship and the timing of the headache to the trauma is very controversial. So the ICHD criteria specifies that it has to occur within seven days of the injury. But then there are others who think that this is a little too restrictive.
[00:01:44] And maybe we should even allow up to three months after the injury called delayed post-traumatic headache. It specified that if the person has a pre-existing headache disorder, this headache is different. There’s no defined headache phenotype and no defined headache frequency. So in theory, somebody could walk in with a headache that occurs once every two or three weeks and say, This is from the trauma which opens a whole other set of doors.
[00:02:09] About 30 to 60% of people have persistent headaches after a traumatic brain injury. It’s considered acute if it resolves in three months. Persistent if it goes on for longer than three months, which occurs in up to half of the cases. And when you look at the literature in this, it’s primarily based on case studies because most people never seek medical care.
[00:02:29] So these are the official criteria from the ICHD about acute headache attribute to TBI. If it you’re thinking post-, again, persistent is just longer than three months, but it’s the same headache. So the ICDH3, you have to keep in mind, defines the headache. It doesn’t — they were not really meant to define the condition causing the headache.
[00:02:50] So you have to have trauma. The injury has to be to the head and or the person regained consciousness following the injury to the head. Or the medication that was impairing their ability to report the headache has to be present. One of the three as far as the onset, and then either one of the following, the headache resolved within three months, if it’s acute or, more than three months, if it’s persistent or you’re seeing the patient hasn’t been three months, but
[00:03:19] their headache is still persistent. And then again, every single secondary headache disorder in the international criteria for headache diagnosis is a get out clause. Not accounted by any other diagnosis. Okay. Then they have a headache that’s attributed to mild TBI to the head. And it’s basically just the inverse.
[00:03:39] The headache should not have occurred after some major head injury causing. a low Glasgow coma scale or prolonged loss of consciousness. It has to be associated with one of the following. In number two, you can see confusion, disorientation, impaired consciousness, or anterograde or retrograde amnesia.
[00:03:58] And then here’s where it gets tricky for us, two or more of the following, suggestive of a mild TBI, nausea, vomiting, dizziness, vertigo imbalance, trouble with memory. Okay, so this is where it starts to sound like it could be a CSF leak which we know can start after trauma. Okay. What about post-concussion syndrome?
[00:04:19] This is most likely to occur in females. People who have either a personal history or a family history of headache or migraine, a history of prior concussions and a history of mood disorders, and this is substantiated throughout the literature. The phenotype of post-concussion headache sounds most like migraine by far and away.
[00:04:40] Next up is tension type headache. Then occasionally we’ll see someone that has cluster headache after a concussion. It’s usually a bilateral headache and it can be moderate to severe in intensity, and it’s treated just like headache with either acute or preventive treatment as needed. But the other things that come along with it, again, are the things that make us start wondering, is this a leak or is this from a concussion?
[00:05:05] So cognitive difficulties are pretty common. Trouble sleeping, cervical injury, people start talking about their neck pain. Maybe that’s a leak. Vestibular and ocular motor problems. Now, we don’t see ocular motor problems too often a leak, but vertigo and dizziness are common after TBI.
[00:05:22] And then the psychological ramifications of which the things that predict the post-concussive syndrome at three months are a pre-injury, psychiatric history of either depression or anxiety, acute post-traumatic stress that’s manifested within five days of the injury, other life stressors, and pain, resilience, and coping styles.
[00:05:43] Then we get to whiplash. Now whiplash is a big mess. So whiplash, acceleration, deceleration head movement with flexion and extension of the neck, it is very common, much more common than CSF leaks. And following whiplash injury, 60% of people report headache within a week. 23% at three months, and still pretty stable, 30% at six months.
[00:06:07] You can see on the graph down there, the green is neck pain and the kind of burnt orangy color is headache. Going from seven days to 12 months after injury. So this kind of plateaus out after time. Most common causes being rear-ended in a motor vehicle accident. It is the number one reason for personal injury, compensation claims and malingering after a traffic accident.
[00:06:31] So it really is a big mess in a meta-analysis again of data that are not all that great, neck pain occurred in about 84% of people within seven days. Declined to about 40% after a year. And then headache again went to from about 60% to roughly 20% or so over time. So the literature’s really weak about whiplash.
[00:06:55] There’s not a lot you can say. Headache is more prevalent with those who had pre-existing headache. Then there’s this classification of the grade of whiplash and which is used by people like in rehab medicine who take care of these folks. So what is it? I’m gonna end every of the individual diagnosis with like, how do we sort this out?
[00:07:15] Okay. History of head or neck trauma. To have a TBI, you have to have head and neck trauma, right? CSF leak. We sometimes see that too. History of surgery. TBI can occur after having brain surgery if they had an open fracture. CSF leak can happen after a spine surgery. The headache is usually migraine, like with a TBI.
[00:07:35] It can sound like anything in a CSF leak. Neck pain, common in both vestibular symptoms, common in both, but cochlear symptoms I think are pretty well isolated to A CSF leak. Cognitive dysfunction common in both. Sleep difficulties also common but different. So TBI, it’s usually insomnia and with leak it’s usually nocturnal awakening from pain,
[00:08:00] if they get that. Mood and behavior, that doesn’t really help us. And the MRI of the brain, it should be normal in a mild TBI. And hopefully it’ll help us out and be abnormal in the CSF leak. New daily persistent headache is considered a primary headache disorder. It arises outta the blue.
[00:08:17] There’s no other cause for it. The ICHD require that the onset be distinct and clearly remembered. And within 24 hours of that onset of headache, it’s persistent and unremitting. Has to last for at least three months. And again, no other cause. It usually resembles either migraine or tension type headache.
[00:08:37] And there are two types. One is self-limiting and one is refractory. So SIH is actually considered a cause, a secondary cause, of new daily persistent headache. And whenever we see somebody in headache medicine who has a new daily persistent headache, The first thing we do is we start looking for a secondary cause.
[00:08:56] So how do we tell ’em apart? NDPH has to have a clearly recalled onset. Many people with CSF leaks also will recall exactly when it started. Thunderclap onset is possible in either, orthostatic headache is pretty much only CSF leak, and all those other symptoms are only CSF leak.
[00:09:14] So NDPH is just, it’s just a headache. Not other neurologic symptoms. I don’t think occipital neuralgia is all that difficult to distinguish from a CSF leak. Maybe your experience is different but occipital neuralgia, in order to make a diagnosis you’re gonna have to have paroxysmal attacks, lasting seconds to minutes.
[00:09:33] CSF leak headaches really don’t do that. Severe intensity shooting, stabbing sharp. And then both of the following, either it’s uncomfortable or it’s painful to touch the area in the back of the head in the distribution of one of the nerves involved, or there’s tenderness over the affected branches.
[00:09:52] Or there are trigger points at the emergent emergence of the greater occipital nerve or at the C2 distribution. The pain is eased temporarily by a local anesthetic block and again, not accounted for by any other diagnosis. And I see actually this diagnosis I think overdiagnosed in general, in the headache world. People with migraine and other kinds of headaches, they have tenderness.
[00:10:14] You press on their head, it hurts. You press on their great occipital nerve, it hurts. And that doesn’t mean they have great occipital neuralgia. The phenotype of the headache has to fit. The pain can sometimes extend to the front of the head on the ipsilateral side. And you have to distinguish it from pain that’s arising from the upper cervical region.
[00:10:34] So again, overdiagnosed in the headache world, there are a lot of things that respond to anesthetic blockade, which are not occipital neuralgia, including migraine cluster cervicogenic headache, and even post-dural puncture headache. So which one is it? Occipital pain? Yes, it happens in both, but the characteristic of the pain is very different.
[00:10:55] So in occipital neuralgia, we’re looking for brief paroxysmal attacks. In CSF leak, we’re looking for orthostatic headache. In occipital neuralgia, it’s usually stabbing shooting. That’s not very common in A CSF leak. I won’t say it never happens, but I don’t think it’s very common. Anesthetic relief is a hallmark of occipital neuralgia, but it can also happen with a CSF leak.
[00:11:20] And again, other neurologic symptoms, which I think is really gonna help you and the cervical imaging really doesn’t help you at all, but the brain imaging hopefully will. All right, now let’s count down to the things that are really problematic for us. And the first, which was mentioned briefly by Dr.
[00:11:36] Wang is POTS. And this is not so easy. So POTS, it’s a cardio autonomic disorder. There’s no apparent underlying disease. And it affects one to 2% of the population. And we usually, it’s young white women that have joint hypermobility syndromes, including Ehlers-Danlos, including Marfans.
[00:11:56] It’s typically associated with gastrointestinal disorders like irritable bowel syndrome, and then other comorbidities like mast cell activation syndrome and migraine. So in order to diagnose POTS, you need to have either a 30 beat per minute increase in heart rate in adults, a 40 beat per minute heart rate increase in children, or an absolute heart rate of at least 120 beats per minute, within 10 minutes of standing— not twenty; ten.
[00:12:30] But you have to exclude secondary causes. And here’s where things get messy. So the secondary causes include medications, including a lot of medications that we use to treat headache. Tricyclic antidepressants, for example, beta blockers, for example. Deconditioning. And there was a whole surge of POTS that occurred during COVID.
[00:12:50] And it’s questionable whether that also was related to deconditioning, dehydration, hyperthyroidism, anemia. You have to rule out orthostatic hypotension as well as cardiac arrhythmias. So the headache in POTS is, it’s common. At least a third of people have headache who have POTS. But you can see the confidence interval spans the whole gamut between zero and a hundred, almost so between two and 70% in the literature.
[00:13:17] Orthostatic headache, again, has a very wide range depending on the report. So anywhere between two and about 60% of people have orthostatic headache, but most people with POTS have non orthostatic headache and it’s usually migraine. There was a nice case control study that looked at nine people with confirmed SIH and 48 people with POTS.
[00:13:42] And they found that the things that were more common with SIH were a shorter disease duration; presence of orthostatic headache, which you could see is highly statistically significant; as well as neck stiffness, also highly statistically significant; an older age. The characteristics that were more common in people who had POTS were syncope.
[00:14:04] And I think syncope really helps you. Most people with SIH are not passing out, worsening with menses and the presence of other myofascial pain. So next stiffness, as it turns out, was only present in those nine people who had SIH. Now granted this is a small n, but many of the symptoms were present in both groups.
[00:14:24] So there’s different kinds of POTS, and I don’t pretend to be an expert in POTS. But there’s hyper adrenergic POTS, there’s neuropathic POTS, hypovolemic POTS, and then adolescents get completely different symptoms. But you can see neuropathic POTS is the most common type, and you can see that the major symptoms of neuropathic POTS, some of them might sound like a leak, right?
[00:14:47] Dizziness. I just feel a little weak, can’t sleep, but then anhidrosis, feet are turning blue when I stand up. That doesn’t happen during a leak. Hyper adrenergic POTS, sometimes our patients not only complain of headache, but they complain of feeling tremulous, even if you can’t see it.
[00:15:04] So there is some overlap in the symptoms, but it is complicated. And I think it’s really hard to distinguish these things clinically. The orthostatic vital signs and the tilt table test should be done if you’re considering a diagnosis of POTS, but they’re not always a hundred percent conclusive.
[00:15:23] So again, studies have been done with both people who have POTS and people who have CSF leaks, and they did all these autonomic tests, including tilt table tests. And first of all, in patients with POTS, they found that not all of the patients who complained of orthostatic headache in real life
[00:15:41] developed an orthostatic headache during their tilt table test, and then vice versa. The presence of headache during a tilt table test didn’t correlate with an orthostatic headache during regular activities. Okay. That’s straightforward. And then SIH patients can also have an increase in their blood pressure with head up tilt and increase in heart rate and heart rate variability with headache, with upright tilt, and with deep breathing, identical to people with POTS.
[00:16:12] So it’s not really a diagnostic test that separates the two disorders. And then to complicate the issue even more, people with POTS, just like people with SIH, they learn pretty quickly that they feel better when they’re lying down. And their heart rate’s back to normal. So they get deconditioned.
[00:16:31] SIH patients they do the same thing, right? And so people with SIH can develop POTS. So how do you figure this out? Not so easy. So I just tried to diagram it a little. So spinal fluid leak can lead to physical deconditioning. Physical deconditioning can lead to POTS. POTS can also lead to physical deconditioning.
[00:16:54] And then you have joint hypermobility, which can occur in either one of the disorders. So. It’s a mess. Okay, so what do they have? I don’t know. POTS, CSF leak, or both? Okay. So headache type. Headache type in POTS is usually migraine. It’s usually either in the front of the head or the whole head. And.
[00:17:17] Who knows how many have orthostatic headache. Everything in POTS almost is orthostatic. It all gets worse when they stand up. Spinal fluid leak: the headache can sound like anything. It’s usually posterior, involves the neck, but there’s no specific location. People can even get facial pain. Over 90% have an orthostatic headache at some point with CSF leak. Joint hypermobility doesn’t help you: present in both.
[00:17:43] Eighth nerve involvement. Similar to the TBI headache. People with POTS, they usually describe lightheadedness. They usually don’t describe vertigo and imbalance. People with a leak will describe vertigo, imbalance, and again, those cochlear symptoms, syncope, common with POTS; not so common with a CSF leak.
[00:18:05] Tremulousness, again, common with POTS, not so much with a CSF leak. Abnormal tilt table. You have to have an abnormal tilt table test to have a diagnosis of POTS, but it’s still possible to have an abnormal tilt table test and have a CSF leak—maybe ’cause those people also have POTS. I don’t know.
[00:18:22] And this is like one of those things where I’m like, Please God, make the MRI be abnormal, the brain. Because otherwise it can be really hard to tell. Next, the one that I think gets confusing is a Chiari malformation. A lot of people in this room are experts in Chiari malformation.
[00:18:39] They Often are associated with connective tissue disorders and atlantoaxial instability. I went online to look on for a picture of a Chiari malformation, and most pictures online now are copyrighted. So I ran across this one and I actually, unfortunately know the person who submitted this was submitted online as a Chiari one malformation.
[00:18:58] I’m like I don’t think so. Okay. But this I think is really a Chiari malformation. The other person has a CSF leak. Okay. So different types of Chiari malformations. Basically the cerebellar is herniating down through the foramen magnum. If it’s a primary malformation, there are different types of Chiari malformations.
[00:19:19] The one that’s usually mistaken for a CSF leak is Chiari one, and, which can occur with or without a syrinx and with or without obstructive hydrocephalus. Secondary causes of this Chiari—which is not really a Chiari, it’s tonsillor descent or brain sag —include intracranial hypotension
[00:19:37] or increased intracranial pressure. So people who have idiopathic intracranial hypertension can have tonsillor descent as well as other abnormalities that you see. And the official definition is at least five millimeters of tonsillor ectopia. So symptoms of Chiari one, they arise either from obstruction of CSF
[00:19:58] or from compression of the brainstem or compression of the cerebellum. And it involves the lower cranial nerves and often downbeat nystagmus. And they can also get spinal cord symptoms and signs related to a syrinx, if that’s present. In the in children, they most likely have brainstem dysfunction, that if they’re very young children may just be manifested as sleep apnea or trouble with feeding and headache, which again may be manifested as like crying and irritability.
[00:20:27] Adults, the pain is usually it’s in the back of the head and it’s worse with Valsalva. That’s one of the hallmarks of the headache from a Chiari. Is it orthostatic intolerance? Can you have that with a Chiari one? Basically, yeah, you can. So that kind of messes up the pot too. You can have coexisting POTS, you can have coexisting connective tissue disorders with Chiari one as well.
[00:20:49] So you may see all of those symptoms that we saw in the patients who report them from POTS. There’s not a lot written about that. So lastly, how do you tell the difference? Chiari one. Yes, the headache’s occipital where it has to be. But it may be, as with a CSF leak, coexisting POTS may happen with a Chiari one.
[00:21:09] It’s not really well reported, but it’s in the literature. It’s pretty common I think with patients who have CSF leaks, neck pain in both syncope with Chiari one. Dizziness in both, but again, Chiari one is usually lightheadedness. And we’ve already been through what happens with the CSF leak and this time the MRI is definitely gonna help you the, so you can look for all those abnormalities on the brain.
[00:21:33] Hopefully your radiologist will read the image correctly and realize that it’s a Chiari one or a CSF leak, but not both. So thank you very much for your attention.