S3:Ep10 – Pediatric Migraine Disorders

TRANSCRIPT

Spotlight on Migraine

Season 3, Episode 10

The Professional Series

Pediatric Migraine Disorders

Dr. Jacob Brodsky

[music]

Voice-over: Welcome to Spotlight on Migraine, the Professional Series, hosted by the Association of Migraine Disorders. Join us as we dive deeper into migraine topics with guests from the medical field.

This episode includes educational content intended for medical professionals but may be interesting to some patients as well. 

In this episode, Dr. Jacob Brodsky, of Boston Children’s Hospital, will talk about three types of pediatric migraine disorders in children: benign paroxysmal torticollis of infancy, benign paroxysmal vertigo of childhood, and vestibular migraine. For each disorder, he discusses diagnostic criteria, potential treatments, management strategies, and expectations for recovery.

This episode is brought to you in part by our generous sponsors Amgen and Novartis.

Dr. Jacob Brodsky: Hi, my name is Dr. Jacob Brodsky. I’m the director of the Balance and Vestibular Program at Boston Children’s Hospital, and I’m here to talk to you today about paroxysmal pediatric migraine disorders.

We’re going to talk about a few different disorders today, and for each one, we’re going to talk about a few things. We’re going to discuss how to recognize each disorder; how to make the diagnosis and what the diagnostic criteria are; what things you should consider, including differential diagnosis and other potential concurrent diagnoses; expectations for time course, recovery; and also treatment and ways to manage the disorder.

We’re going to talk about three disorders in chronologic order. The first is benign paroxysmal torticollis of infancy, or BPTI, which typically presents from around the first few weeks of life until about three years of age. And then we’ll talk about benign paroxysmal vertigo of childhood, which typically starts around three years of age until about six years of age. And then we’ll talk about vestibular migraine, which typically presents in preadolescents, adolescents, and adults.

And this is data from a study that we did in 2018 of patients with these disorders, showing that about close to half of patients with BPTI go on to develop symptoms of BPVC and that about 17 percent of patients with BPVC go on to develop vestibular migraine.

This is a patient of mine with BPTI, or benign paroxysmal torticollis of infancy, which is the first disorder we’ll talk about. Her sister does not have BPTI. And, as you can see, Abby, whose mom has given us permission to use this video, has a genuine torticollis. There’s contraction in the sternocleidomastoid muscle, probably from stimulation of the vestibulocollic reflex. You can see she has some light sensitivity, some imbalance, and also some irritability. And this is a disorder that is intermittent, so this comes and goes.

This is the diagnostic criteria outlined in the International Classification of Headache Disorders, third edition, and the diagnosis includes recurrent attacks that typically last minutes to days at a time. So this is not congenital torticollis. It’s not present from birth continuously. It usually comes on a few weeks into life and comes and goes in periods. And it can alternate sides as well. And it’s usually associated with at least some migrainous features, and patients usually have an otherwise normal exam in between attacks.

This is data from a study that we did in 2018 showing that nearly half of patients have some degree of vestibular loss on vestibular testing and more than half have some degree of motor delay. And Rosman and colleagues at Boston Medical Center found similar findings in their study. Also we’ve found that a large proportion of these patients have issues with recurrent otitis media and that often their episodes are exacerbated by episodes of otitis media. And so in these children, we will have a lower threshold for placing tubes, and often once they get tubes, their episodes will become much less frequent and much less severe.

Another consideration is a disorder called EVA, or enlarged vestibular aqueducts, and for those who are not familiar with that disorder, it is the most common structural cause of congenital hearing loss that can be seen on imaging. And it is a disorder that causes hearing loss that is often mixed and can often be progressive in nature. And so it also often has vestibular issues as well, and so we wanted to look at what the incidence was of paroxysmal torticollis symptoms in these patients.

And so my colleague Dr. Kawai and I did a study that was just recently published this year looking at patients that we had diagnosed with any of these disorders listed here: EVA; benign paroxysmal vertigo of childhood; vestibular migraine; GJB2, which is connexin 26 mutation associated hearing loss, which is the most common inherited cause of hearing loss in children and is not typically associated with vestibular problems; and in a group of control patients with a history of epistaxis. 

And we did a parental survey looking at symptoms of paroxysms of torticollis. And so we found that in EVA and BPVC patients, approximately one in five in each group had a history of paroxysmal torticollis, while very few in the migraine group and none in the control group and in the hearing-loss control group had any history of paroxysmal torticollis.

What’s particularly interesting is that in EVA patients, the paroxysmal torticollis typically started around a month or so of age, while the hearing loss was not diagnosed until over three years of age on average. And so the mean time in between diagnosis of paroxysmal torticollis and hearing loss in these patients was nearly three years. 

So the paroxysmal torticollis could be a sign of eventual progressive hearing loss, and so a very low threshold for getting a hearing test on these patients should be considered. And based on this data, I have been in the habit of now getting audiograms on all of my BPTI patients and typically monitor their hearing until they’re at least about three to four years of age to make sure that they don’t develop any progressive hearing loss over time.

This is combined data from our study and from the Rosman study from Boston Medical Center, and you can see that the female predominance of migraine is there, but it’s not as strong as it is with adults or adolescent migraine disorders. The onset age can vary anywhere from birth to three years of age, and resolution is typically by five years of age at the latest, usually closer to three. The duration of episodes can vary quite a bit, anywhere from days to weeks, and the intervals in between can also vary greatly, anywhere from days to weeks.

So in terms of management and workup, this is an algorithm that’s included in our 2018 paper on this. And so in patients with a history of paroxysmal torticollis, the workup should include an audiogram and a good neurological and motor evaluation, and if you have access to it, then vestibular testing. Very few centers are able to do vestibular testing in children this young, so if you are interested in pursuing that and you are in the New England region, you can certainly let us know, and we could certainly facilitate that at our program if you felt it was warranted. That would usually consist of rotary-chair testing and cervical VEMP testing, which is vestibular evoked myogenic potential testing.

And in these patients, you want to consider recurrent or chronic otitis media, and if they have that, you may want to have a lower threshold for recommending tympanostomy tubes. You want to look closely for a history of motor delay or signs of vestibular loss, in which case physical therapy would be beneficial. If they have very frequent or severe episodes, we have found that cyproheptadine can be very helpful, and that is described in our 2018 paper. And then also patients with hearing loss, you definitely want to have a low threshold for starting them with hearing aids and for considering a CT scan to look for evidence of EVA.

Moving on to benign paroxysmal vertigo of childhood, this is one of my patients with BPVC demonstrating what she feels when she gets a headache, which is a room-spinning sensation. And so this disorder is typically characterized by room-spinning vertigo that’s sudden onset without warning, no change in consciousness, usually lasts for a few minutes, and then there’s typically no postictal state, so usually kids just go back to what they were doing before. Even if they’re upset during the episodes, they recover really quickly, unlike a seizure disorder or a lot of other migraine disorders. 

They have to have at least five attacks to meet criteria, and they should have at least one other migrainous feature with the episodes, though that is not typically headache. They should have a normal neuro exam in between episodes and typically normal hearing and normal vestibular function. If they do not, then, similarly, EVA would be something that would be worth considering, because they can also often present with these symptoms. And, again, just like the other criteria, ruling out other disorders.

Considerations — one is differentiating this from benign paroxysmal positional vertigo, or BPPV, which, as most of you probably know, is the most common inner-ear-related cause of vertigo in adults. This is often thought to not happen in kids and teens, but we’ve actually found that it’s actually incredibly common in adolescents and preadolescents, although it’s less common in this very young, three-to-six-year-old age group, where BPVC is much more common. 

We have had a few patients with BPPV, but it’s less typical, and we’ll talk more about BPPV later when we talk about vestibular migraine. The key differentiating factor with that is that that is more of episodes that are specifically triggered by particular position changes and are not just spontaneous vertigo.

Otitis media, similarly, can have increased tendency in these patients, and often they will have exacerbations of episodes with otitis media episodes, and so a lower threshold for tubes is worth considering. And also epilepsy is important to differentiate, although typically that’s going to be fairly obvious relative to this. The big difference with that would be changes in level of consciousness during episodes. Historically, the ICHD criteria for BPVC used to include an EEG, but that has been eliminated with the third-edition criteria because that’s so rare and because the difficulty with differentiating the two is not typically a major issue.

This is data from our 2018 study on this disorder, showing that the male-to-female ratio is fairly similar to what we see with BPTI and again shows that female predominance. The interval between episodes and the duration of episodes is highly variable, and the age of onset is typically around three years of age. And resolution varies, but as you can see from this study in 2013 from Spain, that no matter when the onset is, typically the resolution of episodes is about two years from the time of onset on average.

In terms of treatment, specific triggers like dietary triggers or other migraine-hygiene-type triggers that we see with adult or teenage migraine are less common with [this?] and are harder to manage with [this?] or to nail down. Usually, no treatment’s required because the episodes are so intermittent and so brief, but if they are really frequent and really bothersome, we have found that cyproheptadine can be very effective for treating this and preventing the episodes. Unfortunately, the episodes are so short that abortive therapies like triptans or even ibuprofen or other NSAIDs is typically not very effective.

So, lastly, we’ll talk about vestibular migraine. Vestibular migraine is well known at this point to be the most common cause of vertigo and dizziness in adults and is also well established as the most common cause of vertigo and dizziness in kids and teens; however, the medical literature on the management and diagnosis of vestibular migraine in pediatric patients is extremely limited. 

The main differences in terms of symptoms with migraine in kids is that the headaches are typically more commonly bilateral; they are more often nonthrobbing; there is less tendency towards aura symptoms as there would be in adults; and also because kids have a particularly high incidence of concussion, especially teen athletes, there’s a high association between concussion and vestibular migraine in teenagers and some young kids.

In terms of the diagnosis, this is the ICHD criteria, which was just added in the third edition as an appendix. Hopefully, with the next edition, it will be part of the primary criteria. And the key, again, is that they have to have had at least multiple episodes and there needs to be vestibular symptoms associated with the episodes, which would be either true vertigo with a sensation of motion or movement, or motion sensitivity with nausea. And the episodes have to last minutes to hours, they have to meet ICHD criteria for a current or prior history of migraine with or without aura, and they have to have at least one migrainous symptom during the episodes. 

However, it’s important to recognize that patients with vestibular migraine, during their episodes, don’t necessarily need to have a headache during the episode. If they do meet criteria for migraine in general, and the episodes are associated with true vestibular symptoms along with other migraine features like light sensitivity or visual aura, then they do meet criteria for this diagnosis. And we have a lot of patients like that, where they will get very clear vertigo with severe light and sound sensitivity, sometimes a scotoma or scintillating lights, but they may not necessarily have a clear headache with those episodes. And many of those patients do respond very well to migraine treatment.

Some considerations — there are a lot of potential concurrent diagnoses with vestibular migraine that may make the diagnosis more confusing. I mentioned earlier BPPV, and BPPV is actually very common with migraine in general but especially with vestibular migraine. 

And for those that are not familiar, to review, BPPV occurs from dysfunction of the utricle organ in the inner ear, where calcium carbonate otolith crystals that are typically embedded in a gelatinous mass of the utricle organ can become displaced from anything that disturbs the inner ear, so old age, concussion, infection, or things that cause inflammation. This will cause the crystals to become displaced into one of the semicircular canals, and every time the head is moved in the plane of that canal, it will cause room-spinning vertigo and a nystagmus that can be diagnosed on exam to identify the diagnosis. And the treatment is repositioning maneuvers of the head to get the crystals back to the appropriate position. 

There have been a number of studies in animal models showing evidence that there are inflammatory changes in the inner ear with migraine, including vestibular migraine, that over time can cause vestibular loss or can cause things like BPPV to happen, and this is probably triggered by the trigeminal vascular pathway.

This is one of our patients with vestibular migraine. She was seven years old at this time. You can see in the right Dix-Hallpike maneuver, she has the torsional twisting nystagmus of her eyes. This is usually easier to see with video nystagmography goggles or Frenzel lenses, and typically we do use VNG goggles when we’re testing for this in the office. But sometimes the nystagmus is visible enough that it can be seen even without goggles.

Another diagnosis to keep in mind is persistent postural perceptual dizziness, or we colloquially refer to this as “3PD” or “triple P-D.” This is a somatoform disorder, or a functional neurologic disorder. It is incredibly common and is increasingly being considered one of the most common causes of dizziness in adults. And we’ve actually found it to be incredibly common in adolescents as well, especially adolescent females. 

This is a disorder that develops after an initial precipitating event, which is typically a vestibular disorder like BPPV or vestibular migraine, though it can also be triggered by any major medical event or anxiety- or stress-related event that causes a sensation of dizziness initially, such as a syncopal event or a panic attack. And the initial precipitating vestibular disorder or dizziness episode triggers changes in the brain that have been well demonstrated in a number of studies at this point that cause symptoms of chronic dizziness that are often there all the time, with frequent flares that are triggered by things like standing or motion, and things like visual flow, especially places like a grocery store or a shopping mall, and often also the setting of schools. 

There’s typically a constant sensation of feeling like the patient is going to fall; however, they never actually do fall. And a history of anxiety is certainly a risk factor for developing 3PD, although once patients develop the disorder, they almost ubiquitously will develop anxiety from the disorder, and it creates a vicious feedback cycle that further exacerbates the disorder.

And so the management of this problem is with vestibular therapy with a physical therapist, cognitive behavioral therapy or biofeedback, and SSRI or SNRI therapy. And so, very often vestibular migraine can trigger 3PD to develop, and so, often, these diagnoses will occur concurrently and can confuse the symptom presentation.

This is from a study that we very recently published looking at over a thousand patients in the pediatric age group seen in our program over the past eight years, and what you can see is that there is a large proportion of patients that have overlap between multiple of these three diagnoses: the vestibular migraine, BPPV, and the 3PD. And some patients actually even have all three of these disorders, and so I’ve come to refer to that as the vestibular triad. And so it’s very important in vestibular migraine patients to keep these other diagnoses in mind, as they can often confuse the clinical picture, and they may also often explain why some patients may not fully respond to migraine therapy and may need additional treatments.

Additionally, as I mentioned, inflammatory changes in the inner ear in a lot of vestibular migraine patients can cause vestibular loss, which can also cause some chronic dizziness symptoms. So in patients that are not responding appropriately to therapy or that have interictal dizziness, doing vestibular testing may be worth considering.

And another diagnosis to consider in the differential is episodic ataxia, especially EA type 2, which typically presents with episodic vertigo and nausea and vomiting and headaches. Usually, EA2 patients, their episodes are much more dramatic and much more severe. They often have interictal balance impairments, and these patients also typically will have down-beating nystagmus in between episodes, which can sometimes be seen on exam in the office, but more typically needs to be seen with video nystagmography. So if a patient’s not responding to treatment appropriately, getting vestibular testing may be beneficial. 

EA2 is typically due to a mutation in the CACNA1A gene and can be confirmed with genetic testing, especially with an ataxia panel. And, typically, this disorder does not respond to traditional migraine meds but responds very well to acetazolamide or 4-aminopyridine. And so this is certainly a disorder to keep your eye out for even though it’s less common than vestibular migraine. We do have a handful of patients in our practice that we’ve diagnosed with this disorder and have responded well to treatment after not responding well to vestibular migraine and subsequently getting this confirmed with vestibular testing and genetic testing. So it’s something to think about.

Ménière’s disease can have some overlap with vestibular migraine, and they can often be mistaken for each other. And also Ménière’s often can occur concurrently with vestibular migraine in adults; however, Ménière’s is very rare in kids. Out of over a thousand patients that we looked at in this study, only one of them had likely Ménière’s disease, and that was a 15-year-old female. So that’s very rare, including in other medical literature.

Vestibular migraine is usually in females. Again, there is that female predominance, where there’s that close to 2:1 ratio. And the age of onset varies widely, but the average is typically around the peripubertal age. And the female predominance and the peripubertal onset mirrors a lot of what we see in the adult population, where there is the female predominance and often onset or exacerbation in the perimenopausal time frame, which further supports that estrogen changes may have a role to play with vestibular migraine.

In terms of treatment, the big mantra that I like to tell all of my migraine patients is that migraines thrive on inconsistency. So for kids in particular, we really try to focus on migraine hygiene first and try to optimize consistency of sleep, hydration, activity, stress, or diet, and especially in our adolescents, those things can be very inconsistent. For any of you that have your own adolescent kids at home, you know that maintaining all these things is probably impossible consistently, and so it’s best to really try to find specific ones of these areas that we feel like we can make good progress with with a particular patient and focusing on one or two of these at a time to really try to make progress.

Pediatric patients do respond to migraine treatment for vestibular migraine. Unfortunately, there is only one study in the medical literature looking in detail at treatment of vestibular migraine in kids and teens, and that was a study that we published back in 2015 and by now is also probably very out of date and up for newer data. And so we’re still continuing to look at how kids respond to different medications for vestibular migraine.

We did find that they do often respond to triptans well, and we found about 70 to 80 percent do have significant improvement or resolution of episodes with triptans, both in terms of dizziness and headache symptoms. Rizatriptan’s FDA-approved for kids 6 and up, and the other triptans are approved for patients 12 and up. 

And then in terms of prophylactic medications, there’s a lot of choices that we find to be beneficial. In particular, venlafaxine is one that we often like for adolescents, especially those that have overlapping 3PD, because 3PD tends to respond well to SSRIs or SNRIs. And in the medical literature in adults, the SNRIs actually are the medications that have had the most consistently positive findings in the literature for management of vestibular migraine. So they have that double benefit.

The CGRP inhibitors, as most of you know, are not FDA-approved for patients under 18 years of age yet, but they’re showing some promise for treatment of vestibular migraine in adults. And, hopefully, eventually we’ll be able to use them for pediatric patients as well.

So to summarize, be aware of the “vestibular march”; that’s the BPTI progressing to BPVC to vestibular migraine. It helps families to be prepared so that if they’re having issues with torticollis or with vertigo and then they’re asymptomatic for a couple years and all of a sudden they start having issues with headaches and vertigo and stuff like that, they don’t have to freak out and go through a big medical workup, because they’ll know that they were going to expect that as a possibility.

Consider for BPTI patients getting an audiogram. Look for history of otitis media, and look for signs of vestibular loss or motor delay. Benign paroxysmal vertigo of childhood is common and self-limited. BPTI and BPVC may respond well to cyproheptadine. Make sure to check pediatric vestibular migraine patients for BPPV and also consider 3PD. And for vestibular migraine patients in the pediatric population, focusing on migraine hygiene and consistency is important; however, pediatric vestibular migraine can be treated with triptans and with prophylactic medications as well.

I thank you all for your time today.

[music]

Voice-over: Thank you for tuning in to Spotlight on Migraine. For more information on migraine disease, please visit MigraineDisorders.org.

*The contents of this podcast are intended for general informational purposes only and do not constitute professional medical advice, diagnosis, or treatment. Always seek the advice of a physician or other qualified health provider with any questions you may have regarding a medical condition. The speaker does not recommend or endorse any specific course of treatment, products, procedures, opinions, or other information that may be mentioned. Reliance on any information provided by this content is solely at your own risk.