A male doctor in scrubs analyzing brain scans on his computer.

Webinar: Transforming Data into Seizure Control with Learning Healthcare Systems

“Learning healthcare systems” is a method of improving clinical outcomes in patients by collecting and analyzing privatized electronic health data, then rapidly disseminating findings to change medical practices.  This approach is highly collaborative, bringing together patients, families, doctors, and researchers from institutions around the country and even globally.

Two learning healthcare system initiatives are actively working towards this goal specifically for people with epilepsy, one focused on care for adults and one focused on care for children.

This webinar will discuss the progress and potential impact of the Pediatric Epilepsy Learning Healthcare System to epilepsy patients, their families, and to the entire epilepsy research community. At the end of the presentation, audience viewers asked Dr. Zach Grinspan questions on how data and collaboration is being used to improve patient care and outcomes.

You can download the full transcript below.

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Dr. Zach Grinspan

This webinar is presented by Dr. Zach Grinspan, Associate Professor of Population Health Sciences and Pediatrics, and Director of Pediatric Epilepsy at Weill Cornell Medicine.  He is primary investigator of the Pediatric Epilepsy Learning Healthcare System project and the Rare Epilepsies in New York City project, and currently serves as chair of the steering committee for the Pediatric Epilepsy Research Consortium.

Audience Q&A with Dr. Grinspan

From a digital perspective, what are your biggest data-gathering needs at this point?

Let me be a little wordy with my response. We’ve had many conversations with IT groups around the country about how do we standardize process of getting electronic health records. Other groups have done that and I think we need to get better at that.

Right now, it’s a lot of phone calls and a lot of specifications. It works. We’re getting there, but we could certainly be more efficient. We have a good system to transfer the data. Now that we have the data, we’re starting to run into some bottlenecks with the processing. We have one analyst working through it. As we scale up, we’ll need more people processing the data and getting it ready. A lot of the technology is free, so we have a good pipeline to get the reports out.

We have electronic health record system questionnaires deploying over the next few months. Epic is going to release our questions this month. Cerner is not far behind, and someone has already built the questionnaires in Athena. We’ll want to expand to other electronic health record vendors, because we really want to be vendor agnostic. Then, we’d like to bring more data in.

Data from EEGs, MRIs, devices, and patient-reported outcomes would be amazing. People who wear devices are walking around collecting data moment to moment with an RNS or a VNS, and so we want to explore partnering or working together to include that data. Some of it is humanware. I think technology is relatively straightforward. It’s just a matter of all of the conversations and figuring out how to get the data out, move it, and link it.

Have advancements in implants, watches, and other devices helped you understand more about medication efficacy for patients? Has this new data increased the whole picture of an epilepsy patient’s day and changed the way you view potential versus multi-drug prescription regimens?

It’s a great question, because what it gets at is that, in the digitized world, you have people who are creating oceans of data, begging the question, “Can we do anything with it?” For some patients, the answer is absolutely. Certain kinds of data, like VNS and RNS data, can help doctors make very targeted changes. The question is, how do we scale that process and how do we learn from it? And the question is very compelling. We are not there yet, because we don’t quite know how to do this yet at scale. It’s a long-term goal of ours.

Can the collected, aggregated data be individualized? Or can that data be available to doctors and patients for a fee? For example, if RNS data is collected, would somebody need to pay in order to see their own data?

I’ve never had anyone ask me for a copy of their VNS or RNS data. It’s certainly doable and the data is, after all, coming from the patient. I don’t see any obstacle to that.

Other learning health systems use identified data, so their databases know your name, date of birth, everything. These systems actually do provide as the questioner poses direct services. “Here is a patient-level report about how your patient is doing.”

We shied away from that for privacy reasons. Data breaches can be devastating for so many reasons, so we opted to use less personalized data. We don’t know anyone’s name, date of birth… we do know zip codes, but we don’t know where patients live or their medical record number. That was intentional.

Our thought process here is that we can send information to individual centers, which can identify the patient. The report might say, “Patient ABC had this happen.” Then the center has the ability to say, “ABC is actually John Smith.” They have to do that extra step. I don’t know it’s John Smith. I know it’s ABC.

This method helps make the data more secure, but we’re very much about data sharing, and so we have promised all of our collaborators that they can have their own data with no questions asked. We’ll just give it back to them. We’ve crunched it and processed it a little bit because we want to promote your new faculty, we want to promote residents, and fellows who do research projects. Then, for the network, if one investigator says, “I have an idea. It works on my own data. Can I do it on everyone else’s data?” Then, we have a very straightforward process to allow that to happen, too. We really all want to learn together.

You’ve talked about data sharing. Will data sets be made publicly available?

I don’t think we’ve thought about that really. The data sets we have qualify as limited protected health information (PHI), so we can’t share information like dates of birth and zip codes. But the full data sets… I mean, theoretically we could. I think we’d have to talk and think a bit more about that.

A lot of networks want to make sure that patient data is used for a purpose that’s aligned with the mission of the organization, so there’s often a process. We don’t have such a process in place right now, but if that became something of interest to the community, there’s no reason we couldn’t start planning.

How have you had to overcome the barriers of institutions not wanting to share their data with other institutions?

I thought that was going to be a huge problem, but it doesn’t seem to be an issue. Everyone’s so happy to share. It’s really nice. As much as I’d like to say I’m a pioneer, I’m not. People have been working on this in other fields for more than a decade, so the ground has really shifted and we’re in a new world.

The Pediatric Hospital Inpatient System has data from roughly 45 centers, including data from Cornell, Columbia, NYU… and you can walk from one to the next in an hour. I think that the culture, particularly in pediatric hospitals, is very mission-driven, so these potential issues of competition and “you can’t have my data” has just not been a problem.

What are you doing to monitor and measure the impact of diet on seizure control?

It’s not easy to figure out who is on an epilepsy-related diet and who isn’t from the data we have. I showed you that one question about the seizure frequency, but we built in some questions also about diet. It’s pretty epilepsy-specific, so the options we list are the ketogenic diet and modified Atkins, low glycemic index, or other. That’ll give us some high-level information about who’s on what diet, if it’s working, and similar information. More detailed information about specific foods and specific exposures would mean a whole different level of data collection.

Are you familiar with the Observational Health Data Sciences and Informatics (OHDSI)?

Let me nerd out for a bit! One of the major questions we’ve had is, “What does a tables look like in the database?” A lot of people have spent whole careers thinking about that for health data. The Patient-Centered Outcomes Research Institute, has advocated use of PCORnet, the PCORnet Common Data Model.

Our data looks a little bit more like PCORnet mixed with the OMOP model. Currently, our data model is our own, which could be sort of seen as a simplified version of OMOP and PCORnet. What we told our sites is, “If you have the data in PCORnet or if you have the data in OMOP, just send that. Don’t reinvent the wheel.” No one’s taken us up on the offer, so it seems like operationally, a lot of the sites are finding it easier just to kind of make a custom extract for us and just sending us what we want, which we’ve been fine with.

Would it be beneficial for a healthcare provider to have the PELHS questions answered before the visit versus during the visit?

Yes and no. We really want curated data reviewed by a clinician. The workflow that this question proposes is a good one, in which the parents or the young adult enters the data in prior to the visit, and then the clinician and the family review it together. That would be totally okay!

We’ve spoken with Rob Moss, who runs SeizureTracker, and he’s very excited about this idea. He’s been working to link his application with Epic, which is one of the electronic health record vendors. What we’ve asked is, “If you get that workflow there, can the SeizureTracker data populate the learning healthcare system data?” We’re agnostic on how the data gets into the system. If the data gets in there and the clinician vouches for it, then we’re good.

Are patients and their families aware you are collecting these data? How do they feel about participating?

We’ve been very deliberate from the beginning in maintaining communication with advocacy groups and including parents and people with epilepsy at the highest levels involved in developing this system, so there are representatives. That being said, if a parent bring their child to one of the centers involved in this project, they wouldn’t know that the information from that visit is being brought into our Learning Healthcare System.

The reason we forgo getting patient and caregiver consent is that the labor required is too much work for the kind of data we’re gathering. The way electronic health record data is used for research in this country tends to support that. Institutional review boards granted us an exemption from the federal regulations from HIPAA, which allows us to look at the data without getting explicit permission from a hundred thousand people.

We’re comfortable with because we feel the good things we’re going to learn far outweighs the risk to loss of privacy. We’ve been quite intentional, as I said, about making sure that the data that we have doesn’t have a lot of personal information – no names, no addresses. We have dates of birth, but lots of people share same dates of birth. When we’ve spoken to advocacy groups, most people are in agreement that the labor required is too much work to get those consents. We’d spend all of our effort doing that and the groups would rather us do the learning . We got all of the approvals. We have data use agreements. We have all of the legal and ethical infrastructure, but it’s true that you wouldn’t know that your data is going to be in there necessarily.

How have you involved patients in designing this system, the process, and the governance? Are differences between the Pediatric Epilepsy Learning Healthcare System and the other system that you mentioned in your presentation?

Both systems are quite deliberate and have made a big effort. The ELHS, the Epilepsy Learning Healthcare System, is run out of the Epilepsy Foundation, which at its heart is an advocacy organization. I think the DNA there I think is much more about patients’ perspectives. We were aware of that. We wanted to make sure we were listening and including that voice, which is why we bring everyone on the calls.

As a example, when we put our forms together, we wanted to have a scale like, “How often are you having seizures?” In the original scale, the most you could say was multiple per day. A couple of parents were like, “My kid has more than that.”

“What do you mean?” we asked. “Multiple per day, that’s it.” Parents said, “Yeah. I can’t even count because there’s so many.”

We said, “Oh, okay. We missed something important.” Now the highest level is “too many to count.”

Is there the possibility for an international collaboration? Could it be even better with hundreds or thousands in the wider group?

I love that. Whoever wrote that question, we are like mind melded. We’ve had some conversations about collaborating internationally. Building the questionnaire into the healthcare records vendors’ system, then all of their customers internationally can then use the form. Then, if you get a collaborator, then the data’s already there. The collaborator just has to send it.

How do I encourage my neurologist to participate in The Pediatric Epilepsy Learning Healthcare System?

Tell them to email me. We have some funding and we were provided 20 participating centers some seed funding. That money has run out, but some sites are willing to join with internal resources. At present, if there are sites that are enthusiastic, they can just find me and I can have a conversation with them.

The other thing is that 54 of the US Pediatric Epilepsy Centers are part of PERC, and I am pitching and giving updates on this through all of our PERC calls. We just had our annual meetings last week and we have calls every other month. Find out if your center is part of PERC, find out who the PERC representative is, and then having that person reach out to me.

The CURE Leaders in Epilepsy Webinar Series has covered many topics related to epilepsy and innovations in research. Check out our full list of available webinars here.

The information contained herein is provided for general information only and does not offer medical advice or recommendations. Individuals should not rely on this information as a substitute for consultations with qualified health care professionals who are familiar with individual medical conditions and needs. CURE strongly recommends that care and treatment decisions related to epilepsy and any other medical condition be made in consultation with a patient’s physician or other qualified health care professionals who are familiar with the individual’s specific health situation.

Female psychologist working with boy who has autism and epilepsy.

The Epilepsy-Autism Connection: Research, Diagnosis, and Treatment

It is estimated that over 30% of people with epilepsy also meet the diagnostic criteria for autism.1 In this webinar, presented by Dr. Jamie Capal, hear leading theories on the interconnection between autism and epilepsy. Learn about the most common seizure types in people with autism and dive into the current research on why so many children with autism develop epilepsy.

Dr. Jamie Capal is Associate Professor of Pediatrics and Neurology at Cincinnati Children’s Hospital Medical Center. Dr. Capal has been an integral member of the multidisciplinary Tuberous Sclerosis Clinic Center of Excellence and maintains a busy clinical practice diagnosing and treating children with autism spectrum disorders and comorbid neurological disorders. Her current research is focused in the areas of autism spectrum disorder and Tuberous Sclerosis Complex.

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1 Spence, S., Schneider, M. The Role of Epilepsy and Epileptiform EEGs in Autism Spectrum Disorders. Pediatr Res 65, 599–606 (2009). https://doi.org/10.1203/PDR.0b013e31819e7168.

To hear an author, journalist, and mother discuss how her family has navigated the dual diagnosis of autism and epilepsy to help her son live his best life, watch or listen to the Seizing Life episode Raising A Child with Both Autism and Epilepsy featuring Liane Kupferberg Carter.

Audience Q&A with Dr. Capal

Dr. Jamie Capal

Your presentation shared a lot of information about tuberous sclerosis complex. Can you speak to the information we’ve gathered there, and how that might translate to other neurodevelopmental disorders? 

That’s a great question. Currently what we’ve learned is that the earlier your brain gets disrupted (by seizures for example), you’re at a much higher risk for overall disorganization of the brain. And so, what we’re trying to figure out is, are there other things that can tell us what else is going on from a structural standpoint, from an EEG standpoint? Because the goal is prevention.

There are studies going on now, the PREVeNT trial for example, looking at early seizure treatment in babies with Tuberous Sclerosis Complex who have not had seizures yet, but do have abnormalities in their EEG. If you treat the abnormalities before the seizures come, will you get better results with development? And maybe better results as far as preventing autism? That study is closing right now, so we are very interested to learn the results, because really that’s the next step.

Many other neurodevelopmental disorders are also looking at similar things, and what we know is looking earlier is better. The earliest we can try to advocate for prevention, the better off we are to disrupt these mechanisms that result in developmental delays and autism.

EEG testing is not standard when screening for autism. What sort of information would need to be gathered in order to make that a more standardized approach?

I think what we really need is a protective studies – basically doing screening EEGs on all kids that are newly diagnosed with autism, and then follow them out longitudinally. But the problem is that this is a very expensive and long study, but I really think it’s something that needs to be done. That way, we have a true idea of what the percentage of kids with autism have abnormal EEGs, and what their risk of eventually developing epilepsy is. That will give us the evidence we need to say, “Everybody with autism needs to get an EEG as screening.” Right now we just don’t have anything.

In focal epilepsy, if seizures are emanating from a specific area of a brain, is a person more likely to develop autism?

A lot of people have looked at this. There is some evidence showing that maybe epilepsy in the frontal region of the brain, or the temporal region of the brain may predispose you to having autism, but it’s not universal. There’s some evidence showing that.

Has any research looked at if children with autism can improve cognitively with increase seizure control? 

The study I referred to in my presentation that is looking at the natural history of the development of autism is working to understand this. We collect all the seizure diaries form patients in this study, and we are going to see if anyone’s scores improve with treatment. There’s also a group looking at whether or not improving seizure control with epilepsy surgery increases cognitive development. Again, the goal though is to look at this early in life. That is where the field is going right now. Nothing has come out of it yet, but that’s we’re looking.

We know epilepsy can impact cognitive function, and then cause cognitive decline in a way that might make an adult look like they have some aspects of autism. Is this due to the seizure activity? Is there a way to protect the brain from that seizure activity?

That’s a difficult question to answer. Think about the epileptic encephalopathies, for example. Those are the patients who are having lots and lots of seizures, and even when they’re not having seizures, they’re background brain activity is abnormal. Their neuronal connections are not allowed to form correctly, so those patients are going to develop cognitive impairment. In those cases, by controlling the seizures, you would expect cognition to improve.

In other cases though, it’s less clear. I think you have to think about epilepsy as not causing necessarily causing the delays. There may be two things happening simultaneously, and epilepsy is just making it worse. So treating epilepsy may help, but it’s not going to reverse cognitive impairment.

Are there links between epilepsy, autism, and Alzheimer’s?

I can’t say that I know a lot about the literature for Alzheimer’s, but I can say there is a lot of interest in looking at the connection between Alzheimer’s and autism. I think there are a lot of shared genetic mechanisms between these conditions. That connection is definitely something I think needs to continue to be looked at as we do more genetic studies. We need to look at; what are the shared genetic links between individuals with Alzheimer’s and autism? That being said, research has found that there are many similarities in the connectivity of the brain in both of those disorders.

Many people with epilepsy, including those with autism, are not responsive to medications, and antiepileptic drugs can cause side effects, like mood changes, GI issues, anxiety, increased repetitive behaviors that worsen…. What are your thoughts about the VNS, and more specifically the noninvasive VNS that is not approved in the US as yet? Does it help with autistic behaviors at all?

I like VNS. I think there are some folks it works very well with. I had one patient, for example, who I was thinking about doing a VNS on, but because he is so active and his behaviors were so erratic, he wasn’t deemed a good candidate for it. Another patient I’m thinking of really did well with VNS,  because they were having so many negative side effects from antiseizure medications.

Should all children diagnosed with epilepsy, especially learning disabilities, be screened for autism?

Ideally, yes. The American Academy of Pediatrics has set up a guideline to screen young patients for autism with the M-CHAT, which is a questionnaire that parents get at 18 months, 24 months, and then again between ages two and three.

One problems is that when young individuals are diagnosed with epilepsy, other aspects of their development aren’t really paid attention to as much. So, clinicians are finding that individuals who are being diagnosed with autism much later, because maybe the doctors were spending more time really focused on the seizures. Those folks really should have good surveillance by their pediatrician. If there are any concerns for development, they should be referred on to developmental pediatrician for further work up.

So, this is really a place where parents could be advocating for that.

Very much so.

What evaluations are being done outside the brain and EEG? Are people looking at the gut, the autonomic nervous system, or sleep disruption that are implicated in both epilepsy and autism?

Yes. There are definitely folks looking at the gut-brain connection. I think there’s a lot of interest there. It’s almost like these subtypes of autism. Abnormal EEG is one subtype, then there are patients with GI disturbances in another group. Sleep can be disrupted for many reasons and you see this problem in various subtypes of autism. There’s an autoimmune interest in individuals that potentially have an autoimmune component to their autism, which I again think is another subtype worth studying. So, I think the more we learn about the underlying causes, the better able to study the clinical features we are.

We historically have been looking at autism as a set of symptoms. But if we study autism as the symptoms only, even though there could be hundreds of causes behind it, we’re not really going to learn anything.

There was a question about what type of preventative treatments would be given to a person with autism and abnormal EEGs, but it sounds like we really need to understand the biology.

Correct. We don’t know. There have been some small studies that have put kids with abnormal EEGs on depakote, for example, and really haven’t found a lot of benefit. The interest is: do we put these patients on medicine to prevent epilepsy? Do we put them on medicine to improve their EEG? We don’t know.

If we look at benign rolandic epilepsy, for example, those individuals may have a few seizures, but they have a lot of underlying EEG abnormalities when they’re sleeping. Some groups found if you treat the EEG abnormalities, cognition may improve. So, the same thought present here, but nobody has ever done a big enough study to tell us that treating EEG abnormalities is worth it. This area definitely needs to be studied.

It could be very difficult to get a non-sedated EEG on children with autism due to sensory and other issues, especially an overnight. Are there other ways to perform EEGs with a headband or other nontraditional approach? 

Some researchers are using the EEG cap. One group is actually desensitize the children in their study by having them wear a hat, so they can get used to the feeling. Those EEGs are pretty accurate versus the traditional leads.

Now, a lot of times we don’t do that clinically. I’m sure there are a lot of reasons financially and training-wise. But I know in research, to get all of these children to have EEGs, the scientists become really creative at desensitizing the kids. For all of our studies we do EEGs on all of our kids, and the tests are actually pretty successful.

What’s involved with genetic testing, and where can we direct people for more information?

Genetic testing can be done several ways. Typically and historically, it’s been a blood test. Your neurologist or developmental pediatrician can order it. What we tend to do is a “chromosomal microarray,” which looks at any deletions or duplication in genes. This test is a good place to start. There are many companies which have developed genetic panels that can be done by blood or saliva. Each panel is different and geared toward a certain set of genes – there’s a autism and developmental disability panel, there’s an epilepsy panel…. So, those are targeted tests.

Then you have the bigger whole exome sequencing. Currently, the Simons Foundation has a big study going on throughout the country called the SPARK study. The study team is collecting saliva from the patient and both parents to look at their exomes and really understand the genetic underpinnings of autism. You can even go to the Simons Foundation’s SPARK study, and can get a kit sent to your house. That’s a great, free way for families to get genetic information, since often genetic tests are not covered by insurance.

The CURE Leaders in Epilepsy Webinar Series has covered many topics related to epilepsy and innovations in research. Check out our full list of available webinars here.

The information contained herein is provided for general information only and does not offer medical advice or recommendations. Individuals should not rely on this information as a substitute for consultations with qualified health care professionals who are familiar with individual medical conditions and needs. CURE strongly recommends that care and treatment decisions related to epilepsy and any other medical condition be made in consultation with a patient’s physician or other qualified health care professionals who are familiar with the individual’s specific health situation.

Webinar: The ABCs of EEGs: An Evolving Tool for Epilepsy Diagnosis

This webinar explores the intricacies and advancements in a well-known diagnostic tool; an EEG.

An electroencephalogram – better known as an EEG – is a test that records electrical brain patterns from the scalp. EEGs are critical for the diagnosis of epilepsy and other neurological conditions. While this diagnostic tool has been available for nearly a century, there have been great advances in the portability and signal detection properties.

This webinar discusses how epilepsy patients have benefited from advances in EEG technology, and the role of the EEG and other neuroimaging tools in the future of epilepsy diagnosis and seizure localization.

This webinar is presented by Dr. David Burdette, Epilepsy Section Chief for Spectrum Health Medical Group in Grand Rapids, Michigan. He has been at the forefront of EEG education having served on the American Board of EEG Technologists (ABRET) and the LAB-EEG board of the American Board of EEG Technologists (ABRET).  Dr. Burdette’s clinical interests include neurotelemetry, long-term EEG trending, treatment of refractory epilepsy, treatment of status epilepticus, and electroencephalography.

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Dr. David BurdetteAudience Q&A with Dr. Burdette

How often should a patient get an EEG if a past EEG was abnormal? At what point do you elect to have a 24 hour versus a 60 minute sleep-deprived EEG?

That is an interesting question, for which there’s not necessarily a right answer or a wrong answer. First, a question: why do people get EEGs? If someone has what I would consider a prototypical seizure…. The old expression goes, “If it walks like a duck and quacks like a duck, it’s probably a duck.” And if someone has seizures that walk and quack like seizures, it’s probably seizures. And if they walk and quack like a focal seizure (what we used to call a partial seizure), then I’m going to treat it as a partial seizure. I may not even need an EEG to do that.

I’ll get an EEG, just a one off type EEG, to make sure I’m not just totally out to lunch. But that EEG may end up being normal, even though that person is probably still have seizures. So, if a patient responds to the first medication I prescribe, as arguably 47% of people do, then that’s great. There is no compelling reason in my mind to repeat that EEG. If that person doesn’t respond to the first medication, I’ll usually have a plan B, so we’ll try the plan B. If that doesn’t work, I need more information.

I need to get some insight into how a person who hasn’t responded to the second medication is doing, so I may get a sleep deprived EEG or a two hour long EEG, so that way I can see those brain waves when they’re awake, drowsy, and asleep. Often times we need that sleep in order to really see the rhythmicity of the brain and for me to figure out, “Ah I was wrong. It was walking and quacking like a partial seizure, but in fact it was a generalized seizure and I chose the wrong medication.” Ideally that doesn’t happen, but it could. So, in that instance, I would get a sleep deprived EEG or I might get an ambulatory EEG so that over 24-48 hours, I can see that waking, drowsy sleep rhythmicity and see what’s going on.

If the individual still has seizures or the EEGs are unhelpful, then I will have the person come into the epilepsy monitoring unit. The place in the hospital where the healthiest people are, and we bring them in, it’s the one time in your life we want you to have a seizure, so we crash the person off their medications, sleep deprive them every other night, and ideally record a seizure.

That being said, in children there are many genetic forms of epilepsy which may appear in childhood and be outgrown later in life. In this case, serial EEGs are necessary to identify that progression.

Regarding the rhythms you showed at the end of the presentation, are those patterns in patients with epilepsy only or do healthy patients have similar multi-day rhythms?

There will be an element of speculation to my answer because we don’t know. But I don’t think it’s too much of a leap of faith to say that multi-day variation, the diurnal variation we tend to see in seizures,  is being driven by a rhythm that is intrinsic to the brain itself. So in essence, the likelihood is that, seizures or no seizures, epilepsy or no epilepsy, we all have those rhythms. How they manifest though is difficult to say unless you have seizures.

Can you explain the subclinical seizures and EEG signatures called PLEDs, burst, and birds? This is a very detailed question about these different signatures.

Seizures are typically divided into “generalized seizures,” in which one second the brain is fine and then the next second both hemispheres are seizing, and “focal seizures,” in which seizures begin in a specific area of the brain. You’ve probably heard this notion that we only use ten percent of our brain. If we could use 90% we could do telekinesis, we could do whatever. Who knows, maybe people could develop telekinesis, I wouldn’t know. But I do know that we use our entire brains. A PET scan will light up the glucose metabolism the brain cells that are working, and we know that the whole brain lights up.

We’re using all of our brain, but we can only define what 10% of the brain does. When I do brain mapping on someone in anticipation of epilepsy surgery, in 90% of the parts of the brain I map, I can’t identify anything that happens when I stimulate it. Further, the person with epilepsy cannot identify that I’ve done anything. So, 10% of the brain is what we call “eloquent.”

If you have a seizure in eloquent cortex, it’s going to cause a symptom. If you have a seizure in a part of the brain that activates if you heard an oncoming train, then your seizure will cause an auditory hallucination of an oncoming train. But for other 90% of the brain, if the seizure starts there and stays focally there, then there’s a reasonable chance you’re going to have no symptoms. We would call that a “subclinical seizure.” In this case, we can see it on the EEG, particularly if we’re recording from directly within the brain itself. A seizure is happening, but it is subclinical – it’s causing no symptoms.

With PLEDs or LPEDs – we change the name sometimes but it’s the same phenomenon – this is a sign of an excited brain. If either some badness happens to the brain, a stroke for instance, then the area around the stroke will have excessive excitability. Or, if someone has known epilepsy and they go into status epilepticus – one seizure after another after another – the excitability of the brain really goes up in that area. The end result of this happening is that the brain keeps pushing toward a seizure, causing a burst of activity that shuts down that part of the brain for a second as it recovers, and then it happens again, boom, and then it shuts it down, and then boom…. Seizures during status epilepticus are periodic, like a metronome: fires, fires, fires, fires, it’s lateralized, it’s over one half of the brain, epileptic form discharges. So these are boom, boom, boom. This state is highly epileptogenic, but it is transient. So once you correct whatever is the underlying issue, it should resolve.

Bursts are a more descriptive term. In that case the brain is going about its business, then there’s a burst of activity, kind of like we saw earlier in my presentation where it’s burst, suppression, burst, suppression. So that is a descriptive term applied to any sudden outpouring of electrical activity within the brain. We will see bursts in a broad array of clinical situations from burst suppression to various epileptic or epilepsy related phenomena, and they are in essence this large outpouring of synchronous brain activity.

And then the final term, birds, has been applied in a few ways, but these are these brief, rhythmic discharges that are not quite seizures, but show a strong tendency towards seizures. This is a term most commonly used in neonatal EEGs. In adults, I see the brain waves going along, I see a burst of activity, looks like a spike, we call it a spike.

In the neonatal brain, development is happening really fast. Newborns get these spikes all the time and they can be normal. To differentiate abnormal bursts of activity, we use various terms to describe he specific kinds of spikes. If you see those spikes but they’re rhythmic and they last a certain period of time, then that is more worrisome for seizures. So that is most commonly how birds are used.

How sensitive are ambulatory EEGs? Is there a difference in the ability to pick up seizures between ambulatory versus in patient monitoring?

Ambulatory is more sensitive than a routine EEG. A routine EEG lasts 20 to 30 minutes. What are the odds that we’re going to pick up some abnormality in 20 to 30 minutes? It depends how active someone’s seizures are. If they’re having a seizure every five minutes, we’ll probably pick it up in a 30 minute EEG. Most people, however, are not having seizures as often as that. Most people have more widely dispersed seizures and therefore, by extension, more widely dispersed abnormal bursts of activity associated with seizures.

The longer we can record an EEG, the greater our likelihood of coming up with an answer to better inform our treatment options. A 20 to 30 minute EEG gives us some information. A 24 hour EEG gives us much more information, because we see those brainwaves in wakefulness, drowsiness, stages one, two, three, four, and REM sleep. The next step is being admitted to the epilepsy monitoring unit. If you go into an epilepsy monitoring unit and there are no medication changes made, you might as well have it done at home, because you’re less restricted at home.

But typically what we do in the epilepsy monitoring unit is evaluate situations when the ambulatory EEG didn’t give us the answers we needed. The in-patient epilepsy monitoring unit EEG allows us to take you off of medications, not necessarily to induce a seizure, but to remove your protection from seizures, so that we can record an actual seizure. That would be dangerous to do in many situations at home because there are risks of having multiple seizures. You could go into status epilepticus, you could (god forbid) have Sudden Unexpected Death from Epilepsy – it’s a scary situation. But in the hospital, it’s a monitored situation. An EEG tech is watching the screen 24-7 and when a seizure happens, they push a button, nurses come running, and they give medications to abort the seizure.

That’s the main difference – for an ambulatory EEG you’re probably on medication, and in the epilepsy monitoring unit we’re taking away the medication.

How do I know if my doctor knows the latest information about performing an EEG? Are there any questions I can ask?

I would ask if they have done an EEG or epilepsy fellowship. When someone goes into training in neurology, they do their internship right out of medical school. During that time, they get some basic training and learn more about treating patients with various maladies. Then they do a neurology residency, and focus on just brain-, spinal cord-, nerve-, and muscle-related issues. Part of that training is learning some basics of EEG, and learning the basics of taking care of a broad range of issues from Parkinson’s disease to tremors, to peripheral neuropathy to epilepsy.

Typically after someone develops seizures they will start with seeing a neurologist. And frankly, the majority of people do very well with seeing a neurologist. If, however, a person continues to have seizures, then it is time to move it up a notch. And that next notch takes the form of neurologists who did extra training for one or two years in either clinical neurophysiology, EEG, or in epilepsy (which also includes an EEG component). That by itself means that person is going to have a greater level of comfort and more in-depth knowledge of seizures and epilepsy.

In addition, you can check if your provider is board certified. Board certification will establish that a person has a minimal amount of knowledge. It doesn’t say that they’re the greatest thing since skim milk, but it assures to some degree a minimum level of competence. I tend to check if my doctor is board certified in the area in which I am seeking their opinion. That being said, some of the best epileptologists I know have never taken a board exam. Because you don’t have to take a board exam to practice in epilepsy.

If your seizures are well controlled, and by well controlled I mean you are seizure free, then your general neurologist is more than adequately capable of taking care of you. If you are still having seizures – once a month, a week, a day, a year – and adjustments are not effective, then it is time to seek the opinion of a specialist, who has done that extra training.

If that doesn’t work out, you kick it up a notch and you see someone who is in an NAEC level four epilepsy center. So that’s the National Association of Epilepsy Centers. They have a certification process whereby they evaluate who the epileptologists are, the neuropsychologists, the nurse practitioners, the entire epilepsy team, and determine if they have all of the credentials that indicate them to be highly competent in their field. If they do, those individuals will get a level three or level four (the highest level) designation. And if you’re having ongoing seizures and have tried multiple approaches, then ultimately you want to end up at an NAEC level four center.

The CURE Leaders in Epilepsy Webinar Series has covered many topics related to epilepsy and innovations in research. Check out our full list of available webinars here.

The information contained herein is provided for general information only and does not offer medical advice or recommendations. Individuals should not rely on this information as a substitute for consultations with qualified health care professionals who are familiar with individual medical conditions and needs. CURE strongly recommends that care and treatment decisions related to epilepsy and any other medical condition be made in consultation with a patient’s physician or other qualified health care professionals who are familiar with the individual’s specific health situation.

Webinar: Rescue Medication Delivery Methods and Future Therapies

Seizures can be both unpredictable and unrelenting. When a seizure becomes an emergency, rescue medications provide immediate relief and help prevent the need for emergency care. While existing therapies do stop these epilepsy emergencies in many patients, some are still searching for an option that works for them.

In the second part of this two-part webinar series, gain insight from Dr. Nathan Fountain of the University of Virginia on how different rescue medications can be administered, promising research, and what rescue therapies are currently in the pipeline. His presentation includes a look at the rescue medication pipeline and the new delivery methods which may become available to patients.

Dr. Nathan Fountain, Professor of Neurology and the Director of the Comprehensive Epilepsy Program at the University of Virginia.

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Audience Q&A with Dr. Fountain

Dr. Nathan FountainIs there any reason not to use Diastat before a seizure in a child who has been seizing for five minutes? And if so, how long should you wait?

There are exceptions to every single rule. As a general principle, if you’ve been prescribed rectal diazepam gel to stop seizures, and you have – let’s say – a child who’s been seizing for five minutes, there’d be few situations in which you wouldn’t give them the rectal diazepam gel. There’s a safety concern, and there’s a treatment concern in this circumstance.

The safety concern is; imagine that you’ve already given the child other benzodiazepines, let’s say clonazepam or lorazepam, or even diazepam in a pill form an hour ago. And so, it’s already in their blood. You might be hesitant to give them more without any kind of supervision. But as a general principle, we give rescue medications and tell them, “If you actually time a seizure, five minutes is a really long time.” We do this in the epilepsy monitoring unit. We admit people to the hospital and observe their seizures to figure out where they’re coming from and so forth.

A common exercise is to ask people when we watched the seizure on the video, “How long was that?” And they almost always say, “Oh, that was five minutes.” But you know what? It’s almost always under 90 seconds – a minute and a half. The point: while you’re watching someone seize for five minutes, it’s kind of forever, especially the big convulsive seizure. For convulsive seizures or medically serious seizures, you might say, usually we would advise giving the medicine after five minutes of seizure activity.

Now, if it’s a non convulsive seizure, or if you’re not sure it’s a seizure, that’s a different situation. I suppose we could imagine a situation where you wouldn’t give them medicine. It was in five minutes because it’s a non-convulsive seizure and you think it’s not causing any harm. Let’s say it’s just a staring seizure, an absence seizure. It is unusual to have one for more than five minutes, but there are specific situations when you might. Or if you’re not sure that it’s a seizure, so if you haven’t figured that out yet, then maybe it would be okay to not give the medicine if it’s something, as in, convulsive activity.

Are there any reasons to administer rescue medications to individuals who have one tonic clonic seizure without a cluster?

That goes back to really defining what it means to have a cluster. As a casual observation, we can easily make the statement, “Sure. Give the medicine to prevent the next seizure.” But if you think about this in detail as the FDA does when they think about exactly what the medicine is used for, this starts with a careful history to determine what is a typical seizure for that individual.

If that individual has big convulsive grand mal or generalized tonic clonic seizures typically lasting three minutes, but they suddenly have three in one day or even twice in one day and you want to prevent the next seizure. Usually you’d let that seizure complete because it would almost take three minutes just to administer whatever you’re going to administer. Typically, we’d let that seizure complete itself, then give whatever medicine is appropriate to prevent the next seizure.

I’m glad that question was asked because in general, we wouldn’t treat an acute seizure unless it lasted more longer than usual or more than five minutes. Now, as I said, five minutes is a long time. By three minutes, everybody’s getting all excited and getting ready to do something – call the rescue squad or given a board of therapy or do something – because it will take your five minutes to figure all that out. But for most people with epilepsy, they don’t have a seizure that lasts longer than three minutes. In fact, if you measure it, it’s usually less than 90 seconds and a half.

If you define the typical seizures as less than five minutes, we typically wouldn’t recommend the currently available abortive therapies. Maybe that’ll change. Maybe if it turns out intranasal medications really worked very quickly and are very effective and maybe we’ll get for ongoing seizures, but at the moment we would say no.

Do you have any suggestions for rescue therapies in children younger than two,  because the FDA approved cutoff for Diastat is two years old.

That’s a situation where you really need to talk to your doctor. There are alternatives. Everyone gets nervous when treating young children. When they’re less than two, it gets a little complicated because the dosing changes as well as the method of administration.

I’d say talk to your doctor about that. For a prolonged seizure in those who are less than two years old, we have the same concerns that we have in those older than two. Although the medications are only approved down to a certain age, doctors can use them in different situations. For example, the IV form of midazolam is definitely not approved to be blown up the nose until now. Still the IV form is not, but yet we would use that in certain situations when we knew the details warranted it. Being less than two, you could still use rectal Diastat for instance, or could use some other form.

Talk to your doctor about that and what might be best in that particular situation.

The CURE Leaders in Epilepsy Webinar Series has covered many topics related to epilepsy and innovations in research. Check out our full list of available webinars here.

The information contained herein is provided for general information only and does not offer medical advice or recommendations. Individuals should not rely on this information as a substitute for consultations with qualified health care professionals who are familiar with individual medical conditions and needs. CURE strongly recommends that care and treatment decisions related to epilepsy and any other medical condition be made in consultation with a patient’s physician or other qualified health care professionals who are familiar with the individual’s specific health situation.

A doctor explains what epilepsy is and what causes it

Webinar: Epilepsy Emergencies and Current Rescue Medications

Seizures can be both unpredictable and unrelenting. When a seizure becomes an emergency, rescue medications provide immediate relief and help prevent the need for emergency care. While existing therapies do stop these epilepsy emergencies in many patients, some are still searching for an option that works for them.

In this webinar, Dr. Kamil Detyniecki of the University of Miami provides an overview of the different types of seizure emergencies, while also discussing the currently available rescue medications.

View our follow-up webinar on the latest advances in rescue medications.

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Dr. Kamil DetynieckiAre these medications combined with other treatments or solo treatments?

These medications are not supposed to replace the daily antiepileptic medications that you take. These are only to be used in situations of emergencies. When you are prescribed a rescue therapy, you continue taking your daily medications. Rescue meds are extra protection in case of a situation that requires treatment.

Are there differences in effectiveness between the different delivery methods of rescue medications such as buccal or nasal or…

The important thing is that these medications get to the system as fast as possible. There are not good studies comparing one to another, because it’s very hard to make a study like that unbiased and blinded (a blinded study is one in which the participants don’t know if they are taking a placebo or not). But there have been some studies comparing rectal diazepam with nasal midazolam, and they seem to be compatible.

During your presentation, you mentioned the side effects of some of the rescue medications. Does delivery method affect these side effects?

Yes, absolutely. So as I mentioned, the nasal administration has the potential for irritation in the nose. That’s one type of side effect. In addition, medications have different half lives, which means they may stay in your system for different lengths of time. For example, diazepam stays in your system longer. After taking it, the effect of sedation may last for a longer time than midazolam. In some patients this may be beneficial, because there maybe longer lasting protection. So one may be better for one patient then versus others.

Is current research suggesting that rescue medications, such as Nayzilam, will be effective?

Yeah, absolutely. For the nasal midazolam, there was a large, multicenter study comparing this medication with placebo. This study showed that the nasal midazolam was much more effective than using the dummy medication. That’s what we use and that’s what the FDA used to approve this medication.

When is a patient considered to be in status epilepticus? Does the length of time vary between children and adults?

Definitions have this problem that they’re never perfect and this is what we have right now. And I think that, again, in terms of when to treat, it’s different. One definition may say that status epilepticus is five minutes, but this doesn’t mean that we need to wait five minutes in order to start treatment.

That is what has been shown based on animal and human data; if a seizure goes for five minutes or longer, there’s a less likelihood that it will stop on its own. For some patients, if a seizure is one, two minutes long, it may be too long for them. Every patient is different, but not aware of any that we’re changing that definition.

What would you say to say school teachers or school personnel who refuse to administer a rectal medications?

There’s been a lot of debate about having the school administer those medications. I think that the problem with the rectal administration maybe resolved now that we have nasal. Again, rectal is troublesome in patients’ privacy. But now that we have the option of nasal, hopefully that won’t be a problem.

Can diastat be given twice at one time if the seizures persist after the first dose ? Or do you have to wait a certain period of time between doses?

Typically the dose is calculated by weight. There is a possibility of giving another dose, but I would normally wait at least 10 minutes or more to know if the first dose had an effect. We have to always assess whether the patient may be overly sedated, having any difficulty breathing, etc. But those discussions about the dose need to be specifically addressed with with the neurologist. I can’t give you an answer for everyone.

But to answer shortly. Yes. In some patients, we can.

Is there an average length for how long it takes a rescue medication to take effect?

Yes. And really it depends on the type of rescue medications. Oral medications can take much longer. That’s why we’re so excited to have different routes of administration. If you swallow a pill, it may take 20 minutes or more to start working. A convulsion going on for a long time, it’s unacceptable.

Nasal may work as fast as 10 minutes, and there are new medications that are being searched in and are being researched that may work even faster as fast as IV. So there’s different times depending on the type on the medication and the route of administration. But the fastest we have right now are the rectal and the nasal.

What are the differences between Versed and Nayzilam?

The active compound is the same. Versed is the brand name of midazolam, which is the same compound which in this brand name Nayzilam. The main difference is that when you use the off label midazolam, like I showed this picture of this young kid getting the Versed with a syringe or a spray. That is because we’re using a product that is being developed for IV, it’s very diluted. You need to use much more volume or much more amount of liquid that it actually doesn’t all get absorbed in the drips behind the nose, so it’s not ideal.

This product that was FDA approved, Nayzilam, is a much smaller concentration, so it’s just one dose, and so that is an improvement compared to the off label, but the actual medication that is being used is the same.

If you can use the nasal spray to stop a seizure and it’s not effective, can you switch rescue medications and give Diastat?

These are great questions and that’s why it’s important to have a rescue plan. Every rescue plan needs to have an option. What if the rescue medication doesn’t work? When can you use another dose, should you call 911… Again, this is not an answer for everyone, and it really depends on the age of the patient and the dose that is to be given.

Using different types of rescue medication at the same time is possible, but it’s unusual. There are rescue medications that you can repeat after five or 10 minutes. It’s not that commonly used but it’s possible. But this is something that should be discussed and patients should ask that of their neurologist.

Again important to have a rescue plan where we discuss these situations… what to do with one medication doesn’t work. Can I use a second dose? When should I call 911? And so on.

If a patient already has a benzo, such as Onfi, as part of their daily AED regime, are the rescue meds effected?

The good answer is: it’s possible. There’s a phenomenon of tolerance to benzodiazepines, and so if a patient is on Onfi or clobazam, it’s possible that they may require a higher dose of rescue medication.

And this is something that is going to need more research for the newer medications. For the new medication, Nayzilam, the patients in the study were not allowed to be on benzodiazepines. And so we need more information about it. It’s definitely not a contraindication, but it may be that the patients may notice that they may require a higher dose.

Are there resources available for school personnel or other professionals on how to use rescue medications? 

I think that the Epilepsy Foundation is a great resource. They have examples of rescue medication plans.

Are there sublingual rescue medications that would work more quickly than a pill?

People are using lorazepam or clonazepam buccally or sublingually. I think the exact absorption has not been studied as well, but there’s a potential that it works faster than swallowing the pill. Although many of these pills are not meant to be used sublingually, so they may not dissolve as fast.

There are companies trying to develop different products, for example, a film that goes into the cheek to get absorbed faster. It’s an active area of research looking for different routes of administration.

For a child who has had a history of refractory epilepticus, when would it be ideal to administer the rescue medications or call 911? This person in particular is not comfortable waiting five minutes.

So, the answer is right there. If you’re not comfortable waiting five minutes, you shouldn’t do that. There is not an answer for everyone. And that’s why I continue to mention that which rescue medication you use, when to administer it, etc. should be a decision between the patient, caregiver, and the doctor depending on the user’s seizures, what kind of seizures they have.

For example, you may be willing to wait longer if you’re having a focal motor seizure, which is a seizure where you have, for example, motor activity without loss of awareness. These seizures can go on for several minutes without causing any significant harm to the patient, but a tonic-clonic seizure going on for four or five minutes, it can be potentially a concern.

The CURE Leaders in Epilepsy Webinar Series has covered many topics related to epilepsy and innovations in research. Check out our full list of available webinars here.

The information contained herein is provided for general information only and does not offer medical advice or recommendations. Individuals should not rely on this information as a substitute for consultations with qualified health care professionals who are familiar with individual medical conditions and needs. CURE strongly recommends that care and treatment decisions related to epilepsy and any other medical condition be made in consultation with a patient’s physician or other qualified health care professionals who are familiar with the individual’s specific health situation.

Epilepsy Surgery: Advancements, Options, & Considerations

For some people with uncontrolled seizures, epilepsy surgery is an effective and important option to consider. This webinar will help patients and caregivers approach this difficult and sometimes confusing conversation with their doctors.

Webinar presenter Dr. Kate Davis explores recent advances in epilepsy surgery, which have increased not only the types of surgeries available to patients but expanded who is an appropriate candidate for surgery. Dr. Davis also discusses the different tests performed as part of an epilepsy surgical evaluation and review current surgical options including resective surgery, laser ablation, and implantable devices.

Dr. Kate Davis is Medical Director of the Epilepsy Surgical Program and Epilepsy Monitoring Unit at the University of Pennsylvania.

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Plus, get the patient perspective on epilepsy surgery from this episode of our Seizing Life podcast.

Audience Q&A with Dr. Davis

How do you make sure that someone won’t have any new problems with memory or function after surgery?

There are some parts of the brain that we don’t worry about it that much. However, there are a lot of parts that are really critical real estate. The neuropsychological testing is really helpful for us to determine whether there’s risk. There are also clinical factors that come into play. The age that the seizures started is really important. If seizure started really early in life, most people will have had a lot of reorganization of the brain and the areas of the seizures are usually not functioning. We can do some testing for that.

The functional MRI can look at where language is. We can also look at where motor function is, or what allows you to move your hands or your legs. With patients who have intracranial EEG, we can use those electrodes to map function. We do a combination of these things, plus our knowledge of what brain regions do in most people’s brains, and that can help us make an assessment of risk. Then that should be discussed by your treatment team with the family and the patient, if there is risk.

Are the ketogenic diet and AEDs are tried before surgery even becomes an option?

I briefly mentioned that the ketogenic (keto) diet is mostly used in pediatrics. I’m not a pediatric epilepsy specialist, but many pediatric patients will try the keto diet with seizure medications before considering surgery. Not all. That’s something definitely should be discussed with your treatment team. In adults, there’s very little evidence that the keto diet and other dietary therapy substantially helps with seizure. We do not have a trial of a diet therapy before we consider surgery because the data is really not there to support that decision.

Can deep brain stimulator (DBS) or RNS can ever be implanted in pediatric patients?

I don’t know the specific age cutoffs because I don’t treat pediatrics, but the NeuroPace device is designed for the skull or the head of a seven-year-old or older. I think they are trying to change labeling to push the age back to pediatrics, and there are some centers that are implanting NeuroPace devices in pediatric patients. It is being done. Some of it is off label use, meaning it’s not under the FDA-approved label. The deep brain stimulator device is much newer, and I honestly don’t know if there are pediatric sites that are implanting that yet. I’m sure that we’ll learn that soon. I know that the pediatric epileptologists are very passionate about bringing these kind of technologies to their patients when they think it’s indicated.

Can you ever skip any of the tests, or is there a certain order that you always have to go through?

In the presentation, I went through a whole laundry list of tests. Not every patient needs each one of those tests. There is some variation in what centers use which tests. Some centers may not have availability of some tests, or have more experience with certain ones.

One test that I did not cover is a Wada test, which is done less frequently, but still at many centers is done. We will sometimes do Wadas in some patients. We will frequently do a functional MRI before doing that. Wada, just as an offside, is a test that’s been done for a very long time in epilepsy that is more invasive than the functional MRI, and that’s really one of the reasons there’s a move away from that test. Because it involves an injection of a drug that puts one side of the brain to sleep for a few minutes, during which time the neuropsychologist can do testing to look at function. Then you do it for the other side of the brain. That can give us information trying to determine the risk of the memory or language problems after a surgery, going back to the first question. In isolated cases, we are still doing Wadas.

I hesitate to say there’s a certain group of tests that each patient really needs. At a minimum, a brain MRI and EEG data is really done I think at every center. Then there’s some variation.

The CURE Leaders in Epilepsy Webinar Series has covered many topics related to epilepsy and innovations in research. Check out our full list of available webinars here.

The information contained herein is provided for general information only and does not offer medical advice or recommendations. Individuals should not rely on this information as a substitute for consultations with qualified health care professionals who are familiar with individual medical conditions and needs. CURE strongly recommends that care and treatment decisions related to epilepsy and any other medical condition be made in consultation with a patient’s physician or other qualified health care professionals who are familiar with the individual’s specific health situation.

A cute little girl in a yellow shirt holding a tomato in front of a bowl of salad.

Epilepsy and Dietary Therapies: How What You Eat May Help Control Seizures

For individuals with epilepsy – particularly refractory epilepsy – change of diet can be a recommended therapy for seizure control. While the ketogenic diet has been around for almost a century and is the arguably the most well-known dietary treatment option, today, there are multiple diets used to treat specific epilepsy types and syndromes.

In this webinar, two neurologists present both the research and clinical perspectives of dietary therapies. Dr. Jong Rho speaks to the science backing the use of dietary therapies to control seizures, and Dr. Eric Kosoff discusses how doctors determine which patients to recommend these therapies for, as well as how patients can work with their doctors to navigate these options.

Dr. Jong Rho is Professor of Pediatrics, Clinical Neurosciences, and Physiology and Pharmacology at the University of Calgary and Dr. Eric Kossoff is Professor of Neurology and Pediatrics at Johns Hopkins Children’s Center.

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Plus, listen to our Seizing Life podcast episode featuring registered dietician Robyn Blackford and advanced practice nurse Breanne Fisher for more information about using the ketogenic diet to treat epilepsy.

Audience Q&A with Dr. Kossoff and Dr. Jong Rho

How long does the average person stay on the ketogenic diet? And then, the second part of that question is, what are some of the factors that that you look into when coming off the ketogenic diet?

The duration of the ketogenic diet is a question that has been looked at with some research both in terms of the minimum time, how long you should give it and perhaps the case where it’s not effective. But also, maybe more interestingly, the maximum time at which point is maybe the ketogenic diet outlived its usefulness or maybe it’s led to the benefit you’re going to see and you can come off the diet without negative effects. And so, most of the time, we will tell families give the diet at least two to three months.

There is some scientific evidence that if you’re having seizures frequently enough, even within two to three weeks, you’re going to see potential benefit. Unlike some therapies, the ketogenic diet seems to work relatively quickly, maybe again within a week or two. And so, we generally do still recommend for families that make the commitment and engage in the ketogenic diet as a therapy to give it two to three months before giving up if they perhaps don’t see any benefit. On the opposite end in terms of how long to stay on the diet, the tradition, at least in the pediatric world is two years similar to medications. So, if you’re doing well or especially if you’re seizure free, at about two years on the ketogenic diet will often check in EEG. And if you have an epilepsy that potentially could be outgrown, we will slowly wean the diet by reducing the ratio usually every week or two until the diet has been stopped and you’re back on regular foods.

For certain epilepsies like Glute 1 deficiency where diet may be helpful even into adulthood, you may stay on the diet much, much longer. For some epilepsies like infantile spasms, there was a randomized trial comparing six months to two years and they found six months was just as effective in terms of diet duration. For that epilepsy, we may go shorter periods of time. But in general, on average, it’s about one or two years in the pediatric world. In the adult world, they often stay on for longer if it’s helping. But in pediatrics about two years.

What are the steps that someone might take to initiate the diet? Meaning, blood work, other tests, et cetera.

It really is important that this should be done with supervision at an epilepsy center. We do have families that are starting on their own and sometimes it works out, but other times they can have adverse effects or even just not the efficacy they were hoping for. So, it really should be done in an epilepsy center. There are labs that are recommended to be done in advance to make sure there’s no reason that the diet could potentially cause a problem.

As Dr. Rho mentioned it is a metabolic big change to the neurons into your body. And so, it can have some adverse effects if there is a problem in metabolizing the fats. So, we usually recommend labs to be done in advance. We usually as well spend a lot of time talking to the families just about goals, expectations. What’s going to happen during the week, maybe changing their medications to a tablet formulation, so there’s less carbohydrates. We often will do that. And so, there’s a fair bit that goes in before the ketogenic diet is started. And a lot of that does involve neurologists, it involves dieticians and really families just reading and getting information before jumping down the road of the ketogenic diet.

Is there potential harm be it cardiovascular risks, renal risks, hepatic risks of somebody on the ketogenic diet long term?

This is actually a topic that we’re really actively looking at. There are a lot of investigators trying to look into the long-term side effects. At least right now, the three major long term effects that have at least been reported are bone density changes and bone fractures can happen if you’re on ketogenic diets for over five to six years. This is true for a lot of our anticonvulsant drugs. And it may be, again, as many patients are on both more related to drugs. But we do certainly see that this can happen with ketogenic diet therapy. So, bone health is one. Growth is something that we do see as a problem in children who were on ketogenic diets for prolonged periods of time.

This may have more to do with again the bones than the actual diet components. But children who are on the ketogenic diet, sometimes their height velocity can be affected if they’re on it for many years. And then, the last one, at least so far appears to be kidney stones. And so, if you’re on the diet for long periods of time, we make sure that your kidneys are being monitored. We often will use a supplement called polycitra to help prevent kidney stones. And so, those are the three at least current in 2019 long term side effects that we’re aware of. But there’s a lot of research being done looking at cardiovascular health, carotid artery changes and looking at long term effects that we really just don’t know about. But not knowing doesn’t mean it’s not a potential risk or side effect. So, stay tuned. And I think we’ll have more research in this to come.

If there has been success on a medically managed ketogenic diet i.e. they’ve become seizure free on the die, has there been any research or does science suggest that a person could have a higher chance of experiencing seizures again?

There’s been a little research into how you wean the diet and what happens. And before I jump to that question I guess, most of the studies would say about 80% of the time, what happens on the diet will remain when you come off a diet. So, for example, if you’re seizure free, you stop the diet, and this is again in pediatrics. 80% of the time, your seizures will remain gone when you come off the diet. And if you’re 90% better and you wean the diet in odds, you’ll stay 90% better without it. But there certainly are children, it tends to be more in the short term as you wean the diet, their seizures can get worse and you go back on the diet.

There are some patients who maybe years later depending on the epilepsy they have may have a recurrence. We are certainly seeing in our adult epilepsy diet center some patients who were on the ketogenic diet when they were young children were taken off the diet, maybe tried a few more medications over their adolescence. Now, they’re adults and they go back on the diet in adulthood. It’s a small number of patients, but certainly, it happens in the diet. Again, it seems to be equally effective in adults as in children.

Are there ways to increase ketosis when you follow a diet that’s more based on meat?

So, there are several ways you could do that. Obviously, fasting calorie restriction is the classic way you induce ketosis. If you take the diet and it’s high in fats then the fatty acid oxidation will produce the ketone bodies. There’s a growing number of sources for ketone supplements that are currently available through the web. And the number of experimental studies looking at various formulations of ketone bodies such as ketone esters that have been shown in animal models to be effective. We don’t have any human data yet, but those are being planned. The ketone esters are interesting because they’re orally ingestible. They can be broken down by the body through enzymes that are resident in the gut as well as in the bloodstream, the so-called ester ACES.

And that what that does is it produces ketones to be elevated in your blood. There are also certain foods that have a higher tendency, perhaps to produce ketone bodies, the so-called MCT diet, for example, has been historically observed to maybe induce greater ketosis. But again, in terms of whether that’s a proven fact or not. I think Eric can certainly comment on that better than I can. So, I think ketones because of their pleiotropic mechanisms that have been described in the last five years, there’s a growing interest in trying to figure out a way of providing ketone supplements, enhancing ketosis without necessarily going to the traditional diets themselves.

It’s still early days. I would be cautious in the sense that not all available formulations are necessarily safe in large quantities and these things are constructed also with various salts and ways of making them ingestible. So, they may produce some degree of toxicity as well. And keep also in mind that the relative amount of ketosis is important. Mild to moderate degrees of ketosis that we see in clinical therapy with the diet is tolerated. Although, certainly diabetics who go into major ketoacidosis, something we don’t see in ketogenic diet treatments, high levels can actually induce health problems and in the extreme case, coma. So, dose does differentiate a remedy from a poison. So, as a famous pharmacologist from centuries ago would say.

Is there research into the effectiveness of the diet for those who experience nocturnal seizures versus those who experienced seizures during the day?

It hasn’t really been looked at in that manner before. We certainly see a lot of pediatric patients who have more seizures at night than during the day. And the diet seems to be effective for those types of epilepsies as well as those that have more during the day. It doesn’t seem to make much of a difference. We do sometimes target the way we provide the diet based on when the seizures are happening. So, in some children who have predominantly nocturnal seizures, we may give a higher ratio or an extra fat supplement at bedtime to try to get their ketones higher during the evening that can sometimes be successful. But to my knowledge, I don’t know if Jong knows. I don’t know anyone who’s looked specifically at treating those only with nocturnal seizures versus those with daytime or with maybe a combination. I think we just don’t know that yet.

It’s a very interesting question, certainly. The topic for more focused future clinical studies. What I can say from the basic science side is that it’s known that the epileptic brain has derangements in sleep wake cycling and rest activity cycling. So, there’s perturbations that occur and it’s not surprising that many forms of epilepsy actually manifest at night for example or during sleep wake transitions. When you actually feed epileptic mice or animals with the ketogenic diet, there are restoration of the sleep wake cycling and the rest activity cycling such that the prediction would be that would have perhaps efficacy, perhaps on those that tend to manifest mostly during sleep, for example. But we don’t have any real strong clinical data to show that, that’d be a subject for an interesting clinical study for sure.

Are there quantifiable EEG changes once somebody is on the diet?

Yeah, that’s actually somewhat controversial topic. There has been some evidence that supports that. That suggests that there can be changes in the EEG sometimes within a few weeks. There’s one study out of Texas that suggested that if there was a decrease in the amount of slowing, slowing someone suggesting a diffuse change to the brain, not necessarily an epilepti-form one. But if there were changes to slowing within that first month, they were much more likely to respond in terms of seizure reduction later on to the ketogenic diet. And there have been some studies that have looked at certain epilepsies where they follow the EEG and shown that yes, the EEG can improve just like the clinical seizures can improve.

On the other hand, there actually have been other papers that have really said there’s a big disconnect. The seizures may seem like they’re clinically improved but the EEG may actually not have changed very much. There’s one epilepsy called ESES or Epilepsy with Status Epilepticus and Sleep. Where really the goal of that treatment for that condition is to improve the nocturnal EEG. And the data is actually relatively mixed about how just affected the diet is and improving that EEG. Patients may be better but the EEG doesn’t always change very much. So, it’s a hot topic. There’s a lot of debate and discussion. When we see families and we talk to them about the ketogenic diet at our center, we really focus primarily on the clinical seizures. And actually, there’s some good data now about cognitive benefits out of the Netherlands. But usually, we don’t promise or guarantee for any patient that the EEG is necessarily going to change.

Based off the effectiveness of the ketogenic diet with certain medicines and things of that nature. Has there been research into the use of the ketogenic diet with AEDS? And also, does the ketogenic diet work when somebody is not on any AEDs?

It’s usually a partnership. We tell families that for most patients, and actually we have a recent study that suggests that it’s again, about 82%, 83% of our families still remain on medications, it might be fewer medications, but it’s usually not the ketogenic diet by itself. Although, that did happen in about 20% of patients who usually did very well. We were able to wean them off their medications. But we tell families for the most part, it is a partnership. There is no one drug that is negatively interacted per se by the ketogenic diet.

We know that certain drugs you have to be a little bit more cautious for side effects. So, topiramates, zonisamide, you have a slightly higher chance of acidosis. Valproic, you may see a higher chance of carnitine deficiency. You might see reduced ketosis if you’re on valproic. But these drugs are not contra indicated so to speak, it’s not a situation where they have to stop those medications. And on the other hand, we also don’t have any evidence that any one drug seems to be really effective with the ketogenic diet.

There are lots of drugs often tried. But we don’t have any one drug in particular, that seems to be particularly beneficial. If anything, we have some older data that the Vagus nerve stimulator may in combination with the ketogenic diet be potentially synergistic, but that’s obviously not a medication. So, I think as more patients are put on different therapies, we can look for those beneficial synergistic effects. But at least right now, we tell families that any drug is fine, no drug is perfect with the diet, and we’ll just do it case by case.

Is there a range that’s recommended for ketosis in terms of blood meters?

I think much like when we use anti-seizure drugs and we do blood levels of the drugs, we have a rough idea based on clinical experience where patients should be or could be, but these are guides. There are no absolute numbers and everyone’s response is going to be a little bit different. In fact, the same level in two different patients, one may tolerate very well, the other one may get very sleepy or tired. So, I think the levels themselves are really a guide. We tell families that the ketone levels are like a drug level and every child is different. Some children only need to have relatively low ketone levels and the low glycemic index, which is one of our dietary therapies.

You have extremely low ketone levels and actually in the urine, they’re undetectable, yet these children do very well. And so, I think, especially when we hear the mechanistic potential options for why the diet works as Dr. Rho presented, there’s so many mechanisms at work that ketones may just be part of it. And it may suggest the body has made this metabolic change but it may not really be the true aspect of why the diet’s effective. So, I think every child is different. We try to keep a calendar of what their ketone levels are, and in a sense for where maybe, okay, the ketone levels seem to correlate with seizure control and try to achieve that for every child individually.

For somebody who is considering the ketogenic diet or is maybe new to the ketogenic diet, what resources are available to those patients and to those families?

One major website that I would recommend is the Charlie foundation for ketogenic therapies website. So, https://charliefoundation.org. All one word, charliefoundation. It’s a good place to start. They’ve been around for a long, long time. And the fact the Charlie Foundation was founded in the mid-1990s. And it was really seminal in catalyzing both clinical interest in research in the ketogenic diet. And we’ve actually looked at this in a research way and really families find out about the ketogenic diet from the internet nowadays is not all from books and magazines and other resources. It really is the internet. So, I always recommend the Charlie Foundation webpage.

There’s another organization in England called Matthews Friends. It’s site, S-I-T-E, .matthewsfriends.org. That like the Charlie Foundation is a parent support with great resources and articles that patients can download. I also have helped the Epilepsy Foundation through their webpage, which is epilepsy.com. Good information about ketogenic diet along with all the other therapies as well. But those are probably my top three.

The CURE Leaders in Epilepsy Webinar Series has covered many topics related to epilepsy and innovations in research. Check out our full list of available webinars here.

The information contained herein is provided for general information only and does not offer medical advice or recommendations. Individuals should not rely on this information as a substitute for consultations with qualified health care professionals who are familiar with individual medical conditions and needs. CURE strongly recommends that care and treatment decisions related to epilepsy and any other medical condition be made in consultation with a patient’s physician or other qualified health care professionals who are familiar with the individual’s specific health situation.

Webinar: Post-Traumatic Epilepsy (PTE)

Dr. Ramon Diaz-Arrastia, Professor of Neurology, University of Pennsylvania
CURE presents a leading expert on Post-Traumatic Epilepsy

In CURE’s Leaders in Epilepsy Research webinar, participants hear from a leading expert on Post-Traumatic Epilepsy (PTE), epilepsy resulting in physical trauma to the brain. The webinar reviews efforts underway to advance our understanding of PTE, as well as the exciting new therapies being developed that may one day result in a cure.

It is presented by Ramon Diaz-Arrastia, MD, PhD, of the University of Pennsylvania. Dr. Diaz-Arrastia is an expert on the molecular, cellular, and tissue level mechanisms of trauma-induced neuroregeneration and injury-related synaptic plasticity. He works to develop effective therapies for Post-Traumatic Epilepsy.

Plus, hear more about the history and current state of PTE research from this episode of our Seizing Life podcast.

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Audience Q&A with Dr. Ramon Diaz-Arrastia

What strategies may be developed to help prevent post-traumatic epilepsy?

Through our work on understanding the mechanisms, I did point to some strategies that may be very attractive. So, I do think we now know that anti-epileptic drugs are not epileptogenic necessarily, and we rather need drugs that are going to promote the integrity of the axonal connections into the hippocampus. We may also need drugs that promote the repair of the blood brain barrier and perhaps block some of the long acting or long duration inflammation that occurs in the brain. So, the goal of developing or the mechanism of developing anti-epileptogenic drugs is really going to be tied in to these strategies to promote the integrity of resilience and recovery of these particular neural structures.

If you have decades of seizures and epilepsy and you’ve had multiple brain surgeries, would you say that you have a brain injury? Would that be an accurate statement? Obviously, it’s not a TBI, but would you call it an ABI?

Of course, I mean, I think obviously epilepsy can occur from many different consequences, but certainly anything that injures the brain, be it a traumatic insult to the head, or be it an ischemic insult or an inflammatory insult or an infectious insult can result in epilepsy. Now, many cases of epilepsy, such an insult is not recognized, which is not to say that it wasn’t there, right? It may have been a subclinical or a subtle insult, but nonetheless resulted in an injury. So, now, I do think the answer to the question is right, that in most cases epilepsy does result from some kind of injury and that discovering how that happens is potentially a value in preventing the development of epilepsy and also potentially treating the seizures after it already develops.

Are there specific symptoms that indicate if epilepsy is due to a brain injury as compared to other epilepsy causes?

Most patients who develop epilepsy after brain injury develop a focal epilepsy syndrome although it can be anywhere in the brain, right? It turns out that temporal lobe epilepsies appear to be the most common. There is something about the temporal lobes, particularly the mesial temporal structures that make it uniquely pro epileptogenic, but we certainly can have frontal lobe epilepsy, parietal lobe epilepsy, occipital lobe epilepsy. It can start from anywhere where there is an injury and the manifestations of the seizure obviously depends on where in the brain the seizure is starting. So, the manifestations of the frontal lobe seizure are going to be different from a temporal lobe seizure, or an occipital loop seizure. So, it’s the kind of stuff that requires careful evaluation, and in most cases require admission to an epilepsy monitoring unit for video EEG monitoring. But yes, one can determine what kinds of epilepsy develops from what.

We have a question about veterans and the prevalence post-traumatic epilepsy in the veteran population to other types of populations with head trauma?

Veterans are at a particularly high risk of suffering traumatic brain injury, and this is a consequence of their military service. Veterans or military personnel are at high risk of traumatic brain injury, obviously in combat settings but even during practice or during training and just living in a harsh environment is risky and there’s a high incidence of brain injury from that. Brain injuries are very common in a general population, but individuals who have served in the armed forces are at approximately a three to four fold increased risk of having a brain injury and a correspondingly increased risk of developing post-traumatic epilepsy. So, this is a major problem to the Department of Defense, Military Health System, as well as for the Veterans Administration Health System. A lot of the research in this area historically has been funded by the DoD and the VA.

Do you happen to know the percentage or approximate percentage of post-traumatic epilepsy patients that are candidates for brain surgery?

Unfortunately, that’s a very hard number to know. It’s certainly not the majority, but it could be as much as 10, 20%. I think everyone who has epilepsy that is refractory to medications, which means that they have been on good doses of at least two anti-epileptic medicines and they continue to have frequent disabling seizures. That is someone who regardless of the etiology of their epilepsy, regardless of the cause of their epilepsy, should be referred for Epilepsy Monitoring Unit Admission and Video EEG Monitoring to determine if they are surgical candidates. I think surgery remains an underutilized therapy option in post-traumatic epilepsy, as well as in many other causes of focal epilepsy.

What are your thoughts about the predisposition to Alzheimer’s after one has had a closed head traumatic brain injury?

This is another area my laboratory is very busy in investigating. So, it appears that individuals who sustain a moderate to severe traumatic brain injury are at greater risk of developing late life dementia. That risk is somewhere in the order of about three to four fold for severe traumatic brain injury. But then even mild traumatic brain injury increases risk of late life dementia modestly, but probably by around 30 to 50%.

Now, that doesn’t sound like much. On the other hand, given that late life dementia is so common, even a modest increase in relative risk does translate into a large number of cases. Now, whether that dementia is Alzheimer’s disease or whether it is some other kind of late life dementia, that remains to be proven, right? Again, that’s one of the things that we very much need to investigate is if we can identify what is the mechanism of that risk of late life dementia after a TBI, what can be done to prevent it? Do we have any strategies that could help in promoting the resilience or promoting the recovery of the brain after a brain injury? And we just don’t know yet, but it’s an area of very active research.

Do you feel that there is an ideal anti-epileptogenic drug profile?

The ideal anti-epileptogenic drug profile is one that controls the seizures 100%. It doesn’t produce significant side effects, right? Now, that ideal drug is going to be different for every patient, right? But many patients are able to find a drug or drug combinations that is ideal for them, meaning that it controls the seizures and allows them to continue their life without significant side effects.

Unfortunately, that only happens about 60% of the time. So, that is a significant number of people and many of the drugs that we have are good drugs, but that remains that about 40% of patients with epilepsy and that includes post-traumatic epilepsy as well as other causes are unable to find an ideal drug or drug combination that works for them. That remains a big problem, which certainly stimulates our work in the area trying to develop preventative therapies and also trying to develop better symptomatic therapies.

What about starting a ketogenic diet post TBI as a possible seizure prevention protocol?

I think that’s a potentially good strategy, right? There are some work primarily so far in animal models that a ketogenic diet appears to be neuroprotective, appears to prevent the death of neurons in some of the aberrant synaptic plasticity. Research in this area is very, very early, I must say. It’s so far mainly in animal models, but it certainly looks promising. Not an unreasonable approach to try.

What about researching the space dealing with devices such as brain cooling?

Likewise in animal models, there is very good evidence that cooling the brain soon after the injury prevents the development of post-traumatic epilepsy. In fact, helps in preserving neural structures and preventing neuro degeneration. So, I think that is likewise an attractive strategy. So far, it’s really only been tried in preclinical models. It appears that focal brain cooling is what’s important. There have been several studies in patients with TBI doing whole body cooling, and that does not appear to work as well. It’s mainly because cooling can have deleterious effects on pulmonary function and cardiac function and renal function. But focal brain cooling is certainly a promising strategy.

Do you feel that preventing hippocampal neurogenesis after TBI is a viable therapeutic direction for preventing mesial temporal lobe epilepsy?

So, there is neurogenesis in the hippocampus after TBI. In most cases, that is a good thing because that allows the recovery of memory function and attention and things that the hippocampus does. So I think one would have to be very, very careful about a strategy where one tries to prevent the normal healing pathway.

On the other hand, it is likely that as I mentioned earlier in my talk that post-traumatic epilepsy may result from those attempts of the tissue to rewire and repair itself, that some of those repairative processes may not be totally perfect and result in a circuit that is epileptogenic. So, I would say that one has to be very careful and one has to be very precise on strategy, such as this, and obviously develop strategies that target the aberrant neurogenesis and the aberrant synaptogenesis while leaving the neurogenesis and synaptogenesis that are important for more recovery in place.

Are sodium channel blockers therapeutic in post-traumatic epilepsy?

Dilantin for examples is one of channel blocker and it does not appear to be effective, although it blocks the early seizures, does not appear to be effective as an anti-epileptogenic agent. I would say that although not every sort of general blocker has been tested, more likely we are going to need alternate strategies to come up with a truly anti-epileptogenic compound. Those alternate strategies are going to have to rely on things like blocking inflammation or promoting repair of the blood brain barrier, or perhaps promoting integrity of the external pathways that become disconnected. I’m personally not that hopeful that just because a drug is anti-epileptic, that it will be anti-epileptogenic. I think the data we have so far seems to indicate that it’s not the case.

Are there any specific group of neurons implicated in post-traumatic epilepsy?

I think that there’s not any one specific group. I think that depends a lot on the location. I think what tends to happen is that when you have a brain injury, many neurons are lost and are damaged, and then the neurons that remain attempt to rewire in order to repair the function of the circuit. But that rewiring is sometimes not perfect and that’s how an epileptic circuit presents itself. So, it appears to be mostly the neurons that are relatively resilient to injury that may be responsible.

Again, I think it’s a two-edge sword. You would not want to prevent epileptogenesis with a strategy that also prevents the repair and recovery. We would have to be a lot more precise and a lot more clever to come up with strategies that prevent the aberrant synaptic plasticity while allowing the functional or the positive synaptic plasticity to persist.

Can seizure type after a traumatic brain injury be a basis for predicting the risk of recurrence?

I don’t think we know the answer to that yet, right? I think what very frequently happens that it often takes a while for the diagnosis of post-traumatic epilepsy to be made. When we talk to patients who have developed epilepsy after a brain injury in reality, they often have subtle behavioral problems or subtle memory problems that proceed the development of clinically apparent seizures. In retrospect, those were probably very small, very focal seizures that were occurring before the clinically apparent seizures presented themselves. So, I think the answer is that we’re going to need strategies that allow the diagnosis of post-traumatic seizures very early on in many cases before it’s very clinically apparent in order to develop effective therapies.

Are you hopeful about the near term future for patients with TBI?

I’ve been working in this field for about 25 years and we have had great progress. On the other hand, when I started working on it, I certainly would have hoped that we would have been further ahead by now. So, I have long accepted and I’ve told my wife and children this that optimism is one of my personality flaws. So, I do believe that we will develop some effective anti-epileptogenic therapies before I retire, right? Obviously, that’s only a belief in terms of having very solid therapies.

I do know that the amount of research in this field has skyrocketed over the last several years. This is mainly through the support of the Department of Defense, but also through the CURE Foundation and many others. We now have much better animal models. We have much better understanding of what’s going on at a cellular molecular level. We have much better biomarkers, be the imaging biomarkers or molecular biomarkers. So, I do think we have the tools in place, and we certainly have some strategies that appear to be successful in animal models. Obviously, translating those into humans is not necessarily an easy or even a linear proposition. But I am an optimist, and I think the answer is that we will have something in the next 10 to 15 years or so.

Webinar: Sudden Unexpected Death in Epilepsy (SUDEP)

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A webinar discussing the population at risk for Sudden Unexpected Death in Epilepsy (SUDEP) and what risk factors may be involved. Dr. Elizabeth Donner explores the personal impact of SUDEP, the latest research statistics, and what resources are available to the community. Part of the CURE Leaders in Epilepsy webinar series.

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Audience Q&A with Dr. Elizabeth Donner

What are some of the barriers that healthcare professionals have when talking about SUDEP?

One of the big issues has been the fear of healthcare providers that learning about SUDEP, that if they’re patients or parents of kids with epilepsy, learning about SUDEP, that they will actually be causing more harm than good, that the anxiety produced by learning about SUDEP will surpass any potential benefit. But there’s actually been some nice studies about this. There’s really no evidence that learning about SUDEP causes any undue anxiety or ha There has been some qualitative research done by my colleague Rajesh Ramachandran Nair at McMaster University where he’s talked to parents of children with epilepsy and people living with epilepsy about their interest in SUDEP information. They clearly have expressed a need for more information about SUDEP.

There has been a study out of Susan Duncan’s group in the UK that showed that, upon learning about SUDEP, there was no significant change in anxiety or worry related to their risk. That’s probably been the biggest barrier.

Talking about face down position, does this indicate suffocation? If so, are there things that can be done to prevent that?

I want people to think about the fact that when a person is sleeping and they’re in otherwise good health, if they roll over and their face goes into the pillow, and this probably happens to us a few times a night, what happens is, we’re not breathing well. Actually, our brain wakes us up and we roll over. You don’t suffocate while you’re sleeping because your brain has a mechanism to know when you’re not breathing well when you’re sleeping, and you wake up. What might be happening in some SUDEP cases is that, after people have a seizure, and many of you will know that often in the immediate period of time after a seizure, people can be, what we call a postictal state. Quite sedated, unresponsive like they’re in a very, very deep sleep.

It’s quite possible that the brain in that state is unable to respond to the cues that allow us to roll over when we’re breathing into our pillow. We don’t exactly call it suffocation because what’s happening is the effect of the seizure has made it such that the brain doesn’t wake up because the person is face down in their pillow. I hope that answers the question, and I recognize it’s a bit of a complicated explanation. With regards to the more practical side of the question around, well, does that mean that people would be safer if they just slept on their back all the time? There is some newer research coming out that shows that actually some SUDEP deaths people are found on their side or on their back position as well. Even if that was the case, it wouldn’t completely fix the problem, but there’s two important considerations.

One is that when we look at people who are having seizures, and we have the opportunity through our video EEG monitoring to review lots of people having seizures on videotape, we can see that most of the time in a generalized tonic-clonic seizures, the movements of the body causes the person to roll over onto their stomach. Even if you could convince the body to always stay sleeping on the back, it’s quite possible that during a seizure, a person flips over onto their stomach. The other is we move around while we’re sleeping. There are some tricks, different things you can wear and stuff that make it uncomfortable to sleep on your stomach, but even if we were to do that, it’s quite likely that the body flips over during a seizure. Unfortunately, I don’t think that’s been very well developed as a prevention technique.

Are there any wearable devices that could monitor epilepsy and that risk for SUDEP?

Something that gets a lot of attention, for sure, is the advent of all these new epilepsy monitoring devices. There are quite a few devices available to the general public that monitor for seizures. There really is no evidence that these devices actually reduce SUDEP risk. Now, of course, it’s difficult prove that because, practically speaking, you would need to give devices to so many people and then wait to see whether it reduces the risk of SUDEP. What we know about some of these epilepsy devices is that they can alert a caregiver when a seizure occurs. Then, what we need to then extrapolate from that is whether going to a person when they have a seizure, so the person who had the seizure, whether they benefit from the attention of a caregiver in the immediate post seizure period and whether that will reduce SUDEP risk.

I think it’s going to be almost impossible to prove that the devices can reduce SUDEP risk, but I definitely think that the devices have a place in alerting caregivers that a person has had a seizure, and that those can, at times, give peace of mind to caregivers. But we need to be cautious because devices can have both false positive and false negative alarms. Meaning sometimes the devices don’t pick up all the seizures and sometimes the devices pick up all sorts of movements that aren’t seizures and alarming all the time. Also, I’ll remind you that 10 of the 27 pediatric cases that I reported back in 2001, there was someone present with the child when they died. Just because there was someone there, they still are not able to save that life even if we’re alerted to seizures, it doesn’t mean we’re going to be able to save every life, unfortunately.

A doctor examines brain scans while a girl sits on her father's lap in the background.

Transitioning from Pediatric to Adult Epilepsy Care

Transitioning from pediatric to adult care is a major milestone for individuals with epilepsy and their families. Unfortunately, this process can be delayed due to the multitude of health and comorbid conditions which can accompany epilepsy, as well as the personal bond between the family and their pediatric care team.

This webinar discusses how epilepsy patients, their families, and pediatric neurologists can develop a plan to prepare for the transition of care. The presenter, Dr. Joseph Sirven, explores what factors to consider when transitioning care, established research guidelines for transitioning care, and the resources available to assist everyone involved.

This webinar is conducted by Dr. Joseph Sirven, Professor of Neurology and Chair Emeritus in the Department of Neurology and Director of the Epilepsy Program at the Mayo Clinic’s Arizona Campus. Dr. Sirven serves as editor-in-chief at www.epilepsy.com.

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Audience Q&A with Dr. Joseph Sirven

If you have a neurology team that really hasn’t been following this plan, then how do you actually communicate this as a need as the parent?

This is considered a vital aspect of the care of a child with epilepsy or with any neurological condition. And so, it’s important to know that this is what Dr. Sirven and CURE Epilepsy and other teams are putting together. Hey, what a cool concept. This is actually considered a guideline of standard of care. And so, if you’re not having this discussion, I would say you’ve got to bring this up. You’ve got to ask, say, “Can we have a formal transition type of meeting?” Now to a pediatrician, they’re probably going to get that and they’ll understand.

But maybe you’re not seeing a pediatric neurologist. Maybe you’re seeing an adult neurologist already because that’s who’s in the community, or you’re seeing your family care. This is to be brought to them. That’s why I gave you some of those resources. Some of those are actually handouts you can print out, especially at the transition site, that you can actually even bring to your physicians with questions so you can begin answering these.

So as I mentioned earlier, a lot of this has to do with self-advocating and also planning. Like any good parent, ask the questions. If they’re not coming to you with this, know that by the age of 13, this should begin to be brought up and bring this up, because even for busy clinicians, they will stop, they care, they want to help. They just needed to be reminded sometimes, because you’re so busy and focused on the acute issue that you forget that there’s a future big picture that we need to address. Sometimes asking for a time appointment for that is going to be the way that you get there.

Are there already insurance issues or even possible legal ramifications, if people wish to stay with actually their pediatric clinicians if they’re very comfortable with them?

Yeah. There’s no insurance ramification per se. The pediatricians will be covered under the same plans as an adult neurologist. I’ve not seen in my career a situation where I have seen lack of payment or not paying because an adult is with a pediatric neurologist. It doesn’t work that way. It certainly doesn’t work that way in the current laws. Having said that, there are some pragmatic issues, and I’m delighted for the person who has that pediatric neurologist. But the fact of the matter is if there is a huge shortage of pediatric neurologists, and then if you expand that out, there’s an even bigger shortage of pediatric epilepsy neurologist out in the United States.

And so, oftentimes you may see that it’s not that the pediatrician wants to let go of you or anything along those lines. Most pediatricians, they love taking care of their patients. It’s just that, at some point, because the lines are so long and there are so few of us, so few of those physicians out there, that the transition may come up to you. Now maybe you’re in a community that that’s who you’re going to see because that’s all you have, but know that that’s the reality. Insurance sticks its neck in many things, but, fortunately, has not stuck its head in this equation just yet.

What do you suggest if your pediatric team has not yet identified, an adult neurologist and does insurance play a role in choosing those physicians?

First of all, you ask the pediatric team to help choose who are my choices out there. You don’t have to ask them for a specific person, even though you’re going to get one, but can you give me a list of names that you think may be good for my transition? Now insurance, they indirectly play a role here. One of the things that is occurring in modern healthcare now is fairly large networks of doctors. If you think about it, most of us get insurance through our employers, and employers, they get their insurance coverage through a network. So you work for a company, the company has networks.

Well, the network may say, “These are the people in my plan.” So you may be on the kid plan, the pediatric plan, these are the doctors on it. But then for adults, the plan is whatever they spelled out that they’re going to cover as being within network. So I guess, indirectly, how your insurance is structured, how tight do you have to go with the network. If you have a PPO insurance, that means you get to choose fairly broadly. If you’re in a health maintenance organization or a version of that type of organization, you may not have a broad choice as that, and that may impact how much out-of-pocket you have to put out for continued care in the hospital. So the network, the insurance plan may not do it, but the network may. I hope that I picked out that nuance for the listener out there.

To what extent are records transferred over to you providers? Do they have to handle this or does their pediatric neurologist take care of this?

That should be a simple answer, and it’s not going to be. So I’m going to try and talk it through at the end.

Medical records are technically owned by the patient, but housed by the physician, hospital, and all those folks. They can only travel to a new doctor with the permission of the patient. So the patient has to give permission. Patient or parent, whoever is in charge, have to give permission for those records to travel from doctor A to doctor B, from pediatrician to adult. So that has to be instituted from there.

In some cases, it’s really easy. For instance, where I work, I’m on a large electronic medical record known as Epic. One of the benefits of Epic is the fact that anyone who’s on Epic, by clicking a button with the patient, the records will automatically open up from one institution to a completely different institution if they’re both on. Now if you’re not on Epic, then physical records have to be sent, either paper or electronically, to the doctor in some way. But the permission, regardless if it’s electronic or paper, is the same. It has to come from the patient. The patient technically owns those records.

Is a legal power of attorney required for joining her adult child who’s over the age of 18 at her doctor’s appointments?

Well, the fact of the matter is that at the age of consent or the age of majority, which is defined state-by-state. 18 is the typical one for most states in the United States. I can’t think of one that isn’t, so I’ll just say age 18. They’re legally adults unless there’s a power of attorney that says that someone else is there. Now to your listener’s question, if they don’t have a power of attorney and the child or your child, even though as a parent, they’re always your children, but your child can say they don’t want you there and the healthcare group has to honor that.

So to your listener, yes, legally, if they’re 18 and the child has consent regardless and there’s no power of attorney, it’s up to the child to say whether they want them there or not. That’s legally. I mean it doesn’t have to be ugly or anything along those lines, but, yes, that is true from a legal perspective.

Are there different steps involved when transitioning a child who might be diagnosed with both epilepsy and autism spectrum disorder?

I get a lot of kids or young adults that have both. In fact, as an adult epileptologist, autism comes into the exam room, the clinic office very frequently together. What the transition is in that situation is that you spelled out not just what you’re doing for the epilepsy, but what’s the plan for the autism, because we focus so much on the epilepsy that sometimes the autism piece gets almost placed on the side, and yet that may be the bigger issue and the epilepsy’s not such a huge one.

So the short answer to the question is that those have to both be laid out. What do we do when there’s issues with behavior? What do we do when there’s issues with regards to management? That has to be spelled out because oftentimes they get conflated in the exam room, in the office, and they’re not. It’s just laying out what’s my course of action for each of these. That’s important because the management are very different, and understanding where the medications may play a role, what’s been tried, what’s worked, laying those things out can be a godsend in that reaction.

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The information contained herein is provided for general information only and does not offer medical advice or recommendations. Individuals should not rely on this information as a substitute for consultations with qualified health care professionals who are familiar with individual medical conditions and needs. CURE strongly recommends that care and treatment decisions related to epilepsy and any other medical condition be made in consultation with a patient’s physician or other qualified health care professionals who are familiar with the individual’s specific health situation.