Epilepsy Discussion
Chris Inglese & Susan Heighway
Unit 1: Questions for Child Neurologist
(Slide 1) – Epilepsy in the Intellectually and Developmentally Disabled
(Slide 2) – Video of Inglese and Heighway
Chris Inglese: Susan, I understand that you take many, many phone calls from communities all over the state. You take phone calls from teachers, parents, social workers, physical therapists, occupational therapists, psychologists; everybody. Many questions are asked of you that require the expertise or involvement of a child neurologist. What are the types of questions that you’re having a hard time getting answered from us? How can we be more useful to you, to individuals like you, that assist and coordinate care at the WaismanCenter?
Susan Heighway: I think one of the main questions that we get is - Families and other care providers you mentioned wander off and specifically related to seizure activity: When and what data they should collect, or maybe what their indications are, or what children might be appropriate to refer to you?
(Slide 3) Questions for Child Neurologist
Chris Inglese: Yes. I think the first question is: Is this child having a seizure? Is it a seizure or not? Is it behavioral staring? Is it something else? Is it the inattentiveness of a very overwhelmed child with a learning problem or a very anxious child?
So, the first question is to define whether or not the child has a seizure. The short answer definitely is that - and it may sound self-serving, but all these children should be referred to somebody who has a good sense of what a seizure is and what it represents. I don’t think that any parent or any teacher should be poo-pooed or ignored with regard to that question in spite of how busy and important we might think that we are. I think it’s important to answer the question negatively rather than not direct attention to it at all.
That is a tough question. It’s also tough when seizure disorders have already diagnosed because there are so many different variations on the theme of the seizure. It becomes: Is the child exhibiting a new kind of seizure? Is the child exhibiting a behavior as a result of a side effect from the medication? Is the medication suppressing seizures but is it having a negative effect upon attention, memory, learning and social interactions? So, the initial question, whatever it is, should be asked and it can lead toward the answering of, hopefully, the solution of a lot of different problems; if that answers your question somewhat.
(Slide 4) Q: Are there developmental/genetic diagnoses…
Susan Heighway: It does. Would you say that there are certain diagnoses, certain developmental diagnoses or genetic diagnoses that would be associated with seizures or that would automatically signify to care providers that a neurological evaluation would be important?
Chris Inglese: Yes, definitely. Specifically, for example tuberous sclerosis, neurofibromatosis, trisomy 21, Down syndrome, fragile X. If you think about those four syndromes or conditions alone, they’re highly associated with seizures and epilepsy. Tuberous sclerosis is almost universally associated with some measure of epilepsy, almost 90%. Fragile-X, similarly, increases the incidence of seizures. Any child who has had prenatal or perinatal damage, any child who has neuromotor motor problems who has CP plus cognitive impairments is hugely at risk for having seizures. It could approach 50%, according to some studies.
(Slide 5) Q: Are children with autism spectrum disorder…
Susan Heighway: How about children with autism spectrum disorders? Are those children also at risk for seizures?
Chris Inglese: Tremendously since autism, at least in my view, is a symptom of a variety of brain insults. If autism is an expression of the brain changes that occur in tuberous sclerosis, then again, 90% of the time, there’s likely to be seizures, but even in the so-called idiopathic or primary autisms. By that, I mean children who are developing in an otherwise normal fashion. Even in children who don’t have a genetic or metabolic label, the incidence of seizures is relatively high. The incidence of behaviors that mimic seizures is high as well; some of the things that I mentioned in the discussion in the previous lecture.
Susan Heighway: So, that would make it more difficult for the teachers or the parents to sort out what behaviors might be or might not be seizures. So, that would be helpful for them to have a consultation with a neurologist to help with that.
Chris Inglese: Yes. There could be so much overlap between seizures and behaviors. I think an over-valued or over-questioned expression of epilepsy is aggression. I think it originates from, perhaps, the medicolegal community that somebody commit a violent act because of their epilepsy or because of misperceptions that occur after a seizure. So, what occurs then is that in the classroom there is this notion that people with epilepsy act out in an aggressive way and characteristically. Is that an expression of their seizure disorder or is that a seizure itself? Kids with developmental disabilities and seizures on medications act out as we all do for a variety of reasons.
Unit 2: Seizure Back Ground Information Helpful for Evaluation
(Slide 6) Q: What kinds of information related to seizures…
Susan Heighway: In this whole area of thinking about families and professionals referring a child to you as a neurologist, what kinds of information is helpful for you for them to collect or for them to be prepared to share with you about their concern related to seizures?
Chris Inglese: I think that if the question is whether the child is having seizures is to be answered. I think that the caretakers or parents or teachers need to be literally the eyes and ears of the clinician making the diagnosis. It’s very rare. It’s very unusual for a neurologist to see a seizure. Many of us don’t see them even when we’re in our training. We usually see the aftermath of a seizure, the post ictal state. So, we insist upon, or we should - that there’s very, very careful precise documentation of what the witness saw.
Often times, there is not a witness at the beginning of the event. A parent will come into a room and find the child is in the after-seizure state, the post ictal state. There could be froth on their lips or there can be blood tinged saliva on the pillow and they could be very limp or in a certain posture, but if family members or caretakers are lucky enough to see the event evolve from its beginning; the most useful information for clinicians such as Roger Seth, myself and others, Carl Stafstrom, I say, is a blow by blow description of the event with as much precision as possible.
I don’t know it it’s a good analogy or not but it’s like the way radio announcers for football games used to have to precisely describe everything happening on the field before we had the multimedia, the video part of it all. So, you can envision what’s happening on the field while you’re raking the leaves or something or washing the car.
That’s key. It’s more key than an EEG. It’s more key than an MRI, a CAT scan; that description to do a good comprehensive evaluation in addition to having as much information about the ictus, the event, as possible. If we’re going to take care of the whole child, we want to know what’s happening educationally, behaviorally, socially. Information relative to that is very important in providing a comprehensive opinion about what’s going on.
Unit 3: Referral to Child Neurologist
(Slide 7) When should neurological opinion be sought…
Susan Heighway: One of the very frequent questions that we get are related to absence seizures. Many times, again, parents, teachers or caregivers noticing that the child is spacing off or whatever. Those are some – what I find as some of the trickier behaviors to advice: When should neurological opinion be sought? Do you have any suggestions on looking at those?
Chris Inglese: Yes. I’m simplifying it a little bit. There are basically 2 kinds of staring seizures. There’s the absence seizure, previously known as the petit mal seizure; and there’s the complex partial seizure, otherwise known as the complex partial seizure.
The absence seizure is literally of abrupt onset and offset. I mean there’s a crisp beginning and a crisp ending. If a teacher observes that, if a parent observes that, that’s very important because staring is universal. If it’s just a drifting into a stare and a drifting out of a stare, it could be somebody contemplating or reflecting upon something.
Again, I think that staring is pretty universal, but it it’s abrupt onset, abrupt offset, particularly if there’s the arrest of an ongoing activity like walking or eating or dropping a pencil and having the head nod and scribbling something unusual during test taking, there will be a high degree of suspicion on the part of the teacher to describe it that way and do the appropriate tests.
The complex partial seizure can be a little bit more tricky but some of the clues that help us understand that it’s a seizure or not is the duration. Absence seizure of abrupt onset and offset generally lasts around 10 seconds, 12 seconds, 15 seconds; seldom more than 20 seconds. The complex partial seizure will be closer to a minute to 90 seconds in duration. The child will drift into it gradually and come out of it subtly, and toward the end there will be more than just staring. The child will fumble with the hands. She may smack her lips or drool a bit or have forced involuntary swallowing. Those are what we call automatisms or funny postures with their hands.
A useful tool for parents, teachers and others is to not just wave the hand a foot or two in front of the child’s eyes. Some people that respond to that are deep in thought, but to gently grab their shoulders and give a gentle shake. I think that most people that are staring behaviorally, staring because of anxiety or depression or worries or whatever reason, they’ll come out of it. Somebody who’s in a complex partial seizure will not. They will continue with their automatisms, their lip licking and lip smacking and swallowing and staring. They may even become resistive of that, combative toward it.
Hence, where most of the aggression that occurs, to digress a little bit, with seizures has to do with restraint and misperception on the part of the person that they’re being hurt. But, essentially, the child should not just have a hand waved in front of her eyes but be spoken to, gently shaken to, and maybe have the hand waved in front of the eyes at the same time.
Be more proactive and active about bringing the child out of it. If the child comes out of it and shakes her head and says, “I was just thinking about the next question or the previous questions I couldn’t answer,” then you know what’s going on. It wasn’t likely to be a seizure.
The only thing a person will remember during an absence seizure usually is that they’ve lost time. If there’s recollection of what they were thinking about and they can describe some of it, it’s probably not a seizure.
Susan Heighway: That’s very helpful because that’s really one of the biggest questions that, I think, comes up for families especially people that have learning differences, and they’re really having trouble learning, and they wonder about those different kinds of behaviors. The other kinds of things that come up are: We see a fair number of children that come to our clinic who’ve been diagnosed with seizures or have previously been on medication or maybe they’re currently on medications. I’m wondering what your thoughts are for the frequency of long-term followup or long-term following by a neurologist related to seizure management and treatment.
Chris Inglese: Are you referring to children specifically that were on medications and their epilepsy is considered being in remission or in more general terms?
Susan Heighway: Maybe we should –
Chris Inglese: Because I think the –
Susan Heighway: Divide those out a little bit because it might muddy that a little bit. I think one of the groups of children that come in are children that are on medication, maybe their primary care physician is the person that’s prescribing the medication, there has been a neurologist involved. One of the questions that we have is: How often and for what purposes would it be useful for that child to be seen by a neurologist?
(Slide 8) How often and for what purposes…
Chris Inglese: I think that one of the problems with the lack of enough child neurologists to go around, or pediatricians that are comfortable with epilepsy to go around is that there are many children that fall between the cracks. By that, I mean that they’re properly assessed, their epilepsy is characterized and they’re put on the right medication, but then they stay on the medication often times well beyond the point where it is needed.
It’s impossible to know in most cases when a child’s epilepsy will remit. So, I think one of the good questions that you’re bringing up is in these children that are being followed by a neurologist or a primary care physician: When should we should we ask the question, “Do they still need to be on the medication? Have they outgrown it?” In particular, if they’re struggling with other aspects of their life because it’s not uncommon for the medications we use to negatively impact learning and mood. It’s not uncommon for the medications we use to cause problems beneath the surface metabolically with bone health, for example.
It’s good to get the kids off the medication as soon as you think that it’s safe; if that answers part of the question. I think if a child is still at risk for having seizures, their epilepsy is not considered to be in remission or it’s unlikely that it’s ever going to remit. It’s still good for somebody comfortable with epilepsy and the new medications to be involved because one of the most important advances in the past 10 years in the world of epilepsy management is the newer drugs which are more user friendly both for physician and for the person taking them and the caretaker giving them.
So, we may have a child who still needs to be on medication. Maybe we can transition them to a compound that can help them with another aspect of their life that’s problematic, or maybe we can transition them to one of the newer medications that will not cause new problems in the future such as obesity, osteopenia or osteoporosis having a negative impact upon attention or mood.
Unit 4: Professional Team Participation In Diagnosis and Treatment
(Slide 9) What kinds of things are important for the other team members…
Susan Heighway: One of the other things that I recognize is that children with disabilities and seizure disorders have complex needs, and often there’s a team of people, and we’ve been to alluding to that, talking about families and the professionals. One of the things is a neurologist working with a team. What kinds of things do you see as being important for the other team members related to seizure followup - what kinds of things are helpful to you on an ongoing basis in terms of, maybe, looking at the seizures or reporting back or whatever? I think that would be helpful to hear from your perspective.
Chris Inglese: Yes. I think that important feedback is valuable from teachers relative to whether there has been a plateauing of a rate of learning, or, in particular, regression. That kind of directs attention back toward one of your earlier questions regarding the comprehensive evaluation of somebody with epilepsy. To digress just a little bit, if we get that kind of feedback from the teacher that not only is the child not learning at the rate that she did before but she’s going backwards. That necessitates that we dig a little bit deeper in terms of understanding the cause of their epilepsy. That’s when we involve the John Wolf’s of the world.
Are we missing something metabolic that would be important to know because of a potential treatment or because of the potential for impacting other children or other generations in that family? So, plateauing of rate of learning and regression of learning are important feedbacks from teachers.
Important feedback from teachers also has to do with: Is the child exhibiting, in addition to the epilepsy and independent of the treatment, some of the signs of ADHD? Many people feel that it’s over-diagnosed. Many of us feel that it’s very under-diagnosed. I think it’s really sad when the developmentally disabled with cognitive impairments have their attentional issues ignored because, “It’s all due to their MR.”
A person with borderline intellectual impairments can have comorbid ADHD and can be helped immeasurably by medications that augment attention. So, those are important feedbacks from teachers.
If we’re lucky enough to have an occupational therapist involved, a physical therapist involved, if they can direct their attention toward other challenges for the child that can be remediated by an orthopedist or by an orthotist or somebody who can come up with a way for a child to better handle a pencil or pen, that’s very, very useful.