Dr. Richard J. Konkol is a physician whose specializes in pediatric neurology. He is the chair of pediatric neurology, Kaiser Permanente Northwest Health Plan, and is also an adjunct professor of neurology and professor of pediatrics at Oregon Health Sciences University. He received his Ph.D at the University of Iowa in neuroanatomy and his Medical Degree from Georgetown University.

Yes, I did...

What did that exam consist of?

It consisted of two hours of interview with Kip in a one-way observation room. There were deputies adjacent to that room that I could hear across the one-way mirror. It consisted of an interview, the kinds of questions that I ask about neurologic function, some background activity, some developmental history, a review of systems where one checks out various parts of the nervous system and determine whether there are any signs of trouble or dysfunction, and then I conducted a neurologic examination. The neurologic exam consists of several parts. It consists of a mental status evaluation. This is where one, in a face-to-face, paper/pencil paradigm scans certain kinds of functions like language, constructional abilities, concentration, processing. It consists of a cranial nerve examination, which is a unique part of the nervous system. Each cranial nerve has its own designated function. The motor examination, a strength test, a test of tone and muscle function, coordination testing, sensory testing, and a reflex testing.

Tell me what the results of those examinations were.

Well, I found abnormalities on the examination. They were in several of the subparts of the exam. Just looking at Kip, there was a slight asymmetry of his body habitus. That is how he is structured. The left side was smaller, slightly smaller than the left ... Excuse me, the left is smaller than the right. And I could see this on his face and on his hands.

There is a rule in pediatric neurology that if there is trouble in the brain in what we call the super segmental or the part above the foramen magnum, or the opening where the spinal cord comes up, it can produce atrophic effect or a growth effect on the body, so that the earlier this trouble is manifest in the brain, the greater the degree of asymmetry of growth you will see in the body. So that is just one of the elements that I found is that there seemed to be an asymmetry with the left side being slightly smaller than the right.

Then I found that Kip's language in his mental status part of the exam was not normal. He had difficulty with word production, writing, spelling -- remarkable difficulty. Some naming difficulty, some intermediate verbal memory troubles, and some disfluency in reading, particularly when there were words that he hadn't been familiar with. So that was one category.

And in that, I also had some assessment of his attentional abilities, and I had him do serial sevens, which is starting with a hundred, subtracting seven to get ninety-three, subtracting seven from that and so on down the list until you run out of numbers. It's a standard neurologic test of ability to focus. He had some troubles on that. He lost his place a couple times. So the mental status exam suggested that there were difficulties in higher-level processing.

On the motor part of the examination there were abnormalities. He had a very specific pattern of weakness. The weakness is what we call the parameter pattern or the upper motor neuroparity. By that I mean that when there is weakness in the body, the specifics of what that weakness is very important. If you have a foot drop, it could mean he just had a lesion in one nerve. If you have more than a foot drop and you can't step with your foot, you may have a higher level lesion up that nerve. If you have both legs weak, you may have a spinal cord lesion. If you have a pattern of weakness which fits with the distribution of the input from the brain to the lower centers in the spinal cord, you can identify an upper motor neuropattern of weakness. And he had that, and it was on the left side. So that means the reference is to the right side of the brain. The right side of the brain controls the left side of the body; the left side of the brain controls the right side of the body. And he had a very specific pattern of weakness. He didn't have weakness in all of his muscles. He didn't have weakness in most of his muscles, but he had weakness in a specific pattern of his muscles called the upper motor neuron distribution.

Then on the sensory part of the examination, he also had troubles there. He extinguished when I touched him on the right side proximally and distally. And he didn't recognize when I touched him distally on the right side, but he did recognize that on the left side. And so there was a form of a very mild neglect that I saw, again, referable to the right hemisphere -- this is all on the left side. I don't know if I said that correctly. The left side was the side that that was found on.

Meaning left side body corresponds to...

To the right side of the brain, right. And then his deep tendon reflexes were easier to elicit. These are reflexes that are held in check by the neurons coming down from the brain to the spinal cord, and when the brain is in trouble or when there is a lesion, these reflexes are disinhibited or they become more active. In Kip, this same right-hemisphere related, left-sided asymmetry was seen. So I found subtle neurologic abnormalities in several dimensions.

What you're testing for is brain abnormalities, correct? ...What's a lesion?

A lesion is an abnormality. It can be acquired, it can be developmental. By acquired I would mean something like getting hit in the head, having a contusion, having a bleed, having a stroke. It can be developmental in that you can have malformations that occur in normal development that don't affect the whole brain, but just affect very specific parts of the brain....When there is a lesion, there is usually a dysfunction. And when the dysfunction becomes so troubling that it prevents a person from doing what he should do, what he wants to do, what he would be expected to do in the course of his life and his development and his schooling, that reaches a level of importance that in my practice I pay attention to.

From your motor and mental status examinations, you could draw conclusions about brain abnormalities?

What we're finding now from things like the PET, positron emission tomography, is we can look at the brain and we can see during the ongoing behavior what is actually happening and where. We can compare that with a normal [brain], and we can see what is wrong with somebody, what part is not working in somebody who has a lesion.

Can you show us on the model what abnormalities you noted in Kip Kinkel based on the motor exam and on the mental status exam?

...Most of the findings on the left side would be related to the parietal lobe that would be the atrophic influences...but would be related to the motor output, the development of motor execution, with the weakness in very specific patterns related to the brain. And that would be related to the right frontal hemisphere.

And then problems with language, and as I said before, language in the vast majority of individuals is -- particularly right-handed individuals, which Kip is -- is left-sided in the brain. And so we would have troubles in the left parietal frontal area, in the temporal area, I would predict, from my examination. And also troubles in the right front, based on my examination in the bit of the lobe of the parietal are

Did you also conduct a scan on Mr. Kinkel's brain, SPECT scan?

Yes, I did.

What is the SPECT?

SPECT is an abbreviation for Single Photon Emission Computerized Tomography... I think the best way to explain it is to talk about the procedure itself. This is a radionucleii study where an injection of a compound which is a substance that disintegrates -- and the patient is hooked up to a computer -- the compound suritech, that takes it with the circulation into all parts of the body. And the parts of the brain that are active at the time of the injection for about two minutes are the ones that light up, or take up, the suritech compound. And so that area that was active at the time of injection is bright. That area that was inactive that was not functioning is depressed, or is decreased in activity. So the procedure then, after getting the injection, is to sit underneath a hair dryer-like arrangement, or a set of rotating detectors that pick up the radiation coming from out of the brain and feed it into a computer. The computer has a logarithm that will formulate a three-dimensional picture of where those disintegrations came from in the brain and derive a total picture, a map, so to speak, of where these active areas and inactive areas on the brain are. That map can be then sliced, just like a real brain. If I had this brain, and it was scanned, it should have a continuous pattern of activity throughout the cortex. The activity is linked to the blood flow, and they are in a one-on-one relationship in almost all areas of the brain. So if the brain was normal, one would expect to see a continuous pattern of activity reflecting the anatomy of the brain. And if there was a lesion, it would show it as an area of decreased activity.

And you obtained a scan of Kip Kinkel's brain, ... can you interpret that for us or explain what it is.

...What we have here are five...views of the brain. The center one here is looking straight down on the brain, as if we were holding it like this. The node is up, and the back of the brain is here. So this is the right, this is the left. This view up here is the straight-ahead view, looking like this. Here we have a rotated view, so we're looking at the right side, particularly the temporal lobe here, the parietal lobe and the frontal lobe. Then rotating to this side we have the left side of the brain, the frontal part here, occipital, temporal lobe, cerebellum down here. The top of the brain here. And then the rotation is all the way around, and we're looking at the underside of the brain like this This represents here the frontal lobes, looked at from underneath. And this represents the temporal lobe, the right side -- this is the left side here, and this is the right side here. And here we have the cerebellum, and that represents the arrangement on the illustration.

And on this illustration, there are several major abnormalities that are apparent.

In the orbital frontal area, the prefrontal area here, there are areas of decreased activity. These are not physical holes; these are areas of brain that are not working the same as the rest of the brain. So they are showing up as areas of deficit. They are showing up as holes, with this standardized view setting for the brain study.

When you look at the side of the brain, the right side, you see that this area here, this parietal area, this temporal area is not smooth; it also has areas of decreased activity. And then, it is not that there is a lesion -- a structural part that's missing, a chunk gone; it just means that part is not working, and it's not taking up the dye.

What the SPECT is showing is a decrease in blood flow or an absence of blood flow?

And that correlates to activity in the brain. And the same thing on the left side here. Parietal, frontal, temporal deficits. And when you look at the underside, you can see that the temporal lobe, particularly here on the left, is very ratty, very uneven in its appearance in this scan. That means that what should be a continuous structure and a smooth surface has a lot of areas that are not working, comparable to the rest of the brain. And the same thing here, on the right side of the -- the right temporal lobe. And then we have this huge deficit in the orbital frontal are

And can you correlate your findings on the SPECT with your findings on motor exam and mental status exam and describe to the court what abnormalities you find in Kip Kinkel's brain?

Yes. Well, the frontal lobes up here have been labeled by psychologists and neurologists as the seat of the executive function. The executive function of the brain is, as the name implies, it's where the orders and the structure of behavior come from. It is the agenda-setting region of the brain. You don't see agenda in young children. This is an area of the brain that matures late. Myelinization and development, as I indicated earlier, is a constantly occurring event, and frontal lobes are probably the last areas of the brain to develop in the human. They are also -- they also contain cortex called the prefrontal cortex. The prefrontal cortex is extremely important. It's the largest in phylogeny. That is, if you look at amphibians, reptiles, mammals, chimpanzees, dogs, and cats -- you put a progression on that size and correlate it with a phylogenic placement of these animals, and it gets bigger and bigger the higher up the phylogenic tree you get until it's at its most elaborate and most developed form in the human. It comprises a huge portion of the frontal lobe.

And this is the area of the brain that makes us what we are. That is the basis of our personality. And it has intimate, if not critical, relationship to our abilities to be ourselves and to have our own individual strategies. Without this, frontal lobotomies used to get rid of individual strategies by disconnecting that part of the brain. If that happens, one becomes passive and does not develop strategies, or loses the strategy.

The frontal lobes develop in an adolescent?

Yes. It comes into its own in adolescence. It continues past adolescence, and myelinization continues in this area even into the thirties.

And so, just to be certain we're understanding here, the deficiencies that you've described in the frontal lobe one would expect in an adolescent to be filled in and smooth as you described in the normal brain you showed us; correct?

Correct.

What are the implications of the prefrontal lobe that you describe?

When you have a lesion in a prefrontal lobe, executive function is disrupted, that is, the ability to strategize, to plan, to prioritize, to choose between extrareceptive input -- that is, stuff coming from the outside versus my own motivation coming up from the inside -- is impaired. One cannot, neurophysiologically -- this has been studied -- there are certain neurons that fire just before you have to make a choice, and they seem to be correlated to holding a variety of choices that are available before you actually have to execute and choose something. And when the frontal lobe is damaged, you lose that ability to follow a strategy, to follow a plan, a timeline, a course of individual action and goal direction.

Can you tell us with medical certainty that Kip Kinkel has this deficit, based on your examination?

He has -- there is no way that I can do one test and say that this is a clear-cut, 95 percent abnormality. But taking the totality of the medical method, the history, the review of systems, the family history, the examination, and then the paraclinical testing -- everything seems to point -- when you put the pieces of the puzzle together -- to damage in that are

Let's move down to the temporal lobes. And can you describe what you're seeing there?

Temporal lobes are involved in memory, emotion. They are probably related -- well, on the left side in particular, to language. The right side to relational abilities. Constructional abilities. Your coordination. And when they are damaged, you have troubles with memory. You can have troubles with language. You can have troubles with emotional control.

And so you describe damaged areas in the temporal lobes...And that relates to inhibitions or the failure to properly control emotions and other things?

Yes. Emotional instability, distortions of sensory perceptions, illusions, delusions, visceral sensations -- things that you feel from your gut. And it's often an area of instability in the brain in people with neurological problems like seizures or epilepsy.

And what can you tell us about occipital and parietal?

Well, the occipital lobes here are affected. And this is, again, the sensory processing for vision, primarily. And as I indicated before, that as you go anterior, these perceptions are transfigured and are recombined into more complex experiential units.

The parietal lobe is particularly unique in that it has the primary input from our senses, our sense of touch. We have what we call parietal lobe tests such as touching, drawing figures on hands, putting an object in the hand to see whether somebody recognizes it.

What I did with Kip was draw numbers on his hand while his eyes were closed and see if he could recognize what I was doing, and he had troubles there. And that was again on the left side of his body, referable to the right parietal lobe.

Any other conclusions you draw based on your exam and the SPECT?

Well, what I found was a coincidence of or a congruency of information that pointed and supported the clinical method and clinical conclusions that I had.

I do not rely on the SPECT scan as a sole determinant of a diagnosis; that is in my head after I do the examination. And I do a test, like an EEG, bloodwork, MRI, SPECT scans, to make sure I wasn't wrong.

Summarize your conclusions about Kip Kinkel.

I think that with these multiple areas of problems in his brain, both the left and right side are affected. The frontal part more than the posterior part of the brain is affected. The right frontal part has been shown to be particularly unique in inhibition and inhibiting planned activity, inhibiting ongoing activity, delaying responses. That was the most markedly decreased area of function that I found clinically, and that was also supported by the SPECT scan. He also had marked specific language difficulties that refers to the left temporal parietal, frontal loops that are there for those functions, and was also confirmed here on the SPECT scan.

And then, if I had to conclude as to whether this was an acquired or a developmental lesion, I would probably come down in favor of saying that this was a developmental lesion because of asymmetry on the left side. The right side of his brain had more of the decreased function, and the left side of the body showed more of a hemiatrophy, relative hemiatrophy. And you don't see that in late-acquired lesions; you see that in early developmental events.

So what's the cause of the abnormality... is it something that is secondary to trauma or it's something that is genetic that he inherited or...?

The cause of the abnormality is underlying dysfunction of specific circuits in the brain....I cannot say with absolute certainty that it's one or the other, but if I had to come one way or another, I would say it was probably developmental.

Does the abnormality rise to the level of a mental defect?

Yes... Cognitive defect. A defect in thinking, yes.

Neurologic function is impaired?

Correct.

Would it make him more susceptible to a psychotic episode?

I think it could. As a matter of fact, one of the other sessions that I attended that I found very interesting was the presentation by Dr. Judith Rapoport from the National Institutes of Mental Health. She showed, in the ongoing study that they are conducting at the National Institutes of Mental Health, their imaging results. And what they showed was that there was in childhood onset schizophrenia a presence of neurologic findings that preceded the onset of the symptoms, that there was an effect of a delay in the growth curve of the brain -- just as there has been shown in many, many studies in the adults that schizophrenics have smaller brain sizes -- the effect is greatest in the frontal and in the temporal are These are the areas where the gray matter is most affected in Kip's SPECT scan, and this is seen by the decreased perfusion, decreased activity in the frontal temporal region.

So a number of the criteria fit your conception based on your workup of Kip Kinkel; is that correct?

Correct. There were others as well, if I can recall. One was that there was an unusual, three-fold increase in incidence of other familial neuropsychiatric diseases. I think that is suggestibly present here. And the IQ had to be above 70, and certainly that would fit here too.

And the other history that you reviewed, in terms of the psychological testing Dr. Bolstad did later, on Dr. Bolstad's report, how does that overlay with your neurologic exam?

A: I think it was consistent with it, but it was -- it was different from my approach. And it was another piece of the puzzle, but not related directly to mine. But it would have fit.

And what's the prognosis for someone with the deficits he has?

Based on my experience, with children who I've had similar to Kip -- not exactly the same, because I don't think anybody is exactly the same -- I would be hopeful. Mainly because the effects of proper management, that is, setting up a proper environment, where there is a recognition of a deficit, where there is a bypass strategy around the deficit, where there is development of positive reinforcing habits and behavior to sort of train the mind -- this is everything a parent would do with a normal child, but you have to do this more laboriously and with smaller steps with a child with a lesion. There is great hope that medication could help. In my experience, at least 75 percent, and depending on how hard you push and how meticulous you are, you can maybe get that up to 80, 90 percent in some groups, children, to get a positive response from medication. And then I think counseling, to deal with the broader issues that surround a neurologic dysfunction.