Feb 15 2011
I have not been shy about discussing journalistic behavior that I despise – so here’s another one. Take an unusual medical case and report the following about it: 1) Doctors are baffled, 2) this challenges everything we thought we knew, 3) some are calling this remarkable case a miracle, 4) the patient (or their parents) did not listen to the doctor’s negativity, and bravely persevered.
In the reporting of the case make sure you emphasize the unknown as much as possible. Doctors are just besides themselves with how dang impossible the whole thing is. Then find a physician or other expert who is relatively clueless about how to deal with the media and goad them into saying all kinds of irresponsible but very sensational statements. In order to showcase the triumph of the human spirit, exaggerate as much as possible how much better the patient is doing than they should be, according to those nasty skeptical doctors.
Now before someone accuses me of being a curmudgeon, let me say that I get the human interest angle of unusual medical stories. I have no problem with showcasing brave and optimistic patients or parents, or even overly enthusiastic therapists. But I do object to rank mystery-mongering, getting the facts wrong, and not talking to proper experts. I also find it annoying when physicians or scientists who are not media savvy ram their feet down their throats.
The story of Chase Britton is certainly an interesting one, and it’s unfortunate that the reporting on this case has been so dismal. Chase has pontocerebellar hypoplasia. According to reports he has an absent pons and cerebellum, although I could not find any imaging online. It is possible that his cerebellum is atrophied to the point that it is not visible (or easily visible on MRI scan), but there is probably a remnant there. The pons also cannot be missing. That’s like saying someone’s neck is missing. It is just atrophied – perhaps the ventral pons is missing or atrophied.
The picture above is an MRI scan of a patient with pontocerebellar atrophy – not Chase Britton. I include it to help you visualize the anatomy. This is a sagittal view, meaning a vertical slice down the middle from front to back. The cerebellum is a separate structure at the base and back of the brain. In this image it is visible, but very atrophied – just a shriveled walnut. Immediately in front of the cerebellum is the pons – part of the brainstem. In this image the dorsal (back) part of the pons appears atrophied. As further evidence for atrophy there is a big fluid-filled black space where the cerebellum should be.
The story of Chase Britton is actually consistent with published case series – despite the fact that headlines declare doctors “baffled”. For further background there are different types of pontocerebellar hypoplasia. One type is genetic – mutations that result in abnormal brain development. These children tend to die either in infancy or young childhood, usually from impaired respiration. Chase does not appear to have this variety.
The other is acquired – in this type early sonograms reveal the presence of a cerebellum, but the child is born with a severely atrophied cerebellum and pons. At some point the development of these structures is impaired. This category can be further divided, with one subcategory who have suffered from a bleed, stroke, or infection in the brain that damaged that part of the brain impairing its development. Chase also has no evidence of this.
The category that Chase does fit into is pontocerebellar hypoplasia resulting from prematurity. Here is a case series that fits Chase very well. He had evidence of a cerebellum on early sonograms, and he was premature. There are other possible factors as well. Here is a case of a child with pontocerebellar atrophy apparently resulting from vitamin A deficiency.
So what is so unusual about Chase’s case that baffles doctors? I don’t know.
Perhaps it is that Chase is able to function as well as he does. Again – I don’t want to sound like the curmudgeon. I hope that Chase exceeds all expectations, and his parents seem to be highly motivated to give Chase every opportunity to maximize his function. Perhaps this has allowed him to do better than average.
But his degree of neurological function (as evidenced by the video, not by the description of what he can do) seems to be in line with his anatomical deficits. He can move, but he has very poor coordination. He walks with a walker, and does not seem to have the balance to walk without assistance. He is also legally blind.
The cerebellum is primarily involved with motor coordination. It is not part of the primary motor pathways. It is like a parallel pathway that processes motor information and helps coordinate different groups of muscles together in order to produce balance and precise movements. It is also involved in memory for learned motor tasks – like shooting baskets.
So without the cerebellum you can still move. Your brain will do the best it can without the dedicated coordination processing of the cerebellum, but the best it can do will be clumsy movements. You can think about it this way – imagine if walking were not an automatic function for you and you had to think about every move. You might be able to stumble along, but it would never be graceful and you would never be able to walk on a balance beam or do backflips.
Take a look at the video of Chase – that’s basically what we are seeing. There’s nothing baffling or miraculous about this.
I understand optimism, but the interview with the therapist seemed disconnected from reality. She said that Chase can use scissors better than her, but the video shows someone helping him use scissors in an obviously clumsy manor. She says he can ride a bike – without further clarification. This would be remarkable for any 3-year old, and there is simply no way Chase can ride an actual bike. In one shot, however, you can see in the background a four-wheeled cycle (not a “bike”) and that is probably what the therapist was referring to. I’m sure if you strapped him into that cycle, or held him in place, he could manage to work the peddles.
The story of Chase Britton and his parents is interesting from a neurological point of view. It’s a shame the reporting missed out on the real story here and chose instead to go for cheap sensationalism and the tired cliches of “baffling miracles.”
Also, we can be uplifted by the courage and strength of Chase’s parents, who seem to be meeting this challenge with optimism and a very productive “can do” attitude, without exaggerating the truth. I’m sure the real story is more complex than this, but we can acknowledge human strength and virtue without turning a blind eye also to human frailty. We’re not children, despite the journalist’s apparent attitude toward their viewers.
I am tempted to be critical of the doctor in the report, but I have learned from past experience that often time such doctors (or other experts) were horribly misrepresented by the journalist. This is possible here. It is almost certain that he was filmed for a couple of hours, and we are seeing those bits that were plugged into the narrative the journalist had already written. But at the very least, scientists and experts have to learn how to deal with the media. You have to give crisp soundbites that can all stand on their own. You have to resist the urge to take the bait when a reporter invites you to make a sensationalistic statement.
It also seems this doctor, while being an expert in fetal medicine, was not a neurologist. It did not take me long to find a case series that put the case of Chase Britton nicely into context. He could have done this in prep for the interview, or deferred to a neurologist with experience in this syndrome.
This story does not, as we are told, force us to reconsider what we thought we knew about the brain. It is an uncommon, interesting, but wholly unremarkable case that conforms to existing knowledge. It is, however, further evidence for the terrible state of science reporting. This report would serve only to confuse and misinform the public, which the journalist would likely justify (if they bothered to do so) with the fact that they were making people feel good.
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