NPR Story
4:41 pm
Tue February 25, 2014

Researchers Look For Clues To Polio-Like Illness In California Children

Since late 2012, between 20 and 25 children in California have developed sudden, permanent paralysis that looks similar to polio. Doctors and public health officials are looking for causes and similarities in the cases.

Dr. Keith Van Haren, a pediatric neurologist at the Lucile Packard Children’s Hospital at Stanford University, looked closely at five cases. Two of the samples tested positive for enterovirus 68, a rare virus which is from the same family as the polio virus.

Van Haren joins Here & Now’s Meghna Chakrabarti to discuss his research.

Interview Highlights: Dr. Keith Van Haren

On what links these patients’ cases

“What we describe in the case series are five patients and I’ve since seen a few more at Stanford and we’ve heard of quite a few more in the larger state of California, although the additional cases we haven’t been able to view and confirm in any greater detail – we’ll be doing that shortly. However the interesting thing about the cases is that none of the children had any contact with each other so what we think is that this is a rare manifestation of a rare virus. And again we’re presuming this is a virus because we know of other viruses that can do this.”

On what parents should do

“I actually would encourage parents to do nothing differently. Any parent noticing acute paralysis in their child would, by instinct, consult a doctor promptly and that’s exactly what they should continue to do.”

On what doctors should do

“That’s actually the audience that I think can make the biggest difference here, is that if we get doctors to begin thinking more broadly about diagnoses and to consider the possibility of a polio-like syndrome, I think we’d be make headway. What doctors need to look for is two simple things. One is the acute onset of flaccid weakness or paralysis in one or more limbs, and the second is an MRI lesion affecting the central part of the spinal cord. Those two items together are quite specific and can really help narrow the diagnosis quite quickly. Now, there are many things that can do that as the CDC and others have said, and that’s an important thing to consider.”

Guest

  • Keith Van Haren, pediatric neurologist at Lucile Packard Children’s Hospital.
Copyright 2014 WBUR-FM. To see more, visit http://www.wbur.org.

Transcript

MEGHNA CHAKRABARTI, HOST:

It's HERE AND NOW.

Researchers are looking for clues to what's causing a rare polio-like illness in some children in California. Since late 2012, between 20 to 25 children have developed sudden, permanent paralysis. The illness is being investigated. And while it resembles polio, doctors say it is not the same disease.

Dr. Keith Van Haren is a pediatric neurologist at Lucile Packard Children's Hospital at Stanford University. He's the primary author of an abstract that describes five of these mysterious cases, and he joins us now. Dr. Van Haren, welcome to the program.

KEITH VAN HAREN: Thank you.

CHAKRABARTI: So first of all, doctor, I wonder if you might begin by describing to us a little bit about the case of one of these children, Sofia Jarvis, who right now is a curly headed, beautiful little four-year-old. But she first fell ill at the age of 2 and eventually ended up in your office. Can you tell us a little bit about what happened to her?

HAREN: Yeah. I met Sofia back in the fall of 2012, and she came to our office referred with a diagnosis of possible transverse myelitis. And transverse myelitis, when we normally see it, is considered an autoimmune phenomenon, meaning that the immune system inappropriately attacking the body itself. And it is a reasonably common diagnosis, most common setting we see it is in the spectrum of multiple sclerosis.

The things that were different about Sofia's case is that she had a sudden onset of weakness, which we see in other instances as well. But her muscle strength was - and muscle tone was completely flaccid. So her arm was dangling rather than stiff, and that was unique. Most of the cases we see, when we have an injury to the spinal cord as we do with transverse myelitis, the muscles become very stiff. The reflexes become very strong and brisk. And this - so this struck me as very different.

Looking at her MRI with some colleagues, we noticed that the part of the spinal cord that was affected was not the part that's normally affected in traditional transverse myelitis. The part of the spinal cord that was affected in Sofia was the very center of the spinal cord - actually the parts of the spinal cord that control motor function.

So Sofia had an injury to the anterior horn cells. Those are the same cells that are affected in old-fashioned polio, and those cells can also be affected by other viruses. Other viruses include West Nile virus and other viruses in the enterovirus family. Enterovirus family is a family - a very large family of viruses...

CHAKRABARTI: So, doctor...

HAREN: ...that includes polio virus and those others.

CHAKRABARTI: Forgive me for interrupting, doctor. But if I may, you - because of your findings, you eventually had to tell Sofia's family that she may not get the function back in her arm. And, well, she looks like a happy little girl today. That, indeed, has happened. She - her - parts of her arm - one of her arms at least still remain paralyzed.

HAREN: Yes. That's the tough thing about what we're seeing in these cases, is that, again, in contrast to the more routine cases of myelitis that we see, these cases - in these cases, the children do not appear to recover...

CHAKRABARTI: Well, let me ask...

HAREN: ...much of bodily(ph) function.

CHAKRABARTI: Let me ask you - because you mentioned the enterovirus 68, which I believe your team has determined may be the cause of two of these cases, but overall, there's some 20 to 25 polio-like cases in California. Are there any other things that are linking all of these children?

HAREN: Yeah. That's - so what we described in the case here is - are five patients. And we've - I've since seen a few more at Stanford, and we've heard of quite a few more in the larger state of California. Although the additional cases, we haven't been able to review and confirm in any greater detail, although, we're doing that shortly.

However, the interesting thing about the cases is that none of the children had any contact with each other. So what we think is that this is a rare manifestation of a rare virus. And again, we're presuming this is a virus because we know of other viruses that can do this.

CHAKRABARTI: Now, I'm reading that in Asia at least, there have been similar cases of enterovirus, 68 at least. But they - and while it may be infectious, there was never an epidemic outbreak as far as we know?

HAREN: Well, yeah. I think in Asia, in Australia, there have been quite a large number of cases. The outbreaks are sporadic. They occur and they sort of pop up in clusters and appear for a few months or year at a time and then disappear and move to other parts of, apparently, Southeast Asia and Australia. So the number of cases affected there has been much higher than we think we're seeing here. And the numbers are in the - can be up to the hundreds or much smaller as well. That outbreak was actually caused by a different of enterovirus. In most cases, it's - they're numbered consecutively in the order of their discovery, and that virus was enterovirus 71.

CHAKRABARTI: I see. I see. I'm also seeing here that Dr. Jane Seward of the Centers for Disease Control has said that a number of illnesses could be at work, and until officials get more information, the CDC isn't looking around the country for similar cases of EV68 at least. All the children in - that you've sort of talked about so far, they all were vaccinated for polio. So what do you think parents should do, if anything?

HAREN: Yeah. I actually would encourage parents to do nothing differently. Any parent noticing acute paralysis in their child would, you know, by instinct, consult a doctor promptly, and that's exactly what they should continue to do.

CHAKRABARTI: And finally, doctor, I'm curious. There are, you know, polio has been eradicated because of the vaccine for, you know, more than a generation now. So there may be doctors out there who have never even seen polio-like symptoms in their office. So for doctors who are hearing this, what would you tell them if a child came in with sudden acute paralysis?

HAREN: Well, that's actually the audience that I think can make the biggest difference here is that if we get doctors to begin thinking more broadly about diagnosis and to consider the possibility of a polio-like syndrome, I think we would be making headway.

What doctors need to look for is two simple things. One is the acute onset of flaccid weakness and paralysis in one or more limbs, and the second is an MRI lesion affecting the central part of the spinal cord. Those two items together are quite specific and can really help narrow the diagnosis quite quickly. Now, there are many things that can do that as the CDC and others have said, and that's the important thing to consider.

CHAKRABARTI: And the key is to act quickly if those two things show up in the doctor's office. Well, Dr. Keith Van Haren is at the Lucile Packard's Children's Hospital at Stanford University. Dr. Van Haren, thank you so much.

HAREN: Thank you so much.

CHAKRABARTI: This is HERE AND NOW. Transcript provided by NPR, Copyright NPR.

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