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A PANDAS Story

One of the skills I try to teach medical students on their journey to becoming experience clinicians is to consider and address the patient’s “narrative.” Patients have a certain understanding of their illness, its cause, and its role in their life. They make sense of their situation as best as they can, resulting in a story they tell themselves. This is how humans generally deal with the complexities of life.

There is a potential problem when the clinical narrative of the health care provider conflicts significantly with the illness narrative of the patient. Patients, for example, often feel that a highly specific diagnosis is necessary for optimal treatment of their condition. Until they are given such a diagnosis they feel they need to keep looking – for better diagnostic tests or different specialists (what I call the “Dr. House” narrative). The appropriate diagnostic and therapeutic algorithm for that patient, however, may not require a specific diagnosis, but rather eliminating certain diagnoses and then treating the probable category or clinical syndrome that remains. The clinical narrative, in other words, may be one of considering risks vs benefits with incomplete and imperfect knowledge.

Increasingly, it seems, the conflict of narratives is taking on a larger scale – not just between one doctor and one patient, but between the medical community and patient communities. Perhaps this is one manifestation of the new social media generation. Take, for example, a recent controversy over a patient diagnosed with PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections).

What is uncontroversial about this story is that Elizabeth Wray is a 16 year old girl who developed the rapid onset of some form of tic or obsessive compulsive disorder. She was diagnosed with PANDAS and treated, for a time, with antibiotics (for the presumed Strep infection). She was recently transferred to Boston Children’s Hospital (BCH) for treatment, but her doctors there apparently disagreed with her previous management. They petitioned the state to take custody of Elizabeth partly so that they can have her transferred to a locked psychiatric unit. A judge recent decided that the state of Massachusetts would take temporary custody of Elizabeth while the case is being sorted out, but did not allow her transfer to the locked unit.

As far as I can tell, those are the basic facts of the case. However these facts are woven into very different narratives. Part of the PANDAS patient community has taken up the call to free Elizabeth Wray. Most are quoting the family’s attorney, such as this report:

“Their daughter Elizabeth was admitted to BCH due to eating issues. This is the second family I have represented within two months regarding BCH with the same fact pattern. In both cases, BCH has immediately told the parents that PANDAS/PANS does not exist, cut off the child’s antibiotics, reported the parents to Mass child protective services, and had the parents trailed by guards at the hospital. In both cases, BCH has actively encouraged children’s services to remove the child from the custody of the parents unless the family signed a voluntary agreement to place the child in a locked psych unit.”

This is sickening. So very sickening.

Please forward this to everyone you know. Parents who are dealing with PANDAS are going through so much. To have their child taken from them, by ignorant doctors who claim the condition doesn’t even exist?

According to this narrative, PANDAS is a very real syndrome that affects many children and responds to treatment. However, most doctors are dismissive, ignorant, and arrogant and “just don’t get it,” so they deny the existence of PANDAS and as a result refuse needed treatment to desperate patients. There are a few mavericks willing to buck the system for their patients, and they are champions of a populist crusade against entrenched professional ignorance. Sometimes insurance companies also get to wear the black hats, or Big Pharma, or government regulators, or all three.

It’s a great story, complete with villains and heroes, and has at its core the hope of a treatment for a serious and frightening illness. It is perhaps naive to think that this powerful narrative can be addressed by something as cold and dry as scientific evidence. The narrative, in fact, shields itself from such evidence.

The medical community, as you might imagine, has a very different narrative, one that you will read here on Science-Based Medicine quite often (yes, we have our own narratives that guide our thinking about often complex topics). According to the SBM or mainstream narrative, medicine is a complex business. It is especially difficult to identify new diseases and clinical entities. The creation of a new diagnosis must be done very carefully, with thoughtful scientific studies used in an open-minded way to explore all the complexities involved. Only when all the ducks are in a row can we confidently conclude that a new diagnosis is, in fact, a distinct entity, and not just a variant or misidentification of existing known diseases. Meanwhile, even before all the diagnostic questions can be answered, we need research to answer the basic question of how patients respond to specific treatments – what clinical features predict what kind of respond to which treatments? This is, as I alluded to above, not necessarily the same question as one of diagnosis.

Often new ideas in medicine are controversial for years, even decades, before a consensus can be reached about this fundamental claims. And most new ideas in medicine turn out to be wrong, but slowly our medical knowledge grinds forward.

How does PANDAS relate to the above narrative? A recent review found:

The relationship between obsessive-compulsive disorder (OCD) or tics/Tourette’s syndrome in childhood to antecedent group A streptococci (GAS) is unclear. One recent prospective cohort study found that more than 85% of clinical exacerbations in OCD/tic behavior in patients who met criteria for PANDAS had no relationship to GAS infection. Another study found no correlation between clinical exacerbations and changes in a variety of markers of brain autoimmunity, the proposed pathogenesis of PANDAS. A third recent study concluded that, compared with specialty clinic diagnoses, patients diagnosed with tics or Tourette’s by physicians in the community were significantly more likely to be diagnosed with PANDAS without meeting the proposed criteria, most lacked supporting laboratory evidence of GAS infection, and they were more likely to be treated with unjustified short-term to chronic antibiotic and/or immunomodulatory therapy.

Despite continued research in the field, the relationship between GAS and specific neuropsychiatric disorders (PANDAS) remains elusive. It is possible that GAS infection may be but one of the many stressors that can exacerbate tic/Tourette’s or OCD in a subset of such patients.

I have to stress that I am not an expert in this area, but reading as much of the published literature as I can, the above is essentially what I found. The syndrome of sudden onset neuropsychiatric symptoms certainly exists, and is reported in the literature. However, the pathogenesis is unclear – specifically its relationship to GAS. GAS may not be a specific cause, it may just be one of many possible triggers of an exacerbation of a syndrome that has another underlying cause or causes. Further, the more specific question of whether or not patients who meet diagnostic criteria for PANDAS respond to antibiotics has not been settled, but certainly those advocating for such treatment have not met their burden of proof that it is safe and effective. One researcher, Singer, has proposed moving to a new diagnostic label, CANS (childhood acute neuropsychiatric syndrome), which has the advantage of not making any assumptions about pathophysiology that are as yet unproven.

The bottom line is that PANDAS remains uncertain and controversial. It’s not implausible and I (I think like most clinicians) remain open minded about this disorder and are just waiting for better evidence to settle the issue. We’re happy to listen to whatever the evidence has to say.

It’s difficult to express all of this complexity to a community of parents of children with a scary neuropsychiatric disorder who are just looking for treatment. The internet narrative of heroes and villains makes the job that much more difficult.  BCH seems to be caught in the middle of this controversy. They are not allowed to talk about this case, but a statement from BCH has stated that they do treat PANDAS. This suggests, as I suspected, the notion that they categorically deny the existence of PANDAS is a gross oversimplifcation of their position – suitable for a comic book villain, but not the complexities of modern health care.

Conclusion

Confronting common narrative in the public is critically important for proponents of science-based medicine. I do think this gulf can be bridged, mainly because there is vast common ground. Patient, their families, and the medical community do all want the same thing in the end – to do what is best for patients. Further there is still vast agreement that the scientific evidence is ultimately the best guide to what is best for patients. If we can agree to this common ground, then a much more productive discussion can be had about what the current evidence says, what further studies are needed, and what is the optimum management at present given our state of knowledge.

Posted in: Neuroscience/Mental Health

Leave a Comment (61) ↓

61 thoughts on “A PANDAS Story

  1. tgobbi says:

    At the risk of being accused of straying from the thrust of Dr. Novella’s comments I want to point out my concern over the following:

    “Patients, for example, often feel that a highly specific diagnosis is necessary for optimal treatment of their condition. Until they are given such a diagnosis they feel they need to keep looking – for better diagnostic tests or different specialists (what I call the “Dr. House” narrative). The appropriate diagnostic and therapeutic algorithm for that patient, however, may not require a specific diagnosis, but rather eliminating certain diagnoses and then treating the probable category or clinical syndrome that remains. The clinical narrative, in other words, may be one of considering risks vs benefits with incomplete and imperfect knowledge.”

    I’m afraid these words can be misconstrued by purveyors of, and believers in, quackery. Someone please correct me if I’m wrong, but it looks to my layman’s eyes like it might be used as a corroboration of the claim that mainstream doctors are only interested in treating symptoms, not in addressing the cause of the problem and the “whole person.”

  2. cervantes says:

    I’m not getting the part about seizing custody of the patient and putting her in a locked psychiatric ward. That seems completely off the wall here. The BCH docs obviously don’t have to give her antibiotics, but why does someone with Tourette’s or OCD symptoms belong in a locked psych ward? That’s just nuts.

  3. tgobbi – you are correct – this can be willfully, or perhaps naively, misinterpreted. That is kind of my point. There is a lot of complexity to the diagnostic and therapeutic approach to a syndrome that does not fit cleanly into one specific pathophysiological disease. This does not mean that we treat symptomatically only, that therapeutic treatments or preventive treatments are not possible. Further, even when all we do have are symptomatic treatments the value of that should not be minimized. Not knowing the exact cause is also not the same as not being interested, just that we acknowledge we have to operate with less than medical omniscience. The context of what I am talking about is usually after a very thorough workup for specific causes.

    But yes – CAM practitioners have their black and white narrative and they will shoe-horn all of this complexity into that tiny box.

  4. Cervantes – the doctors and BCH have not defended themselves or explained their management of the case because of confidentiality issues and because it is also now a legal case. So – we don’t know what, exactly, they are thinking and what all the relevant medical information is.

    I would recommend avoiding any assumptions or premature conclusions. I specifically avoided speculation in my article. Certainly do not buy into the internet narrative that they are being heavy handed – we just don’t know.

  5. rork says:

    Damn interesting topic. Thanks. http://intramural.nimh.nih.gov/pdn/web.htm was also helpful to me.

    I had written a long note about bayesian specification searches and revision of priors by us mortal non-super-bayesians but decided to spare you except for this summary: Priors move more easily for patients than for docs. Here’s my firmware: nerdy note, else exception.

  6. windriven says:

    I’m with cervantes. There is either an important bit of story going unreported (i.e. is the child a threat to herself or others) or, rather less likely, a clinician with an axe to grind who has gone half a bubble out of plumb. Either way, whomever manages Boston Children’s public relations ought to be considering a new career. What a CF.

  7. cervantes,

    If you read the linked story, it might shed a little light on that. It’s not “just” Tourettes or “simple” OCD, according to the article she is experiencing “bouts with anorexia or trouble eating, as it has in the case of Elizabeth, according to her family.”

    I don’t think that seizing custody in cases of anorexia is extremely unusual (though still not typical), especially if the parents are refusing recommended treatment.

    It seems likely the court believes Elizabeth’s behavior may represent a danger to herself, and the parents may not be taking adequate steps to get her proper treatment.

  8. tgobbim

    “but it looks to my layman’s eyes like it might be used as a corroboration of the claim that mainstream doctors are only interested in treating symptoms, not in addressing the cause of the problem and the “whole person.”"

    It may indeed be be used that way, but there’s several reasonable responses to that.

    Mainstream doctors prefer to treat the underlying condition, of course, but it is not always possible (or sometimes even necessary) to do so, especially if the underlying condition cannot be determined.

    Until that cause can be found, it is often better to treat the symptoms than it is to either do nothing or treat the patient for a condition that has not even been proven to exist with treatments that have not been proven to be either safe or effective.

    Science based medicine is at a disadvantage to CAM because it is not allowed to invent a condition out of whole cloth which it then can claim to treat.

    Homeopathy, for instance, is a modality that is really only concerned in practice with treating the symptoms and not the underlying cause.

    If a person has influenza, treat them with a solution containing (no molecules of) a substance that causes flu like symptoms, regardless of the fact that the way the flu and that substance cause those symptoms is completely different. Also regardless of the fact that that substance probably only causes some of the symptoms that are similar to, but not identical to, some, but not all of the symptoms caused by the flu.

  9. mousethatroared says:

    I can’t help but suspect that the hospital overstepped in this case. As I understand it when serious allegations are made of child endangerment, it’s not usual for the courts to rule for state custody during investigation. This is to error on the side of safety for the child, which is good. BUT the judge ordered that the child NOT be placed in the locked psych ward. If the hospital made a good case to the judge that a locked psych ward was essential for her safety, then why didn’t the judge go with it?

    If the judge didn’t believe the hospital’s argument, then is it any surprise that the parent weren’t convinced with the hospital’s similar advice to them?

    But, maybe this is a temporary investigation and the judge or state guardian will precede with the hospital’s recommendation.

    But I do think that it’s important to remember that the state taking custody of the child is not proof of wrong doing on the parent’s part. It is only evidence that the state saw fit to fulfill it’s obligation to error on the side of caution in CPS complaints.

  10. I have learned not to judge a colleagues actions until I get their side of the story. It is amazing how things can be misinterpreted, and factually misrepresented. Clinical decision making is complex – there are always many possible factors to consider, and until you know what they are all it’s hard to second guess. All we can really do is speculate, which is likely to reflect mostly our own biases (or “narrative”).

  11. mousethatroared says:

    @ SN – Which colleagues actions are we not judging, fellow doctors or fellow parents? ;)

    But, seriously I think I wasn’t clear. I did not mean to say that I thought the doctor’s were more likely to be wrong here. Only that sometimes people make the mistake of thinking that a judge intervening and taking state custody of a minor is good evidence or proof that the parent’s were negligent or abusive. That’s not case. The CPS judge has the obligation to error on the side of caution. The laws are not “innocent, til proven guilty” here, for good reason.

    So maybe the parent’s screwed up or maybe the doctor screwed up or maybe a bit of both. We won’t know for a while. In news reports like this, I like to make a note of red flags that I want to follow up on. Your point about the furor over PANDAS is one of those red flags. But I see the request for the “locked psychiatric ward” to be another curiosity. It’s not surprising to me that parent’s would drag their feet on such a recommendation. Is that the only science based recommendation for a serious eating disorder, or did the parents reject all eating disorder treatment? Was the recommendation actually for a “locked psychiatric ward” as in general psychiatric ward or was there some suggestion of a inpatient eating disorder clinic. It just is something I’d like to know more about to understand this situation.

  12. evilrobotxoxo says:

    My view on this as a psychiatrist: I agree with Dr.N: we simply don’t know the facts of the case, so it’s pointless to speculate. I do know that hospitals would not go to court to try to get custody of a minor unless there was some reason to believe that the parents were endangering the child, so there’s obviously something going on that the family’s lawyer didn’t post on the internet. Big surprise there.

    Second, everyone keeps saying “locked” psych unit, implying some sort of sinister one flew over the cuckoo’s nest-type environment, but pretty much all psych units are locked, even units that are all voluntary patients. Keep in mind that we’re also talking about a minor; I can’t imagine that there is such a thing as an “unlocked” adolescent psych unit, where mentally ill teenagers would be free to run away in the middle of the night unimpeded. So basically the hospital wants to transfer the patient from a pediatrics unit to a psychiatry unit. Again, no big surprise, particularly if there is an eating disorder component. They generally can’t monitor a patient to prevent purging on a regular peds unit.

    Third, as far as PANDAS goes, I’ve never seen a case of it, but then again I’m not a child psychiatrist, and I think there is a tendency of clinicians to dismiss things they haven’t actually seen. I went to a really interesting talk at the NIH a couple of years ago that was given by a child psychiatrist there who specializes in PANDAS and apparently gets lots of cases referred to her from all over. IIRC, she said that Sydenham’s chorea and PANDAS seem to be on a continuum, and that a number of cases have waxing and waning motor and psychiatric manifestations that overlap. She also said that she spent years collecting a bunch of serum samples from PANDAS and Sydenham’s cases, then used those to probe rat brain slices. She said that she found clear evidence of autoantibodies that react with the striatum, and that there was a correlation between the clinical picture and where in the striatum the autoantibodies bound. In other words, patients with more OCD symptoms had more labeling of ventral striatum, while patients with more motor symptoms had labeling in dorsal striatum. Then apparently someone in the lab stored the samples improperly, and everything was lost. I don’t know if it’s true or not; I assume she wasn’t lying, but I don’t think it’s been published either.

  13. bgoudie says:

    In the past year my niece sought treatment for an eating disorder. She was placed in a psychiatric program run by the university she attends. During the first few weeks of the program she was not allowed to leave the clinic, followed by several more weeks where she was allowed time outside but had to return at night. All this was part of helping learn new eating habits and avoiding opportunities to slide back into the behaviors that brought her there.

    I can see how a group looking to create controversy would describe this as “a locked psychiatric ward.”

  14. Robb says:

    Interesting case – with regards to narratives, it seems clear that the parents believed the PANDAS diagnosis to be correct. It isn’t clear who came up with the initial diagnosis though, just that BCH disagreed. If it is true
    that BCH does recognize and treat PANDAS, then it would be interesting to know the basis of their conclusions vs. the original diagnosis.

    Pure speculation here, but I’d say that PANDAS is a more “acceptable” or “preferable” diagnosis to parents as it places the cause directly on bacteria rather than other factors. OCD/Tourette’s/eating disorders in a child can be a scary, complex condition so I can see how it would be appealing on some level to hope for it all to be due to a bacteria that can be treated with a simple medication.

    I can understand patients wanting second opinions and trying to get different perspectives or feeling unsure one opinion is covering all possible bases, especially when it comes to complex conditions and I can only hope that with both diagnoses they were clearly presented with the reasoning behind them. I can see though how their narrative would develop as initial diagnosis + reading information online + more acceptable diagnosis in their minds = strong resistance to any second opinion.

  15. evilrobotxoxo:

    “Then apparently someone in the lab stored the samples improperly, and everything was lost. I don’t know if it’s true or not; I assume she wasn’t lying, but I don’t think it’s been published either.”

    Interesting, but slightly odd. It’s sort of like Columbus returning to Spain and saying he made it to the East Indies, but he forgot the route and neither he nor anybody else tried to go back.

  16. evilrobotxoxo says:

    @Karl: I agree, it’s suspicious, less for dishonesty than for lack of reproducibility. As in “we got this experiment to work once.” Nevertheless, I found it very interesting.

  17. Robb:

    “Pure speculation here, but I’d say that PANDAS is a more “acceptable” or “preferable” diagnosis to parents as it places the cause directly on bacteria rather than other factors. ”

    Without any further speculation on this particular case, I think many people generally find it more preferable to have an external cause of a condition that is “not their fault”, especially with psychiatric conditions. There’s still a lot of stigma with psychiatric conditions in regards to sufferers being perceived as crazy or weak willed or that’s it’s some sort of personal deficiency that makes them responsible for their own condition.

    I think we see this a lot with autism and vaccination. Parents don’t like the idea that the genes they passed on to their children could be responsible for their children’s autism, and they seek out other external factors to blame that are someone else’s fault.

  18. lilady says:

    I absolutely deplore the fact that child’s parents have embarked on a media campaign, supported by a petition online, that puts the Boston Children’s Hospital and the physicians there, in a bad light.

    IMO, the parents have done well to hire a lawyer to represent them…but they do not have the *right* to exposure their child to unfavorable publicity (no matter how the situation is resolved, her identity and her medical issues are in the public domain).

    The parents and their lawyer have told their story, in their own *style*, yet the hospital is constrained from discussing the details of the child’s condition, because of patient confidentiality and because of the pending lawsuit.

    I got myself embroiled on a blog on the Huffington Post. http://www.huffingtonpost.com/lisa-belkin/denying-transplant_b_1207630.html

    Here, the child’s medical condition (Wolf-Hirshhorn Syndrome with failing renal function), was quite different. The parents decided to go the same “publicity” route with multiple TV appearances, a petition online and multiple blogs on the Ho-Po and other websites, when their child was denied a kidney transplant at a Children’s Hospital.

    I commented twice about the child’s overall medical condition and the unfair advantage they had by telling “their side”, while the hospital was constrained from discussing the child, due to patient confidentiality. In turn, a commenter who has her own blog about autism and who has no medical background, posted back at me, because her sole concern was the right of a disabled child to have a kidney transplant.

    That same commenter posted at me, on the Respectful Insolence blog, where we verbally *duked it out*.

  19. mousethatroared says:

    ….and another thing.

    I got so caught up in my concerns over the custody issue that I forgot to comment on how much I enjoyed the first point of this article.

    As a patient who has been experiencing some, as of yet, undiagnosed unpleasant symptoms for the last few months, I wanted to praise the first couple paragraphs of this post.

    “Patients, for example, often feel that a highly specific diagnosis is necessary for optimal treatment of their condition. Until they are given such a diagnosis they feel they need to keep looking – for better diagnostic tests or different specialists (what I call the “Dr. House” narrative). The appropriate diagnostic and therapeutic algorithm for that patient, however, may not require a specific diagnosis, but rather eliminating certain diagnoses and then treating the probable category or clinical syndrome that remains. The clinical narrative, in other words, may be one of considering risks vs benefits with incomplete and imperfect knowledge.”

    Yes I do often wish for a highly specific diagnosis. Not only because then, maybe a highly specific (and effective, as long as we’re wishing) treatment would be in order, but also because it seems like it would be easier to plan my life that way.

    But it’s really helpful to be reminded that the specific diagnoses is not necessarily needed or desirable. Better an uncomfortable uncertainty than a mistaken certainty. Really if my doctors and I keep the above approach in mind, I don’t think I could ask for anything better. I will keep reminding myself that. :)

  20. evilrobotxoxo,

    With nothing published, the cynical side of my skepticism suspects that the most likely possibilities are there was no attempt to reproduce (for some mysterious reason) or the effort to reproduce failed and no publication was made of that effort (for one or more various obvious reasons).

    Failures to produce desired results are not necessarily failed studies. Since such failures (in well designed and performed studies) add to the body of knowledge, the circular file effect of not publishing such studies is depressing and skews the body of published studies.

  21. mousethatroared

    “Better an uncomfortable uncertainty than a mistaken certainty.”

    Well said, indeed.

  22. mousethatroared says:

    @Karl – just so I’m not unintentionally plagiarizing someone, I think that might be at least partly a quote, but for the life of me I can’t find the source.

  23. lilady says:

    @ Karl Withakay:

    “I think we see this a lot with autism and vaccination. Parents don’t like the idea that the genes they passed on to their children could be responsible for their children’s autism, and they seek out other external factors to blame that are someone else’s fault.”

    Just look at this “Google” search “Age of Autism PANDAS”.

    https://www.google.com/search?q=age%20of%20autism%20PANDAS&ie=utf-8&oe=utf-8&aq=t&rls=org.mozilla:en-US:official&client=firefox-a&source=hp&channel=np

    “Google” any other anti-vaccine website to find a number of blogs about autism and PANDAS. :-)

  24. Robb says:

    @mousethatroared,
    Yes, well said – a confident, accurate, and thorough diagnosis is what one would hope for – whether highly specific or not. I would like to think if I were a patient in a similar situation I would not run with a narrative that was quick to jump to a conclusion and that really, what I want it to be, is basically irrelevant and could only get in the way. Easier said than done sometimes though when it’s you in the hotseat. A good friend of mine was experiencing nerve related dysfunctions in one of his hands and was initially diagnosed with gliomas. Luckily he had a very thorough team of docs and it was later found to be tumefactive MS (a rare form of MS that can be easily misdiagnosed as brain tumors). He went from one highly specific to another even more highly specific. I don’t know if he had time to form much of a narrative other than “Shit…fml…”. Kind of funny when you sigh with relief that it’s “only” MS – and currently a very asymptomatic form for him.

  25. evilrobotxoxo says:

    @Karl: what was actually said was that the expt was done once with positive results, and then the serum samples were lost before the experiment could be replicated. I’ve done work with human samples before, and it’s definitely true that the availability of the samples is the rate-limiting step. The story is plausible.

  26. mousethatroared says:

    I read about PANDAS years ago and honestly I thought it was just a standard diagnoses with a proven treatment. But here’s a question. Even if Strep did cause some neurological change that cause OCD symptoms, it does not necessarily follow that antibiotics would reverse those changes, yes? Just as antibiotics would not reverse the damage of rheumatic fever.

    Forgive me if my approach is overly simplistic, just wondering if I am misinterpreting the diagnoses.

  27. evilrobotxoxo

    It is often said elsewehre on the internet: “Photos, or it didn’t happen.” (As in show us photos, or we won’t believe it happened.)

    In regards to any experiment/trial/study, I have to take the position, “Publication, or it didn’t happen.” :)

    Just curious, do you know if this was an actual registered trial or just some kind of semi-formal/informal experiment?

  28. weing says:

    Is it a form of Sydenham chorea?

  29. doughnut says:

    This sounds like a complicated case clinically, ethically and legally. I doubt the possible diagnosis of PANDAS has much bearing in the complexity, as the major issues seem to relate to child protection and duty of care.

    I would be surprised if BCH outright dismissed PANDAS. Empirical evidence exists for PANDAS in the form of syndenham chorea/ rheumatic fever related ‘neuropsychiatric’ symptoms, i.e. anxiety disorders. I don’t think there is any dissent on the existence of this phenotype, it is the broader PANDAS phenotype where controversy exists. Harvey Singer (John Hopkins) is pretty much the world expert in this sort of paediatric movement disorder, and even his textbook and articles don’t dismiss the existence of PANDAS, but question concepts formulation.

    I would also be surprised if they had taken the alleged action they have without fairly significant reason for concern- the potential implications for everyone involved are pretty big.

    It’s unwise to exclude any potential diagnosis in neuropsychiatric presentations, without good evidence- there is a high rate of misdiagnosis in this presentation, and there is often a delay in diagnosis of treatable causes, especially neurometabolic disorders. Even in chorea, GAS-mediation is typically a diagnosis of exclusion unless there is other evidence of rheumatic fever (i.e. valvular involvement etc.). The list of auto-immune causes of neuropsychiatric presentation has also expanded in recent years, particularly the antibody mediated channelopathies.

  30. BillyJoe says:

    Michele,

    “Even if Strep did cause some neurological change that cause OCD symptoms, it does not necessarily follow that antibiotics would reverse those changes, yes?”

    You are not getting any expert help, so I’ll give it a go:

    Antibiotics could possibly prevent further damage. The presumed pathogenesis is autoimmune. If GAS is still present, or re-infection occurs, the antibody level would rise, thereby possibly reactivating or exacerbating the autoimmune reaction leading to further damage. The antibiotics could eliminate GAS or prevent re-infection with GAS and hence prevent further damage.

  31. mousethatroared says:

    BillyJoe – Oh geez, I had no idea PANDAS had an autoimmunity component…I might have avoided the topic had I known, I have my own is it an auto-immune disorder or is it “just” anxiety.

    My heart goes out to the poor girl, either way.

  32. The Dave says:

    Sorry I’m a little late to the game but I wanted to comment on @evilrobotxoxo:

    Let me preface this by making it clear that I am not actually involved in any research so I don’t know the ins-and-outs of it all, but I was wondering. Even though the biologic samples were lost, wouldn’t they still have the raw data of all their observations and their calculations? Wouldn’t they then be able to submit a paper, but in the discussion section explain that the results are preliminary, explain why they weren’t able to replicate the experiment, and invite other researchers to redo the experiment themselves to try to validate the results?

    I’m not saying they are lying or that I don’t believe PANDAS is a real diagnosis (I’m withholding judgement until more evidence is presented), but that just seems like a little bit of a red flag.

  33. If PADNAS is a post-infectious syndrome, than treating the infection with antibiotics will not help. If there is an active infection driving the immune activity then it’s an infectious syndrome and treating the infection will help.

    Strep is not usually a hard infection to diagnose and treat.

    Same situation exists with Lyme vs post-Lyme syndrome.

    There are chronic infections that occasionally flare and need intermittent treatment, such as herpes. As far as I know, they are generally viruses. Bacterial infections cam be chronic, but they can generally be eradicated by adequate antibiotics.

    I am not aware of any (uncontroversial) chronic bacterial infections that need recurrent antibiotics to treat (Mark, any come to mind?).

    Regarding my comment about treating with uncertainty – context is important also. It’s one thing to have demonstrable pathology but just not know the etiology of the pathology (tumor vs MS). It is another situation to have symptoms without demonstrable pathology. Our posture will also be different for stable chronic symptoms vs a progressive syndrome.

  34. The Dave says:

    Correct me if I’m wrong (I probably am :) ) but isn’t necrotizing fascitis caused by MRSA sometimes a chronic bacterial infection that’s hard to treat?

    1. Harriet Hall says:

      @The Dave,
      ” isn’t necrotizing fascitis caused by MRSA sometimes a chronic bacterial infection that’s hard to treat?”

      No, it’s an acute infection that is hard to treat because of the extensive tissue damage and the resistance to most antibiotics.

  35. weing says:

    “Strep is not usually a hard infection to diagnose and treat.”

    Unless it’s asymptomatic. I thought that was the whole point of benzathine penicillin prophylaxis for rheumatic fever.

    1. Harriet Hall says:

      @weing,
      “Unless it’s asymptomatic. I thought that was the whole point of benzathine penicillin prophylaxis for rheumatic fever.”

      Penicillin prophylaxis is intended to prevent strep infections, not to treat existing ones (regardless of whether they are symptomatic or asymptomatic).

  36. lilady says:

    Did you you know that the LUAT (Lyme Urine Antigen Test) performed by Igenex Labs, has more specificity than the two-step ELISA and Western Blot tests, to determine “chronic” Lyme disease or post-Lyme Syndrome.

    It must be the truth because I see the LUAT test being promoted on “chronic Lyme disease” patients support groups on the internet…

    http://www.medhelp.org/posts/Lyme-Disease/Lyme-Urine-Antigen-Test-Anyone-know-about-it/show/1238297

    Months/years long treatment regimens with oral antibiotics or IV ceftriaxone, *seem* to relieve patients’ suffering…much *safer* than injections of bismuth…

    http://cjonline.com/stories/120608/loc_364328990.shtml :-)

  37. weing says:

    “Penicillin prophylaxis is intended to prevent strep infections, not to treat existing ones (regardless of whether they are symptomatic or asymptomatic).”

    Yes. That’s why it is started as soon as you finish treating the acute infection. I thought the recommendations were to continue 5 years or to age 21, whichever is longer. According to Steve’s post, I got the impression, ?mistaken, that prophylaxis is not needed. I would prefer to err on the possibility that it is like Sydenham’s chorea and treat it also with prophylaxis.

  38. Sialis says:

    This Boston parental rights protest is in part being organized by the chronic Lyme disease activists, Lyme disease support group founders, as well as LymeDisease.org’s Founder and President. Rumor is that the patient was diagnosed with chronic Lyme disease in addition to PANDAS by a so-called ‘Lyme-literate’ physician in New York. It’s interesting that these political type relationships were not mentioned in the mainstream news story, as the New York activists have been once again alleging that their doctors are under persecution by the state medical board. Overall, these protests and allegations are intriguing, and certainly make a statement about the social behavior of activists. What justification do these activists have to make such accusations when they are not privy to the intimate details of any specific patient’s medical history and records, nor to any particular medical board complaints and investigations?

    The DAN! and ILADS physician featured earlier this year on the Dr. Phil Show or at least her image is being used by the same activists on various Facebook pages to garner support for the Boston patient, On the show, the DAN! and ILADS doctor agreed to treat a patient with similar symptoms. It will be interesting to see if in another year or two, or even more, if this patient does indeed appear to be ‘cured’. She seemed to be self-treating her pain with Whippets, dozens of ‘hits’ a day!

    This reminds me of another parental rights case that was played out in MI by a chronic Lyme disease activist and attorney who was involved in the recent controversial Romney Lyme disease campaign mailings in Northern Virginia. Orac wrote about that parental rights case on the Respectful Insolence blog.
    http://scienceblogs.com/insolence/2012/03/26/another-case-of-chemotherapy-refusal-for/

    Orac wrote,
    “Yet, unfortunately the Stielers have been lulled into a false sense of security because of the excellent response to surgery and chemotherapy, and now is not the time to be complacent. It’s hard, horribly hard, but Jacob needs to complete his therapy. He might get away without any more therapy. Might. Isn’t it better not to rely on “might”? Isn’t it better to listen to pediatric oncologists who have devoted their lives to treating cancers of this type when they say that stopping therapy now is dangerous, rather than relying on prayer, which, if the WND story is to be believed, appears to be what the Stielers are doing now?

    The answer should be obvious.”

    Sadly, the answer was not so obvious to everyone. History repeated itself, and once again Orac proved right and the parental rights and alt-med activists proved wrong. You can read more about it here. http://www.mlive.com/news/grand-rapids/index.ssf/2012/10/cancer_returns_legal_case_dism.html

    Point being, it seems the misguided and uninformed rantings of the activists are indeed counter-productive to safe, effective, quality medical care. This is not the kind of help patients need.

  39. The Dave says:

    @HH:

    Thanks for the clarification. I’m not sure where I heard that, obviously not from any science-based source. :)

  40. Sialis says:

    @Dr. Novella, you mentioned that “Our posture will also be different for stable chronic symptoms vs a progressive syndrome.” I would very much like to understand more about the meaning of this comment. I have been giving a lot of thought as to what the different treatment regimen’s should be for these types of chronically ill patients with progressive symptoms.

    In discussing patients with PANDAS, which I know little about, or so-called ‘chronic’ Lyme disease, may I ask your opinion? Assume a mainstream (not a quack) physician has a patient that was previously diagnosed with having an infection, possibly Lyme disease, in the past, and such infection was indeed treated with high doses of prolonged antibiotics. If the patient’s symptoms return years later, progressing in severity and degree of disability, despite negative repeat test results, do you feel it acceptable to treat with a trial of oral antibiotics when all other tests fail to provide any indication as to the cause of their newly flared and progressively debilitating symptoms?

  41. Calli Arcale says:

    The Dave — MRSA can be a chronic infection, and flare-ups can result in necrotizing faciitis and other stuff too. Something along those lines was plaguing my grandfather for years; one flare-up cost him a lot of the function in his left shoulder (where he’d had rotator cuff surgery some time ago), and another attacked his heart but was beaten back in time. Several flare-ups in his knee eventually drove doctors to actually remove the artificial knee, stick it in an autoclave, pack the (now joint-less) knee with antibiotics, then put the sterilized knee back in. There hasn’t been another flare-up in the years since that surgery. The doctors think they’ve finally gotten all of the MRSA, but proving a negative is tricky business.

  42. mousethatroared says:

    The other situation I’ve heard about with MRSA is with my brother. He’s had two serious MRSA abscesses where he had to be hospitalized with IV antibiotics. With him the situation appears to be the staph that we typically carry around on our skin and in our nose, etc was antibiotic resistant variety. I don’t think it’s considered a chronic “infection”, though. He is supposed to wash daily with some sort of antiseptic which he says, seems to do the job of preventing new infections. That’s my laymen’s understanding of secondhand information, though.

  43. evilrobotxoxo says:

    @Karl, Dave: I didn’t say I believed the whole story about the autoantibodies, but I found it pretty interesting. A working scientist would be very unlikely to publish data that can’t be replicated. It would tarnish your reputation, so people often sit on a result until they can really prove it.

    I know other people have looked, and they didn’t find an association between PANDAS and autoantibodies, but that association exists for Sydenham’s chorea, and I guess it depends on what population you’re studying. I think this talk I went to was about PANDAS as on a spectrum with Sydenham’s, so it’s not crazy. One thing in general about something like PANDAS is that it if you don’t have a test for it, then you can’t know who has it, so you don’t have the positive and negative controls you need to validate a test.

  44. The Dave says:

    About 8 years ago, I had a minor case of community-acquired MRSA on my cheek. This was back when nobody (non-medical professional) had heard about it and it was still mostly hospital-acquired. Needless to say, I did a lot of “research” on the internet to find out as much about it as I could. Fortunately, all they had to do was lance it, culture it, and perform a susceptibility test to find a sulfa-antibiotic that would work. But I still frequently wonder how I got it because I hadn’t been to a hospital, and the 2 microbiology classes I’ve taken since has shown that I’m not even a carrier for s. aureus, just s. epidermidus.

  45. The Dave says:

    @evilrobotxoxo:

    Thanks for the clarification. I just assumed (in my ignorance) that it would still be valuable to publish as preliminary findings with the disclaimer of the difficulties in replicating. But I can understand how they would want to protect a reputation. And, although I don’t know anything about Sydenham’s, I like the possibility of it being on a continuum, since so much else is (autism, mood disorders, etc.)

  46. Calli Arcale says:

    On reflection, I think a better word for situations like my grandfather’s isn’t so much chronic MRSA infection as chronic MRSA carrier. You can carry a pathogen without having an active infection.

  47. mousethatroared says:

    SN – I think that the uncertainty portion of your comment was directed at least partly to me. Sorry, I’m afraid I only understand it, maybe 50%, at best.

    With myself, I have symptoms, I have some abnormal tests, The doctor seem to say they don’t really match or make a definitive picture at this point, but because of the possibility of an auto-immune disorder it could be new symptoms or test could clarify things later…or not. I’m told and (it make sense to me) that in this case, nothing definitive is good, because it means a much lower chance of serious disease developing.

    I’m not sure which context that fits in. But no worries, if you don’t have time to clarify.

  48. BillyJoe says:

    Steve,

    Just to clarify:

    “If PADNAS is a post-infectious syndrome, than treating the infection with antibiotics will not help.”

    Did you mean: If it is a post-infectious syndrome, then there is no infection to treat and, therefore, antibiotics will not help.

    “If there is an active infection driving the immune activity then it’s an infectious syndrome and treating the infection will help.”

    Isn’t that the same as saying: If there is an autoimmune disease being driven by an active infection, then antibiotics will help (and prophylactic antibiotics could be expected to prevent aggravations of the autoimmune disease by preventing the infections that drive it)

  49. daedalus2u says:

    The evidence for PANDAS being a real disorder is quite strong. Group A strep is known to cause auto-immunity toward the heart (that being what scarlet fever was) and there are many movement disorders caused by acute infection. Sydenham’s chorea (also caused by group A strep) has been known for hundreds of years. Various movement disorders, collectively known as choreas are known

    https://en.wikipedia.org/wiki/Chorea_%28disease%29

    And they can have multiple causes. All of them are characterized by neuroinflammation. When that neuroinflammation is caused by autoimmune cross-reactivity with GAS, it is called Sydenham’s chorea. When it is caused by a buildup of mutant huntingtin with too long a polyglutamine tail on the protein, it is called Huntington’s chorea. People with Rett Syndrome sometimes exhibit something similar called stereotypies. The movement disorders of Parkinson’s are also associated with neuroinflammation.

    I suspect that neuroinflammation around the movement centers is actually a “feature”, to protect those neurons from damage during an infection. Some infectious agents (toxoplasmosis) seem to specifically target the movement centers so as to compel their host to be eaten.

    If movement disorders are caused by neuroinflammation, then anything that exacerbates neuroinflammation will make them worse. Psychosocial stress does exacerbate neuroinflammation, which is why (I think), that stressful situations tend to unmask things like tics.

    Just speculating here, but to invoke children’s protective services and the court and seek custody of the 16 year old, they must suspect something like abuse or Münchausen syndrome by proxy.

    https://en.wikipedia.org/wiki/Munchausen_by_proxy

    This diagnosis is extremely controversial and there have been a number of cases where parents have been accused and even convicted and imprisoned for murder for the deaths of their children, which deaths have then later been shown to due to actual medical issues.

    Still just speculating, but if the patient’s movement disorder gets worse during contact with parents, there may be some parent-child psychosocial stress dynamic that is exacerbating the neuroinflammation and is being interpreted as Münchausen syndrome by proxy. How to do a differential diagnosis under those circumstances is difficult and tricky. Throw in clinician, parent, lawyer and internet onlooker (friends and trolls) egos and it becomes extremely difficult.

    If it is PANDAS, there aren’t any good treatments once the bacteria are dead and cleared. Antibiotics will only kill the bacteria, but if there has already been autoimmune sensitization, antibiotics won’t do much, you have to wait for the normal and eventual attenuation of immune cells producing self-reactive antibodies. Antibiotics might be causing a Jarisch-Herxheimer reaction (from killed gut bacteria), which acutely releases anti-inflammatory cytokines which temporarily “balance” the pro-inflammatory autoimmune antibodies and temporarily relieve symptoms. I think this is the basis for the long term antibiotic use in what is called “chronic Lyme”. If antibiotics give temporary symptom relief, there might be a tendency to continue those antibiotics long term, which would be bad and might delay the eventual resolution by delaying the attenuation of immune cells producing self-reactive antibodies. If the parents have gotten caught up in the various chronic lyme, anti-gluten, Morgellons, DAN! quack-fests, then the hospital really does need to get the patient away from them.

  50. BillyJoe says:

    …so, if GAS causes PANDAS via an autoimmune reaction, wouldn’t re-infection with GAS cause an exacerbation of PANDAS via an aggravation of that autoimmune reaction and, therefore, wouldn’t prophylactic antibiotics help.
    I’m not suggesting this is the case, I’m asking if this is possible.

  51. weing says:

    @BillyJoe,

    That is the case with St. Vitus’s dance and rheumatic carditis. I think the problem may be assuming that antibiotics are all that you need. That is definitely not true.

  52. daedalus2u says:

    It might, but prophylactic antibiotic treatment for PANDAS is not the same as the chronic antibiotic treatment that some clinicians give for things like “chronic Lyme”.

    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3440267/

    We don’t know what kind of antibiotic treatment she was on, but if it was being given in-patient in a hospital, it is likely not anything that would be considered standard for GAS (which requires treatment in any case), or PANDAS. If she had already been given antibiotics for months (which is what it sounds like), then additional antibiotics are very likely not going to help.

  53. BillyJoe says:

    Okay, thanks.

  54. mousethatroared says:

    DU2 “If the parents have gotten caught up in the various chronic lyme, anti-gluten, Morgellons, DAN! quack-fests, then the hospital really does need to get the patient away from them.”

    I’m sorry folks, but as an adoptive mom who reads a bit about custody, CPS, talks to foster parents, some of the way this dispute is expresses concerns me. Hospital have an responsibility to report, they may even have a responsibility to take temporary protective custody. But the child has a right to an impartial guardian appointed by the courts to look at the medical options critically and proceed. The hospital is not an impartial guardian in this case or pretty much any case.

  55. daedalus2u says:

    MTR, when quack medical treatments rise to the level of child abuse, then (in my opinion), every human being has the obligation to the child to intervene. Health care providers are mandated reporters, it is a crime for them to not report suspected child abuse.

    It is my understanding that the hospital did not seek to become the legal guardian of the child, only that the court appoint someone.

    I think the legal standard is not “impartial”, but rather the legal standard is “reasonable”. In the case of medical care, the “reasonable” standard is pretty close to what is the “standard of care”. Imposing treatments that are not “standard of care” is not reasonable, even if done by someone being impartial.

    We don’t have enough information to know what is actually going on. If the parents are trying to give long term, chronic antibiotic treatment, as some advocate for things like “chronic Lyme”, then (IMO), that rises to the level of child abuse. The parents may be well meaning, and want only the best for their daughter, but some quack treatments are harmful both acutely and over the long term.

    I don’t know what treatments the parents are trying to get, but the articles report antibiotics and problems with insurance companies balking at paying for them. That suggests non-standard of care usage.

  56. mousethatroared says:

    DU2 – First you said the hospital need to get that patient away from them (the parents). Now you say the hospital are mandated reporter.

    You are correct in the second statement. The hospital reports. The courts are the ones who decide what is in the best interest for the safety and well being of the child.

  57. mousethatroared says:

    Also DU2 – you’ve brought up Münchausen syndrome by proxy a couple times on this blog. Have you looked into the controversies surrounding this diagnoses?

  58. daedalus2u says:

    MTR, yes, I know that there is controversy about the diagnosis, and it can go both ways, false positives and false negatives. When quacks are involved it can be even more difficult. Everyone can be wanting what is best for the child, but if the parents are in the thrall of a quack, it can be as bad as if they are actively trying to hurt their child. A bad doctor could be just as bad too.

    Apparently it is not an easy case for people with first hand knowledge. Multi-hand accounts that have been filtered through the media are not reliable enough to do anything but speculate.

  59. lktease says:

    I work with children with ASD in a school and counseling setting, and we have parents that swear by PANDAS as a signal to their children’s behaviors. One parent takes her daughter to numerous professionals to work towards understanding her PANDAS. I have seen the increase in problematic behaviors (increased aggression, tics, OCD-like compulsions), but it seems very much like a correlation to me. I believe that the clients I see take their children in for regular strep tests, and are able to “predict” problem behaviors via strep like symptoms that the child reports.

    Yet, I agree with many of the postings that it seems to be used as an explanation for the behavior as having a medical basis, other than just the symptoms of a psychological disorder. While my experience with PANDAS is limited to ASD clients and a few other “odd” children, I still am not sure my thoughts on the syndrome due to the lack of convincing research.

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