Of all the reasons parents bring sick children in to see me, concern about strep throat is easily in the top ten.
The most typical presentation of this illness is a combination of fever and sore throat, the latter appearing bright red and inflamed on examination. After a short course of an antibiotic like penicillin, the vast majority of kids are back to good health in no time.
For an unlucky few, however, strep might cause something else—the opening act of a much more serious condition that attacks not the throat but the mind: Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections, or PANDAS.
Characterized by the rapid onset of neurological or psychiatric symptoms, PANDAS can significantly alter the mental state of a sufferer seemingly overnight. Children who have been diagnosed with the illness show signs ranging from unusual arm or finger movements, to obsessive-compulsive behaviors, to outright psychosis.
One day they are behaving and developing normally, the next, they’re incontinent, or afraid of a hug, or battling a tic, or losing the ability to write. “We watched the child that we knew disappear in front of our eyes,” the mother of a 12-year-old in England diagnosed with PANDAS (and now in recovery) told local news of his battle last month.
A controversial diagnosis, PANDAS is thought to be related to infections with group A streptococcus, the bacterium more commonly known as “strep.” But much like the condition itself, the exact cause of it remains a mystery—two factors that have led to a question that’s far from settled in the medical world: does PANDAS exist?
My own clinical experience with the diagnosis is limited to one patient I barely recall from residency, not long after a seminal study about the condition had been published. I couldn’t say with any certainty now what his symptoms were, only that this new condition was mentioned over the course of his treatment. I remember the boy himself, a sweet and funny grade-schooler, but not if PANDAS was ever even confirmed.
Despite having no patients diagnosed with the disorder in the years since, I’ve absorbed a lot of the skepticism about the condition from fellow pediatricians. When my editors approached me about writing an article about PANDAS, I responded that there is a lot of controversy about whether strep infections really do trigger the symptoms some attribute to it, but I’d see what I could find.
What I found, at least initially, is that the topic is so fraught some people don’t even want to talk about it. When I reached out for comment to one of the most well-respected pediatric research hospitals in the country, I was told that nobody there felt comfortable offering thoughts about PANDAS at all, either about the diagnosis itself or the controversy surrounding it. Given that one of the members of its faculty is a well-known and forceful advocate for the safety and efficacy of vaccines, the institution’s reticence about discussing PANDAS is unlikely to be due to a fear of controversy, per se.
What sets PANDAS somewhat apart is that, unlike some other controversial diagnoses where a scientific consensus actually comes down on one side or the other, one can find prominent physicians and researchers on both sides of the issue.
Dr. Susan Swedo, the Chief of the Pediatrics and Developmental Neuroscience Branch at the National Institute of Mental Health, is both the author of that first major PANDAS study and a member of the advisory board of the PANDAS Network, an advocacy group devoted to the diagnosis. While credentials aren’t the same thing as outright evidence, it’s hard to dismiss the support of NIMH and a researcher of Dr. Swedo’s stature out of hand. (Dr. Swedo did not respond to my request for comment.)
One physician who does question the diagnosis is Dr. Nicholas Bennett, the Medical Director of Infectious Diseases and Immunology at Connecticut Children’s Medical Center. In his view, the support for the diagnosis is thin, and the evidence showing a causal relationship between infection and symptoms weak.
“I think it’s silly to say that some children aren’t suffering from acute-onset neuropsychiatric symptoms when they clearly are,” Dr. Bennett wrote in an email to me. “But it’s been difficult to prove a causal link with any specific infection, or that it’s an autoimmune process that is tying it all together. The testing that has been done for things like strep infections are notoriously unreliable and don’t take into account that so many people catch these infections and do fine.”
From Dr. Bennett’s perspective, there is a divide between different specialists when it comes to accepting the PANDAS diagnosis.
“I don’t think there are any infectious disease specialists who are completely sold on the idea, it has largely been driven by neurologists and psychiatrists,” his message continued. “In the same way that an ID doc wouldn’t go far postulating a new cause or treatment for schizophrenia, neurologists and psychiatrists haven’t got much traction postulating a new infectious disease.” He went on to note that many people who get treated for PANDAS do so with regimens that are not typically used for strep, after supposedly failing treatment with antibiotics known to be effective against the bacteria.
Dr. Saul Hymes, a pediatric infectious disease specialist at Stony Brook University Hospital, raised similar concerns. He has seen many patients given extensive, poorly supported treatments by providers at the fringes of medicine.
“This is where the dark side of PANDAS really is,” he wrote to me. “I have seen many, many children who have typical OCD or Tourette’s—with a clear onset, not associated with an infection, in the usual way these disorders come on—but are told this is because of strep. And they are placed on chronic antibiotics, or are given antibiotics every time their symptoms worsen even if a strep test is negative.”
Hymes continued: “These children become poster children for bad medicine and pseudoscientific behavior because nobody bothered to question the diagnosis or at least the causal attribution.”
But his skepticism about PANDAS isn’t one that’s necessarily shared by the majority of the infectious disease community, at least not from what I can see. The Infectious Diseases Society of America, which has not shied away from issuing strong statements about other controversial diagnoses despite vehement opposition, has no official statement on its website.
When I spoke with Diana Pohlman, Executive Director of the PANDAS Network, about some of the concerns physicians had raised about the diagnosis, she was quite ready to agree with a number of them. She expressed dismay at the inappropriate use of antibiotics to treat it for longer than is necessary, and suggested that the usual antibiotics for strep work well for many children with the illness.
She attributed much of this overuse of antibiotics to “Saving Sammy,” a book written by the mother of a child diagnosed with PANDAS, which brought more attention to the condition but promulgated a one-sided view about it and advocated for long-term antibiotic use. She also noted that there are unscrupulous providers making hay with indiscriminate PANDAS treatment, many with large social media followings. (She declined to specify names.)
Pohlman became involved in PANDAS advocacy after both of her children were diagnosed with it, as were many other children she knew in her area. Despite many advocates for the diagnosis now investigating a broader category of disease called pediatric acute-onset neuropsychiatric syndrome (PANS), similar to PANDAS but possibly linked to other infectious triggers, she was quick to stipulate that she only felt confident about the role of strep in the illness. (Given how frequently proponents of a vaccine-autism link change the supposed culprit in immunizations every time one ingredient is debunked as a trigger, I must admit I found her unwillingness to move the goalposts with any great speed somewhat refreshing). She also made a point of saying every child she knew with the illness had gotten better with treatment, and did not require indefinite care.
Dr. Elizabeth Latimer, a Bethesda, MD pediatric neurologist who specializes in the treatment of PANDAS, expressed similar reservations about long-term antibiotic use. She first became involved in the treatment of the disease after caring for the sibling of a patient described in Dr. Swedo’s paper, who also had symptoms of the illness.
“I looked at the data. It seemed to make perfect sense to me given what we have known about the link between strep and autoimmunity in the basal ganglia for over a century,” Dr. Latimer wrote to me. PANDAS is often described as a variant form of Syndenham chorea, a rare but well-established complication of strep infections characterized by irregular and involuntary movements of the extremities, trunk and facial muscles, and caused by an autoimmune reaction that affects part of the brain controlling motor movements.
“I treated that child and she was cured almost immediately,” Dr. Latimer continued. “I thought this was probably uncommon or rare, but then saw one of my own long standing patients who came in with the same syndrome. Knowing the signs, it was pretty easy to pick up and he had the highest strep titre I have ever seen.”
The controversy surrounding PANDAS is a source of bemusement to Dr. Kiki Chang, a professor of psychiatry at Stanford School of Medicine and Director of PANS Psychiatric Research. He attributes it in part to the history of how the condition was first studied.
“As people were [initially] investigating the diagnosis, some people published papers saying it didn’t exist,” Dr. Chang told me. “They looked at their own patients who they thought met PANDAS criteria, but didn’t.”
Many of these subjects lacked the defining acute-onset aspect of PANDAS. When they failed to improve with treatment for the condition, this was taken incorrectly as evidence that the diagnosis itself was spurious.
Dr. Chang conceded that using strep titers as a biological marker for PANDAS is specious. (Psychiatric diagnoses in general lack biological markers.) However, for patients who meet the criteria, these tests can guide diagnosis. Dr. Chang maintains that the rise in PANDAS is consistent with other neurodevelopmental phenomena manifesting over the past several decades, including the rise in autism. (I politely elided mention of my own recent writing about alternative explanations for the latter during that part of our conversation.)
As a provider, it’s hard to know what to make of all of this. After reviewing a special issue of the Journal of Child and Adolescent Psychopharmacology focused on PANDAS forwarded to me by Pohlman, I can’t simply dismiss it outright. The connection to Sydenham chorea in particular strikes me as plausible. And it certainly didn’t hurt that among the authors listed on one article was a pediatric infectious disease specialist whose work I know and respect a great deal.
Further, the people I spoke with who champion the PANDAS diagnosis were quite ready to agree with many concerns their ostensible opponents raised. Though it may be mere observer bias, they certainly didn’t strike me as the kind of zealous advocate I’ve encountered surrounding other controversial diagnoses, more prone to shout down opposition than brook any criticism of their fellow travelers.
That said, it’s not a diagnosis I could make with confidence myself. (Perhaps it’s lucky that experience has mooted that point thus far). Were a patient to seek treatment for it, I would pay particular care to the specialist he or she wanted to see, and how they came to hear of him or her. I know where I’d direct them and what questions I’d want answered, but would be unlikely to treat it myself. And as I keep my eyes open for more research on the subject, that will just have to do for now.