Strep infection may prompt a severe reaction in some children, causing their own immune system to attack cells in the brain. What appear to be extreme psychological symptoms result from what is really a physical disorder. The disorder, known as PANDAS, is often misdiagnosed.
- J.C. Konecny, Executive Director, PANDAS Network and parent of PANDAS sufferer
- Dr. Elizabeth Latimer, Clinical Assistant Professor of Pediatrics and Neurology, Georgetown University
Links for more information:
Reed Pence: A couple of years ago, J.C. Konecny’s daughter, Greta, was a normal five year old looking forward to starting kindergarten. But it changed in an instant after a case of strep throat.
Konecny: We had her treated and we thought that she was better. But we noticed over night that she became extremely obsessive about her throat, specifically. And her belief was that something was stuck in her throat. She literally believed there was a bone or a piece of tissue that was lodged in her airway. She was very insistent about this and would mention it thousands of times a day. So of course we became very concerned. We went down the medical path, had her seen by an ENT; and the ENT cleared her and said her throat looks great. She doesn’t have strep; she looks good. I would recommend that this might be a psychological problem.
Pence: The Konecnys took Greta to a therapist, who thought she had obsessive-compulsive disorder. But treatment didn’t help. She was put on four different SSRI drugs… but the symptoms only got worse.
Konecny: During this time she began having these attacks where she would be completely normal, we’d be driving down the road and all of a sudden she would say I’m dying and start screaming and have what adults would call a panic attack — a very very upsetting, emergent type situation where she really believed that her heart was going to jump out of her chest, that she was dying. She had deteriorated to the point of, what we can’t call psychosis because psychosis is a complete break from reality, but in our daughter’s case, she was completely out of control of her body. She would scream and cry and run around the house pulling things off walls. She was completely out of control. There would be days when she wouldn’t be able to swallow her own saliva because she couldn’t coordinate her swallowing reflex. She began to lose the ability to walk, so she would crawl everywhere. And again I have to emphasize this was a completely normal child prior to this illness.
Pence: About a year and a half later, Konecny found a doctor who finally connected Greta’s strep throat to the onset of her symptoms. He believed she had not a psychological disorder… but damage to a part of the brain called the basal ganglia. It’s a disorder whose full name is “pediatric autoimmune neuropsychiatric disorder associated with streptococcal infection.” People call it PANDAS for short.
Konecny: In PANDAS when a child contracts strep there is the misdirected immune response. Basically the body becomes autoimmune. So the immunities that the child produces to strep attack the child’s own brain and affect specifically the basal ganglia of the brain. The basal ganglia controls movement that’s where we see these tick disorders, problems swallowing, issues with urination, and then also the basal ganglia controls mood. Obsessive- compulsive disorders, anxiety are thought to originate in the basal ganglia, and that is the part of the brain that seems to be most impacted by PANDA.
Dr. Elizabeth Latimer: The only reason we really treat strep is to prevent the immune reaction to it. Strep normally goes away in three days whether you treat it or not, but there are serious consequences that have been well known for a long time, that strep untreated has consequences to the brain, the kidneys, the skin and joints in general. And to the heart obviously, which is rheumatic fever.
Pence: That’s Dr. Elizabeth Latimer, clinical assistant professor of pediatrics and neurology at Georgetown University. She says the hypothalamus and amygdala may also be attacked by the body’s immune system, creating a whole host of possible symptoms.
Latimer: A lot of the kids have difficulty with noise, bright lights. That can occur at school as well and their issue at the same time is that they [have] severe separation anxiety, so a lot of these kids can’t go to school at all. And then it’s a minor issue that can come up like worrying or collecting things. But a lot of these children become cripples within their own home. Severe impulsivity bring their children more across the main highway, aggression, rage episodes, which the kids don’t recall by the way, people just go completely black, they have deterioration of their handwriting, they have inability to fall asleep, initiate sleep at night and difficulty maintaining sleep at night.
Pence: Many children with the disorder suffer from facial tics, or may yell out when they don’t intend to. Konecny, who is now executive director of the PANDAS network, says kids may also suffer from severe obsessive-compulsive disorder.
Konecny: And that in children can work many different ways; it can look like the need to repeat things or ask something over and over and over again; the classic germ phobia or hand washing, a belief that food is contaminated is a very common OCD symptom we’ll see in children with PANDAS. And that usually leads to restricted eating, so, “Mommy, I cannot eat any food that is red, it is poison.” And a true belief in the child that that is true.
Pence: Konecny says parents should take note of symptoms like these, particularly if they start suddenly. Latimer says a child can have a Jekyll and Hyde transformation from completely normal to out of control literally overnight. However, that’s sometimes hard for doctors to believe.
Latimer: Patients are attacked by their physicians, they are told that your child couldn’t possibly have been normal before this. Even though the parents bring video with them. And then they get them into psychiatric hospitals and one of the things that they put them on medications and sometimes the medication makes the kids worse. And then they get bipolar disease or some other psychiatric illness. And then they spiral into a psychiatric world and it’s tough to get the out of that.
Pence: Officially, PANDAS afflicts an estimated one in 200 children, but Latimer says the real number is likely higher because so many are misdiagnosed. And the longer PANDAS goes on, the worse the symptoms get. Strep is often never diagnosed… and it may take as long as six weeks after a strep infection before PANDAS symptoms set in. So doctors and parents may never connect the two. But once they do, doctors often start treatment with antibiotics.
Konecny: That is the first line of therapy. And really our hope is that if kids are caught early, if we increase awareness and children will be caught in the beginning of their illness, and then they can be treated usually with a course of antibiotics, typically a little longer than you see for strep. And we see kids treated for about three weeks with low cost generic antibiotics typically run $10-$20.
Latimer: You treat the infection so you get rid of the trigger of the immune reaction. Whatever antigen or protein is triggering your body to response immunologically you get rid of the trigger. You just have to treat the strep. Parents, if that does not resolve the problem, then you have to deal with the immune reaction, so if you use the analogy of asthma, if a child comes in with an asthma attack, you give him antibiotics if it’s a bacterial infection. If that doesn’t work then you give them steroids.
Konecny: A course of steroids is usually introduced as well to reduce any swelling that we might see in the brain. This is really encephalitis, if you will. It’s the brain being attacked by antibodies and then the brain swells, so there’s an encephalitic process going on and that’s why steroids are needed.
Pence: Doctors may also suggest a treatment that might surprise you — taking out the tonsils. A tonsillectomy was almost a rite of passage decades ago for kids with recurrent sore throats. Doctors were concerned about essentially the same thing as PANDAS — strep throat prompting an immune reaction. But back then it was a fear that antibodies would attack the heart.
Latimer: We do a lot of tonsillectomies at Georgetown. Again it’s removing the trigger. So, in the 1950s, in 1959 was the peak year of tonsillectomies in the United States. People were really afraid of strep in 1959, because people had rheumatic fever. If someone had strep too many times they would take their tonsils out. I had many parents actually just make the decision to take the kid’s tonsils out and pay for it on their own, because many insurance companies wouldn’t pay for it.
Konecny: I think if your child has a strep mediated illness it’s a really good idea to remove the organ in the body, the tonsils, that harbor strep very often. And in many kids we think of it as cleaning out the house and dusting everything before you put the furniture back in. So before your deliver treatment to a child, you take out this tissue that in many cases, and in my daughter’s case, is full of bacteria.
Pence: However, children with more severe cases of PANDAS, who’ve been sick longer, may need more. That’s when doctors may try treatment with IVIG, intravenous immunoglobulin therapy.
Konecny: In this therapy, antibodies taken from healthy donors, so you go and donate blood, thankfully millions of Americans do that, the healthy antibodies are removed from that blood product and they are cooled into warm baths and then infused into the sick patient. Those healthy antibodies, there’s usually 900 of them, between 700-900 depending on the weight of the child, go in and may dilute the child’s system so that the disease antibodies that the child’s carrying are less effective, not able to go in and attack the child. So it’s a reset for the immune system and it’s quite effective.
Pence: Konecny says after receiving IVIG, her daughter returned to her normal self almost immediately after 18 months of illness. But if rebooting the immune system doesn’t work, doctors may try purifying it, with what’s called plasmaphoresis.
Konecny: And this is usually reserved for the most severely ill children, because it’s done in the hospital. During this process a central line is placed in the child and the child’s blood is actually removed and filtered, so that about 98% of antibodies are removed from the child’s blood. And then the blood, the clean blood that has been cycled through this plasmaphoresis machine is put back into them.
Latimer: Plasmaphoresis has been used for decades for treatment of all autoimmune neurologic disorders. It’s like dialysis of the immune system. You just take out the plasma that contains all the antibodies and the process that you do over several days. And that’s probably the most effective treatment. It is absolutely the gold standard for the treatment of a variety of things. And the American Academy of Phoresis has approved that plasmaphoresis is the first line of therapy of PANDAS.
Pence: Latimer says the vast majority of children can be treated successfully. But researchers don’t know if PANDAS prompts long-term damage to the basal ganglia. Latimer says repeated strep infections are probably necessary to trigger PANDAS… along with a genetic predisposition. So it’s important to know — do pediatricians know PANDAS when they see it?
Konecny: I would say 10 years ago, absolutely not, but thanks to the work of our organization and many others and really, quite frankly parents, it’s becoming common that when you go in and mention this to a pediatrician and they’ve at least heard of the disorder. What’s troubling is that yet the American Academy of Pediatrics has not made a statement about the disease, and we hope that that will happen soon, that they will come out with a best practice, and how to diagnose PANDAS and then how to treat it.
Pence: Konecny invites you to find out more at her organization’s website, PANDASnetwork.org, or through the PANDAS physicians network at PANDASppn.org. You can find links on our website, radiohealthjournal.net, where you’ll also find archives of our programs. You can also find them on iTunes and Stitcher.
I’m Reed Pence.