One morning in 2012, 7-year-old Katelyn Pohlman woke up with a stomachache and a high fever. A throat culture at the doctor’s office came back positive for strep throat, so she stayed home to rest. But by the next day, instead of lying on the couch, Katelyn was zipping from one place to another, insisting she saw spiders everywhere. She kept crying for no reason and seemed to be suffering from anxiety and mood swings. Her pupils were dilated. She refused to eat. She also felt compelled to jump in place. “Jump, jump jump,” recalls her mom, Diana, “for hours.”
Katelyn was diagnosed with a form of Pediatric Acute-onset Neuropsychiatric Syndrome, known as PANS. It’s an uncommon and controversial disease that is believed to occur when the microbes that cause an infection—usually strep, but sometimes chicken pox, Lyme disease, or walking pneumonia—ignite the bizarre, sudden onset of a psychiatric disorder. (It’s confusing, but when the symptoms are caused by strep infections, the disease is instead called PANDAS, or Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections.)
The condition was first recognized almost 30 years ago, but doctors are still debating the evidence and how to identify it. Due to lack of research, no one knows for sure how many children have PANS disease, but it’s estimated that the disorder affects one in 200 children each year. The first official diagnostic guidelines for PANS were published in the Journal of Child and Adolescent Psychopharmacology in 2015, and treatment standards followed in 2017. These steps have been crucial for kids like Katelyn, who until recently might have gone months or even years without a diagnosis.
How could one child’s strep merely cause a sore throat while another’s unleashes a full-blown psychiatric disorder? Research suggests that children with PANDAS and PANS have inflamed basal ganglia, the parts of the brain that process information and decide what to do with it. (They also play a role in memory and reasoning, and tell your muscles when and how to move.) When strep enters the body, antibodies emerge in the bloodstream to fight it. But some scientists believe that in certain children, rogue antibodies trickle through the blood-brain barrier and react with basal ganglia cells. This ambush inflames the brain, causing its functioning to go haywire.
“It really is as if a child’s brain were on fire,” says psychiatrist Kiki Chang, M.D., lead author of the diagnostic guidelines. Experts suspect that this reaction happens only with some strains of strep and only in children whose immune systems have been primed—for unknown reasons—to overreact to those strains, says Susan Swedo, M.D., chief of the Section on Behavioral Pediatrics at the National Institute of Mental Health.
Unfortunately, no lab test can definitively confirm whether a specific infection has caused a child’s strange behavior. Doctors may suspect PANS disease whenever a child has an extremely abrupt “overnight” onset of refusing to eat or other obsessive-compulsive symptoms (like asking for reassurance, or handwashing), accompanied by two or more of seven other behavioral changes (such as separation anxiety, frequent urination, sleep disturbances, or sensory sensitivities). “If parents can’t say on which day their child changed, then it’s probably not PANS,” says Dr. Swedo, who wrote the first paper on PANDAS in 1998.
For Chandler Wendland, that day was October 10, 2016. A typically well-behaved kid, the 7-year-old suddenly started blinking constantly and doing things in threes. Still, his journey to a PANS diagnosis was long and bumpy. After a month of taking him to a psychologist, his mother stumbled across PANDAS in a book and made a pediatrician appointment.
Katelyn’s diagnosis had been much quicker. Her mother, Diana, recognized the PANS symptoms instantly because her older son, Garrett, had contracted the condition ten years earlier. (That’s what prompted Diana to found the PANDAS Network, pandasnetwork.org, a national nonprofit for PANDAS patient advocacy.) Having a parent with any kind of autoimmune disorder or a sibling with PANS is one of the few known risk factors.
The treatment doctors use for infection-triggered PANS seems almost too simple: antibiotics. Katelyn’s psychiatrist prescribed six weeks of azithromycin, followed by thrice-weekly pills for six more months. For some kids, a typical ten-day dose is all they need. “Patients can go from being mentally ill to completely well in a few days,” says Dr. Swedo. However, most require a longer course (which can cause its own set of problems), often along with other drugs.
Alas, not everyone recovers completely, and kids who have seemingly gotten over PANS may be at risk of developing it again months or years later. For example, Chandler improved while taking penicillin for a month. “But after catching a flu virus at a holiday gathering, he developed neck spasms and movements that interfered with his walking. “The theory is that once a child’s immune system is primed by an infection, anything that stimulates it in a similar way could lead to a recurrence of symptoms,” says Dr. Chang.
For persistent PANS symptoms, some doctors are now trying an immune-modifying treatment called intravenous immunoglobulin, or IVIG. Put simply, IVIG is a sterile solution made of antibodies from a diverse pool of donor blood. And while it can have a miraculous effect—it put Katelyn into remission last year after she relapsed at age 12—the treatment doesn’t work on every child and carries serious medical risks, says Margaret C. Fisher, M.D., chair of pediatrics at The Unterberg Children’s Hospital at Monmouth Medical Center, in Long Branch, New Jersey. But desperate parents are willing to take the gamble, even at a high price. For most patients, IVIG is not yet paid for by insurance.
Advances in imaging could change that. Unlike a broken leg, inflammation in the brain can’t be seen on an MRI. But research shows that positrone mission tomography scans may be able to capture it. Says James F. Leckman, M.D., Ph.D., professor of child psychiatry at Yale School of Medicine, “It’s the most compelling insight we have into the underlying biological basis of PANS, but it needs to be replicated.”
In the meantime, the best thing children with PANS can do is try to stay healthy, act quickly if they catch strep, and relax. “It’s important to avoid added stress,” says Dr. Chang. “After all, stress itself leads to inflammation.”
Learn More about PANS. You can find a complete list of symptoms and the names of specialists around the country at the website of the PANDAS Physicians Network.