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When my middle son, Sammy, was twelve years old he was suddenly struck with an increasingly bizarre series of behaviors, just before the start of sixth grade. He was diagnosed first with obsessive-compulsive disorder, next Tourette’s, and then I was told that he might be in and out of hospitals for the rest of his life. He went from totally normal to completely dysfunctional in the space of weeks while doctors shook their heads and prescribed increasing doses of psychotropic medications that never helped. Confined to our home by the severity of his illness, his world shrank to a couch in our den. Month after month passed as I struggled to come to terms with the illness that had come out of nowhere. I kept asking doctors to explain what had happened. He’d been a completely normal boy until he turned twelve.

“It happens,” I was told.

Sammy was sick for a full year before I found the missing piece of the puzzle: a link between strep, OCD, and tics. Another mom told me about it. No doctor had ever mentioned the possibility that perhaps Sammy had caught something that was making him mentally ill. We ran a simple blood test, and there it was with startling clarity. The results showed that an elevated level of strep antibodies surged through his system. His brain was under attack. The disorder is called PANDAS: Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections.

I thought that was the end of our journey, but it was only the beginning. It was extremely difficult to find treatment. Doctors dismissed the link between his infection and his mental illness. Even with blood work indicating strep, doctors said there was no connection.

“The best thing you can do for your son,” said a well-respected pediatric neurologist in Boston, “is to take him home and accept the diagnosis of OCD.”

Instead of doing that, I redoubled my efforts. I located a physician with the correct expertise; she was four hundred miles away. Month after month, we made the trek from Maine to southern New Jersey to see Catherine Nicolaides, MD, a developmental pediatrician. Sammy would lie across the back seat of my van, too weak to move. He had already missed all of sixth grade and the start of seventh grade. But after a few visits he could ride sitting up and, a few months later, banter with his brother as we drove. Under Dr. Nicolaides’ tender care and guidance, along with the insight of Boston psychiatrist Daniel Geller, MD, to whom Dr. Nicolaides referred us, slowly and steadily Sammy began to recover. With appropriate antibiotic treatment (Augmentin XR), a gradually reduced prescription of psychotropic medications, and cognitive behavioral therapy, Sammy was finally able to rejoin his classmates in eighth grade. It took four full years of antibiotics taken every day for the disorder to be fully treated. And for two years after that he occasionally had compulsions when he was exposed to strep. But by the time Sammy entered college, he was fully recovered and took no medication at all. He has now graduated from Carnegie Mellon University with a degree in computer science and mathematics: no meds, no behaviors, just fully recovered. The future is brilliant for this young man who had been destined to be in and out of mental treatment facilities for the rest of his life.

What is PANDAS?

In a nutshell, researchers believe that PANDAS occurs when strep antibodies cross over the blood-brain barrier, into the brain, and attack areas such as the basal ganglia and produce particular behaviors. The presence of a strep infection correlates with the presence of the behavioral disorders in PANDAS but similar symptoms can result from other triggers and this is know as Pediatric Acute-onset Neuropsychiatric Syndrome (PANS).

PANDAS is not as new and controversial as you might think. Descriptions of infections triggering disturbing behaviors began as early as the 1800s. In the 1920s, reports linked the behaviors to sinusitis. In the 1950s, strep was linked to the movement disorder Sydenham’s Chorea. And in the late 1990s, PANDAS was officially identified. In 2011, the NIMH proposed criteria for a much broader, related disorder known as PANS—proposing that behavioral disorders can be triggered by a number of infections, not just strep. In my book, Childhood Interrupted: The Complete Guide to PANDAS and PANS, I most often refer to PANDAS because it is a more established diagnosis, but the principles of the two disorders are basically the same. If you understand PANDAS, you will also be able to understand PANS.

Why It’s Confusing

One of the confusing things is that PANDAS and PANS don’t fit into our framework of what to expect from an infection. We think of strep as giving our children sore throats and temperatures. We may remember hearing that a relative had rheumatic fever and heart problems that were caused by strep. We might even have heard news reports about an unusual case of strep that attacked the skin. Those all fit within the common understanding of what an infection does: it makes us physically ill in the traditional sense. What isn’t widely known or accepted is that sometimes a child’s concerning behaviors can be symptoms of strep or another infection.

We need to trust our instincts when it comes to PANDAS because nothing about it is black and white. Medicine is just at the beginning of recognizing this disorder, so PANDAS is still in its infancy. There isn’t a checklist yet for whether a child has PANDAS, and there isn’t an established protocol or a series of easy diagnostic steps to follow. I tell parents that if they have a child whose behaviors are concerning, and they are not satisfied with the answers they have received to explain those behaviors, then they are right to wonder if an infection might be the root cause of what’s troubling their child. What they are doing is following their instincts. Our instincts guide us every day. They help us choose friends, avoid strangers, and know when a problem is looming. Instincts are what enable us to sometimes know what our children want or need before they do. Our instincts gnaw at us when we know something is wrong, but we can’t put our finger on it. Instincts help us sort through confusing situations.

This helps explain another odd thing about PANDAS: parents are often the first to raise PANDAS as a possibility. We usually look to doctors to guide us regarding health concerns. If you think it through, though, it’s not necessarily surprising that parents may be the first to mention PANDAS. We take our children to the doctor when we suspect a strep or ear infection. We take them when their bellies won’t stop aching. We take them when we spot a rash or a tick bite. Parents are the first to wonder about their child’s well-being because they know their child better than anyone else. So when parents don’t feel that they have found the answer to what’s troubling their child, they may suggest PANDAS. Generally it’s because the children are acting so differently from the past. Friends and relatives may reassure parents that the child is simply going through a rough patch or will “grow out of it,” but parental instincts may question whether there might be another explanation for what is going on with their child.

Behavioral Symptoms

There are behavioral symptoms that suggest a child may have an underlying infection. There are some children who will suddenly have a full-blown tornado of behaviors. They happily drift off to sleep one night and wake up the next day a totally different, unmanageable child. But their parents and the doctors who treat them say that, in retrospect, there were early behavioral hints, or soft signs, along the way that a bigger problem was brewing. These soft signs, sometimes called microepisodes, are behaviors that are new or unexpected for a child, but still manageable. They whisper that a problem may be coming.

Any of the behavioral changes listed below may be soft signs of PANDAS. If a child is experiencing any of these difficulties, and especially a combination of more than one, you want to make it a point to notice whether they are becoming more prominent.

  • Obsessions
  • Compulsions
  • Tics
  • Regressions
  • Touch, hearing, sight, taste, and smell issues
  • Refusal to eat
  • Inability to concentrate
  • Impulsivity/distraction
  • Separation anxiety
  • Bedtime fears
  • Rapid mood swings
  • Episodes of stuttering
  • Raging, sobbing, screaming
  • Threatening or worrying about harm
  • Irritability
  • Sleep disturbance
  • Urination issues
  • Changes in handwriting
  • Joint pain—consider also rheumatic fever, rheumatoid disorder, or Lyme disease
  • Personality changes at home and/or school

Abnormal movements, sometimes called “choreiform movements,” may indicate PANDAS, but their presence should always cause consideration of whether the child may have rheumatic fever or another neurological disorder. Such movements may include jerking, writhing, lack of coordination, tics, and twitching (myoclonus).

Three Examples of Early Signs

Parents report that many PANDAS children have separation anxiety, get stuck and regress, or have tics. Some children may have all three together. Because these three are some of the most common behaviors, I am using them as examples. All the behaviors listed above may grow into much bigger problems, but these offer a good illustration. And I chose separation anxiety to more fully describe how an early sign might escalate from difficult into something far more challenging and disabling.

Separation Anxiety: Ordinarily, when our children don’t feel well, they don’t like us to leave them. They want mom around to put a cold cloth on their foreheads and kiss their sick tummies. They want dad to tell them a special story. They’d rather not be left with a babysitter. They want you. This is all normal behavior. Suppose though that your second-grade daughter had what seemed like a minor cold two weeks ago. She’s clingier this year than she was last year, but still loves going to school and went even while a bit under the weather with her cold. This week she suddenly does not want to go at all. She says her clothes are uncomfortable; they don’t “feel right.” None of your suggestions make her happy. It takes her two hours, but she finally gets dressed and agrees to go to school. When it’s time for you to leave her there, the teacher has to peel her off you as she sobs. She can’t be consoled. You’re upset and confused. The teacher wonders if something is going on at home.
Your fears fade when your daughter is back to her old self in a matter of days. She’s enjoying school and playing with her friends. But what if it happens again three months later in a much bigger way? This time she refuses to go to school. She screams and cries, and there is no amount of persuasion that will change her mind. You stay home with her because she is so upset. She won’t leave your side, not even when you need to use the bathroom. She cries all day. Now you start to question what is really going on. Could it be related to what happened three months ago? Was it just a cold that she had? Could she have PANDAS? Maybe, because a PANDAS child will often have a series of behavioral episodes over the course of childhood. Usually each episode is more extreme than the last and that is called an exacerbation.

In very young children it’s especially important to be alert to behavioral patterns because those children lack a long-established history. Not many people know a young child well, so it’s easy to attribute changes to personal growth. If you have a kindergartener who suddenly starts demonstrating extreme separation anxiety and wets the bed at night, the logical explanation is that your kindergartner is anxious about school. The other possible explanation may be that these are often overlooked signs of an infection. Left unchecked, an infection can become quite serious.

Getting Stuck/Regression: Another sign to look for is whether your child is getting “stuck.” Each child is different, but it seems the simplest tasks, the ones taken for granted, have become difficult. The child regresses. You find yourself looking at your child and thinking, “This just isn’t like him.”

Each child has his or her own version of getting stuck or regressing, but here are some “before” and “now” examples.
A child who…

  • raced out the door, now can’t leave.
  • regularly washed up before dinner, now can’t touch the faucet.
  • was a good eater, now doesn’t like what you serve.
  • wrote clearly, now has trouble writing.
  • loved school, now doesn’t want to go.
  • spoke clearly, now stutters.
  • was toilet trained, now wets the bed.
  • zipped through homework, now can’t finish due to painfully rechecking.

While getting stuck may be manageable in the beginning, it can grow to become disabling. Checking and rechecking may progress to the point that it’s hard for your child to leave the house because there are so many things that she must do first. You never want it to get to that point. This is why knowing the early signs helps.

Tics: Another way that strep can present itself may be a motor or verbal tic. Tics can present themselves in a number of different ways and are four times as likely to show up in boys. Some of the most common are: Eye blinking; facial grimaces; coughing; snorting; tapping; neck rolling; head jerking.

Even sneezing can be a tic, but the other tics are far more prevalent.

The presence of a tic may indicate a neurological problem, but tics can also be the sign of a strep infection. Often when parents raise concerns about tics to the pediatrician, the doctor feels the child will grow out of it. Sometimes that does happen, but even if a persistent tic is mild and does not significantly impact daily life, it should be taken seriously. For example, sneezing is something that we do occasionally but not repeatedly for hours. A persistent sneeze indicates a problem. You want to make sure there’s no infection because you don’t want it to escalate to the point that your child is sneezing every few seconds.

Beyond the Early Signs

When an early sign becomes concerning, it may indicate that this is more than a phase the child is going through. Listed below are ten broad categories of behaviors that have been linked to PANDAS. If a child has an increasing level of behaviors within these categories, then parents should make sure there is no underlying infection that has gone undetected. PANDAS is particularly prevalent in boys—pre-puberty OCD affects about three times as many boys as girls, although practitioners report that it is recently on the rise among girls.

Common behaviors linked to PANDAS:

  1. Obessive-compulsive disorder (OCD)
  2. Motor and verbal tics (Tourette’s)
  3. Attention deficit hyperactivity disorder (ADHD)
  4. Enuresis
  5. Fine/gross motor changes (dysgraphia)
  6. Joint pains
  7. Mood changes
  8. Sensory defensiveness
  9. Separation anxiety
  10. Sleep disturbances

 

Overlooked Signs

There are eight other diagnoses and conditions that should cause us to question whether a child may be plagued by an infection. These diagnoses are sometimes given when PANDAS is present but overlooked. If a child is diagnosed with one or more of these, you might want to consider whether an underlying infection may be present.

  1. Autism
  2. Bipolar disorder
  3. Depression
  4. Dysthymia
  5. General anxiety disorder
  6. Learning disabilities
  7. Oppositional defiant disorder
  8. Other autoimmune disorders

 

How can a strep infection cause a behavioral disorder?

When strep antibodies pummel the area of the child’s brain that controls behavior, the child’s behavior changes. This does not happen to all children who have strep infections, just as not everyone with untreated strep will develop rheumatic fever. But the effect of strep antibodies on the brain may be overlooked and mistakenly excused to childhood misbehavior rather than recognized as symptomatic of the infection. Susan Swedo, MD, of the National Institute of Mental Health is one of the leading researchers in the country. She has reported studies showing that about 25 percent of first graders have tics, particularly during the winter months when strep is most prevalent. And in collaboration with another of the country’s top researchers, Tanya Murphy, MD, of the University of South Florida, she reported that all of those children who had classroom tics and behavioral problems tested positive for strep.

If a child has a strep infection, two things happen: inflammation develops and the immune system produces strep antibodies. With increased inflammation, the blood-brain barrier (BBB) may become more permeable. Swirling through our body’s blood in pursuit of the strep, the antibodies cross the BBB and enter the brain. Once there, they form an imperfect lock with tissue in the basal ganglia that is mistaken for the strep bacteria; then the onslaught begins. At first the child may merely exhibit a mild tic or odd behavior. The children who are lucky enough to have traditional symptoms of strep may go to the pediatrician, turn up positive on a throat swab, take a ten-day course of antibiotics, and the whole thing subsides. The mild tics or behavioral outbursts are quickly forgotten.

The children who are not so lucky suffer because the strep is never detected or eradicated. As PANDAS progresses, the children may eventually be given mental health diagnoses such as OCD, ADHD, Tourette’s, and/or bipolar disorder. They may be prescribed psychotropic medications, fall apart on a regular basis, and have difficulty functioning in a normal setting. Their heartbroken parents wonder what happened to their child and remember how he or she “used to be.” The entire family revolves around the needs of the ill child. Siblings grow resentful, parents impatient, and the family structure teeters.
Parents who raise PANDAS as a possibility may be challenged by physicians who “don’t believe” in PANDAS. Correct treatment may be further complicated by the information posted on the website for the National Institute of Mental Health. http://intramural.nimh.nih.gov/pdn/web.htm The criteria posted at the time of this writing are:

  1. Presence of clinically significant obsessions, compulsion, and/or tics
  2. Unusually abrupt onset of symptoms or a relapsing-remitting course of symptom severity
  3. Prepubertal onset (with recognition that this is an arbitrary criteria chosen because post-strep reactions are rare after 12)
  4. Association with other neuropsychiatric symptoms
  5. Association with streptococcal infection

Some doctors insist on strict compliance with the criteria. Keep in mind that my son had PANDAS, but he would not have fit the NIMH criteria. His symptoms developed over a period of weeks. He had no relapsing-remitting course of symptoms; he only grew consistently worse. And I had no way of associating his behaviors with a strep infection because we never knew he had one. He’d never had any traditional symptoms of strep, not even a sore throat. Moreover, as described by parents in the thousands of e-mails I have read, there are many other children who do not fit the NIMH criteria. They do, however, respond when appropriately treated for PANDAS.

What Is PANS?

Also posted on the NIMH website is a suggested criteria for a disorder to be known as PANS: Pediatric Acute-Onset Neuropsychiatric Syndrome. PANS was proposed by three researchers in early 2012 based on clinical observations made by treating physicians that bacteria and viruses other than strep can cause behavioral disorders. Although broader recognition of infectious triggers is a welcome change, the defined criteria for afflicted children is limiting. The threshold for fitting the criteria is a child who has OCD, which may manifest as “severely restricted food intake.” If the threshold test is met by a child who has 1) OCD and/or an eating disorder, then 2) the child must have a severe and acute onset of at least two out of seven other specified concurrent symptoms, and 3) there can be no better explanation for the behavioral disorder such as Sydenham’s chorea, lupus, or Tourette’s with the suggestion that spinal taps, EEGs, and MRIs may be appropriate to rule out these other possibilities.

The seven other neuropsychiatric symptoms specified by the NIMH for fulfilling part two of the PANS criteria are:

  1. Anxiety (particularly, separation anxiety)
  2. Emotional lability (extreme mood swings) and/or depression
  3. Irritability, aggression, and/or severely oppositional behaviors
  4. Behavioral (developmental) regression (examples, talking baby talk, throwing temper tantrums, etc)
  5. Deterioration in school performance
  6. Sensory or motor abnormalities
  7. Somatic (bodily) signs and symptoms, including sleep disturbances, bedwetting, or urinary frequency

Parents are disappointed by the PANS criteria because the three-part rubric is so difficult for everyday pediatricians to apply. In particular, the children must be incredibly ill—essentially having reached a critical point and perhaps needing hospitalization—to be identified with PANS. Most acute PANS children do have a firestorm of these symptoms at onset and, therefore, an MRI, EEG, and LP are warranted. But many PANS children will not reach the acute stage if there is appropriate early intervention. And while most of the children do have OCD (perhaps manifesting as an eating problem), not all do. Some have only tics. For certain though, by the time a child has OCD, along with two out of the seven other behaviors, and a paper trail of diagnostic testing, that child will be unable to function. Not to mention that you will probably be broke. You are seeking intervention well before reaching that point. The PANS definition offers criteria to examine an extreme phenotype, but a solitary and new behavior in a previously asymptomatic child should be assessed for strep or other triggers. Early detection, immediate recognition, and prompt treatment are the keys to any neuropsychiatric disorder attributable to an infection.

This was an adapted excerpt from Childhood Interrupted: The Complete Guide to PANDAS and PANS by Beth Alison Maloney, author of Saving Sammy: Curing the Boy Who Caught OCD.
To learn more go to Beth’s website:
www.BethAlisonMaloney.com