PANDAS: Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococci
After a bout with a sore throat, nine-year old Jason started to cling to his parents and obsess over noises outside his bedroom window. He was sure that there were robbers outside that would break through and harm him and his family. Jason said he knew his fears weren't "real," but still refused to sleep in his own room unless his parents helped him perform an elaborate ritual - checking under the bed, in the closet and other places to make sure he was safe. The symptoms started suddenly and then subsided gradually over the ensuing weeks. Jason seemed to be over the problem, but he developed the same symptoms again several months later, following a bout of the flu.
Eight year old Jessica thought monsters would emerge from the bathroom pipes to take her away from her parents. She resisted taking baths and complained about going to school. Abruptly, she began to twirl and pull at her hair, and display facial tics, so much so that her teachers recommended a psychologist. These behaviors emerged suddenly after a course of strep throat.
Given these symptoms, many parents would consider counseling for their children. Such obsessions and/or compulsions are observed in about 1% of children of elementary school age and often bring kids into the counselor's office. However, in some cases, physicians determine that the symptoms stem from an unlikely source - the aftermath of an untreated strep throat infection. Doctors are tipped off by how suddenly the behaviors came about, and by how closely they show up in connection with a strep infection. These children may be suffering from a little-known disorder called PANDAS or Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococci.
Dr. Susan Swedo is the National Institute of Mental Health physician and researcher who is credited with the discovery of PANDAS. In an interview, she told me that "these children explode with symptoms before they come into the physician's office. Typically, the OCD symptoms and movement disorder symptoms don't come by themselves." In addition to obsessions, compulsions and tics, children affected by this autoimmune disorder also display attachment fears, deficits of attention and a host of other behavioral symptoms, including irritability and excessive mood swings.
Dr. Swedo, who is also Chief of the Pediatrics and Developmental Neuropsychiatry Branch noted, "We get reports of children who had no problems separating from parents who now need to follow them everywhere they go. For example, one 11 year old boy begged to sleep with his parents because he feared something might happen to him."
What is PANDAS?
The course of PANDAS is often confusing since there is little warning or predictability to the behaviors. No one knows the prevalence of PANDAS but for families affected by it, the disorder can become all consuming. What separates PANDAS from non-strep related psychiatric symptoms is the sudden and abrupt emergence of the symptoms. Without warning or prior history of psychiatric illness, children become fearful, obsessed with danger, develop compulsions or other movement disorders. A typical pattern is for a pre-pubertal child to experience strep throat and then within several days of the occurrence of the bacterial illness, the child changes dramatically. Normally happy, sunny children often develop worries, obsessions and/or extreme mood swings. They may refuse to leave the room where a parent is. Children who previously had no problems going to bed will throw tantrums to get a parent to stay in the room or will repeatedly crawl into bed with parents.
The behavioral symptoms usually persist for several days to weeks. The tics or OCD then seem to go away, and the children often enjoy a few weeks or several months without problems. However, for reasons described below, these children remain vulnerable to relapses, even from non-strep illnesses.
How Does Strep Lead to Psychiatric Symptoms?
The discovery of PANDAS came through observing the reactions of patients. Initially, Swedo's team noticed a group of patients who were ill with infections and then suddenly developed dramatic psychiatric symptoms. At first, they were not sure streptococcus was the culprit but research soon isolated the strep antibodies as an important focus of interest.
PANDAS is controversial in medical circles with some physicians questioning whether such extreme psychiatric symptoms can be triggered by strep antibodies. However, according to Dr. Swedo, there is little doubt that the body's efforts to fight the strep bacteria set off a chain of events leading to PANDAS symptoms. "The science is clear now," Swedo asserts. "We not only have a direct relationship between the anti-strep antibodies and the anti-neuronal antibodies, but also have demonstrated that the antibodies interact with receptors in the brain that could produce the symptoms observed."
Normally when the strep bacteria enter the body, they defend themselves by mimicking healthy cells and elude detection by the immune system. Using the stealth strategy, the strep bacteria create an infection. In response, the body naturally creates antibodies - cellular components designed to find the bacteria, bind with them and direct the immune system to destroy them. When this happens as designed, the antibodies find the strep bacteria and the immune system wins the battle.
However, antibodies are not terribly discriminating and sometimes cannot tell a streptococcus bacterium apart from a healthy cell. In some children, these antibodies pass into the brain and look for something to connect with. In PANDAS, the strep antibodies are unable to discriminate between strep and healthy brain cells. However, the union of strep antibody and neural tissue is not a match made in heaven. Recent research, reported last fall by University of Oklahoma researcher, Madeleine Cunningham, suggests that the antibodies connect with parts of the brain which regulate repetitive behaviors and movement, such as compulsions and tics. According to Dr. Swedo, such autoimmune actions may have the direct result of producing obsessions, compulsions and tics; and in addition, they might indirectly offset the critical balance of brain chemistry which regulates mood and emotion.
Compounding the problem is the durability of strep antibodies. They can remain on the prowl for a hook up weeks after the strep is gone. Subsequent infections, the flu or even colds can stimulate the immune system to produce more of the same kind of antibodies which the triggered the original symptoms. PANDAS children can then abruptly re-experience the same symptoms after a symptom-free span of time. Dr. Swedo explained, "We see a saw-toothed pattern of symptoms, extreme outbursts of behaviors for a brief period in response to the infections and then the child may be relatively symptom-free for a period of time."
What can parents do?
The primary advice for parents is to treat strep throats promptly. This means a visit to the primary care provider at the first signs of strep throat. Symptoms of strep throat include:
- Severe and sudden sore throat without coughing, sneezing, or other cold symptoms,
- pain or difficulty with swallowing,
- high fever,
- swollen lymph nodes in the neck,
- white or yellow spots or coating on the throat and tonsils,
- bright red throat or dark red spots on the roof of the mouth at the back near the throat and/or swollen tonsils, although this symptom may also be caused by a viral infection.
- Vomiting without diarrhea, headache and other nonspecific signs of illness can also signal a strep infection.
Some children dislike having a throat culture done and will deny that they are in pain, rather than risk getting "the swab." However, for reasons other than PANDAS (e.g., rheumatic fever), it is important to investigate and treat these symptoms promptly. For some children, the sudden PANDAS behavioral changes are the tipoff that an otherwise unclear symptom picture is really strep throat or a relapse related to an illness. Thus, any sudden changes in behavior should involve a trip to the pediatrician to rule out a biological trigger.
Not long ago the advice was to cease antibiotic use when the symptoms were gone. However, current research suggests maintaining a course of antibiotic treatment may be beneficial. Furthermore, for severe cases, parents and physicians might consider using immunoglobulin treatment (IVIG) or plasmapheresis. IVIG administration produces a vigorous immune response that appears to counteract the PANDAS symptoms. Plasmapheresis cleans the strep antibodies (and others) from the blood via plasma exchange. These interventions are considered experimental and are not without side-effects. In addition, they are expensive and insurance companies often won't pay for this "off-label" use of the treatments. However, for extremely symptomatic children, the procedure may offer relief.
Psychological counseling can also be helpful as means of helping both parents and child understand the condition. Since there are no traumas to uncover, cognitively based counseling which focuses on helping children better understand and reframe their obsessions and fears is most helpful.
Parents might also consider calling a regional medical school or facility to find out if any PANDAS research studies are underway. The National Institute of Health provides up to date information on PANDAS and a helpful Q & A on this website: http://intramural.nimh.nih.gov/pdn/web.htm. Currently there is a study accepting participants.
Warren Throckmorton, PhD is Associate Professor of Psychology and Fellow for Psychology and Public Policy at the Center for Vision and Values at Grove City College (PA). His personal blog is at www.wthrockmorton.com.
An earlier version of this article was published in Oct. 2009 in the Christian Post. This version recommended a reduction of antibiotics. The article above has been updated to reflect more current research.