Doctors love abbreviations and acronyms. For a myriad of reasons, we don’t like to use the actual words of a medication or dosage or disease or syndrome or anatomical part or instruction or etc. So, Amoxicillin becomes amox; milligram becomes mg; Diabetes Mellitus becomes DM; Latissimus Dorsi becomes lat; once daily becomes qd; etc. These shortcuts have become so ubiquitous that there are national organizations trying to limit there use for safety reasons.
And I have to admit, that I have become so used to some of the abbreviations or acronyms that I have ‘forgotten’ the real word(s)! Imagine my embarrassment during a patient visit when I tell a parent “Your child has XYZ disease.” “What does XYZ stand for, Dr. Cattaneo?” “Uh…ummm…let me Google that!”
That kablooey moment came to head when a working group recently met to clarify the criteria for PANDAS. In the end, they formed a new entity – PANS. Both PANDAS and PANS are related to PITANDS. Whew…but, wait a second…what exactly are PANS and PITANDS and PANDAS?
What are PANS and PITANDS and PANDAS?
-PANS = Pediatric Acute-onset Neuropsychiatric Syndrome
-PITANDS = Pediatric Infection Triggered Autoimmune Neuropsychiatric Disorders
-PANDAS = Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections
At the core, all three diagnoses involve an acute onset of obsessive-compulsive disorder and/or tic disorder symptoms. PANS encompasses a process preceded by infectious or non-infectious triggers. PITANDS is preceded by an infectious disorder such as chickenpox or the flu. PANDAS is a subset of PITANDS preceded by a streptococcal infection, specifically.
All PANDAS are PITANDS, which are all PANS. But, not all PANS are PITANDS, and not all PITANDS are PANDAS. (Click here for pictorial explanation: PANS – PITANDS – PANDAS)
How is PANDAS diagnosed?
Focusing on PANDAS, the diagnosis is strictly clinical. There is no one test that a doctor can order and evaluate to diagnose PANDAS. While laboratory and diagnostic tests are an adjunct to the history and physical, they will not make the diagnosis for the doctor. A thorough history and physical is a must. All other causes of OCD/Tic disorders must be ruled out.
The criteria for PANDAS is as follows:
-Abrupt, significant onset of obsessive-compulsive/tic disorder
-Relapsing-remitting course (i.e. it comes and goes)
-Pre-pubertal onset (although PANDAS can rarely be seen in adolescents)
-Association with other neuropsychiatric symptoms (eg anxiety, bed-wetting, school problems, ADHD)
-Association with streptococcal infection (positive rapid strep test –or- positive strep culture –or- elevation in strep antibodies in the blood).
*PANS is basically the above criteria minus the association with streptococcal infection. The key to diagnosing both PANS and PANDAS is to not miss a more concerning neuropsychiatric disorder such as Lupus, Tourette Disorder, Chorea, etc. which may require a much more in-depth work-up, including possible imaging, blood tests, muscle function tests, and brain wave tests.
How does strep cause PANDAS?
No one is exactly sure how strep causes the OCD/Tic symptoms. However, the most common theory is “molecular mimicry”. Basically, very smart strep bacteria put molecules on their cell walls that are very similar to the molecules found on human heart, kidney, and brain cells. This enables the strep to stay undetected for a long period of time. Eventually, the body deduces the trickery and starts attacking the strep. It, however, also attacks the similar molecules on the heart, kidney, and brain. This cross-reactivity is what causes rheumatic fever and kidney disease. When mild cross-reactivity occurs in the brain, it can cause the OCD/Tic disorder characteristic of PANDAS.
How common is PANDAS?
Fortunately, PANDAS is extremely rare. The most recent estimation is 10/30,000 positive throat cultures will lead to this disorder (i.e MUCH less than 1%). However, some investigators are suggesting that 10% of all childhood tic disorders are associated with PANDAS. The bottom-line is this: if your child has an OCD/Tic disorder, it is most likely not PANDAS.
How do you treat PANDAS?
As you would expect, the main treatment for PANDAS is to treat the strep. Your doctor will usually prescribe a medication such as Amoxicillin, Cephalexin, or in rare cases, Azithromycin*. Usually with eradication of the strep bacteria, the OCD/Tic symptoms will resolve.
*Azithromycin is one of the most resisted antibiotics that doctors use. Yes, it is great that it is only five days. But, due to huge overuse, common bacteria have figured out how to not be killed by Azithromycin. Further, in children, there are very few bacteria that are treated by this medicine anyway. Amoxicillin is still the most common antibiotic used and for good reason: it is cheap and it works most of the time.
If OCD/Tic symptoms are persistent, or are greatly effecting the child’s life, cognitive behavioral therapy and/or neuropsychiatric mediation has been suggested as a possible treatment. The most common form of medication is a Selective Serotonin Re-uptake Inhibitor, or SSRI (Zoloft, Paxil, Celexa, etc). When used together, these children do extremely well.
There is ongoing and promising research suggesting two other forms of treatment: immune modulating therapies and steroids. Given that PANDAS could be caused by a immune system gone awry, treatments such as IVIG and plasmapheresis have been tried in the past with some success. Larger studies are currently being performed to evaluate the benefit of these treatments. Therefore, regular use of these treatment options is NOT recommended.
Steroids, such as prednisone, have also been shown to alleviate problems in some children suffering from PANDAS. Some concerns with using steroids, however, have developed. First, sometimes symptoms worsen with the steroids. Second, once the steroids are stopped, the symptoms have a tendency to return. Studies are being performed to evaluate the best use of steroids in PANDAS. Therefore, regular use of this treatment option is NOT recommended.
Some physicians recommend prophylactic antibiotics to prevent further episodes of strep, therefore, preventing further episodes of PANDAS. There are no reliable studies to support these claims, as of yet. Therefore, generalized use of prophylactic antibiotics is not recommended. However, physicians may decided on a case-by-case basis to use prophylactic antibiotics if the benefits outweigh the risks.
So, what are the take home messages?
-If your child suddenly develops OCD or Tic disorder symptoms with or without signs of strep, please see your doctor.
-Understand that most cases of OCD and Tic Disorders are NOT caused by PANS, or PITANDS, or PANDAS.
-Antibiotics are the best treatment option, but only in dosages to treat the acute infection.
-Adjunct therapy (therapy/neuropsychiatric medication) to treat the OCD and/or Tic symptoms may be necessary.
-Say “no” to IVIG or plamaphoresis (unless enrolled in a NIH study).
-Say “no” to steroids
-Say “no” to prophylactic antibiotics (most of the time).
*Remember – reading this blog does not constitute a physician-patient relationship. All information provided is not to be used for medical decision making. If you have concerns/questions – please call your doctor.