WHAT IS PANDAS?
PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections) occurs when strep triggers a misdirected immune response results in inflammation on a child’s brain. In turn, the child quickly begins to exhibit life changing symptoms such as OCD, anxiety, tics, personality changes, decline in math and handwriting abilities, sensory sensitivities, restrictive eating, and more.
PANDAS Network estimates that PANDAS/PANS affects as many as 1 in 200 children.
The hallmark trait for PANDAS is sudden acute and debilitating onset of intense anxiety and mood lability accompanied by Obsessive Compulsive-like issues and/or Tics in association with a streptococcal-A (GABHS) infection that has occurred immediately prior to the symptoms. In some instances, the onset will be 4 to 6 months after a strep infection because the antibiotics did not fully eradicate the bacteria. Many pediatricians do not know the latent variability of strep – Rheumatologists and Streptococcal Experts do.
When strep cannot be linked to the onset of symptoms, the NIMH states one should look into the possibility of PANS (Pediatric Acute-onset Neuropsychiatric Syndromes).
The acute onset means a Y-BOCS (Yale Brown Obsessive-Compulsive Scale) score of >20 and or a Chronic Tic Disorder YGTSS (Yale Global Tic Severity Scale) often with multiple tics. Below is the symptom criteria for PANDAS. Additional symptoms may be present.
PANDAS has an encephalitic-like onset. Some childrens’ onsets are clearly debilitating and they become near catatonic and homebound. Other children can function at school and then fall apart at home for hours on end. BUT IT IS CLEAR – THE FORMERLY NORMALLY FUNCTIONING CHILD IS GONE.
PANDAS symptoms may have flared in a lesser manner for weeks or years prior to the acute onset but often readily disappeared or lessened over time. If untreated with antibiotics generally we have seen a myriad of other symptoms will intensify in the weeks and months post-acute onset. If the severe symptoms do not stop and persist over many months, permanent cognitive damage can occur.
PANDAS children may have moderate to dramatic improvement with antibiotics within one week of treatment, however, further interventions may be needed. How to stop the entire syndrome is still debated, but many parents and doctors report prolonged antibiotics (two months to one year) and/or IVIG (intravenous immunoglobulin) treatment or plasmapheresis.
1998: Dr. Susan Swedo and associates first described the PANDAS syndrome in “P.A.N.D.A.S.: Clincial Description of the First 50 Cases”.
2012: Revised criteria and guidelines for PANDAS was established by the NIMH.
2015: The Special Edition JCAP is published. It is the first collection of research papers on PANDAS/PANS written by a Consortium of researchers and physicians.
LINGERING RELUCTANCE TO DIAGNOSE
Many doctors assume, if they have heard of PANDAS at all, that it is only the sudden onset of “typical” pediatric Obsessive Compulsive Disorder or Tics. We believe Our Collective Voice of experiences will help them understand this illness reaches beyond that assumption.
PANDAS is currently a clinical diagnosis and open to interpretation and subjective observation. There are currently no conclusive diagnostic blood or neurological tests to be relied on.
Many doctors have not seen it. Nor have they seen Rheumatic Fever which is a close-cousin to the PANDAS autoimmune syndrome occurring and they don’t appreciate the variability of streptococci and its potential virulence.
More is written about this in the Diagnostic Test portion of this website. Getting a rapid throat swab and 48-hour strep culture is a good first step. A strep ASO and D-nase Titer test is also beneficial in helping to establish the strep connection. But, many times the titers will be only moderately elevated – and at times not elevated or extremely elevated. This is the variable nature of the strep bacteria.
THE CUNNINGHAM PANEL OF TESTS
A series of 5 tests to help determine the “likelihood of the patient’s condition being auto-immune in nature”, including possible PANDAS, is commercially available by Moleculera Labs. This test is derived from the research done by Dr. M. Cunningham.
Even though a variety of tests can aid in determining the root cause of symptoms and what course of action may be appropriate, PANDAS IS A CLINICAL DIAGNOSIS.
PRESENTING THE CLINICAL PICTURE
We have found in our group of 100+ that about 90% of the families have a history of the following in first or second degree relatives on either mother’s or father’s side (parents/grandparents/aunts or uncles):
1 Autoimmune Illness
2 Bad Relationship w/Strep Infections (Repeat strep infections, Rheumatic or Scarlet Fever)
3 Child has a History of Upper Respiratory Cough, Sinus Issues or Allergies
4 Family Mental Health History of Anxiety Issues or Other Related Issues
Please see the Table of Family Histories where approximately 20 parents were randomly asked to explain their family medical history. In the interest of time, we did not put all 70+ parents results on this website but in informal conversations these similarities were nearly always found. Bring this table to your doctor if it fits your family’s situation.
Most of the PANDAS children in the original PANDAS Network group have never had an autoimmune workup –testing immunoglobulin (IGg) levels. Of the 15 families that have done so – 10 of the children had various IGg deficiencies that were extreme to moderate. Some of the families also reported that other family members had extreme immune deficiencies. Research has specifically found that mothers have a 25% chance of having an autoimmune disorder themselves (Clinical Psychiatry News 2011).
In the Diagnostic Test section there are other suggestions given for tests to be taken that often indicate a child’s “taxed” immune system and scales to help provide a clinical picture of the child’s symptom severity.
ARE RESEARCHERS STUDYING PANDAS?
Yes! The number of studies involving PANDAS and PANS has grown significantly over the years.
The 2015 JCAP Special Edition is the first collection of research papers on PANDAS/PANS written by a Consortium of researchers and physicians. It is pivotal to the advancement of treatment and research for this subset of children. Among the published articles, is the groundbreaking Consensus Statement. Anyone who may be affected or interested in PANDAS or PANS, needs to be familiar with this work. Click here for more information.
Other primary research to know:
• Kirvan, et al (2003) Mimicry and Auto-antibody mediated neuronal Signaling Cells in Sydenham Chorea (NOTE: See the Discussion Section regarding Dopamine and CaM Kinase Released into the brain.) And, Kirvan, et al (2006) Antibody-mediated neuronal cell signaling in behavior and movement disorders. (NOTE: See page 3 figure a. – showing elevation of Lysoganglioside, Tubulin and CaM Kinase in PANDAS children at Acute Stages of Exacerbations.) Briefly, it explains that our children are making… an immune response to group A streptococci that causes brain cells to make too much dopamine. It begins in brain cells exposed to the anti-streptococcal immune response. The brain cells make too much CaM kinase and this leads to too much dopamine. Too much CaM kinase was originally discovered in children that have Sydenham Chorea, a movement disorder usually found in Rheumatic Fever that may disappear in 4 to 9 months. The PANDAS children are also making too much CaM kinase. Anti-brain antibodies induced by streptococci react with lysoganglioside and tubulin and cause the increase in CaMkinase and subsequently dopamine release. Thus these anti-brain antibodies may create movement and mood disorders.
• Dr. Dritan Agalliu‘s research has the goal to explain the mechanisms that break down the blood-brain barrier in PANDAS. This, in turn, will enable the development of therapies to prevent immune cell or antibody entry into the nervous system, thereby providing long-term treatment for PANDAS. This research is currently underway.
IMPROVEMENT AND RECOVERY
PANDAS and PANS children can recover! What is important to remember is every child is different and there are numerous factors that affect the outcome.
Most families have to stay on daily prophylactic antibiotics to beat back a resistant strain of strep and/or protect the child while they healed from the exacerbation from getting another strep infection.
Many have found our children have a profound autoimmune response to strep microbially – the children did not get a strep infection but they reacted with PANDAS symptom increase while a friend or family member had strep infection.
Many children have an immune system that is so heightened that they react to other bacterias or viruses. Families ma need IVIG or plasma-exchange, realizing the antibiotics were not keeping the autoimmune reaction at bay. We have followed a protocol similar to Perlmutter, et al’s (1999), IVIG and Plasma Exhange Study – where 30 children were administered these treatments and had very good outcomes.