PANS (Paediatric Acute onset Neuro-Psychiatric Syndrome) is a diagnosis associated with sudden onset OCD, tics or severe eating restrictions with at least two other associated cognitive, behavioural and neurological symptoms. PANDAS (Paediatric Auto-immune Neuro-psychiatric Disorder associated with Streptococcus) is a subgroup diagnosis of the above, but associated with a sudden onset of symptoms after an infection. The diagnosis is difficult to recognize in autistic children, as there is often an overlap of symptoms.
A little bit of history
The diagnosis of PANDAS was first described in the 1990’s by dr. Susan Swedo (MD), a researcher in the field of pediatrics and neuropsychiatry, and currently Chief of the Pediatrics & Developmental Neuroscience Branch at the US National Institute of Mental Health. Since then several further developments around the diagnosis have taken place, leading to the publication of a series of well-reviewed articles with an overview of treatment options in the Journal of Child and Adolescent Psychopharmacology in 2017.
Getting the diagnosis
Recognising the diagnosis is challenging and, in most cases, takes quite a while. According to the PANDAS Parent Survey in 2014 it took longer than 3 years in about 35% of children to get to the diagnosis and most children saw 3 or more doctors before they got the correct diagnosis. As there are no specific tests to help to make the diagnosis, a comprehensive medical and psychiatric history, with a physical examination, certain blood tests and some other special investigations, like an EEG and or an MRI all need to be taken into account. There are some other neurological diseases that can be confused with PANS/PANDAS. These have to be excluded before a diagnosis of PANS/PANDAS can be made. Response to therapy often is the final confirmation of diagnosis.
A high percentage of cases can never be associated with a specific trigger. If an infective trigger is suspected, an array of blood tests for different infective agents, such as Streptococcus, Herpes 1&2, Parvo virus, HHV6(Roseola), Influenza, Corona virus, Epstein Barr virus, Cytomegalo virus, Coxsackie virus, Rickettsia, is available, but the association often doesn’t present any further treatment options.
An auto-immune panel, known as the Cunningham panel, can be done as a blood test in Washington DC to test for 4 neuronal auto-antibodies, namely anti-dopamine receptors D1 and D2L, anti-lysoganglioside GM1 and AntiTubulin, as well as the activity of an associated enzyme, CaM Kinase II. There is an association with increased antibodies and elevated CaM kinase II in PANS patients.
So, what does PANS/PANDAS look like?
The history of a child being “normal” one day and breaking down into a bundle of OCD behaviours, irritability, hyperactivity, fear, anxiety, tantrums, rages, and eating restrictions the next day could be alarming. This, together with concentration problems, tics, frequent urination, acute sensory dysregulation, sleep disturbances and bedwetting are the scope of presentation of PANS/PANDAS. Other symptoms that we often come across are: loss of intellectual functioning with slower processing, memory problems, motor skills regression with clumsiness and issues with balance, deterioration in handwriting, tremors, emotional regression with mood swings, baby talking, excitement of the adrenal system with excessive perspiration, pupil dilation, flushing.
The important factor is that there is a change in presentation. Strictly the diagnostic criteria refer to a “sudden onset” of symptoms, but in some cases it could be less dramatic. The change can be after some emotional event, as the birth of a sibling, parents being absent for a while, or changes in the environment. For PANDAS there can be a history of an infection: a sore throat, upper respiratory tract infection, ear infection, impetigo or a red ring around the anus (peri-anal strep). I have seen children regress into PANS after a Ricketsial infection (Tick bite fever).
How can we treat it?
As the diagnosis of PANS and PANDAS is still in its baby shoes, treatment options will probably evolve as new research evidence is presented. The basis of treatment revolves around 3 pillars:
- The treatment of symptoms by behavioural interventions, as well as psychiatric (or alternative) medications.
- The treatment of the disturbances in the immune system, by treating the neuro-inflammation and/or underlying auto-inflammation.
- The treatment of the etiological aspect, which in PANDAS would be the relationship with the streptococcal infection and subsequent treatment with antibiotics or alternatives.
PANS/PANDAS is known as a relapsing disease, so treatment can be stopped when symptoms remit and restarted when necessary. The presentation as “flares” precipitated by different and new triggers needs to be addressed appropriately.
Working with an integrative doctor who understands and has experience in treating PANS/PANDAS is very important. There is no “blanket-type” PANS protocol. One can adhere to the principles of treatment mentioned above, but each patient responds differently. Just so, not one single practitioner has all the answers. We have to embrace all treatment modalities to optimise the outcome.