Our grateful thanks to the charity PANS PANDAS UK for the information below and for their contribution to our radio report (which you can hear again via our audio player at the bottom of this page – or you can hear an extended interview on the Word on Health Podcast – click here to access)
PANS is an acronym for Paediatric Acute-onset Neuropsychiatric Syndrome.
PANDAS is an acronym for Paediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal infections.
PANS is a neuropsychiatric condition - is triggered by a misdirected immune response to an unknown trigger causing inflammation in the brain, leading to both physical and psychiatric symptoms. Despite its name, PANS can affect any individual at any age.
There is currently no specific test which will prove or disprove the condition - and a diagnosis is to be made based on the analysis of the patient’s medical history, a review of their current symptoms and a physical examination. Lab work and additional testing can be ordered to identify an infectious trigger, rule out other diagnoses, and inform treatment plans.
PANDAS is a subset of PANS. Similarly, it is a neuropsychiatric condition which is triggered by a misdirected immune response to a Group A Streptococcal (GAS) infection which disrupts a child’s normal neurologic activity. GAS infections can occur in many parts of the body, not just the throat and can result in the inflammation of a child’s brain. Symptoms can appear whilst the infection is still present or several months later.
PANDAS usually starts with an acute onset of obsessive-compulsive disorder and/or tics, particularly multiple, complex or unusual tics. PANDAS symptoms can then appear to wax and wane with the child experiencing flares periodically, following illness or periods of stress.
The child is usually between the ages of 3 and puberty when first symptoms occur and usually appear following a streptococcal infection such as Sinusitis, Ear infections or Scarlet Fever. In some cases children can carry the streptococcus bacteria without showing signs of illness.
What causes PANS and PANDAS? It is a misdirected immune response to common infections, targeting the brain.
With PANDAS the infection is identified as group A Strep, whereas with PANS the initial trigger for the misdirected immune response may be ‘Walking pneumonia’ (Mycoplasma pneumoniae), the flu (Influenza H1N1, Glandular Fever (Epstein-Barr virus), Lyme disease (from an infected tick bite), Chickenpox, bacterial infection (such as Bartonella) or Toxin exposure (such as exposure to industrial pollutants).
This disrupts a patient’s normal neurologic functioning, resulting in a sudden onset of Obsessive-Compulsive Disorder, motor tics and/or eating restrictions.
PANS and PANDAS can include a variety of other symptoms such as (but not limited to):
Is PANS PANDAS the same as Autoimmune Encephalitis? Encephalopathy is a disease that affects the functioning of the brain. PANS and PANDAS are encephalopathies.
Autoimmune Encephalitis (AE) refers to a group of conditions that occur when the body’s immune system mistakenly attacks healthy brain cells, leading to inflammation of the brain.
More research is needed to determine whether PANS is a form of AE, but some children who are diagnosed with PANS are eventually diagnosed with AE Treatments for the two conditions have many similarities.
Why do some children get PANDAS after a Group A strep infection while others do not - Science has not yet answered this question. Possible hypotheses include the following:
Strain Differences - There are over 150 strains of Group A streptococci (GAS) and only 10–12 of these cause acute rheumatic fever and Sydenham chorea, the medical model of PANDAS. It is reasonable to hypothesize that only certain strains of GAS trigger symptoms of PANDAS. Geographic clusters of new cases of PANDAS have been reported, which would give credence to this possibility.
Genetic Vulnerability - Defects in clearing Group A strep, resolving inflammation after group A strep, differences in neuro-circuitry, cytokine receptors in the brain, or abnormal expression of neuro-signalling molecules/receptors during infection are all hypothesized to play a role in neuro-immune disorders including PANS and PANDAS. No genetic marker has been determined as of today.
Location of Strep Infection - Strep infections typically occur in the throat, tonsils, and anus, and each area should be examined and swabbed for culture. Although “strep throat” infections are the most common trigger, PANDAS has been reported to occur in association with perianal strep infections. Additional sites may be involved; for example, animal research suggests that strep in the nasal cavity may enter or influence neuro-immune cells along the olfactory nerve, providing access to the brain through the third ventricle.
How many children have been diagnosed in the UK so far- Currently, we simply do not have enough information to answer this question. The PANS PANDAS Steering Group is in the process of rolling out a UK-wide surveillance study in order to ascertain a clearer picture of the prevalence of these conditions within the UK.
Is there a blood test that tells you if you child has PANS/PANDAS - No, there is no single blood test for PANS or PANDAS. Common bloodwork used when working up a suspected case includes looking for possible infectious triggers, immune system function, and levels of key micronutrients.
An antibody to PANDAS has been identified; however a suitable test has not yet been produced for use by the medical profession.
So, how are PANS and PANDAS diagnosed - Both require a clinical diagnosis and as mentioned above, there are no specific tests which will prove or disprove the condition. The diagnostic criteria for PANS and PANDAS differ slightly and are outlined in detail below:
PANS Diagnostic Criteria - In order for a diagnosis to be made for PANS, presentation of the following symptoms must occur:
An abrupt, acute (abruptly and without warning), dramatic onset (usually within 24-48 hours) of obsessive compulsive disorder or; severely restricted food intake along with two or more of the following symptoms which are not better explained by a known neurologic or medical disorder, these include;
Whilst not part of the diagnostic criteria, in approximately 25% of cases there have been reports of Psychosis and/or Hallucinations. Parents frequently report dilated pupils when their child is in a PANS flare.
PANDAS Diagnostic Criteria - As identified earlier, PANDAS is a subset of PANS.
Usually starting with an acute onset (abruptly and without warning) of obsessive compulsive disorder and/or tics, particularly multiple, complex or unusual tics. PANDAS symptoms can then appear to wax and wane with the child experiencing flares periodically, following illness or periods of stress.
In addition to obsessive compulsive disorder and/or tics, children can also experience a range of symptoms similar to those listed under the PANS diagnostic criteria but they are not necessary for a diagnosis. Parents also frequently report dilated pupils when their child is in a PANDAS flare.
What is a clinical diagnosis - A clinical diagnosis is one made based on specific signs and symptoms observed by a medical provider rather than on lab tests or biomarkers. Both PANS and PANDAS are clinical diagnoses based on a set of specific criteria. They are also diagnoses of exclusion, meaning that a patient should be fully worked up for other known illnesses or diseases that could cause the observed symptoms. The workup should rule those other illnesses in or out.
What if my child didn’t have an ‘acute onset’ - Does that mean s/he doesn’t have PANS/PANDAS? No, your child might still have PANS. Given that many parents and providers don’t know what they are looking for (or looking at) when a child has a new onset, it is possible for an acute onset to be missed. Some children’s initial symptoms are mild, or they start when a child is very young, and the behaviour is labelled as “typical” or “developmentally appropriate”.
My child has never had a positive strep test - Does that mean s/he doesn’t have PANDAS? Not necessarily. Many children with PANDAS don’t present with classic strep signs – sore throat, fever, headache, and stomach-ache. Strep infection can also infect other areas of the body, such as the anus. If children aren’t swabbed thoroughly or in the right places, an infection might be missed.
In addition to the infection not always showing up in the way parents and providers expect, rapid strep tests miss 15%-20% of active infections and blood titers (ASO and Anti-DNASE B) tests have a false negative rate of nearly 40%
To find out more click here to link through to PANS PANDA UK - click here
https://www.panspandasuk.org
Listen to this weeks radio report
All material on this website is provided for your information only and may not be construed as medical advice or instruction. No action or inaction should be taken based solely on the contents of this information; instead, readers should consult appropriate health professionals on any matter relating to their health and well-being.
