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PANDAS & PANS



PANDAS & PANS

Introduction

PANDAS ( Paediatric Autuimmune Neuropsychiatric Disorder associated with group Streptococcus) is a group of disorders seen in the paediatric population which consist of either tics or obsessive compulsive disorder either exacerbated or brought on by a common bacterial infection called Group A streptococcus (GAS). This infection may present with a sore throat or chest infection.

This group of disorders has been given a International classification of disease (ICD-11) code in the next set of codes to be released.

This group of disorders is thought to occur after the body produces an antibody to fight a common everyday infection.  This antibody then goes on to stimulate a part of the brain involved with movement control (or regulation of obsessive compulsive behaviours) which in turn results in the abnormal movement, noise, feeling or compulsion.

PANS (Paediatric acute-onset neuropsychiatric syndrome)  describes a clinical syndrome that may be caused by noninfectious or infectious triggers.  Proposed diagnostic criteria for PANS include: the sudden onset of OCD or severely restricted food intake,  severe neuropsychiatric symptoms (eg, anxiety, depression, emotional lability, etc) or symptoms not better explained by a known neurologic or medical disorder. The key words in this are “sudden onset”. Symptoms appear dramatically and there is a rapid shift in the child’s behaviour.

Background

Rheumatic fever has been a less commonly recognised condition over the past 30-40 years. Part of the diagnostic criteria for rheumatic fever included a condition called “Sydenham’s chorea” or ‘St. Vitus dance” . This was an abnormality of movement which was associated with streptococcal infection. Individuals diagnosed with rheumatic fever were given long term prophylactic penicillin to treat the condition and prevent disease relapse.

Dr Susan Swedo during her research into Sydenham’s chorea realised that there were a sub-group of patients with tics & obsessive-compulsive behaviour that had a sudden onset of symptoms. This was very different to the movements seen with rheumatic fever. Crucially the symptoms had a very rapid pace of onset. The video below shows how this condition has evolved.

Dr Swedo 2014

Signs & symptoms

PANDAS symptoms typically start abruptly, almost as if a switch has been thrown. Symptoms include tics, sleep disturbance, obsessive compulsive behaviour, deterioration in handwriting, eating disorders (including anorexia but the problems appear to be more centred on difficulty in coordinating swallowing), behavioural regression and urinary incontinence. By definition these occur following a streptococcal infection which then results in the stimulation of antibodies which stimulates the part of the brain involved in movement and behaviour regulation (the basal ganglia). Examination of the child reveals a child who is “trapped” or “psychologically burdened”. They may have reduced muscle power and may show abnormal movements.

Pediatric Acute-onset Neuropsychiatric Syndrome (PANS) is defined by the rapid onset of obsessive–compulsive disorder (OCD) or eating restrictions and comorbid symptoms from at least two of seven categories:

  1. Anxiety (particularly separation anxiety)
  2. Emotional lability or depression
  3. Irritability
  4. Aggression, and/or severely oppositional behaviors
  5. Deterioration in school performance related to ADHD-like behaviors, memory deficits, and cognitive changes
  6. Sensory or motor abnormalities
  7. Somatic signs and symptoms, including sleep disturbances, enuresis, or urinary frequency (Swedo et al. 2012; Chang et al. 2015). Acute onset cases that are triggered by Group A streptococcal infections may meet diagnostic criteria for both PANS and PANDAS.

Diagnosis & Investigations

Criteria for diagnosis of PANDAS include:

  1. Prescence of OCD and/or Tics
  2. Pre-pubertal onset
  3. Acute onset of symptoms with an episodic (saw-tooth) pattern
  4. Association with a neurological abnormality
  5. Temporal relationship with Group A strep infection

Examination of the child should include looking at the throat, ears, skin (eg for impetigo or guttate psoriasis),examining the peri-anal area and genitals for infection, checking for a heart murmur and assessing weight and height.

Disease monitoring

The free downloadable form that we have supplied on this page will allow parents to score their child’s symptoms on a daily basis. You can download this form by clicking on “Patient Information Leaflet” above or by clicking on the following link; PANDAS symptom monitoring chart. This scoring sheet also allows  parents to add detail such as whether or not a child is receiving antibiotics or anti-inflammatories. This information is invaluable in assessing the patients that we see using the Children’s e-Hospital on-line service but can also be used to help parents with any health professionals they see. If you need any support in using this tool please conact us at: admin@e-hospital.co.uk

Treatment of PANDAS

The latest UK PANS & PANDAS Physicians Network treatment guidelines can be downloaded here: 

PANS PANDAS protocol (Final V1.2)

GP’s may prefer to use the UK PANDAS & PANS Quick Reference Guide for GP’s:

e-Hospital PANDAS Protocol GP Quick Reference Guide

In addition, the Children’s e-Hospital has adapted these guidelines into a user friendly protocol for health professionals:

 e-Hospital PANDAS Protocol V1.3

Please email vicki@e-hospital.co.uk to obtain the password to access the documents (You will need to provide proof of your professional medical status)

Treatment can be divided into several stages which are described below.

  1. Induction of disease remission

    • Anti-inflammatory treatment
      • Non-steroidal anti-inflammatory drugs (eg ibuprofen)
      • Steroid pulse therapy (e.g. prednisolone)
    • Anti-microbial treatment
      • Azithromycin (Zithromax)
      • Penicillin V
      • Cephalexin
      • Amoxicillin (Augmentin)
    • Intravenous immunoglobulin
      • Intravenous immunoglobulin (IVIG) can be used to induce disease remission. Drs. Perlmutter and Swedo used IVIG in the 1999 study published in the Lancet where nearly all of the children benefitted from its use. The sampling was small (30 of children). In PANDAS an autoimmune irregularity is causing encephalitic-like inflammation and the use of IVIG interrupts this process. The PANDAS IVIG study (run by the PANDAS physician network) administered 1gram/kg of the child’s body weight per day on 2 consecutive days. Preliminary results were positive but not conclusive. Prophylactic antibiotics should be continued thoughout treatment if IVIG is given. The following link gives access to the NHS IVIG guidelines: NHS IV Immunoglobulin Guidelines
    • Plasmapharesis
      • In this process the harmful auto-antibodies are removed from the blood system itself. Compared to IVIG the success rate for this procedure is very good but it does need to be done in a specialist setting. This technique is therefore usually reserved for severely affected patients with symptoms that would be considered “life threatening”. What is not clear is if the child’s autoimmune system will recreate the negative antibodies. It has been reported that PEX has had to be repeated (as with IVIG) in a few cases.  Prophylactic antibiotics should be maintained throughout treatment. The following link gives access to the UK blood transfusion & tissues transplantation services professional advisory committee recommendations for therapeutic plasma exchange : Therapeutic Plasma Exchange Recommendations
  2. Cognitive behaviour therapy

    • The onset of PANDAS or PANS symptoms can be extremely distressing for the child and family members. It is therefore essential that psychological support with cognitive behaviour therapy (CBT) is initiated at an early stage. On-line CBT in children has been shown to be more effective than face to face therapy and also allows flexibility in seeking consultations. Using CBT gives parents the tools to manage their child during a crisis.
  3. Maintainance therapy

Once disease remission has been achieved the antibody level will gradually fall and symptoms will slowly improve unless the immune system is restimulated eg with another infection. In order to reduce the risk of further streptococcal infections, current recommendations include the use of preventative (prophylactic) antibiotics which are given long term to prevent further streptococcal infections.

Parent support groups

The Children’s e-Hospital recommends PANS PANDAS UK for parental support. This UK charity have worked tirelessly to raise the profile of these conditions and help direct parents to the right medical resources in addition to providing moral support. You can access their website by clicking on the following link: PANS PANDAS UK 

Facebook support

The Children’s e-Hospital  has a very active PANS/PANDAS Facebook page where you can find the latest updates on research & treatment: The Children’s e-Hospital PANDAS & PANS Facebook Group

Final words

This article has been written to try and help parents in the UK get the best treatment for their child. It must be remembered that this is an evolving disease in the paediatric population and therefore other conditions must be excluded before you embark on a pursuit of a diagnosis of PANDAS or PANS.

If you would like to discuss your child’s diagnosis further or wish to know more about PANDAS & PANS please contact the Children’s e-Hospital admin team at admin@e-hospital.co.uk