PANDAS and PANS Sensory Integration and Processing Difficulties
Sensory Systems: Vestibular processing deficits, often low PRN Poor postural control especially antigravity extension Can slouch, slump – extension against gravity is tricky and tiring Likes to move and not stop/fidgeting Can have low levels of alertness when not moving Scared of the dark without visual input to support spatial understanding Altered spatial awareness Poor grading of force May appear ‘low toned’ – but normal Beighton Scale Poor self-awareness – spatial; position in space and body awareness ARFID and picky eating | often poor tactile registration and poor modulation Super sensitive to some tastes Altered temperature perception Delayed cues re ill, nauseous, hungry, full or needing toilet Hyper-responsivity to some textures and light touch eg certain fabrics/textures May dislike light touch; skin, hair, tooth and nail care can be tricky Dislike being touched or held when not on own terms Slow or under-responsivity to pain, Hyper-responsivity in far senses; smell, vision and hearing
The dyspraxic patterns seen can include; Often bumping into things and people Difficulty playing with manipulating tools and toys Difficulty learning new/novel movement/motor skills Fine motor co-ordination difficulties e.g., handwriting, bilateral co-ordination, poor tool use Speech praxis difficulties include stutter, slurred words, poor pronunciation and timing Ideation, planning and execution can all be affected.
Emotion Regulation Rage Anger Irritability Poor frustration tolerance Difficulties with co and self-regulation Poor self-awareness – emotional lability is common Tearful one moment, raging the next 0-100 in 3 seconds
Executive Function Poor processing speed Multi-tasking is hard Poor timing and sequencing Poor concentration and focus Slow to perform tasks ? observed difficulties with language processing ? observed difficulties with more complex and abstract problem solving that is age-appropriate
Fatigues easily and needs lots of reset time May go ‘off legs’ Looks like have regressed
May need much parental encouragement and support Lose resilience to trying new things Low self-esteem
Older children Self-loathing and disgust at self Extreme fear and losing control of agency over the world Awareness of personality change and burden on parents and siblings
These guidelines were first published in May 2018 following several meetings between the PANS Physicians’ Network UK (PPNUK) and the Charity PANS PANDAS UK. Based on the US treatment guidelines originally written by the US PANDAS Physicians’ Network, these guidelines have been modified to adapt to UK medical practice, GP’s are strongly encouraged to start treatment and investigations early as early treatment is likely to improve the long term outcome of these patients.
“There is gradually accumulating evidence that there are some children who experience sudden onset of a neuropsychiatric disorder (usually obsessive-compulsive disorder (OCD) or tics) following a Group A beta-haemolytic streptococcal infection (GABHS). The acronym PANDAS was first cited in 1998 to describe this group of patients.However, neurological sequelae of streptococcal infection have been well recognised (eg, Sydenham’s chorea described by William Osler in 1894).
Doubt remains about the aetiology of the condition and whether it can be considered an independent disease entity.
More recently the term PANS (paediatric acute-onset neuropsychiatric syndrome) has been suggested, as it captures both the sudden onset and uncertainty about the aetiology.”
PANS (Pediatric Acute-onset Neuropsychiatric Syndrome) is when an infectious trigger, environmental factors, and other possible triggers create 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, severe restrictive eating, anxiety, tics, personality changes, decline in math and handwriting abilities, sensory sensitivities, and more.
PANS was introduced in 2012 by Dr. Susan Swedo in the paper From Research Subgroup to Clinical Syndrome: Modifying the PANDAS Criteria to Describe PANS (Pediatric Acute-onset Neuropsychiatric Syndrome).
The PANS Criteria
PANS is a clinical diagnosis. The following is the “working criteria” as listed Dr. Swedo’s paper on PANS:
Abrupt, dramatic onset of obsessive-compulsive disorder or severely restricted food intake.
Concurrent presence of additional neuropsychiatric symptoms, with similarly severe and acute onset, from at least two of the following seven categories: Anxiety Emotional lability and/or depression, Irritability, aggression and/or severely oppositional behaviors, Behavioral (developmental) regression, Deterioration in school performance, Sensory or motor abnormalities, Somatic signs and symptoms, including sleep disturbances, enuresis or urinary frequency
Symptoms are not better explained by a known neurologic or medical disorder, such as Sydenham’s chorea, systemic lupus erythematosus, Tourette disorder or others.
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.
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.
A clinical diagnosis of PANDAS is defined by the following criteria:
Presence of significant obsessions, compulsions, and/or tics
Abrupt onset of symptoms or a relapsing-remitting course of symptom severity
Interestingly no one asked for evidence of Sydenham’s Chorea, which has been well documented for much longer. Perhaps because it has a very physical presence that is clinically easier to diagnose, especially as the condition progresses to full-blown ataxic movement patterns, as well as the neuropsychiatric symptoms.
In the 1930s, if a doctor saw a patient with chorea, especially if the patient were a child or young woman, it was a reasonable assumption that the diagnosis was Sydenham’s chorea. In western societies today, such a presentation is unlikely to be Sydenham’s chorea and considerable thought must be given to the differential diagnosis. The time course of the chorea is useful diagnostically: most previously healthy children with an acute or subacute chorea have an autoimmune aetiology. Additional causes of childhood choreas include:
Other autoimmune causes, such as seen in systemic lupus erythematosus.
Athetoid cerebral palsy.
Drug-induced causes – metoclopramide, phenothiazines and haloperidol are the most important.
Primary and metastatic brain tumours affecting the basal ganglia.
Metabolic – bilirubin encephalopathy and toxins, especially carbon monoxide, manganese and organophosphate poisoning.
*Genetic Causes can include:
Benign hereditary chorea starts in childhood and is a non-progressive chorea. Inheritance is usually autosomal dominant, although rare cases of autosomal-recessive and X-linked inheritance have been reported.
Wilson’s disease is an autosomal-recessive disorder of copper metabolism.
Ataxia telangiectasia and other related conditions.
Huntington’s disease presents most often between the ages of 35 years and 45 years but it can be younger, especially if inherited from the paternal line. There is usually but not invariably, a family history. A juvenile form exists that should be seen as a variation of the normal form and not a distinct entity.
Therapists practicing Ayres’ Sensory Integration should be aware of PANDAS and PANS as part of differential diagnosis. Like Sydenham’s Chorea, the incidence of both these clinical presentations may be becoming increasingly more common as antibiotics are used more judiciously.
It is a possible differential diagnosis for a range of childhood neurodevelopmental and mental health difficulties including autism and rage/anger, and occupational therapists practicing sensory integration are likely to receive these referrals due to the sensory symptoms that may be a prominent marker of PANDAS, PANS and early-stage Sydenham’s Chorea, before frank ataxic movement patterns are observed .
A key symptom of this disorder is extreme sensory reactivity alongside development of tics, changes to participation in activity, altered movement patterns and fluidity of movement, joint pain and changes to mood, focus, attention and difficulties with emotion regulation. Intense extreme emotions and eating difficulties are also reported in the literature.
Symptom presentation and severity can vary from child to child. It can also vary in each exacerbation. Below is a list of possible symptoms a child may exhibit. Not all need to be present. Not all possible symptoms are listed.
OBSESSIVE COMPULSIVE DISORDER (OCD)
OCD can manifest in different ways in young children. Learn more here.
This includes selective eating and food refusal.There can a variety of reasons why the child experiences this, including contamination fears, sensory sensitivities, trouble swallowing, fear of vomiting or weight gain, and more. If restrictive eating is resulting in severe weight loss, call your provider immediately.
Tics are repetitive movements or sounds that can be difficult for a child to control. Motor tics can include eye-blinking, head-jerking, shoulder shrugging, nose-twitching, and facial grimacing. Some motor tics are a series of movements, performed in the same order. Vocal tics can include grunting, humming, throat clearing, coughing, repeating words or phrases. Some children are able to suppress tics temporarily, but doing so can cause extreme discomfort. Relief comes through performing the tic.
Emotional lability includes not being able to control one’s emotional response such as uncontrollable crying or laughing. This is a neurological symptom.
IRRITABILITY AND AGGRESSION
This includes baby talk.
DETERIORATION IN SCHOOL PERFORMANCE
This includes deterioration in math skills, inability to concentrate, difficulty retaining information, and school refusal. School performance can also be a result of another contributing symptom, such as OCD or severe separation anxiety.
CHANGES IN HANDWRITING
This includes margin drifts and legibility.
This can include being sensitive to touch, sounds, and noise. Simple touches may feel like they are hurting. For example: being unable to tolerate the way socks feel or the texture or temperature of certain foods. Sensory processing problems can also cause difficulty in finding an item when it is among a vast selection of items. For example, a child may have a hard time finding a shirt in a full dresser or finding words in a word search.
This includes sleeping difficulties, enuresis, frequent urination, and bed wetting.
Here is an example of Choreiform movements. The child is attempting to hold his hands straight out and is trying not to move his fingers.
SEVERE SEPARATION ANXIETY
Separation anxiety in an older child will present differently. For example, a child may be unwilling to leave the house or their bedroom.
This includes both visual and auditory hallucinations.
FIGHT OR FLIGHT RESPONSE
RHEUMATIC PAIN OF JOINTS
Is often described.
This includes daytime wetting accidents and/or frequent urination.
Parent Facebook group: This is a group run by parents only for parents of children with the illness PANDAS or PANS. Any medical advice should come from your child’s doctors.
Like Sydenham’s Chorea, the onset of PANDAS and PANS follows bacterial infection. However, without the clear cerebellar signs and choreic movements of Sydenham’s Chorea, PANDAS and PAN’s can be easily attributed to childhood developmental disorders, typical developmental changes and everyday environmental stressors.