Paediatric Acute Onset Neuropsychiatric Syndrome (PANS) and it’s better-known sub-condition Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS) are believed to be the result of a misdirected immune response to a pathogenic trigger, leading to the abrupt onset of a range of challenging symptoms, including OCD, tics and restricted food intake, alongside cognitive, behavioural, and neurological symptoms.
The conditions primarily impact Children and Young People, however adults can develop PANS, and if untreated or not fully responsive to treatment, the conditions can also be taken into adulthood.
What are the Symptoms?
PANS Diagnostic Criteria:
- Abrupt, dramatic onset (culmination within 72 h) 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 behaviours,
- Behavioural (developmental) regression,
- Deterioration in school performance,
- Sensory or motor abnormalities and
- Somatic signs and symptoms, including sleep disturbances, enuresis or increased urinary frequency.
- Symptoms are not better explained by a known medical disorder, such as Sydenham’s chorea, systemic lupus erythematosus, Tourette disorder or others.
PANDAS Diagnostic Criteria:
- Presence of Obsessive Compulsive Disorder and/or a tic disorder: The patient must meet lifetime diagnostic criteria (DSM-III-R or DSM-IV) for Obsessive Compulsive Disorder or a tic disorder.
- Paediatric onset: Symptoms of the disorder first become evident between 3 years of age and the beginning of puberty
- Episodic course of symptom severity: Clinical course is characterized by the abrupt onset of symptoms or by dramatic symptom exacerbations. Symptoms usually decrease significantly between episodes and occasionally resolve completely between exacerbations.
- Association with group A Beta-haemolytic streptococcus infection: Symptom exacerbations must be temporally related to group A Beta-haemolytic streptococcus infection, that is associated with positive throat culture and/or significantly elevated anti- group A Beta-haemolytic streptococcus antibody titres
- Association with neurological abnormalities: During symptom exacerbations, patients will have abnormal results on neurological examination. Motoric hyperactivity and adventitious movements (including choreiform movements or tics) are particularly common.
Pfeiffer HCV, Wickstrom R, Skov L, et al, Clinical guidance for diagnosis and management of suspected Pediatric Acute-onset Neuropsychiatric Syndrome in the Nordic countries. Acta Paediatr. 2021; 110: 3153–3160.
The Impact
While it is important to share the diagnostic criteria, as these are what medical professionals refer to, they really do not convey the devastating impact of the conditions on those affected, and their families.
The GIRFEC Toolkit shares more details about the realities of living with PANS, and while it can be difficult to read, please remember it is even more difficult to experience.