PANDAS/PANS Awareness Day 2018: What are PANDAS and PANS?

PANDAS_PANS infographic

Every year on 9 October, patients, families and healthcare professionals come together to raise awareness and spread understanding of paediatric autoimmune neuropsychiatric disorder associated with Streptococcus (PANDAS) and paediatric acute-onset neuropsychiatric syndrome (PANS).

What are PANDAS and PANS?

PANDAS and PANS are autoimmune disorders, meaning that they are caused by the immune system attacking a person’s own tissues. In the case of PANDAS/PANS, the immune system attacks structures in the brain called the basal ganglia, which are found at the base of the forebrain [1]. These structures are thought to be involved in movement and behaviour; therefore, symptoms of PANDAS/PANS include motor tics, obsessive compulsive disorder (OCD), deterioration of handwriting, mood swings, sleep disturbances, restricted eating, irritability and anxiety.

While the exact causes of many autoimmune disorders remain unclear, PANDAS and PANS are thought to be induced by infections. First discovered in the early 1990s, PANDAS was seen to develop in children specifically after exposure to the bacteria Streptococcus pyogenes [2]. Since then, it has been noted that children who have been exposed to other infectious agents can also develop the syndrome, so in 2012, the diagnosis ‘PANS’ was introduced [3]. PANS refers to any syndrome where children have a dramatic onset – sometimes overnight – of neuropsychiatric symptoms, where all other psychiatric diagnoses have been ruled out, and regardless of the causative factor. PANDAS, therefore, comes under the bracket of PANS, but remains a term that is frequently used in the healthcare field.

So, why do these infections cause the body to attack its own brain tissue?

It is believed that in patients with PANDAS/PANS, receptors on their brain cells that have a similar structure to those on an infectious agent. This means that when an immune response is launched to fight the infection, the patient’s brain cells are mistaken for infectious material and are also attacked. This is known as molecular mimicry.

Diagnosis and treatments

Symptoms frequently go unrecognised in children with PANDAS/PANS, often being attributed to personality development, rebellious phases or exaggeration of emotions. Given that the symptoms of PANDAS/PANS mimic psychiatric disorders, children with these syndromes are also often misdiagnosed with mental illness. Many are prescribed psychotropic drugs to manage their symptoms, but these medications do not address the underlying autoimmune condition.

To treat patients with PANDAS/PANS effectively, the latest published standards of care recommend that patients be prescribed antibiotics, non-steroidal anti-inflammatory drugs, corticosteroids, intravenous immunoglobulin or therapeutic plasma exchange, depending on the severity of symptoms [4–6]. Cognitive behavioural therapy is also recommended, alongside medical treatment, to help minimise and control symptoms while the underlying infectious and inflammatory causes are targeted.

Prevalence and outlook

It is thought that PANDAS and PANS are not necessarily rare diseases but, rather, underdiagnosed diseases. The PANDAS Network estimates that they affect as many as 1 in 200 children in the USA [7], and some researchers suggest that they may account for ≥10% of childhood-onset OCD and tic disorders [8]. This is why it is so vital that physicians understand these conditions, and that awareness of them is raised worldwide.

References

  1. Dale RC and Brilot F. Autoimmune basal ganglia disorders. J Child Neurol 2012; 27 (11): 1470–1481.
  2. Swedo SE, Leonard HL and Kiessling LS. Speculations on antineuronal antibody-mediated neuropsychiatric disorders of childhood. Pediatrics 1994; 93 (2): 323–326.
  3. Swedo SE, Leckman JF and Rose NR. From research subgroup to clinical syndrome: Modifying the PANDAS criteria to describe PANS (Pediatric Acute-onset Neuropsychiatric Syndrome). Pediatr Therapeut 2012; 2: 113.
  4. Thienemann M, Murphy T, Leckman J et al. Clinical management of pediatric acute‑onset neuropsychiatric syndrome: Part I – Psychiatric and behavioral interventions. J Child Adolesc Psychopharmacol 2017; 27 (7): 566–573.
  5. Frankovich J, Swedo S, Murphy T et al. Clinical management of pediatric acute-onset neuropsychiatric syndrome: Part II – Use of immunomodulatory therapies. J Child Adolesc Psychopharmacol 2017; 27 (7): 574–593.
  6. Cooperstock MS, Swedo SE, Pasternack MS et al. Clinical management of pediatric acute-onset neuropsychiatric syndrome: Part III – Treatment and prevention of infections. J Child Adolesc Psychopharmacol 2017; 27 (7): 594–606.
  7. PANDAS Network. Statistics. Available at: https://www.pandasnetwork.org/understanding-pandaspans/statistics/. Accessed October 2018.
  8. UpToDate®. PANDAS: Pediatric autoimmune neuropsychiatric disorder associated with group A streptococci. Available at: https://www.uptodate.com/contents/pandas-pediatric-autoimmune-neuropsychiatric-disorder-associated-with-group-a-streptococci#H28. Accessed October 2018.