Paediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS) is a rare disorder that has caused significant debate within the medical community for many years.
It is characterised by the sudden onset of obsessive-compulsive disorder (OCD) or tics in children following infection with Group A Streptococcus (GAS), a bacterium that causes infections like strep throat and scarlet fever.
It is essential to understand the causes, symptoms, diagnosis, and available treatments to aid affected children and their families. In this blog, we will delve into these aspects of PANDAS syndrome to help raise awareness.
Causes of PANDAS Syndrome
While the exact cause of PANDAS syndrome is not yet completely understood, it is believed to be triggered by a faulty autoimmune response. In PANDAS syndrome, it is hypothesized that the body’s immune system, in an attempt to fight off GAS infections, mistakenly attacks healthy tissues in the child’s brain – specifically the basal ganglia, a region responsible for body movement and behaviour. This misguided attack results in inflammation, ultimately leading to the behavioural symptoms characteristic of PANDAS syndrome.
The onset of PANDAS Syndrome is contingent on the child’s age. GAS infections are prevalent; however, PANDAS generally manifests between the ages of 3 and puberty. During these developmental years, the immune system is still maturing, making it more susceptible to errors such as antibody cross-reactivity that triggers PANDAS Syndrome.
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Symptoms of PANDAS Syndrome
PANDAS syndrome symptoms generally appear suddenly, often following a strep throat infection. The most common symptoms include:
1) Obsessive-compulsive disorder: One of the key symptoms of PANDAS is an abrupt onset of obsessive-compulsive disorder (OCD). The child may suddenly fear germs, dirt, or illness and may resort to repeated behaviours such as hand-washing or arranging items in a specific order.
2) PANDAS is also characterised by tics – uncontrolled movements or sounds such as eye blinking, throat clearing, facial grimacing, sniffing, and grunting. These tics can be simple or complex and may change over time.
3) Separation anxiety: The child with PANDAS may display signs of separation anxiety, unwillingness to leave home or stay alone, and may suffer from frequent bouts of irrational fear. Mood changes like irritability, mood swings, depression, and episodes of uncontrollable crying or laughing can also appear, generally in an unexpected manner
4) Changes in motor skills often referred to as motoric clumsiness or deterioration in handwriting skills, and hyperactivity disorders are other notable symptoms in PANDAS. The child might be restless, have difficulty concentrating, and can show signs of “ants in the pants” behaviour
5) Changes in eating habits particularly having restrictions with food and drink ingestion due to fear of choking, throwing up, or difficulties in swallowing is a sign of PANDAS syndrome. Sleep disturbances featuring nightmares, sleep disruptions, and bedwetting are some other symptoms observed in PANDAS-afflicted children
The above-mentioned symptoms may also signify other medical or psychiatric circumstances. A sudden onset of such symptoms should prompt immediate support from healthcare professionals. With the right diagnosis and treatment, most children with PANDAS recover completely and return to their normal life.
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Diagnosis of PANDAS Syndrome
Diagnosing PANDAS syndrome is complicated due to its clinical similarity to other conditions like Tourette Syndrome and classic OCD. Diagnosis is largely based on clinical criteria – typically a sudden onset of OCD or tics following a GAS infection.
Hence, a thorough medical history, examining the correlation between strep infections and symptom onset, is vital. Diagnostic tests, such as throat cultures or blood tests for streptococcal antibodies, may also be used to detect prior exposure to GAS infection.
To identify the preceding GAS infection, healthcare providers may utilise throat cultures or blood tests that measure anti-streptococcal antibodies. However, these tests exhibit limitations as they detect GAS exposure but do not conclusively establish whether the neurologic symptoms are due to PANDAS or a coincidental GAS infection.
Given the complexity of PANDAS, the diagnostic process necessitates a multi-disciplinary approach involving professionals in paediatrics, psychiatry, neurology, and infectious diseases.
The diagnostics criteria include:
1) Onset of OCD: Sudden appearance or worsening of OCD and/or tic symptoms following a streptococcal infection
2) Age range: Check for age group between 3 and puberty
3) Neurological symptoms: Presence of motor abnormalities and behavioural issues
4) Laboratory tests: To check for streptococcal infection
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Treatment of PANDAS Syndrome
Treatment strategies for PANDAS revolve around three primary components: antibiotic therapy, immunotherapy, and symptomatic treatment.
1) Antibiotic therapy is prescribed for the course of streptococcal infection. For recurrent infections, long-term prophylactic antibiotic treatment is an option.
2) Immunomodulatory therapies are emerging as a beneficial treatment for children suffering from PANDAS. The approaches include Intravenous Immunoglobulin (IVIG) therapy and Therapeutic Plasma Exchange. IVIG introduces healthy antibodies into the child’s system, helping suppress the faulty autoimmune response causing PANDAS symptoms. Multiple studies suggest IVIG is a safe and effective treatment for PANDAS when administered properly. However, it often requires repeated treatments.
For severe cases unresponsive to antibiotics or IVIG, therapeutic plasma exchange offers promising results. This process involves removing and replacing the child’s plasma to eliminate the harmful antibodies causing the PANDAS symptoms. This technique appears to have the most substantial clinical response, albeit being the most invasive.
However, both IVIG and plasma exchange are not universally accepted treatments for PANDAS as more research is needed to determine its efficacy and safety. Nevertheless, studies demonstrate their potential as effective treatments and have shown promising results.
3) For symptomatic treatment, cognitive-behavioural therapy (CBT) offers significant symptom relief. CBT is designed to help children understand and manage their OCD or anxiety symptoms associated with PANDAS. In combination with medication such as selective serotonin reuptake inhibitors (SSRIs), the results can be significantly improved.
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The treatment for PANDAS requires a multidisciplinary effort incorporating medical, behavioural, and psychosocial interventions. While a broad range of treatment options are available, the specific treatment choice should be based on symptom severity, the patient’s response to therapy, and the preferences of the child and their family.
Although PANDAS syndrome treatment outcomes can vary, most children experience significant improvement in symptoms after addressing the strep infections and behavioural therapy.
Conclusion
PANDAS syndrome is a complex condition with a profound impact on both children and their families. Swift diagnosis and comprehensive treatment are key to mitigating the often-dramatic symptoms accompanying this disorder, enabling children affected by this syndrome to lead healthier, happier lives.
Further, more extensive research is required in unravelling the enigma that is PANDAS syndrome. Proactive, informed patient advocacy is also crucial for pushing this much-needed research forward.
