The Elusive Ailment: Panda's Disease And Its Rarity

PANDAS, or Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections, is a rare disease. It was first described in 1998 by Susan Swedo and colleagues at the US National Institute of Mental Health. PANDAS is believed to be a non-suppurative complication of infection with Group A streptococcus (GAS). The existence of PANDAS as a clinical entity distinct from Sydenham chorea, childhood-onset obsessive-compulsive disorder (OCD), or tic disorder of childhood has been controversial since its initial description. The controversy has focused, at least in part, on the inclusion of tics as a presenting symptom and the relationship of the syndrome to GAS as an instigator event and a cause of exacerbations of neuropsychiatric symptoms.

The estimated annual incidence of PANDAS/PANS was 1/11,765 for children between 3 and 12 years, with some variation between different geographic areas. The majority of the cases were non-Hispanic Caucasian (85%), 48% were female, and the mean age was 7.1 (SD 3.1) years.

PANDAS is a rare disease

PANDAS, or Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections, is a rare disease. It is a hypothetical diagnosis for a subset of children who exhibit a rapid onset of obsessive-compulsive disorder (OCD) or tic disorders. PANDAS is believed to be caused by group A streptococcal (GAS) infections, specifically group A beta-hemolytic streptococcal (GABHS) infections. The proposed link between infection and these disorders is an autoimmune reaction to the infection that produces antibodies, interfering with basal ganglia function and causing symptom exacerbations.

PANDAS was first described in 1998 by Susan Swedo and colleagues at the US National Institute of Mental Health. They observed 50 cases of children with dramatic and sudden OCD exacerbations and tic disorders following infections. The PANDAS hypothesis suggests that OCD and tic disorders arise in a subset of children as a result of a post-streptococcal autoimmune process.

The rarity of PANDAS is evident in its estimated incidence. A study conducted across three academic medical centers found that the estimated annual incidence of PANDAS was approximately 1 in 11,765 children between the ages of 3 and 12. Another study in a large private pediatric practice in Rochester, NY, identified 12 children with PANDAS over a 3-year period, resulting in an estimated incidence of 1 per 1,000 children.

The controversy surrounding PANDAS has led to differing opinions on its rarity. While some sources claim that PANDAS is rare, others argue that it is not rare but rather rarely diagnosed. This discrepancy has resulted in varying estimates of its prevalence, with some sources suggesting it affects 1 in 200 children, while others estimate a lower incidence.

The diagnostic challenges associated with PANDAS contribute to its perceived rarity. There is no definitive diagnostic test for PANDAS, and the criteria are unevenly applied, leading to potential overdiagnosis or underdiagnosis. Additionally, the symptoms of PANDAS overlap with other psychiatric conditions, making differential diagnosis difficult.

Despite its rarity, PANDAS has significant implications for patients and their families. The sudden onset of OCD, tic disorders, and other neuropsychiatric symptoms can be debilitating and distressing. The lack of definitive diagnostic tools and effective treatments further complicates the management of PANDAS.

It is a paediatric disorder

PANDAS is an acronym for Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal Infections. It is a rare paediatric disorder that typically first appears in childhood, from age 3 to puberty. The disease is characterised by dramatic physical, neurological, and psychological symptoms that interfere with children's daily lives.

The disease is thought to be caused by an autoimmune attack of healthy brain tissue after a streptococcal infection, such as strep throat or scarlet fever. The streptococcus bacteria use a process called "molecular mimicry" to hide from the immune system by putting molecules on their cell walls that look identical to molecules found in the child's heart, joints, skin, and brain tissues. Eventually, the body recognises the molecules on the bacteria as foreign and produces antibodies to fight the infection. However, due to the molecular mimicry, the antibodies also attack the child's own tissues, leading to the onset of PANDAS symptoms.

The symptoms of PANDAS vary widely from child to child and can include a combination of psychological and neurological conditions. The most common symptoms reported are tics, obsessive-compulsive disorder-like behaviour, and anxiety. Other symptoms may include depression, changes in mood or personality, difficulty sleeping, disinterest in food, fidgeting, symptoms similar to attention-deficit/hyperactivity disorder (ADHD), changes in motor skills, difficulty concentrating or learning, poor school performance, reduced coordination, and sensitivity to light and sound.

The diagnosis of PANDAS is based on clinical features and diagnostic criteria, as there are no lab tests available for diagnosis. The diagnostic criteria include the presence of OCD, a tic disorder, or both; pediatric onset of symptoms (age 3 to puberty); episodic course of symptom severity; association with group A Beta-hemolytic streptococcal infection; and association with neurological abnormalities.

Treatment for PANDAS may include antibiotics to treat the underlying streptococcal infection, cognitive behavioural therapy (CBT) to manage mental and emotional difficulties, and habit reversal training for tics.

It is caused by streptococcal infections

PANDAS, or Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections, is a rare disease that affects children from the age of 3 to puberty. It is characterised by a range of psychological and neurological symptoms, including obsessive-compulsive disorder (OCD)-like behaviours, tics, anxiety, depression, and changes in mood and personality. These symptoms occur following a streptococcal infection, such as strep throat or scarlet fever, and can interfere with a child's daily life, including their schooling.

The streptococcal infection is caused by Streptococcus pyogenes, or group A Streptococcus, which can result in a sore throat or skin infection. The infection is typically transmitted when an infected person coughs or sneezes, and others breathe in the droplets or touch contaminated surfaces and then touch their face. While most people recover fully from the infection, some children develop PANDAS, experiencing sudden and severe physical and psychiatric symptoms a few weeks after the initial infection.

The exact cause of PANDAS is still being researched, but one theory suggests that it is due to an abnormal immune response to the streptococcal infection. The strep bacteria are adept at evading the immune system by disguising themselves with molecules that resemble normal molecules found in the body. However, the immune system eventually recognises the bacteria and starts producing antibodies. Unfortunately, the disguise continues to confuse the antibodies, causing them to attack the body's own tissues. This attack on healthy brain tissue results in the psychological and neurological symptoms associated with PANDAS.

The treatment for PANDAS aims to address both the physical and psychiatric symptoms. Antibiotics are used to treat the underlying strep infection, and cognitive behavioural therapy (CBT) is employed to manage the mental and emotional difficulties. In some cases, additional treatments such as intravenous immune globulin (IVIG) or plasmapheresis may be considered to reduce the abnormal immune response and alleviate symptoms.

It is difficult to diagnose

PANDAS, or Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections, is a rare disease that is difficult to diagnose. While the symptoms of PANDAS are usually dramatic and sudden, there are no lab tests that can diagnose the disease. Instead, healthcare providers must rely on diagnostic criteria, including the presence of OCD, a tic disorder, or both, and the onset of symptoms between the ages of 3 and puberty.

The difficulty in diagnosing PANDAS lies in the fact that there is no single test to confirm the disease. Instead, healthcare providers must consider a range of factors, including the patient's medical history, physical examination, and the presence of specific symptoms. Blood and urine tests may be done to rule out other ailments, and throat cultures or blood tests may be used to confirm a previous strep infection.

The diagnostic criteria for PANDAS include the presence of OCD, a tic disorder, or both, as well as pediatric onset of symptoms. The symptoms typically appear suddenly and dramatically, with children experiencing obsessive-compulsive behaviors, tics, or both. Other symptoms may include hyperactivity, mood changes, developmental regression, anxiety, and previous or current strep infection.

The challenge in diagnosing PANDAS is further compounded by the fact that it is a rare disease, and many clinicians may not be familiar with it. Additionally, the symptoms of PANDAS can vary widely from child to child, and there may be a lack of clarity regarding the temporal relationship between infection and symptom onset.

While there is no definitive test for PANDAS, healthcare providers must carefully evaluate the patient's medical history and physical examination, along with the presence of specific symptoms, to make a diagnosis. This process can be challenging and time-consuming, and it requires a thorough understanding of the disease and its potential manifestations.

It is treated with antibiotics

PANDAS, or Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections, is a rare disease that affects children, typically first appearing between the ages of 3 and puberty. It involves the onset of obsessive-compulsive disorder (OCD), tic disorders, or both, following a streptococcal (strep) infection, such as strep throat or scarlet fever. While the mechanism behind PANDAS is not yet fully understood, it is believed to be an autoimmune disorder triggered by the body's immune response to the strep infection.

Regarding treatment, the best approach for acute episodes of PANDAS is to address the underlying strep infection with antibiotics. Here is some detailed information about treating PANDAS with antibiotics:

Antibiotics for Acute Episodes

A throat culture is performed to confirm the presence of strep bacteria. If the culture is positive, a single course of antibiotics is typically prescribed to eliminate the infection and alleviate PANDAS symptoms. The duration of antibiotic treatment is usually a week or two, although some children exhibit improvement within a few days. In some cases, different antibiotics may need to be trialled to find the most effective option.

Addressing Occult Strep Infections

If the throat culture is negative, it is crucial to rule out occult (hidden) strep infections in other parts of the body, such as the sinuses, anus, vagina, or urethral opening of the penis. These infections are rarer but have been known to trigger PANDAS symptoms and can be challenging to eradicate, requiring a longer course of antibiotic treatment.

Preventing Reinfection

To prevent reinfection and potential relapses of PANDAS symptoms, it is essential to practise good hygiene. This includes frequent handwashing, replacing toothbrushes during and after antibiotic treatment, and staying away from sick individuals. Family members may also need to be tested for strep to ensure they are not carriers of the bacteria.

Antibiotics for Prophylaxis

While penicillin does not specifically target PANDAS symptoms, researchers are investigating its potential as prophylaxis to prevent future exacerbations. However, there is currently insufficient evidence to recommend the long-term use of antibiotics for this purpose.

Antibiotics vs. Immunoglobulin and Plasma Exchange

In severe cases of PANDAS, where other treatments have proven ineffective, more extreme procedures like immunoglobulin (IVIG) or plasma exchange (plasmapheresis) may be considered. These procedures aim to reset the immune system, either by infusing antibodies from other people (IVIG) or removing the harmful antibodies from the child's blood (plasmapheresis).

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