Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections (PANDAS)

What is Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections (PANDAS)?

Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections or PANDAS is a rare illness in children, that suddenly brings about mental health symptoms, obsessive behaviors, compulsions, and various muscular tics. These severe behavioral symptoms in children can be triggered by infections, especially Streptococcal (Strep) infections. Strep infections are throat infections caused by a certain type of bacteria. PANDAS was first identified in 1998 and is usually diagnosed in children or young teens who’ve recently had a strep infection.

Initially, PANDAS was put under a category of illnesses caused after getting some other infection. However, in 2010, it was classified under a new category called Pediatric Acute-onset Neuropsychiatric Syndromes (PANS). PANS include new, sudden start cases which show obsessive behaviors, compulsions, and different kinds of muscular tics. These are not only triggered by infections but could also be caused by other factors such as exposure to certain harmful environmental elements, emotional stress, or exposure to certain toxins. PANS may involve severe eating restrictions and a combination of other extreme mental health symptoms, including involuntary movements, sensory disturbances, and hallucinations.

Some physical symptoms of PANDAS can include tics, sensitivity to sensory inputs like sound or touch, deterioration of motor skills, sleep disorders, and joint pain. Mental health symptoms can include obsessive-compulsive behaviors, feelings of anxiety, emotional instability, developmental regression (a loss of previously established developmental skills), and feelings of depression.

Diagnosis of PANDAS is dependent on several factors such as the presence of obsessive-compulsive disorders or tic disorders, sudden onset of symptoms in childhood, symptoms that come and go, association with streptococcal infection, and neurological abnormalities. Diagnosing PANDAS involves several steps including psychological and neurological assessments, infectious disease testing, autoimmune and endocrine evaluations, imaging, and genetic tests.

Strep infection is confirmed using throat cultures and antibody tests, but these are not completely reliable markers for PANDAS. Brain scans in PANDAS patients often show abnormalities in the basal ganglia (areas deep within the brain which help control movements) and can help distinguish PANDAS from other conditions.

The primary treatment for this condition is the administration of antibiotics to deal with the Strep infections, β-lactams, and macrolides are commonly used. These antibiotics also have the additional properties of modulating the immune response, protecting nerve cells, and reducing inflammation. The use of antibiotics to prevent recurrent infections and potential vaccines against Strep infections are also being explored. Understanding how PANDAS occurs, how it affects the brain, diagnosing, and its treatment is vital for improving patient care and outcomes in this rare but complex condition.

What Causes Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections (PANDAS)?

PANS, which stands for Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections, often seems to be linked to infections caused by a type of bacteria known as Streptococcus. This is the same bacteria that causes Sydenham chorea, a movement disorder resulting from a complication of a strep throat infection. Because of this, some scientists think that PANS might just be a kind of milder Sydenham chorea. They suggest this because the symptoms of the two conditions can be similar and they seem to run in families.

However, some researchers pointed out that these two conditions tend to affect different age groups and that there are specific windows of time when kids are more likely to develop problems after a strep infection. Other infections may also trigger PANS. These can include the bacteria that cause pneumonia and Lyme disease, or viruses that cause herpes, mono, flu, coxsackie (hand, foot, and mouth disease), severe acute respiratory syndrome like COVID-19, and chickenpox.

Additionally, studies have found that children with PANS may show signs of difficulty with processing speed, visual-motor skills (coordinating eyes and hands), integrating visual and spatial information, and memory. These problems might be linked to disruptions in how different parts of the brain connect and work together.

Risk Factors and Frequency for Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections (PANDAS)

PANDAS, short for Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections, is a condition that we still don’t know a lot about in terms of how many people it affects. What is known is that it tends to affect boys between the ages of 3 and 12 more than girls, with the average age being around 6 years old. It’s also been found that over half of PANS (Pediatric Acute-onset Neuropsychiatric Syndrome) cases, a broader classification that includes PANDAS, occur in families where another family member has had the condition.

• PANDAS is more common in males, especially boys aged between 3 and 12.
• The average age for PANDAS is about 6 years.
• First-degree relatives (parents, siblings) are affected in over half of all PANS cases.
• Risks factors for developing PANDAS include frequently contracting Group A streptococcal infections and a family history of autoimmune diseases or rheumatic fever, most especially on the mother’s side.

Signs and Symptoms of Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections (PANDAS)

PANDAS syndrome is diagnosed based on symptoms and by ruling out other conditions. Many different medical conditions like autoimmune diseases, neurological disorders, and psychiatric disorders need to be considered and ruled out for a diagnosis of PANDAS syndrome.

In the process of diagnosing PANDAS, several evaluations are carried out. These can include a psychological and neurological assessment, checking for infections and auto-immune diseases, a sleep evaluation, and testing specific organs like the heart. Brain scans and genetic evaluations could also be conducted to support the diagnosis.

The usual time for symptoms of PANDAS to show up is four to six weeks after a strep infection. Symptoms typically get worse in two to three days. The typical signs used to diagnose PANDAS include:

• An obsessive-compulsive disorder or tic disorder.
• Start of symptoms between 3 and 12 years of age.
• Sudden onset of symptoms that get worse rapidly, but also have periods of improvement.
• A link between a strep infection and the start or worsening of symptoms.
• Neuropsychiatric symptoms such as hyperactivity, unusual movements, bedwetting, anxiety, emotional instability, skill regression or mood changes.

Physical symptoms of PANDAS may include tics, unusual movements, sensitivity to stimuli, motor skill problems, memory problems, sleep disorders, weight loss due to not eating, joint pain, frequent urination, bedwetting, and severe withdrawal from reality.

Psychological symptoms can include recurring unwanted thoughts (obsessions), compulsive behaviors, anxiety when away from parents or home, phobia, panic attacks, uncontrolled shouting, emotional and developmental regression, hallucinations, irritability, mood instability, depression, and thoughts of suicide.

Testing for Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections (PANDAS)

Diagnosing patients with PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections) can be quite challenging as specific clinical and immunological criteria are yet to be established. However, commonly used methods often involve laboratory studies to confirm a streptococcal infection and the use of neuroimaging studies.

Streptococcal infection is confirmed through various tests. These tests could involve throat cultures or rapid antigen tests. Sometimes, cultures might also be taken from other affected areas like the nasal cavity, skin, or vaginal areas. However, it’s important to realize that not all positive results for these tests are indicative of PANDAS. While the presence of certain antibodies might indicate an exposure to strep infection in the past, it won’t be able to determine a new infection or a long-standing one.

On the other hand, doctors regularly use neuroimaging for diagnosis. Neuroimaging is a technique that provides pictures of the brain in various ways, helping doctors understand what’s happening inside. Some studies propose a particular sequence of neuroimaging that can help with the diagnosis, especially when comparing with other disorders like obsessive-compulsive disorders or autoimmune encephalitis. In some cases, MRI scans of the brain in PANDAS patients showed an enlargement of specific brain structures (known as basal ganglia). Another method called ‘Positron Emission Tomography’ (PET) imaging and analysis of certain substances from affected individuals have shown inflammation in these same brain structures.

Treatment Options for Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections (PANDAS)

While there’s a lack of agreement on how to best treat PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections), some guidelines have been suggested.

Antibiotics, which are medicines used to treat infections, are often the first course of treatment for PANDAS. This is because these medicines can eliminate the streptococcal infection, which is often associated with this condition, and help manage symptoms. Some studies recommend starting antibiotic treatment in all PANDAS cases, even if no infection is observed.

Antibiotics might help to control the symptoms of PANDAS by acting against the streptococcal infection and also by influencing the immune system and protecting the brain. These medicines include cephalosporins and penicillins, which can protect brain cells, reduce harmful effects of certain brain chemicals, and control the immune response. Another type of antibiotics, known as macrolides, can control the immune response, particularly in chronic inflammatory conditions. They can also decrease inflammation and act as antioxidants, which help to get rid of harmful chemicals in the body.

Preventive treatment with antibiotics, known as antibiotic prophylaxis, is also suggested in some cases, especially those with severe or recurring symptoms. This is similar to the preventive treatment given in rheumatic fever, which is also caused by a streptococcal infection. One specific study showed that this preventive antibiotic treatment reduced neurological symptoms in PANDAS patients. However, the long-term benefits of using low-dose antibiotics to prevent PANDAS are not well known.

However, use of antibiotics must be done carefully, as there’s a risk that the streptococcal infection could become resistant to these medicines. This could make the infection harder to treat and potentially lead to serious complications if PANDAS isn’t treated effectively. Therefore, doctors should carefully monitor patients at high risk.

Lastly, vaccines for the streptococcal infection are also being studied as a way to prevent PANDAS. Vaccines stimulate the immune system to prevent future infections.

What else can Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections (PANDAS) be?

In the process of diagnosing PANDAS, a condition that affects a child’s behavior and mental health, doctors first need to rule out other conditions that may cause similar symptoms. PANDAS is considered a ‘diagnosis of exclusion’, meaning other potential causes must be considered and ruled out first.

Doctors will conduct a thorough evaluation to rule out a range of medical conditions. This often includes:

• Autoimmune and autoinflammatory diseases
• Immunodeficiency syndromes
• Neurological disorders
• Psychiatric disorders, such as obsessive-compulsive disorder and anxiety disorders
• Tic disorders, including Tourette syndrome
• central nervous system conditions, like vasculitis
• Encephalitis including Hashimoto’s disease, that comes with psychiatric features
• Schizophrenia
• Neuromyelitis optica spectrum disorder and other demyelinating diseases
• Infectious diseases and endocrine (hormonal) diseases

Furthermore, some conditions known for causing neuropsychiatric symptoms, like Wilson’s disease and Lyme neuroborreliosis, are occasionally considered if applicable.

What to expect with Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections (PANDAS)

Most kids completely recover from PANDAS, a condition that can cause sudden and dramatic changes in behavior. Research has shown that antibiotics can be helpful in treating this condition by reducing symptoms of Obsessive-Compulsive Disorder (OCD), a mental health disorder where people have recurring, unwanted thoughts, ideas or sensations that make them feel driven to do something repetitively. This is true even when there’s no clear sign of infection.

Additionally, some studies indicate that neurological symptoms, or problems with how the brain and nerves work, tend to improve within 3 to 5 months of antibiotic treatment. However, it’s important to note that antibiotics have not been found to significantly reduce tic symptoms (sudden, rapid, repetitive movements or sounds) and worsened neuropsychiatric symptoms (problems with mental functions that are often related to diseases of the nervous system).

Possible Complications When Diagnosed with Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections (PANDAS)

Investigating PANDAS thoroughly is very important. If PANDAS isn’t diagnosed or treated, it could increase the risk of ongoing mental health symptoms. These could involve obsessive-compulsive behaviors, tics that later result in issues with handwriting, problems with vision and movement, sleep disturbances, and anxiety disorders that carry on into adult life. In addition to this, some individuals might find their symptoms deteriorate after every streptococcal infection.

Common Symptoms:

• Obsessive-compulsive behaviors
• Tics leading to handwriting difficulties
• Visual and motor impairments
• Sleep disruptions
• Anxiety disorders persisting into adulthood
• Potential worsening of symptoms after each streptococcal infection

Preventing Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections (PANDAS)

Kids with certain conditions, such as PANDAS and PANS, can experience intense mental and behavioral issues. These can include thoughts of suicide, extreme aggression, unusual eating habits, and difficulty sleeping. These symptoms aren’t easy to manage, so it’s important that their families play an active role in their overall care process. Their involvement can help ensure that the child receives the most complete and effective treatment possible.