I. Introduction
The sudden and dramatic change in a child’s behavior can be incredibly distressing for parents. When a child who was once happy and healthy begins exhibiting symptoms like obsessive-compulsive behaviors, tics, or extreme anxiety seemingly overnight, it can be a bewildering and frightening experience. These changes may indicate a neuroimmune disorder known as Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS) or the broader Pediatric Acute-onset Neuropsychiatric Syndrome (PANS). Affecting an estimated 1 in 200 children, these disorders require a nuanced understanding and a comprehensive approach to diagnosis and management. This blog post aims to be a thorough resource for parents and practitioners, offering insights into understanding, diagnosing, and effectively managing PANDAS and PANS.
II. Understanding PANDAS and PANS
What is PANDAS?
- PANDAS is an acronym for Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections. This condition is specifically linked to infections caused by Group A streptococcus bacteria. These infections can include strep throat and scarlet fever. It’s important to know that PANDAS symptoms can appear even if a child has not experienced a typical strep throat infection.
- The underlying mechanism of PANDAS is believed to be an autoimmune response. In this process, the body’s immune system mistakenly produces cross-reactive antibodies that target the brain, specifically the basal ganglia. This can lead to the sudden onset of neuropsychiatric symptoms.
What is PANS?
- PANS, or Pediatric Acute-onset Neuropsychiatric Syndrome, is a more inclusive term that encompasses a broader range of triggers for similar neuropsychiatric symptoms. While PANDAS is a subset of PANS, PANS includes other triggers such as Lyme disease, and can be associated with conditions such as OCD and ODD. PANS is diagnosed when the symptom onset is sudden, and no other medical explanation is found.
- The key differentiator between PANDAS and PANS is the association with streptococcal infection. PANS includes cases where the symptoms are triggered by various infectious or environmental factors, not just strep.
Key Differences
- The core distinction is that PANDAS is specifically linked to strep infections, whereas PANS is an umbrella term that includes PANDAS along with other triggers.
- PANDAS has more specific diagnostic criteria and relies on a history of strep infection. In contrast, PANS has broader criteria and can be triggered by various infections, toxins, or other environmental factors.
Historical Context
- The term PANDAS was first coined in 1998, when researchers observed a connection between strep infections and the sudden onset of OCD and tic disorders. The concept of PANS emerged later as clinicians recognized that similar symptoms could be triggered by other factors.
- It’s important to understand that the scientific community’s understanding of PANDAS and PANS is still evolving. Not all medical professionals fully acknowledge or understand these conditions, which can sometimes make it difficult for families to find a diagnosis and appropriate treatment.
III. Recognizing the Signs and Symptoms
Sudden and Dramatic Onset
- A hallmark of both PANDAS and PANS is the sudden and dramatic onset of symptoms. These symptoms tend to appear “overnight and out of the blue”, typically following an infection. Parents often report that their child’s behavior and personality seem to change suddenly.
Core Symptoms
- Obsessive-compulsive behaviors (OCD): This can manifest as intrusive thoughts, fears, and repetitive behaviors or rituals. Children may develop an intense need for order, fear of contamination, or other compulsions.
- Tics: These can be either motor or vocal. Motor tics include repetitive movements such as head jerking, eye blinking, or facial grimacing. Vocal tics include repetitive noises such as throat clearing, grunting, or sniffing.
Additional Symptoms
- Anxiety and separation anxiety can become pronounced, with children showing marked distress when separated from caregivers.
- Mood changes, including sudden irritability, emotional lability, and unexpected outbursts, are common.
- Sleep issues such as difficulty falling asleep, staying asleep, or night terrors, may emerge. Bedwetting may also occur.
- New and intense fears, which can include fears of everyday objects or situations, can be a sign of PANDAS/PANS.
- Depression, violent behavior, hyperactivity, psychotic symptoms, motor problems, cognitive decline, and impaired overall function are also associated with PANDAS/PANS.
Symptom Fluctuation
- Symptoms can vary significantly in severity and may relapse and remit. This means symptoms may worsen, then improve, only to worsen again. The duration and intensity of these episodes may increase with each recurrence.
Importance of Early Recognition
- It’s critical to recognize these symptoms early because untreated PANDAS/PANS can lead to permanent debilitation. Early intervention can significantly improve outcomes and prevent long-term complications.
IV. The Diagnostic Process
Clinical Diagnosis
- PANDAS and PANS are primarily clinical diagnoses, meaning they are based on a combination of signs, symptoms, a thorough medical history, and laboratory findings. The diagnosis is made when these symptoms can’t be explained by any other neurological or medical conditions.
Diagnostic Criteria
- PANDAS has five distinct criteria for diagnosis, which include the presence of OCD and/or tic disorders, prepubertal symptom onset, an episodic course, abrupt onset of symptoms, and association with a GABHS infection. PANS diagnostic criteria include abrupt, dramatic onset of OCD or severely restricted food intake, as well as at least two additional neuropsychiatric symptoms from a list of seven.
Medical Evaluation
- A thorough medical evaluation is essential to rule out other conditions that might be causing similar symptoms. This process often involves a detailed assessment of the child’s medical history, a physical exam, and various lab tests.
Lab Tests
- A series of blood tests is often ordered to identify any current or past infections, immune markers, or other relevant factors. These may include:
- Immunoglobulin Levels: IgE, IgA, IgM, and IgG (including subclasses)
- Complete Blood Count (CBC): To assess overall health and detect signs of infection or inflammation
- Autoimmune Markers: ANA (antinuclear antibodies) and other autoimmune markers
- Iron Levels: Ferritin and B-12 to assess for deficiencies
- Vitamin D Levels: As Vitamin D deficiency is common
- Strep Throat Culture: To identify an active strep infection.
- Strep Antibodies: ASO (Anti-streptolysin O), Anti-DNase B, and Streptozyme to measure the body’s response to previous strep infections.
- Mycoplasma Pneumoniae IgA & IgM: To check for mycoplasma infection.
- Lyme Disease and co-infections: To determine if Lyme or other tick-borne diseases are present.
- Pneumococcal Antibody Titers: To evaluate immune response to pneumococcal bacteria.
- Epstein Barr Virus Panel: To determine if there is an active or past Epstein Barr Virus infection.
- Coxsackie A & B Titers: To check for coxsackie viral infection.
- HHV-6 Titers: To determine if there is an active or past Human Herpesvirus 6 infection.
- Cunningham Panel: A specialized blood test developed to detect autoantibodies associated with PANDAS/PANS
- These tests can help reveal specific antibodies or other immune markers that suggest a diagnosis of PANS or PANDAS.
- A series of blood tests is often ordered to identify any current or past infections, immune markers, or other relevant factors. These may include:
Strep Titers
- It’s important to note that elevated strep titers alone do not indicate an active infection. They only indicate that the patient has been exposed to strep in the past. Antibiotics should not be administered solely based on elevated titers. Instead, antibiotics are only recommended if a rapid strep test or throat culture is positive for strep.
V. Treatment Strategies for PANDAS/PANS
Multifaceted Approach
- Effective management of PANDAS and PANS typically requires a multifaceted approach. A comprehensive treatment plan should address the underlying causes of the disorder as well as the specific symptoms a child is experiencing. Treatment plans need to be individualized, and often require a combination of different therapies.
Addressing Inflammatory Sources
Antibiotics:
- Antibiotics such as penicillin are a first-line treatment for PANDAS, as they are used to eradicate the strep infection. Children with PANDAS/PANS may require longer courses of antibiotics than children with simple strep throat infections.
- Other antibiotics such as azithromycin and Augmentin may also be prescribed.
- It is critical to understand that antibiotics should only be used when there is an active strep infection, as demonstrated by a positive strep test. They are not recommended for elevated titers alone.
Herbal Antimicrobials:
- Herbal antimicrobials can be considered as a safer alternative to antibiotics, particularly for long-term treatment and prevention of infections. These natural antimicrobials are thought to be as effective as antibiotics, without the harmful side effects on the microbiome.
- Herbs like cryptolepsis, artemisinin, oregano oil, and lomatium can be considered when working with a naturopathic doctor.
Regulating the Immune System
Immunomodulatory Therapies:
- Intravenous Immunoglobulin (IVIG) and Therapeutic Plasma Exchange (TPE) are often considered for more severe cases of PANDAS/PANS, particularly when there is evidence of inflammation, immune deficiency, or autoimmunity.
- IVIG is an invasive and expensive treatment that may not be covered by insurance. TPE may be considered for severe cases of PANDAS/PANS.
- Steroids are sometimes used to reduce brain inflammation, and have been shown to reduce symptom severity in patients with Sydenham’s chorea. However, steroids may worsen tics or aggression in some children, making it important to discuss the pros and cons of steroids with your provider.
NSAIDs:
- Nonsteroidal anti-inflammatory drugs (NSAIDs) may be used to reduce inflammation and shorten the duration of symptom flares. Studies suggest that early use of NSAIDs can help shorten flare duration.
Psychological and Emotional Support
Selective Serotonin Reuptake Inhibitors (SSRIs):
- SSRIs are often prescribed to manage OCD and anxiety symptoms. However, they do not address underlying causes of the disorder. Some doctors prescribe them only if medical treatments are not sufficient.
- There is a risk of paradoxical reactions to SSRIs, which can include an increase in rage, anxiety, or OCD. Careful monitoring and dosage adjustments are crucial.
Cognitive Behavioral Therapy (CBT):
- CBT, particularly Exposure and Response Prevention (ERP), is a form of talk therapy that helps children manage their obsessive and compulsive behaviors. It involves gradually exposing a child to anxiety-provoking objects or situations while teaching them how to reframe thoughts and responses.
- CBT is often used in combination with other treatments.
Integrative Approaches
- A “whole body” approach considers the interconnectedness of gut health, immune function, and brain health. Imbalances in the gut can contribute to immune dysregulation and psychiatric disorders. Changes in the gut microbiome can lead to inflammation in the brain.
- Dietary Changes
- An anti-inflammatory diet, rich in whole foods, is often recommended. Working with a healthcare professional can help determine which foods may be inflammatory for your child.
- Including probiotics and prebiotics in the diet is important to promote a healthy gut microbiome.
Supplements
* Probiotics support a healthy gut microbiome.
* Omega-3 fatty acids are essential for brain function and can have anti-inflammatory effects. They can be taken through diet or in supplement form.
* Other supplements, such as magnesium and vitamin D, can also be beneficial.Homeopathy
- Homeopathy is a complementary and alternative medicine (CAM) approach that some individuals find helpful. Homeopathy is based on the principle that “like cures like”.
- Practitioners of homeopathy believe that physical disease often has mental and emotional components. The approach involves highly individualized remedies, with remedies made from plants, animals, or minerals.
- It is important to note that there is a lack of scientific evidence to support the claims of homeopathy. It is essential to consult with your child’s pediatrician before using homeopathic remedies.
VI. Managing PANDAS/PANS: Tips and Resources
For Parents
- Early Treatment: Emphasize the importance of early treatment, as PANDAS/PANS can sometimes remit entirely if treated in a timely fashion.
- Trust Your Instincts: Parents often have the best sense that something is wrong. Seek a second opinion if your concerns are dismissed.
- Keep Detailed Records: Maintain a detailed record of symptoms, flare-ups, and any potential triggers.
- Manage Exposures and Illnesses: Any kind of infection, viral or bacterial, can exacerbate symptoms, so manage exposures and illnesses carefully.
- Build a Supportive Medical Team: Find a team that understands PANDAS/PANS and can provide comprehensive care.
- Know the Immune System: Understand your child’s immune system, and any family history of autoimmune illness.
- Connect with Support Groups: Connect with support groups like the PANDAS Network for community and resources.
- Advocate for Your Child: Stay informed and actively advocate for your child’s needs.
- Be Kind to Yourself: Parenting a child with PANDAS or PANS can be incredibly challenging. Know that you are doing your best.
- Be Patient: Healing is gradual over several months, and patience is important.
- Ongoing Psychological Support: Mild lingering anxiety or OCD may continue even after treatment, making ongoing psychological support helpful.
For Practitioners
- Consider PANDAS/PANS: Be aware of PANDAS and PANS in patients presenting with sudden-onset OCD, tics, and other neuropsychiatric symptoms.
- Identify and Treat Underlying Infections: Thoroughly investigate and treat any underlying infections.
Use a Holistic Approach: Implement a treatment approach that addresses both physical and mental health.
- Individualized Treatment Plans: Recognize that treatment plans should be highly individualized.
- Consult an Immunologist: Consider consulting with an immunologist, as about 20% of children with PANDAS are found to have immune deficiencies.
Additional Resources
- PANDAS Network:
- PANDAS Physicians Network (PPN):
- International OCD Foundation:
- National Institute of Mental Health (NIMH):
- National Center for Complementary and Integrative Health (NCCIH):
- MedlinePlus (National Library of Medicine):
- ClinicalTrials.gov:
VII. Ongoing Research and Future Directions
- Current Research Efforts: There is ongoing genetic research being conducted at PANS research centers worldwide. The development of national biorepositories and patient registries will help improve understanding of the disorder.
- Gaps in Understanding: The long-term prognosis for PANDAS/PANS is not yet known, and there is a need for more high quality research.
- Support for Research: Further research is needed to better understand the underlying mechanisms of PANDAS and PANS and to develop more effective treatments. Supporting these research efforts is critical to improving the lives of those affected by these disorders.
VIII. Conclusion
PANDAS and PANS are complex neuroimmune disorders that can significantly impact a child’s life. It is essential to recognize the sudden onset of symptoms, seek thorough medical evaluations, and implement individualized treatment plans that address underlying infections, inflammation, and psychiatric symptoms. If you suspect that your child may have PANDAS or PANS, do not hesitate to seek help from qualified healthcare professionals. Remember, you are not alone, and with proper care and support, children with PANDAS/PANS can lead fulfilling lives. With early intervention and a comprehensive approach, there is hope for recovery.
