Brief Summary
Dr. Anu French discusses Pediatric Acute-onset Neuropsychiatric Syndrome (PANS) and Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS). She emphasizes the importance of early recognition and treatment to improve outcomes for affected children. She reviews diagnostic criteria, treatment guidelines, and integrative approaches to care, highlighting the need for a multidisciplinary approach involving psychiatric, immunomodulatory, and antibiotic therapies.
- PANS/PANDAS is a disease that exists and early recognition and treatment are crucial.
- Diagnosis is clinical, based on specific criteria, as there is no definitive diagnostic test.
- Treatment involves a three-pronged approach: psychotherapeutic treatment, immunomodulatory therapy, and antibiotics.
Introduction
Dr. Anu French, a pediatrician with 29 years of experience and director of the SSM Health Cardinal Glennon Primary Care office, was introduced. She is double board certified in integrative medicine and pediatrics and serves as a course director for integrative pediatrics at UC Irvine. Dr. French expresses gratitude for the opportunity to share her knowledge on PANS/PANDAS, emphasizing the importance of staying updated with current research and evidence-based guidelines.
Learning Objectives and Resources
The primary learning objective is to increase awareness of PANS/PANDAS as a real disease. Early recognition and intervention can significantly improve outcomes for affected children. Dr. French reviews Consortium guidelines and algorithms through an integrative lens. She recommends the website put together by Dr. SU, which is driven by Panda's parents too, as a comprehensive, evidence-based resource for information, toolkits, research, and advocacy related to PANS/PANDAS.
Personal Background and Approach
Dr. French shares her personal journey, including experiencing professional burnout 15 years ago and finding renewal through integrative medicine. Her primary intention is to build intergenerational resilience for her family and patients. She sees many medically complex, chronically ill children with stressed families in her clinic. She emphasizes the importance of self-regulation to provide tools for self-regulation to patients and families, aiming to change structural systems that don't support resilience.
Patient Examples and History
Dr. French presents several patient examples to illustrate the diverse manifestations of PANS/PANDAS, including a 12-year-old girl with increased anxiety and OCD, a 6-year-old boy with sensory processing issues, a 9-year-old girl with restrictive food behaviors, and a 15-year-old boy with a worsening tic disorder and behavioral regression. She highlights the importance of recognizing changes in behavior that worsen or persist, even in previously neurotypical children or when there are apparent psychosocial stressors.
Diagnosis Delay and Treatment Barriers
A survey reveals that diagnosis is often delayed, with many patients seeing multiple doctors before PANS/PANDAS is considered. Common initial diagnoses include OCD, anxiety, ADHD, oppositional defiance, autism spectrum disorder, and tic disorders. Treatment delays are often due to clinician lack of awareness and skepticism about the disease, leading to medical gaslighting. Other challenges include difficulty finding someone to take charge of care and misdiagnosis.
Definitions: PANS and PANDAS
Dr. French reviews the definitions of PANS and PANDAS, noting that the original definition by Dr. susuo in 1998 has evolved. PANS is the broader term for pediatric acute-onset neuropsychiatric syndrome, while PANDAS is specifically related to infectious triggers, particularly strep. Consensus Consortium guidelines from 2013 and 2017 expanded the definitions to create a common ground for diagnosis and treatment. While she focuses on infectious triggers, she mentions that COVID has highlighted how infections can cause neuropsychiatric syndromes.
Etiopathogenesis of PANS/PANDAS
The etiopathogenesis of PANS/PANDAS is multifactorial, involving genetic predisposition, molecular mimicry, and autoimmunity. Cross-reactive autoantibodies target the basal ganglia, with brain imaging studies showing microglial activation and structural changes. Newer theories suggest that group A strep alters gut microbiota, leading to intestinal inflammation and immune responses. Interleukin-17 (IL-17) is emerging as an important cytokine, suggesting that PANS/PANDAS is the brain's response to a global inflammatory process. High rates of autoimmunity in first-degree relatives and dramatic responses to immunomodulatory treatments further support this.
Key Historical Clues and Symptoms
Key historical clues include a higher prevalence in boys, family history of autoimmune disease, and recurrent upper respiratory infections. Common symptoms include sleep disturbances, behavioral regression, urinary symptoms, ADHD-like symptoms, handwriting deterioration (dysgraphia), restrictive food behaviors, and severe tics.
Diagnostic Criteria for PANS and PANDAS
Diagnosis is clinical, based on major and minor criteria. Major criteria include sudden onset of OCD, severe restrictive food behaviors, and tics. For PANS, one major criterion and two minor criteria (anxiety, lability, aggression, behavioral regression, ADHD symptoms, sleep or urinary symptoms) are required. For PANDAS, OCD or tics must be the major criterion, along with two minor criteria and a history of strep infection. Strep history can include a recent rash, exposure to a strep case, or being an asymptomatic carrier.
Forms and Physical Exam Findings
Dr. French shares forms used in her office to track patient symptoms and progress. Physical exam findings include signs of infection in the ear, nose, and throat (allergies, coated tongue, palatal petechiae, perioral redness, chapped lips, mouth sores), skin issues (warts, molluscum), genital area (yeast infections, perianal strep), and musculoskeletal tenderness. Dilated pupils and choreiform movements may be present during crises. Left side neglect can also be observed.
Visual Examples and Diagnostic Flowchart
Dr. French presents visual examples of handwriting deterioration and coloring ability to illustrate behavioral regression and dysgraphia. She shares a diagnostic flowchart from The PANS Physician Network as a starting point for evaluation. The flowchart guides clinicians through assessing major and minor criteria, strep testing, and comorbid symptoms to reach a clinical diagnosis and determine severity.
Lab Work and Referrals
Currently, there are no definitive biomarkers for PANS/PANDAS, though research is ongoing. Recommended lab work includes throat and perianal cultures, ASO and anti-DNAse B titers, mycoplasma titers (IgG and IgM), ANA, quantitative immunoglobulins, and EKG. Family members should also be swabbed to identify carriers. It's important to rule out other diseases that mimic PANS. Dr. French emphasizes the importance of co-managing patients with various specialists, including psychiatrists.
Treatment Guidelines and Integrative Approach
Consortium treatment guidelines emphasize a three-pronged approach: psychotherapeutic treatment (medications and behavioral therapy), immunomodulatory therapy, and antibiotics. Antibiotics are recommended for new cases, with chronic prophylaxis for recurrent or severe symptoms. NSAIDs and steroids can be used for flares. Other treatments like IVIG and plasmapheresis are typically outside the scope of primary care. Dr. French's approach involves symptom relief, treating the source of inflammation, addressing immune dysregulation, and considering tonsillectomy and antihistamines.
Treatment Protocols in Dr. French's Office
In her office, Dr. French conducts thorough intakes and physical exams, orders necessary swabs and lab work, rules out other diagnoses, makes referrals, and initiates treatment. Treatment typically begins with a trial of NSAIDs and antibiotics to assess response. She also addresses diet, exercise, mindfulness, sleep, and screen time.
NSAIDs and Steroids
NSAIDs are used at 5-10 mg per dose twice a day with food to control inflammation. Stomach protection with H2 blockers or supplements like strip El or DG Alo is recommended. For severe cases, a short burst of steroids or a 30-day taper may be necessary, similar to asthma flare treatment.
Antibiotic Guidelines
Consortium antibiotic guidelines recommend penicillin, though treatment failure rates can be high. Cephalexin is better than penicillin for post-strep exacerbations. Omnicef is a favorite due to its ability to treat tics and OCD. Azithromycin is effective, long-acting, and immunomodulatory, but requires a baseline EKG. Clindamycin and doxycycline are used for allergic patients, resistant mycoplasma or strep, carriers, dental infections, and tick-borne diseases. Early diagnosis and long-term prophylaxis reduce the risk of chronic neurological sequelae.
Integrative Options and SSRIs
Integrative options include diet cleanup, prebiotic and probiotic foods, and antihistamines for histamine dysregulation. Flavonoids can stabilize mast cells. Yeast overgrowth should be addressed with antifungals. Vitamin D and iron optimization are crucial. SSRIs should be used with caution, starting at very low doses (around 25% of the normal dose) due to neuroinflammation-induced lability. Other remedies for anxiety and OCD include theanine, lemon balm, magnesium, omegas, lavender, NAC, inositol, CBD, lithium, and ashwagandha.
Tonsillectomy, Dental Health, and Resources
Tonsillectomy is not a standard recommendation but can be beneficial due to cytokine presence and bacterial reservoirs in tonsils. Mouse studies suggest that strep infections can lead to immune cell migration to the brain, breaking down the blood-brain barrier. Maintaining a healthy oral microbiome is important, with prophylactic antibiotics recommended for dental work. Xylitol mouthwash, nose spray, and Bliss K12 probiotics are used for oral and sinus health. Dr. French recommends several books and websites for further information and research.
Take-Home Points
Key take-home points include recognizing PANS/PANDAS as a spectrum illness with varying symptoms. Consider the diagnosis in children with acute onset of tics, OCD, or anxiety, along with symptoms like frequent urination, dysgraphia, restrictive food behaviors, sleep issues, school avoidance, and baby talk regression. The course is relapsing-remitting, requiring fluid access to school and mental health services. Focus on correct diagnosis, symptom relief, finding the cause, and appropriate treatment, while minimizing unnecessary medications.
