What is the relationship between Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal (PANDAS) infections and Pediatric Acute-onset Neuropsychiatric Syndrome (PANS)?

From the Guidelines

PANS and PANDAS should be treated with a multi-faceted approach, including antibiotics, anti-inflammatory medications, and psychiatric interventions, with a focus on individualized treatment plans based on symptom severity and presentation, as supported by the most recent evidence from 1.

Key Considerations

• PANS and PANDAS are related neuropsychiatric conditions affecting children, characterized by sudden onset of obsessive-compulsive behaviors and other neurological symptoms.
• The conditions are believed to result from an abnormal immune response to infections, particularly streptococcal infections in PANDAS, causing inflammation in the basal ganglia of the brain which controls movement and behavior.
• Early intervention is crucial for better outcomes, and treatment plans should be individualized based on symptom severity and presentation.

Treatment Approach

• For acute cases, a course of antibiotics like amoxicillin (45-50 mg/kg/day divided twice daily for 10-14 days) is often prescribed if strep infection is confirmed, as suggested by 1.
• Anti-inflammatory treatments may include ibuprofen (10 mg/kg every 6-8 hours) or prednisone (1-2 mg/kg/day for 5-7 days) in more severe cases.
• Intravenous immunoglobulin (IVIG) therapy might be considered for treatment-resistant cases, although its use is still a topic of debate, as noted in 1.
• Psychiatric medications such as SSRIs (starting at low doses and gradually increasing) may help manage OCD symptoms.
• Cognitive behavioral therapy is also essential for symptom management.

Important Notes

• The PANDAS hypothesis has stimulated considerable research, as well as controversy, and should be considered only as a yet-unproven hypothesis, as stated in 1.
• Routine laboratory testing for GAS to diagnose, long-term antistreptococcal prophylaxis to prevent, or immunoregulatory therapy to treat exacerbations of this disorder is not recommended, according to 1.
• The most recent evidence from 1 provides the best guidance for treatment, and individualized treatment plans should be based on symptom severity and presentation.

From the Research

PANS and PANDAS Overview

• PANS (Pediatric Acute-Onset Neuropsychiatric Syndrome) and PANDAS (Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal Infection) are emerging autoimmune encephalopathies of childhood 2
• PANDAS is a subset of PANS, characterized by an association with streptococcal infection 2

Clinical Features and Diagnosis

• The mean age at onset of PANDAS/PANS symptoms is around 6.2 years 3
• Neurological and psychiatric symptoms are common in both PANS and PANDAS, with no significant difference between the two groups 3
• Irritability, aggressivity, and food restriction are more prevalent in children with PANS than in those with PANDAS 3
• Anti-streptolysin O and anti-DNAse B levels are higher in PANDAS than in PANS 3

Treatment Approaches

• Antibiotics, such as penicillin and azithromycin, have been used to treat PANDAS and PANS, with some evidence of efficacy in reducing neuropsychiatric symptoms 4, 5
• Immunomodulatory interventions, such as intravenous immunoglobulin and plasma exchange, have also been used to treat PANDAS and PANS 5
• Psychotherapy, particularly cognitive behavior therapy, has been shown to be effective in reducing obsessive-compulsive disorder (OCD) symptoms in PANDAS and PANS patients 3, 5
• Antipsychotic treatments and NSAIDs have also been used in some cases, although the evidence for their effectiveness is limited 3, 5

Management Guidelines

• A working guideline for the management of infection issues in PANS and PANDAS has been proposed, including the use of anti-streptococcal treatment and chronic secondary antimicrobial prophylaxis in certain cases 2
• Guidelines for the assessment of infection at initial onset or during neuropsychiatric exacerbations have also been presented 2
• Standard immunizations and attention to vitamin D levels are recommended for all patients with PANS or PANDAS 2