Clinicians caring for children with sudden and severe behavioral and neuropsychiatric symptoms should take a deliberate, cautious approach and partner with parents and subspecialists, according to a new AAP clinical report.

Due to a limited evidence base, it can be difficult to determine if such symptoms are compatible with a diagnosis of pediatric acute-onset neuropsychiatric syndrome (PANS).

The AAP believes PANS is a valid diagnosis and is providing guidance on diagnosis and treatment in the new report. It also is calling for robust research to improve care for the condition.

The report Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS) is available at https://doi.org/10.1542/peds.2024-070334 and will be published in the March issue of Pediatrics.

“PANS can be a challenging and frightening diagnosis,” AAP President Benjamin D. Hoffman, M.D., FAAP, said. “It’s important for pediatricians to partner with families as they approach a diagnosis and prescribe evidence-informed treatments in concert with specialists and multidisciplinary teams.”

PANS was first described in 2012, but there are large gaps in knowledge about the condition, including its causes and best practices for treatment. The AAP formed an expert panel to create guidance for clinicians and commissioned an external evidence review. The AAP Board of Directors authored the clinical report.

Children with PANS have abrupt and dramatic onset of obsessive-compulsive disorder (OCD) or severely restricted food intake. In addition, they have abrupt neuropsychiatric symptoms in at least two of the following categories:

• anxiety;
• emotional lability and/or depression;
• irritability, aggression and/or severe oppositional behaviors;
• developmental regression;
• deterioration in school performance;
• sensory or motor abnormalities; and
• somatic signs and symptoms.

In some cases, PANS follows an infection. Autoimmunity issues, psychological trauma and genetic predisposition also may trigger PANS. Pediatric autoimmune neuropsychiatric disorders thought to be associated with streptococcal infections (PANDAS) is a subset of PANS.

Clinicians caring for patients who may have PANS should use a family-centered approach in a medical home with care delivered by a multidisciplinary team. The AAP recommends conducting a thorough patient and family history, physical and psychiatric exam on these patients but a laboratory and imaging workup only when conditions such as autoimmune encephalitis are suspected. PANS does not have disease-specific biomarkers.

Children with OCD or tics do not need a PANS workup unless symptom onset is extremely abrupt, according to the report. Clinicians also should rule out other possible conditions such as Sydenham chorea and Tourette syndrome. If autoimmune encephalitis is suspected, a number of tests are recommended, including EEG, a brain MRI, lumbar puncture, blood tests, neurocognitive evaluations and autoimmune panel.

The report reviews additional tests that may be warranted depending on the initial workup as well as tests that are not recommended.

Treatment for PANS focuses on managing a child’s symptoms and is grouped into three categories.

1. Psychiatric and behavioral interventions for OCD, other types of anxiety, tics or avoidant/restrictive food intake disorder. OCD and anxiety often are best treated by cognitive behavioral therapy and selective serotonin reuptake inhibitors.
2. Antibiotic treatment for group A streptococcal Children with pharyngitis and a positive culture should be treated with antibiotics for 10 days.
3. Aggressive therapies for presumed underlying neuroinflammation and autoimmune problems. Invasive immunotherapies usually are not recommended due to lack of evidence and potential for adverse effects. Immunomodulatory therapies should be used only in rare cases after consultation with a multispecialty team.

The report lists additional treatments parents may ask about but are not recommended. Clinicians should communicate clearly with families about the limits of PANS diagnosis and treatment options. The AAP will support well-designed, randomized, controlled trials to understand the condition better.

“The AAP looks forward to engaging and collaborating with a wide range of partners to address the challenges complicating the field,” the report said, “and to meet the goals of robust and unbiased evidence and equitable access to care that will ensure the health and well-being of all children.”

Resources

• AAP clinical report Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS)
• Information for parents from HealthyChildren.org