New consensus guidelines for the symptomatic treatment of children with pediatric acute-onset neuropsychiatric syndrome (PANS) and pediatric autoimmune neuropsychiatric syndrome associated with streptococcal infection (PANDAS) have been published in the Journal of Child and Adolescent Psychopharmacology.
Experts in the PANS Research Consortium discussed existing literature on behavioral, psychotherapeutic, and psychopharmacologic therapies for PANS and PANDAS and came to a consensus on treatment recommendations focusing on psychiatric and behavioral interventions, immunomodulatory therapies, and treatment and prevention of infections.
With regards to treating the psychiatric and behavioral symptoms of children with PANS/PANDAS, the authors write that symptomatic improvement should be directed at reducing suffering, improving functioning, and increasing adherence to treatment interventions. These interventions should be initiated as soon as PANS/PANDAS is identified. The authors urge clinicians to “start low and go slow” when prescribing psychotropic agents due to differing clinical responses among patients. Benzodiazepines may be an appropriate first-line treatment in addressing the child’s anxiety, agitation, aggression, insomnia, and other symptoms as they are generally well-tolerated, however some children may experience severe disinhibition.
The authors note that “psychological, behavioral, and psychopharmacologic interventions tailored to each child’s presentation can provide symptom improvement and improve functioning during both the acute and chronic stages of illness.” Children who are mildly distressed may not need pharmacologic intervention whereas severely distressed children may require education and support in addition to symptom-specific behavioral and pharmacologic interventions. Managing the child’s obsessive compulsive (OCD) symptoms may require use of SSRIs with frequent monitoring; antipsychotics should be limited for cases with incapacitating OCD. The guidelines further recommend treatment options for children who develop tics, unprovoked violent behaviors, anxiety, ADHD symptoms, sleep disturbances, depression, psychosis, and pain.
In Part II of the guidelines, the authors make recommendations on immunomodulatory therapies to target neuroinflammation and post-infectious autoimmunity commonly seen in PANS/PANDAS. For children with mild PANS, the authors recommend “tincture of time” and cognitive behavioral therapies in addition to other supportive therapies. Nonsteroidal anti-inflammatory drugs (NSAIDs) and/or short oral corticosteroid bursts may be used if symptoms persist. For children with moderate-to-severe PANS, oral or IV corticosteroids may be enough, however IV immunoglobulin (IVIG) is often the preferred treatment for these patients. More severe or chronic cases may warrant prolonged corticosteroid rounds (with taper) or repeated high-dose corticosteroids. Children with extreme and life-threatening impairment should be treated with plasma exchange either alone or in combination with IVIG, high-dose IV corticosteroids, and/or rituximab.
“Immunomodulatory therapy should be considered early, because NSAIDs or a short course of oral corticosteroids may be sufficient for symptom remission in recent-onset cases, whereas those with long-standing symptoms often require more intensive and prolonged immunotherapeutic interventions,” the authors write.
Recommendations on managing the infection components of these neuropsychiatric conditions are discussed in Part III of the guidelines. For all new PANS diagnoses, an initial course of anti-streptococcal treatment is recommended. Secondary antimicrobial prophylaxis is recommended for PANDAS patients with severe symptoms or recurrent group A strep-associated exacerbations. The guidelines also contain information on how to assess the infection at initial onset or during neuropsychiatric exacerbations. In addition, children should receive standard immunizations and monitoring of vitamin D levels should be considered.
While children with severe or life-threatening symptoms may require specialized treatment, in most cases, the authors state that “the child’s primary care provider will be able to treat the PANS symptoms effectively, with guidance from these three sets of treatment recommendations.”
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