APPENDIX A

To make treatment for children with Postinfectious Autoimmune Encephalopathy (PANS/PANDAS) consistent across the state of Arkansas:

1. The University of Arkansas for Medical Sciences/Arkansas Children's Hospital Childhood Postinfectious Autoimmune Encephalopathy (CPAE) Center of Excellence, provides information on the latest, evidence-based evaluation and management guidelines for diagnosing and treating Pediatric Acute-onset Neuropsychiatric Syndrome (PANS) and Pediatric Acute-onset Neuropsychiatric Disorders Associated with Streptococcus (PANDAS).

2. The information incorporates the guidance provided by the available evidence-based medical literature on evaluation and management of children with PANS and PANDAS (e.g., Chang et al. 2015, Swedo et al. 2017, Thienemann et al. 2017, Frankovich & Swedo et al. 2017, Cooperstock et al. 2017).

3. Based on the latest evaluation and management guidelines and in order to provide best-practice treatment of PANS/PANDAS, the following considerations will guide recommendations for intravenous immunoglobulin (IVIG) therapy:

A. Only after a licensed physician has treated the patient with two or more less-intensive therapies (e.g., limited course of nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, selective serotonin reuptake inhibitors (SSRIs), behavioral therapy, short- course antibiotic therapy); and these therapies were not effective; AND

B. Only after a consultation and recommendation is issued by a pediatric subspecialist (e.g., pediatric neurologist, pediatric immunologist, pediatric rheumatologist, or pediatric infectious disease specialist) for IVIG treatment; THEN

C. Up to 3 monthly immunomodulatory courses of IVIG therapy may be recommended for treatment of PANDAS and PANS. In addition, a reevaluation at 3 months by the pediatric sub-specialist will be required for continued therapy of IVIG. This evaluation must include objective clinical testing by a specialist trained in structured and/or semi-structured interview assessments, such as a neuropsychologist, which must be performed both pre-treatment and post-treatment to demonstrate significant clinical improvement.

The University of Arkansas for Medical Sciences/Arkansas Children's Hospital CPAE Center of Excellence physicians will be available on a consultation or referral basis to work with referring physicians on the utilization of IVIG prescriptions for PANS/PANDAS patients.