PANS/PANDAS: Clinical Experience in IVIG Treatment and State of the Art in Rehabilitation Approaches

Abstract

Pediatric acute-onset neuropsychiatric syndrome (PANS) is a condition characterized by the abrupt, dramatic onset of obsessive–compulsive disorder (OCD) or eating restriction accompanied by equally abrupt and severe comorbid neuropsychiatric symptoms. PANDAS (pediatric autoimmune neuropsychiatric disorder associated with streptococcal infection) is a heterogeneous syndrome identified as post-Streptococcus pyogenes infection (β-hemolytic Streptococcus group A) complications regarding the central nervous system with specific involvement of neuropsychiatric and behavioral skills. In the first part of our study, we share our experience in the treatment of a group of extreme-grade (according to CY-BOCS severity scale) symptomatic patients with intravenous immunoglobulin (IVIG), following the most recent studies regarding the dosage of the drug. Our contribution is to share our experience made on a sample of 55 patients all in the highest level of a severity grade. In the second part of our study, we also analyze the literature on PANS/PANDAS rehabilitation therapy, since in the literature there is no discussion of union and comparison on this method. Objective: This study aims to evaluate the clinical features of the patients observed from different Italian cohorts, with the attempt at evaluating clinical response to IVIG treatment in children with an extreme severity grade of PANS/PANDAS disease. Furthermore, after having analyzed the literature, we propose rehabilitation therapy as an added value to the pharmacological treatment. Materials and Methods: A total of 55 patients with a diagnosis of PANS/PANDAS, who belonged to an extreme grade of disease, were enrolled. All patients were administered with IVIG treatment at 2 g/kg per day for two consecutive days. Results: From our study, a noticeable improvement (until complete remission) of symptoms was evident for at least one year in 47 out of 55 (85%) observed children, while 11 out of these 43 (25%) showed an evident symptoms remission in a single attempt and the remaining 32 (75%) required a second administration to notice a lasting symptomatic improvement.

Paediatric Acute-onset Neuropsychiatric Syndrome (PANS) and intravenous immunoglobulin (IVIG): comprehensive open-label trial in ten children

“…Consequently, there are some open-label studies published on IVIG treatment in children meeting criteria for PANDAS or PANS [15,16]. Melamed et al 2021, in their open-label study of 21 children with PANS discussed the limitations of open trials and commented that if PANS was not an autoimmune, autoinflammatory disease, then an immunomodulatory intervention, such as IVIG, should not have any impact on psychometric and clinical measurements.…”

“…IVIG was well tolerated and mild to moderate side effects were reported. Another open trial including 55 children who received two successive days of 2 g/kg IVIG treatment, reported substantial improvements in OCD symptoms measured by the Children´s Yale-Brown Obsessive–Compulsive Scale (CY-BOCS), lasting for 1 year in 85% of the patients [ 16 ], but this trial did not report other symptom or functional outcomes.…”