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

Piero Pavone,Raffaele Falsaperla,Giovanni Cacciaguerra,Riccardo Lubrano,Michele Vecchio,Lidia Marino,Maria Grazia Pappalardo,Luca Sabino,Salvatore Cocuzza,Sarah Sciuto,Giulia Messina,Annamaria Sapuppo,Antonino Maniaci,Alessandra Fontana,Claudia Oliva,Rita Chiaramonte

doi:10.3390/neurosci1020007

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.

Highlights

PANDAS collects a specific specter of disturbances linked to group A beta-hemolytic streptococcal infection (GABHS)
From the several cases that came out from literature over time [2,3,4], it is evident that there is a great variety of phenotypes, so allowing to widen the edges of these new disorders including CANS, defined by the presence of obsessive–compulsive disorder (OCD) with abrupt onset and Pediatric acute-onset neuropsychiatric syndrome (PANS), of which PANDAS represents a subgroup of disorders [5,6]
This study aims to assess the clinical features of the patients observed from different Italian cohorts, and to evaluate clinical response to intravenous immunoglobulin (IVIG) treatment in children with an extreme severity grade of PANDAS/PANS disease

Summary

Introduction

PANDAS (pediatric autoimmune neuropsychiatric disorder associated with streptococcal infection) collects a specific specter of disturbances linked to group A beta-hemolytic streptococcal infection (GABHS). It is a post-infectious complication affecting the central nervous system, to Sydenham chorea or rheumatic fever, but with specific involvement of neuropsychiatric and behavioral skills. From the several cases that came out from literature over time [2,3,4], it is evident that there is a great variety of phenotypes, so allowing to widen the edges of these new disorders including CANS (childhood acute neuropsychiatric symptoms), defined by the presence of OCD with abrupt onset and PANS (pediatric acute-onset neuropsychiatric syndrome), of which PANDAS represents a subgroup of disorders [5,6]. Defining criteria for PANS [8] is the presence of sudden-onset OCD or food restriction behavior together with at least two associated manifestations including behavioral disturbances (irritability, aggression, oppositional behavior), anxiety, depression, deterioration in school performance, and other neurologic signs like a motor or sensory impairment, sleep disturbances with enuresis, or incontinence (Figures 1 and 2)

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