Abstract

Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal Infection (PANDAS): Clinical Manifestations, IVIG Treatment Outcomes, Results from a Cohort of Italian Patients

Author(s): Piero Pavone, Raffaele Falsaperla, Francesco Nicita, Andreana Zecchini, Chiara Battaglia, Alberto Spalice, Lucia Iozzi, Enrico Parano, Giovanna Vitaliti, Alberto Verrotti, Vincenzo Belcastro, Sung Yoon Cho, Dong-Kyu Jin, Salvatore Savasta

Abstract

Pediatric Autoimmune Neuropsychiatric Disorder associated with Streptococcal Infection (PANDAS) is characterized with main clinical features including obsessive-compulsive disorders and tics, acute-onset in prepubertal age, relapsing-remitting course, association with neurological abnormalities (mainly choreiform movements and motor hyperactivity), and temporal relationship with group A streptococcal infections. Thirty- four children with a serious- severe grade of PANDAS were enrolled in Italian Institutions with the aim to report clinical manifestations of the patients and their response to the intravenous immunoglobulin (IVIG) treatment. All patients were selected according to the Swedo‘s criteria and specific laboratory investigations as suggested by Chang for the PANS and treated with IVIG at the dosage of 2 g/kg/day for two consecutive days. At the onset, all patients presented with at least, one psychiatric manifestation including anxiety, emotional lability, bedwetting, enuresis, and phobia, and oppositional behavior including temper tantrums, personality changes, and deterioration in math skills and handwriting. At the laboratory investigations, positivity of pharyngeal swab for streptococcal infection in most of the patients and variable titers of anti-DNase B and ASO were found. In 29 patients reduction or disappearing of the motor symptoms were reached after 1 or 2 cycles of IVIG treatment, while in 5 patients the symptoms reappeared after the third cycle of IVIG. PANDAS may present with polymorphic clinical signs involving behavioral and psychiatric manifestations. In this study, IVIG has been shown to be effective in most of the cases and no complications have been reported during the treatment. Since there is no general agreement on PANDAS treatment, further more extensive researches in this topic are to be hoped.