Abstract
Objectives: Pediatric acute-onset neuropsychiatric syndrome (PANS) is a clinical diagnosis in children who have an acute manifestation of varied neuropsychiatric symptoms, including obsessive compulsive disorder, eating disorders, tics, anxiety, irritability, and problems with attention/concentration. PANS may develop as a result of a postinfectious syndrome and may represent a new form of postinfectious autoimmunity. To test the hypothesis that multiple, consecutive infusions of intravenous immunoglobulin (IVIG) for PANS can be efficacious, a multisite, open-label study was designed.Methods: The primary endpoint was evaluation of the efficacy of IVIG [Octagam 5%] in PANS over a period of 6 months (six infusions) based on mean changes in psychological evaluation scores using 6 different assessments, including the Children's Yale-Brown Obsessive Compulsive Scale (CY-BOCS), Clinical Global Impression of Severity, and the Parent-Rated Pediatric Acute Neuropsychiatric Symptom Scale (PANS Scale).Results: The final cohort consisted of 21 subjects (7 per site) with moderate to severe PANS. The mean age was 10.86 years (range: 4–16 years). Results demonstrated statistically significant reductions in symptoms from baseline to end of treatment in all six assessments measured. CY-BOCS results demonstrated statistically significant reductions in obsessive compulsive symptoms (p < 0.0001), resulting in >50% improvement sustained for at least 8 weeks after the final infusion and up to 46 weeks in a subset of subjects.Conclusions: In PANS, which may be associated with an underlying immune dysregulation, sequential infusions of IVIG [Octagam 5%] successfully ameliorated psychological symptoms and dysfunction, with sustained benefits for at least 8 weeks, and up to 46 weeks in a subset of subjects. In addition, baseline immune and autoimmune profiles demonstrated significant elevations in a majority of subjects, which requires further evaluation, characterization, and study to clarify the potential immune dysfunction by which PANS manifests and progresses.
Highlights
Rapid onset of obsessive compulsive disorder (OCD) and/or tic disorder in children following streptococcal infections was initially explored at the National Institutes of Mental Health (NIMH) in the late 1990s
In Pediatric acute-onset neuropsychiatric syndrome (PANS), which may be associated with an underlying immune dysregulation, sequential infusions of intravenous immunoglobulin (IVIG) [Octagam 5%] successfully ameliorated psychological symptoms and dysfunction, with sustained benefits for at least 8 weeks, and up to 46 weeks in a subset of subjects
The criteria established by the NIMH for the diagnosis of PANDAS included ‘‘(1) the presence of OCD and/or a tic disorder; (2) pediatric onset; (3) an episodic course of symptom severity; (4) an association with streptococcal infections; (5) an association with neurological abnormalities, including piano-playing choreiform movements of the fingers and toes, which suggests that PANDAS may be similar to Sydenham’s chorea (SC)’’ (Swedo et al 1998)
Summary
Rapid onset of obsessive compulsive disorder (OCD) and/or tic disorder in children following streptococcal infections was initially explored at the National Institutes of Mental Health (NIMH) in the late 1990s. The preferred terminology is ‘‘pediatric acute-onset neuropsychiatric syndrome,’’ or PANS, in which the key clinical features include, ‘‘acute and dramatic symptom onset of OCD and/or severely restrictive food intake with at least two coinciding abrupt onset, debilitating symptoms (anxiety; dysregulation; irritability, aggression, oppositionality; behavioral regression; cognitive deterioration; sensory or motor abnormalities; somatic symptoms)’’ (Swedo et al 2012). Based on these new criteria, PANDAS is considered a subgroup of PANS
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