Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections: clinical description of the first 50 cases

Am J Psychiatry. 1998 Feb;155(2):264-71. doi: 10.1176/ajp.155.2.264.

Abstract

Objective: The purpose of this study was to describe the clinical characteristics of a novel group of patients with obsessive-compulsive disorder (OCD) and tic disorders, designated as pediatric autoimmune neuropsychiatric disorders associated with streptococcal (group A beta-hemolytic streptococcal [GABHS]) infections (PANDAS).

Method: The authors conducted a systematic clinical evaluation of 50 children who met all of the following five working diagnostic criteria: presence of OCD and/or a tic disorder, prepubertal symptom onset, episodic course of symptom severity, association with GABHS infections, and association with neurological abnormalities.

Results: The children's symptom onset was acute and dramatic, typically triggered by GABHS infections at a very early age (mean = 6.3 years, SD = 2.7, for tics; mean = 7.4 years, SD = 2.7, for OCD). The PANDAS clinical course was characterized by a relapsing-remitting symptom pattern with significant psychiatric comorbidity accompanying the exacerbations; emotional lability, separation anxiety, nighttime fears and bedtime rituals, cognitive deficits, oppositional behaviors, and motoric hyperactivity were particularly common. Symptom onset was triggered by GABHS infection for 22 (44%) of the children and by pharyngitis (no throat culture obtained) for 14 others (28%). Among the 50 children; there were 144 separate episodes of symptom exacerbation; 45 (31%) were associated with documented GABHS infection, 60 (42%) with symptoms of pharyngitis or upper respiratory infection (no throat culture obtained), and six (4%) with GABHS exposure.

Conclusions: The working diagnostic criteria appear to accurately characterize a homogeneous patient group in which symptom exacerbations are triggered by GABHS infections. The identification of such a subgroup will allow for testing of models of pathogenesis, as well as the development of novel treatment and prevention strategies.

MeSH terms

  • Acute Disease
  • Age of Onset
  • Attention Deficit Disorder with Hyperactivity / diagnosis
  • Attention Deficit Disorder with Hyperactivity / epidemiology
  • Autoimmune Diseases / diagnosis*
  • Autoimmune Diseases / etiology
  • Autoimmune Diseases / immunology
  • Central Nervous System Diseases / diagnosis
  • Central Nervous System Diseases / epidemiology
  • Central Nervous System Diseases / immunology
  • Child
  • Child Behavior Disorders / diagnosis
  • Child Behavior Disorders / epidemiology
  • Child, Preschool
  • Comorbidity
  • Female
  • Humans
  • Male
  • Mental Disorders / diagnosis
  • Mental Disorders / epidemiology
  • Obsessive-Compulsive Disorder / diagnosis*
  • Obsessive-Compulsive Disorder / epidemiology
  • Obsessive-Compulsive Disorder / immunology
  • Pharyngitis / diagnosis
  • Pharyngitis / etiology
  • Pharyngitis / immunology
  • Respiratory Tract Infections / diagnosis
  • Respiratory Tract Infections / etiology
  • Respiratory Tract Infections / immunology
  • Severity of Illness Index
  • Streptococcal Infections / complications
  • Streptococcal Infections / diagnosis*
  • Streptococcal Infections / immunology
  • Streptococcus pyogenes / immunology*
  • Tic Disorders / diagnosis*
  • Tic Disorders / etiology