Clinical presentation of pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections in research and community settings.

BACKGROUND The first cases of pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) were described >15 years ago. Since that time, the literature has been divided between studies that successfully demonstrate an etiologic relationship between Group A streptococcal (GAS) infections and childhood-onset obsessive-compulsive disorder (OCD), and those that fail to find an association. One possible explanation for the conflicting reports is that the diagnostic criteria proposed for PANDAS are not specific enough to describe a unique and homogeneous cohort of patients. To evaluate the validity of the PANDAS criteria, we compared clinical characteristics of PANDAS patients identified in two community practices with a sample of children meeting full research criteria for PANDAS. METHODS A systematic review of clinical records was used to identify the presence or absence of selected symptoms in children evaluated for PANDAS by physicians in Hinsdale, Illinois (n=52) and Bethesda, Maryland (n=40). RESULTS were compared against data from participants in National Institute of Mental Health (NIMH) research investigations of PANDAS (n=48). RESULTS As described in the original PANDAS cohort, males outnumbered females (95:45) by ∼ 2:1, and symptoms began in early childhood (7.3±2.7 years). Clinical presentations were remarkably similar across sites, with all children reporting acute onset of OCD symptoms and multiple comorbidities, including separation anxiety (86-92%), school issues (75-81%), sleep disruptions (71%), tics (60-65%), urinary symptoms (42-81%), and others. Twenty of the community cases (22%) failed to meet PANDAS criteria because of an absence of documentation of GAS infections. CONCLUSIONS The diagnostic criteria for PANDAS can be used by clinicians to accurately identify patients with common clinical features and shared etiology of symptoms. Although difficulties in documenting an association between GAS infection and symptom onset/exacerbations may preclude a diagnosis of PANDAS in some children with acute-onset OCD, they do appear to meet criteria for pediatric acute-onset neuropsychiatric syndrome (PANS).

[1]  M. Garvey,et al.  A pilot study of penicillin prophylaxis for neuropsychiatric exacerbations triggered by streptococcal infections , 1999, Biological Psychiatry.

[2]  M. Garvey,et al.  Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections: clinical description of the first 50 cases. , 1998, The American journal of psychiatry.

[3]  Christina M. Morris,et al.  Serial Immune Markers Do Not Correlate With Clinical Exacerbations in Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections , 2008, Pediatrics.

[4]  J. Rapoport,et al.  The pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS) subgroup: separating fact from fiction. , 2004, Pediatrics.

[5]  S. Swedo.  Sydenham's chorea. A model for childhood autoimmune neuropsychiatric disorders. , 1994, JAMA.

[6]  Donald L. Gilbert,et al.  PANDAS: horse or zebra? , 2009, Neurology.

[7]  R. Kurlan.  Tourette's syndrome and ‘PANDAS’ Will the relation bear out? , 1998, Neurology.

[8]  E. Kaplan,et al.  The pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS) etiology for tics and obsessive-compulsive symptoms: hypothesis or entity? Practical considerations for the clinician. , 2004, Pediatrics.

[9]  S. Swedo,et al.  Antibody-mediated neuronal cell signaling in behavior and movement disorders , 2006, Journal of Neuroimmunology.

[10]  J. Leckman,et al.  From Research Subgroup to Clinical Syndrome: Modifying the PANDAS Criteria to Describe PANS (Pediatric Acute-onset Neuropsychiatric , 2012 .

[11]  C. Kallenberg,et al.  Lack of effect of intravenous immunoglobulins on tics: a double-blind placebo-controlled study. , 2004, The Journal of clinical psychiatry.

[12]  E. Storch,et al.  Clinical factors associated with pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections. , 2012, The Journal of pediatrics.

[13]  S. Swedo,et al.  Tubulin Is a Neuronal Target of Autoantibodies in Sydenham’s Chorea1 , 2007, The Journal of Immunology.

[14]  J. Rapoport,et al.  An open trial of plasma exchange in childhood-onset obsessive-compulsive disorder without poststreptococcal exacerbations. , 2000, Journal of the American Academy of Child and Adolescent Psychiatry.

[15]  J. Babb,et al.  Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcus: Comparison of Diagnosis and Treatment in the Community and at a Specialty Clinic , 2008, Pediatrics.

[16]  S. Swedo,et al.  Case study: a new infection-triggered, autoimmune subtype of pediatric OCD and Tourette's syndrome. , 1995, Journal of the American Academy of Child and Adolescent Psychiatry.

[17]  S. Swedo,et al.  Antibiotic prophylaxis with azithromycin or penicillin for childhood-onset neuropsychiatric disorders , 2005, Biological Psychiatry.

[18]  Dwight R. Johnson,et al.  Streptococcal upper respiratory tract infections and exacerbations of tic and obsessive-compulsive symptoms: a prospective longitudinal study. , 2011, Journal of the American Academy of Child and Adolescent Psychiatry.

[19]  M. Garvey,et al.  Therapeutic plasma exchange and intravenous immunoglobulin for obsessive-compulsive disorder and tic disorders in childhood , 1999, The Lancet.

[20]  J. Mink,et al.  Moving from PANDAS to CANS. , 2012, The Journal of pediatrics.

[21]  M. Pichichero,et al.  Prospective identification and treatment of children with pediatric autoimmune neuropsychiatric disorder associated with group A streptococcal infection (PANDAS). , 2002, Archives of pediatrics & adolescent medicine.