Pediatric Acute-Onset Neuropsychiatric Syndrome

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Key points

  • A subtype of obsessive-compulsive disorder (OCD) that consists of an abrupt and severe onset has been described.

  • The clinical presentation of pediatric acute-onset neuropsychiatric syndrome (PANS) can be differentiated from classic pediatric OCD by its course and its more global neuropsychiatric dysfunction.

  • The causal mechanism is unsettled, and it is possible that multiple triggers initiate a neuroimmune process that converges to a common pathway leading to the clinical presentation.

  • Research is

Overview: nature of problem

Pediatric autoimmune neuropsychiatric disorder associated with Streptococcus (PANDAS) is characterized by an abrupt (24–48 hours) onset of obsessive-compulsive disorder (OCD) and/or tics. Associated symptoms include emotional lability, separation anxiety, deterioration in handwriting, poor attention, and attention deficit/hyperactivity disorder (ADHD)-like impulsivity, deteriorating visual-spatial abilities, math and reading deficits, and enuresis (Box 1).1 Neuropsychiatric symptoms tend to

GAS-associated triggers

With increased support of an association between acute-onset OCD/tic symptoms and GAS infection, the diagnostic criteria for PANDAS were formulated based on 50 cases, reported in 1998. Most of the subjects (77%) had some evidence of symptoms being preceded by GAS infection, including exacerbations associated with a positive throat culture or episode of scarlet fever, history of recent upper respiratory infection (URI) symptoms plus known GAS exposure, or sore throat or URI symptoms with fever

Infectious triggers other than GAS

Tic exacerbations following a cold have occurred in pediatric, but not adult, patients with tic disorders,73 suggesting that other infectious triggers in addition to GAS should be considered in the etiology of PANS. Of interest, a temporal relationship between URI and the onset of tics in 2 adult patients was reported in a case study.74 In addition, 3 adult patients reportedly developed dystonia, with sera containing anti–basal ganglia antibodies binding to antigens of molecular weight similar

Clinical presentation of PANDAS/PANS

Although co-occurring conditions are common in OCD (see Table 1), a key feature of PANDAS/PANS is the complexity and number of presenting symptoms. For example, ADHD is a common comorbidity in PANDAS youth. In a recent study by Murphy and colleagues,12 PANDAS subjects (n = 41) were more likely to have comorbid ADHD (61%) than their non-PANDAS OCD peers (n = 68) (31%). In addition, psychosis (PANDAS = 12%, non-PANDAS = 9%) and separation anxiety (PANDAS = 29%, non-PANDAS = 22%) were more

Pathophysiology of PANDAS/PANS

The current hypothesis regarding the pathophysiology of PANDAS is based on molecular mimicry, in that antibodies for GAS may target brain proteins, eventually leading to a clinical presentation of PANDAS. PANDAS children have been found to have significantly higher levels of antibodies that trigger calcium-calmodulin–dependent protein kinase II (CaM kinase II) production when compared with their OCD, ADHD, and TS peers in addition to healthy controls.98, 99 GAS antibodies may directly stimulate

PANS assessment

Acquiring a complete clinical history from the potential PANS child and his or her family is essential for identifying diagnostic features unique to PANS. For example, identifying drastic changes in symptom severity that correlate with illness will assist the physician in accurate diagnosis, and subsequent appropriate treatment. In addition to infectious triggers, course of illness and type of symptoms are the most important factors. Along with historical data, a comprehensive review of systems

Disclosures

Dr T.K. Murphy has received research support from the following: NIH/NIMH: 1RO1MH093381-01A1, 5R34HD065274-02, 1R21MH087849-01A1, 5R01MH079489-04, Centers for Disease Control and Prevention: 5U01DD000509-02, NARSAD, International OCD Foundation, Otsuka Pharmaceuticals, AstraZeneca Pharmaceuticals, Sunovion, F. Hoffmann-LaRoche Ltd, Ortho-McNeil Janssen Pharmaceuticals, Shire Pharmaceuticals, Pfizer Inc, Transcept Pharmaceuticals, and Indevus Pharmaceuticals. She has received travel support from

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