What are the diagnostic criteria for PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal) and PANS (Pediatric Acute-onset Neuropsychiatric Syndrome)?

From the Guidelines

PANDAS and PANS diagnostic criteria are characterized by the sudden onset of obsessive-compulsive symptoms, often in response to a streptococcal infection or other insults.

Diagnostic Criteria

The diagnostic criteria for PANDAS and PANS include:

• Sudden onset of obsessive-compulsive symptoms
• Association with a streptococcal infection or other insults
• Presence of neurological symptoms, such as tics or chorea
• Absence of other explanations for the symptoms According to 1, research has shifted from PANDAS to a broader disorder, PANS, which is characterized by the sudden onset of obsessive-compulsive symptoms that can occur in response to a range of infections and other insults.

PANDAS Specific Criteria

The PANDAS hypothesis proposes that a subset of patients with obsessive-compulsive and tic disorders produce autoimmune responses that cross-react with brain tissue in response to a GAS infection, similar to the autoimmune response believed to be responsible for the manifestations of Sydenham chorea 1. However, the current state of knowledge dictates that the concept of PANDAS should be considered only as a yet-unproven hypothesis, and routine laboratory testing for GAS to diagnose, long-term antistreptococcal prophylaxis to prevent, or immunoregulatory therapy to treat exacerbations of this disorder is not recommended 1. Key points to consider when diagnosing PANDAS and PANS include the presence of obsessive-compulsive symptoms, association with a streptococcal infection or other insults, and the absence of other explanations for the symptoms.

From the Research

Diagnostic Criteria for PANDAS and PANS

The diagnostic criteria for PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal) and PANS (Pediatric Acute-onset Neuropsychiatric Syndrome) are as follows:

• PANDAS is characterized by:
  • Presence of obsessive-compulsive disorder (OCD) and/or any other tic disorders
  • Prepuberal onset (between 3 years of age and the start of puberty)
  • Abrupt onset and relapsing-remitting symptom course
  • A distinct association with group A beta-hemolytic Streptococcus pyogenes (GABHS) infection
  • Association with neurological abnormalities during exacerbations (adventitious movements or motoric hyperactivity) 2
• PANS is characterized by:
  • Abrupt onset of obsessive-compulsive symptoms and/or severe eating restrictions
  • Concomitant cognitive, behavioral, or neurological symptoms
  • No strict link to group A streptococcal infections, but can be triggered by various infectious or environmental factors 3, 4, 5

Key Differences between PANDAS and PANS

The key differences between PANDAS and PANS are:

• PANDAS is strictly linked to group A streptococcal infections, while PANS can be triggered by various infectious or environmental factors
• PANS has a broader spectrum of neuropsychiatric conditions, with a presumed variety of disease mechanisms and multiple etiologies 3, 4
• PANS patients often present with a complex clinical presentation and compromised neuropsychological profile, including obsessive symptoms, behavioral regression, and somatic symptoms 6

Diagnostic Challenges

The diagnosis of PANDAS and PANS can be challenging due to:

• Lack of clear consensus regarding definition, diagnostic criteria, treatment, and follow-up 3
• Absence of biological markers or instrumental alterations, making diagnosis a matter of exclusion 6
• Need for a diagnostic protocol to better define diagnostic criteria, identify possible subtypes, and delineate treatment 4