Criteria

Pediatric acute-onset neuropsychiatric syndrome (PANS)[1]

The proposed diagnostic criteria for PANS are:[1]

  • Abrupt and dramatic onset (culmination within 72 hours) of new obsessive-compulsive disorder (OCD) or severely restricted food intake

    AND

  • Concurrent presence of additional neuropsychiatric symptoms, with similarly severe and acute onset, from at least 2 of the following 7 categories:

    • Anxiety

    • Emotional lability and/or depression

    • Irritability, aggression, and/or severely oppositional behaviors

    • Behavioral (developmental) regression

    • Deterioration in school performance

    • Sensory or motor abnormalities

    • Somatic signs and symptoms including sleep disturbance, enuresis, or urinary frequency

    AND

  • Symptoms are not better explained by a known neurologic or medical disorder, such as Sydenham chorea, chorea, systemic lupus erythematosus, Tourette disorder, or others.

The obsessive-compulsive symptoms must meet Diagnostic and statistical manual of mental disorders, 5th edition, text revision (DSM-5-TR) criteria for OCD.[1][57]

PANS criteria are "agnostic" to the cause of the child's deterioration since causation is not possible to prove on an individual basis. As in the case of Sydenham chorea, patients often present after the window of opportunity to detect group A streptococcus (GAS) infections. PANS criteria are the criteria that are used in clinical practice. Research to understand the fraction of patients with PANS who have a coincidental preceding GAS infection is ongoing; preliminary data from the authors of this topic suggest that a large proportion of patients presenting with PANS have evidence of preceding GAS infection when it was evaluated thoroughly and at the time of the deterioration.[35] Epidemiologic data and animal models strongly suggest that GAS plays a role in neuropsychiatric deteriorations.[31][32][43][45][55]

Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS)[6]

The proposed diagnostic criteria for PANDAS are:[6]

  • Presence of OCD and/or a tic disorder (Tourette syndrome, chronic motor or vocal tic disorder) as defined by DSM-5-TR diagnostic criteria[57]

  • Onset between 3 years of age and the beginning of puberty

  • Episodic course of symptom severity characterized by an abrupt onset (hyperacute) of symptoms or by dramatic symptom exacerbations

  • Association of symptom onset and exacerbations with group A streptococcus (GAS) infection demonstrated by a positive throat culture/rapid antigen detection testing, and/or significantly elevated anti-GAS antibody titers

  • Presence of neurologic abnormalities, most commonly as motoric hyperactivity and adventitious movements (including choreiform movements or tics).

The diagnostic criteria for PANDAS were developed for use in clinical research. In clinical practice, however, proving a temporal association between symptom onset and exacerbations with GAS infection is often difficult. For example, in Sydenham chorea, where the chorea may lag behind the inciting GAS infection by 6 months or longer. At the time of neuropsychiatric symptom presentations, GAS may be cleared from the throat (thus cultures are negative) and the rise in antibody titers has already occurred, so it is no longer possible to establish a causal relationship between GAS infection and the neuropsychiatric symptoms.[1]

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