PANDAS Diagnosis and Management
Critical Perspective on PANDAS
PANDAS remains an unproven hypothesis according to the American Heart Association, and clinicians should approach this diagnosis with significant caution, reserving it only for children who meet all five strict diagnostic criteria simultaneously. 1, 2
The American Heart Association explicitly states that PANDAS "should be considered only as a yet-unproven hypothesis" and emphasizes careful consideration before applying this label. 3, 2
Diagnostic Criteria (All Five Must Be Met)
To diagnose PANDAS, the following criteria must be present simultaneously: 1
- Presence of OCD and/or tic disorder (not just behavioral problems like tantrums or screaming) 1
- Prepubertal onset of symptoms 1
- Abrupt symptom onset or episodic course (dramatic, sudden appearance rather than gradual) 1, 4
- Temporal association with Group A Streptococcal (GAS) infection (symptoms appearing weeks after documented infection) 1
- Associated neurological abnormalities, particularly choreiform movements, which are key distinguishing features 1
Diagnostic Workup Algorithm
When evaluating a suspected PANDAS case: 3, 1
- Obtain anti-streptolysin O (ASO) titer and anti-DNase B titer to document recent strep exposure, interpreting results using age-appropriate reference ranges (school-age children normally have higher titers than adults) 3, 1
- Perform throat culture if the patient has any throat symptoms 3
- Document the temporal relationship between GAS infection and neuropsychiatric symptom onset 1
- Exclude other causes, including Sydenham chorea (a major manifestation of rheumatic fever with similar autoimmune mechanism) and classic OCD (which has gradual rather than abrupt onset) 2
Important caveat: The American Heart Association recommends against routine laboratory testing for GAS solely to diagnose PANDAS in patients with OCD/tic symptoms, as elevated titers are common in school-age children and do not confirm PANDAS without meeting all clinical criteria. 1, 2
Treatment Approach
First-Line: Antibiotic Therapy
The initial treatment for PANDAS is antibiotics to eradicate the streptococcal infection. 3, 2
Preferred antibiotic regimens: 3, 2
- Penicillin V 500 mg four times daily for 10 days, OR
- Amoxicillin 500 mg three times daily for 10 days (or amoxicillin/clavulanic acid for 10-21 days) 2, 5
For penicillin-allergic patients: 3, 2
- Erythromycin, OR
- Clindamycin 300 mg four times daily for 10 days (especially for eradication failures), OR
- Azithromycin (maximum 500 mg once daily for 3-5 days)
Post-treatment throat cultures are indicated only in patients who remain symptomatic or experience symptom recurrence. 3
What NOT to Do
The American Heart Association explicitly recommends against: 3, 2
- Long-term antistreptococcal prophylaxis as routine practice 2
- Immunoregulatory treatments (intravenous immunoglobulin, plasma exchange) as first-line or routine therapy 3, 2
Contradictory evidence exists: One Italian cohort study of 371 children reported that long-term prophylaxis with benzathine benzylpenicillin for at least 5 years resulted in improvement in 75% of PANDAS patients, with symptom improvement within 3-5 months. 5 However, this conflicts with guideline recommendations from the American Heart Association, which should take precedence in clinical decision-making. 2
Glucocorticoid Therapy (If Required)
If glucocorticoids are used: 3
- Taper to the lowest dose that controls symptoms to minimize toxicity risk 3
- Monitor regularly for steroid-related adverse effects (growth suppression, metabolic changes) 3
- Consider steroid-sparing alternatives when long-term use is anticipated 3
Common Diagnostic Pitfalls to Avoid
- Overdiagnosis based on antibody titers alone: Elevated titers are common in school-age children and do not confirm PANDAS without meeting all five clinical criteria 1
- Misinterpreting nonspecific behavioral symptoms: Screaming, crying, and tantrums without specific neuropsychiatric features (OCD, tics) and neurological abnormalities should not be diagnosed as PANDAS 1
- Missing the broader PANS category: Research has shifted toward Pediatric Acute-onset Neuropsychiatric Syndrome (PANS), which encompasses sudden-onset OCD symptoms triggered by various infections beyond streptococcal (including Mycoplasma pneumoniae and Epstein-Barr virus), not just GAS 1, 5
Expected Clinical Course
In prospectively identified cases treated with appropriate antibiotics at the sentinel episode: 6
- OCD symptoms promptly disappeared with antibiotic treatment effective against GABHS 6
- Recurrences occurred in 45% of patients in long-term follow-up, each associated with evidence of acute GAS infection 5
- Notable symptom: Daytime urinary urgency and frequency without dysuria, fever, or incontinence occurred in 58% of one prospective series, with symptoms disappearing at night and negative urinalysis 6