What is the treatment approach for Pediatric Acute-onset Neuropsychiatric Syndrome (PANS)?

Treatment Approach for Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS)

The most effective treatment for PANS requires a three-pronged approach targeting infection, inflammation, and neuropsychiatric symptoms, with antibiotics and anti-inflammatory medications forming the cornerstone of therapy. 1, 2

Initial Assessment and Diagnosis

• PANS is characterized by sudden onset of obsessive-compulsive symptoms and/or severely restrictive food intake with at least two coinciding neuropsychiatric symptoms 3
• When associated with Group A Streptococcus, the syndrome is specifically labeled as PANDAS (Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal infections) 1
• Diagnostic workup should include testing for recent or current infections, particularly streptococcal infection for suspected PANDAS cases 1

Treatment Strategy

First-Line: Infection Management

• An initial course of anti-streptococcal treatment is recommended for all newly diagnosed PANS cases, even when current infection is not confirmed 1
• Broad-spectrum antibiotics with courses >30 days generally produce the best results, with up to 52% of patients achieving a "very effective" response 2
• For children with PANDAS who have severe neuropsychiatric symptoms or recurrent Group A Streptococcus-associated exacerbations, chronic secondary antimicrobial prophylaxis is recommended 1
• All patients should be monitored for intercurrent infections including sinusitis and influenza, which should be diagnosed and treated promptly 1

Second-Line: Anti-inflammatory Therapy

• Anti-inflammatory treatments show significant efficacy in reducing symptom duration and severity 2, 4
• Oral corticosteroids have demonstrated effectiveness in shortening flare durations (6.4 ± 5.0 weeks vs. 11.4 ± 8.6 weeks without treatment) 4
• Earlier use of corticosteroids is associated with shorter flare durations and faster clinical remission 4
• Longer courses of corticosteroids (5 days to 8 weeks) may result in more durable remissions than short bursts (4-5 days) 4
• Non-steroidal anti-inflammatory drugs (NSAIDs) like ibuprofen are at least "somewhat effective" for most patients 2

Third-Line: Immunomodulatory Therapy

• Intravenous immunoglobulin (IVIG) has been used in 28% of patients with PANS and was at least "somewhat effective" for 89% 2
• For 18% of patients receiving IVIG, the effect was not sustained 2
• The highest rate of sustained response to IVIG treatment was observed in immune-deficient patients who received doses of at least 0.8 g/kg IVIG on a regular basis 2

Adjunctive Psychiatric Management

• Psychotropic medications, particularly SSRIs, are commonly prescribed but have limited effectiveness (only 44% found SSRIs "somewhat" to "very effective") 2
• Cognitive behavioral therapy (CBT) is beneficial for a majority of patients and should be incorporated into treatment plans 2
• Family counseling is rated by parents as highly appropriate and should be considered to support the family unit 5

Treatment Considerations

• Parent perceptions indicate strongest support for inflammation/infection mitigation interventions and lifestyle changes, with less enthusiasm for psychiatric/psychotropic interventions 5
• The DICE approach (Describe, Investigate, Create, Evaluate) may be helpful for managing neuropsychiatric symptoms, though this was developed for dementia patients 6
• Standard immunizations and attention to vitamin D levels are encouraged 1

Monitoring and Follow-up

• Regular monitoring for symptom recurrence is essential due to the relapsing-remitting nature of PANS 4
• Treatment response should be assessed within 14 days of initiating corticosteroids 4
• Vigilance for streptococcal pharyngitis or dermatitis in both the patient and close contacts is recommended 1

Pitfalls and Caveats

• Delayed treatment is associated with longer symptom duration; early intervention is crucial 4
• PANS lacks disease-specific biomarkers, which can complicate diagnosis and treatment evaluation 3
• Evidence suggests limited utility of adenotonsillectomy and probiotics in PANS management 1
• The condition spans multiple subspecialties, requiring coordination between infectious disease, psychiatry, neurology, rheumatology, and immunology specialists 3