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Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS) and Pediatric Autoimmune Neuropsychiatric Disorders Associated
Lucia Leonardi1, Camilla Perna1, Irene Bernabei1
1Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy.
Insights
Pediatric acute-onset neuropsychiatric syndrome (PANS) and PANDAS involve inflammation affecting the basal ganglia, leading to sudden neuropsychiatric symptoms. Autoantibodies may play a role in these complex pediatric disorders.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Psychiatry
Background:
- Pediatric acute-onset neuropsychiatric syndrome (PANS) and PANDAS are overlapping disorders.
- Characterized by acute-onset obsessive-compulsive disorder, eating restriction, tics, and cognitive/behavioral decline.
- Often presents with a relapsing-remitting course, but can have persistent progression.
Purpose of the Study:
- To review the immunological features of PANS and PANDAS.
- To explore the role of inflammation and autoantibodies in the pathogenesis of these disorders.
Main Methods:
- Narrative review of existing literature.
- Analysis of immunological mechanisms, including autoantibodies, complement activation, and cytokine production.
- Consideration of neuroimaging and sleep study findings.
Main Results:
- Heterogeneous inflammatory mechanisms, particularly involving the basal ganglia, are implicated.
- Evidence includes imaging studies, abnormal REM sleep findings, and neurological soft signs.
- Autoantibodies, potentially from molecular mimicry with infectious agents like Streptococcus, are noted.
Conclusions:
- Inflammatory processes and autoantibodies are key contributors to PANS/PANDAS pathogenesis.
- These immunological factors contribute to the significant psychiatric, behavioral, and sleep abnormalities observed.
- Further research into immunological features is crucial for understanding and managing these conditions.
Abstract:
Pediatric acute-onset neuropsychiatric syndrome (PANS) and Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS), represent an overlapping group of disorders which is characterized by acute-onset obsessive compulsive disorders, eating restriction, tics, cognitive and behavioral deterioration which typically follows a relapsing-remitting course but some patients have a primary or secondary persistent progress. This condition is likely caused by heterogeneous inflammatory mechanisms (autoantibodies, complement activation, pro-inflammatory cytokine production) involving the basal ganglia as evidenced by imaging studies (patients vs. controls), sleep studies that found movements and/or atonia during REM sleep, and neurological soft signs that go along with basal ganglia dysfunction. The condition causes significant psychiatric and behavioral symptoms, caregiver burden and sleep abnormalities. Autoantibodies resulting from molecular mimicry of infectious agents (namely group A Streptococcus) and neuronal autoantigens that map to the basal ganglia play also a subtle role. This narrative review aims to describe the key immunological features documented thus far and that likely play a role in the pathogenesis and clinical manifestations of this disorder.
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