Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS) and Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS): Immunological Features Underpinning Controversial Entities
RESEARCH IMPACT:
“PANS and PANDAS: Immunological Features Underpinning Controversial Entities” examines the immunological mechanisms underlying PANS and PANDAS emphasizing the role of autoantibodies, cytokine activity, and basal ganglia dysfunction in disease pathology. By reviewing recent research, it highlights how immune-mediated brain inflammation can result in the onset of neuropsychiatric symptoms. The review provides insights into potential diagnostic biomarkers and treatment strategies for these medical conditions.
SUMMARY
“PANS and PANDAS: Immunological Features Underpinning Controversial Entities” discusses the documented immunological features that likely play a role in the pathogenesis and clinical manifestations of Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS) and Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS).
PANS and PANDAS are characterized by the abrupt and dramatic onset of psychiatric and neurological symptoms including OCD and/or restrictive eating, often following infections such as Group A Strep. PANS/PANDAS may be caused by immune system dysfunction, where autoantibodies attack neuronal structures in the brain’s basal ganglia.. Molecular mimicry, in which the immune system mistakenly targets neuronal proteins due to their resemblance to infectious agents, is believed to play a role. Additionally, pro-inflammatory cytokines like IL-17 can weaken the blood-brain barrier, allowing immune cells to enter the brain and cause inflammation. That neuroinflammation then triggers the psychiatric and neurological symptoms associated with the condition. Brain imaging studies have detected abnormalities in the basal ganglia of affected individuals, supporting an immune-mediated cause.
The paper highlights a connection between PANS/PANDAS and autoimmune conditions such as arthritis, thyroiditis, and systemic inflammatory diseases. Some patients show broader immune activation, including joint inflammation and other autoimmune markers, indicating that the condition may extend beyond the brain. Researchers have also identified genetic factors, with certain mutations linked to immune regulation and neuronal function occurring more frequently in PANS patients.
The symptomatology and relapsing-remitting course of PANS and PANDAS may make it difficult for some clinicians to make the differential, clinical diagnosis.Treatment approaches parallel those used for Sydenham Chorea and Acute Rheumatic Fever including identifying and treating infections, anti-inflammatories such as NSAIDs and corticosteroids, and immunomodulatory therapies like intravenous immunoglobulin (IVIG).
Evidence supports that the pathogenesis of PANS/PANDAS is immune-mediated and likely targets the basal ganglia.Additional research is needed to improve diagnostic tools, identify biomarkers, and develop targeted treatments.
LINK TO PAPER: https://www.mdpi.com/2227-9067/11/9/1043
CITATION
Leonardi, L., Perna, C., Bernabei, I., Fiore, M., Ma, M., Frankovich, J., Tarani, L., & Spalice, A. (2024). Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS) and Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS): Immunological Features Underpinning Controversial Entities. Children (Basel, Switzerland), 11(9), 1043. https://doi.org/10.3390/children11091043