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August 2024

Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS) and Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS): Immunological Features Underpinning Controversial Entities

Leonardi L, Perna C, Bernabei I, Fiore M, Ma M, Frankovich J, Tarani L, Spalice A. Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS) and Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS): Immunological Features Underpinning Controversial Entities. Children. 2024; 11(9):1043. https://doi.org/10.3390/children11091043

 

 

 

Cerebrospinal fluid characteristics of patients presenting for evaluation of pediatric acute-neuropsychiatric syndrome

Pooni R, Zheng W, Ma M, Silverman M, Xie Y, Farhadian B, Thienemann M, Mellins E, Frankovich J. Cerebrospinal fluid characteristics of patients presenting for evaluation of pediatric acute-neuropsychiatric syndrome. Front Behav Neurosci. 2024 Aug 19;18:1342486. doi: 10.3389/fnbeh.2024.1342486. PMID: 39224487; PMCID: PMC11367679.

This study found that about a quarter of patients meeting strict PANS criteria who underwent lumbar punctures (LP) had cerebrospinal fluid (CSF) abnormalities, with the most common being elevated CSF protein and albumin quotient. These patients also exhibited signs of inflammation and autoimmunity, such as low complement levels, high immune complexes, non-specific autoantibodies, vasculopathy, and arthritis.

Interestingly, the “other psychiatric deterioration” group—those referred for PANS but not meeting strict criteria—had an even higher frequency (45%) of CSF abnormalities. Many of these patients also showed systemic inflammation, with conditions like thyroiditis and arthritis, and had symptoms overlapping with PANS, including obsessive-compulsive behaviors and food restrictions.

Both groups demonstrated a notable prevalence of arthritis, emphasizing systemic inflammation’s potential role, possibly affecting the blood-brain barrier (BBB). While the study was not designed to assess the significance of these differences, it suggests an overlap in psychiatric symptoms and inflammatory features across the groups, which may involve complement activation and vascular inflammation, similar to what’s seen in Long Covid.

 

PANS PANDAS Triumphs!
PANS PANDAS Triumphs!

Recent PANS/PANDAS Triumphs Thank you to everyone who took a moment to celebrate your recent success. Your stories provide hope to other families and patients! Would you like to share...

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Elevated antibody binding to striatal cholinergic interneurons in patients with pediatric acute-onset neuropsychiatric syndrome
Xu J, Frankovich J, Liu RJ, Thienemann M, Silverman M, Farhadian B, Willet T, Manko C, Columbo L, Leibold C, Vaccarino FM, Che A, Pittenger C. Elevated antibody binding to striatal cholinergic interneurons in patients with pediatric acute-onset neuropsychiatric syndrome. Brain Behav Immun. 2024 Jul 29:S0889-1591(24)00515-4. doi: 10.1016/j.bbi.2024.07.044. Epub ahead of print. PMID: 39084540.
  • Children with PANS had lower levels of IgG antibodies (a type of immune protein) in their plasma during a symptom flare compared to matched controls.
  • PANS IgG drawn at flare shows elevated binding to CINs in both mouse and human brain.
  • PANS IgG binding to CINs reduces their activity using p-rpS6 as a marker.
  • Reduction of PANS IgG binding to CINs parallels symptom improvement during recovery.
  • Elevated IgG binding to CINs is resolved in the same subject during symptom recovery.
  • The study suggests that at least a subset of PANS cases have a neuroimmune pathogenesis.