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Neurological Soft Signs at Presentation in Patients With Pediatric Acute-Onset Neuropsychiatric Syndrome
Jane E Zebrack1,2, Jaynelle Gao1,2, Britta Verhey1,2
1Division of Allergy, Immunology, and Rheumatology, Department of Pediatrics, Stanford University School of Medicine, Stanford, California.
JAMA Network Open
|March 7, 2025
Summary
Neurological soft signs (NSSs) linked to basal ganglia dysfunction are common in pediatric acute-onset neuropsychiatric syndrome (PANS). Increased NSSs correlate with greater PANS severity and symptoms, aiding diagnosis.
Area of Science:
- Neurology
- Pediatrics
- Neuroscience
Background:
- Pediatric acute-onset neuropsychiatric syndrome (PANS) is a clinical diagnosis characterized by a sudden onset of obsessive-compulsive symptoms and/or motor tics.
- Brain imaging and movement studies during REM sleep suggest basal ganglia involvement in PANS.
- Characterizing neurological findings in PANS is crucial for improving diagnostic accuracy.
Purpose of the Study:
- To determine the prevalence of neurological soft signs (NSSs) associated with basal ganglia dysfunction in youth with PANS.
- To assess the correlation between PANS clinical characteristics and NSSs indicative of basal ganglia dysfunction.
Main Methods:
- A retrospective cohort study of 119 pediatric patients meeting strict PANS criteria.
- Evaluation of specific NSSs: glabellar tap reflex, tongue movements, milkmaid's grip, choreiform movements, spooning, and overflow movements.
- Analysis of Global Impairment Score (GIS) and Caregiver Burden Inventory scores.
Main Results:
- Nearly 80% of PANS patients (95/119) exhibited at least one NSS related to basal ganglia dysfunction; the average was 2.1 NSSs.
- Patients with four or more NSSs showed significantly higher GIS scores and more symptoms compared to those with no NSSs.
- The number of NSSs positively correlated with global impairment and symptom count, but not with age or PANS duration.
Conclusions:
- NSSs associated with basal ganglia dysfunction are highly prevalent in PANS patients.
- A higher number of NSSs is linked to increased disease severity and symptom burden in PANS.
- These findings suggest that targeted neurological examinations can aid in diagnosing PANS.
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