Cognitive-Affective Improvement on Cerebellar Neuropsychiatric Rating Scale Scores in Adults and Children After Decompression of Chiari Malformation Type I.

Abstract

This study evaluates the Cerebellar Neuropsychiatric Rating Scale (CNRS) in the preoperative and postoperative assessment of Chiari Malformation Type I (CM1). Adults and children with symptomatic CM1 undergoing surgical decompression completed the CNRS preoperatively (N = 87) and 8-24 months postoperatively (N = 56). Preoperative and postoperative total scores, as well as subdomains of attentional control, emotional control, autism spectrum, psychosis spectrum, and social skills, were compared using a paired t -test. Exploratory factor analysis was used to analyze the CNRS pattern in CM1. CNRS score correlation (Spearman-rank) with predecompression and postdecompression symptoms and surgical success measured by the Chicago Chiari Outcome Scale (CCOS) was assessed and with a standard neuropsychological test battery in a subset of patients (N = 24). Preoperative and postoperative total CNRS scores reflected symptom reduction (mean (SD) 34.7 + 21.8 preoperative vs 29.3 + 22.9 postoperative, P = .01) with significant improvement in attentional and emotional control and autism spectrum subdomains. CNRS attentional control and autism spectrum presurgical subscores were negatively correlated with CCOS scores, indicating that worse performance on attention control and autism spectrum before surgery was associated with worse surgical outcomes. Although higher preoperative CNRS scores correlated with lower performance on a standardized neuropsychological test battery (ρ = 0.56, P = .003), presurgical complaints of neurocognitive symptoms correlated with improvement in reported CNRS attentional control (ρ = 0.37, P = .006). Four of the five hypothesized CNRS factors were observed in our CM1 sample. The CNRS took <15 minutes to administer in all cases. CNRS in CM1 demonstrates preoperative cognitive-affective dysregulation with postsurgical improvement. The observed correlations of predecompression and postdecompression CNRS scores with cognitive symptoms, neuropsychological performance, and the CCOS add a novel clinical tool for the perioperative routine assessment of the Chiari patient, offering a unique focus on patient-reported perspectives of cognitive-affective symptoms.