358 Quantifying Cognitive and Affective Dysfunction in Chiari Malformation Type I Patients With the Cerebellar Cognitive Affective Syndrome Scale and the Cerebellar Neuropsychiatric Rating Scale

Abstract

INTRODUCTION: Cognitive and affective dysfunction in Chiari Malformation Type I (CM1) patients are well-documented, but often rely on extensive neuropsychological tests. METHODS: Symptomatic CM1 patients considering surgical decompression were assessed by a board-certified neuropsychologist using the 105-point CNRS (N = 87) and the 10-task CCAS (N = 23), as well as a full neuropsychological battery (N = 24: Wechsler Full Scale IQ, Digit Span, Coding; WRAML Finger Windows; DKEFS Trail Making; verbal list learning; Grooved Pegboard). Descriptive and correlative measures were computed. RESULTS: The study included 66 adults and 21 children. A neuropsychological battery revealed impairment in 29% of patients on 1 test, 12.8% on 2 tests, and 20.8% on 3 or more tests. The CCAS indicated 26% with "possible CCAS" (1 task), 27% with "probable CCAS" (2 tasks), and 18% with "definite CCAS" (3 or more tasks). The median CNRS composite score was 31.0 (IQR: 18.5, 49.0), with positive and negative symptom sub-scores of 18.0 (10.0, 26.5) and 14.0 (7.5, 23.5), respectively. Moderate correlation with the neuropsychological battery was found between impairment detected by CCAS (Spearman rank coefficient ρ = 0.67, p = 0.002) and CNRS (ρ = 0.56, p = 0.003). CONCLUSIONS: A high prevalence of cognitive and affective impairments in pre-operative CM1 patients was observed by all three testing batteries. In this first report of CNRS and CCAS usage in CM1 patients, increased CNRS and CCAS impairment correlated with impairment on standard neuropsychological testing. These tools show promise for future studies CM1, providing a more concise alternative to traditional testing for cognitive and affective dysfunction.