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Chiari and Cognition: Measuring Postsurgical Change in Neurocognitive Symptoms in Adults and Children with Chiari Malformation Type 1 (CM1)

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Abstract:
Chiari malformation type I (CM1) is a neurosurgical disorder in which one or both of the cerebellar tonsils are displaced below the foramen magnum, causing pressure changes on neural structures including the cerebellum. The cerebellum is known to have a role in motor learning, balance, and coordination, with emerging studies suggesting cerebellar control over cognitive and emotional processing. Cognitive dysfunction in CM1 is well-documented, but assessments have often relied on extensive neuropsychological tests which take hours to administer. In 2016, a concise way to quantify cognition was developed in the form of the Cerebellar Neuropsychiatric Rating Scale (CNRS), previously validated in cerebellar diseases but not reported in CM1 patients. The CNRS is a 105-point survey that rates neurocognitive symptoms on a four-point Likert scale. Here we evaluate the utility of the CNRS to describe neurocognitive symptoms associated with CM1 by comparing these results against a well-validated neuropsychological battery. We then examine post-surgical changes in CNRS scores to evaluate the effect of decompression surgery on emotive and cognitive function in CM1 patients. Patients with CM1 considering surgical decompression were administered the CNRS questionnaire pre- (N=87) and 8-24 months post-operatively (N=49). A subgroup (N=24) was administered a full neuropsychological battery pre-operatively (Wechsler Full Scale IQ, Digit Span, Coding; WRAML Finger Windows; DKEFS Trail Making; Verbal List Learning; Grooved Pegboard). Descriptive and correlative measures were computed. Pre- and post-operative scores were compared using a paired t-test. The results indicate a moderate correlation between the impairment detected by pre-operative CNRS scores and the standardized neuropsychological test battery (Spearman rank coefficient ρ = 0.56, p=0.003). This suggests that the CNRS could be a more concise alternative to screen for neuropsychological symptoms in CM1. The average CNRS composite score showed a statistically significant improvement, with a mean (SD) of 34.7 (±21.8) pre-op vs. 29.3 (±22.9) post-op, p=0.014. Of CNRS sub-domains, there was a statistically significant decrease in CNRS scores for attentional (p=0.003), emotional (p=0.048), and autism spectrum (p=0.025) domains, indicating that cognitive-affective dysregulation can be improved by decompression surgery.

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Citation

Lella E Wirth, "Chiari and Cognition: Measuring Postsurgical Change in Neurocognitive Symptoms in Adults and Children with Chiari Malformation Type 1 (CM1)" (2023). Summer Research Symposium. Brown Digital Repository. Brown University Library. https://doi.org/10.26300/c8zy-6y54

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  • Summer Research Symposium

    Each year, Brown University showcases the research of its undergraduates at the Summer Research Symposium. More than half of the student-researchers are UTRA recipients, while others receive funding from a variety of Brown-administered and national programs and fellowships and go …
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