Type I Chiari Malformation, RBANS Performance, and Brain Morphology: Connecting the Dots on Cognition and Macrolevel Brain Structure

Background: Idiopathic descent of cerebellar tonsils into the cervical spine in Chiari malformation Type I (CMI) is typically associated with occipital headache. Accumulating evidence from experimental studies suggests cognitive effects of CMI. The aim of the current study was to examine the relatio...

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Bibliographic Details
Published in:Neuropsychology 2019-07, Vol.33 (5), p.725-738
Main Authors: Houston, James R., Allen, Philip A., Rogers, Jeffrey M., Lien, Mei-Ching, Allen, Natalie J., Hughes, Michelle L., Bapuraj, Jayapalli Rajiv, Eppelheimer, Maggie S., Loth, Francis, Stoodley, Marcus A., Vorster, Sarel J., Luciano, Mark G.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Arnold-Chiari Malformation - complications
Arnold-Chiari Malformation - pathology
Attention
Brain
Cerebellum - diagnostic imaging
Cerebellum - pathology
Cognitive Ability
Cognitive Dysfunction - etiology
Cognitive Dysfunction - physiopathology
Cranial Fossa, Posterior - diagnostic imaging
Cranial Fossa, Posterior - pathology
Female
Human
Humans
Magnetic Resonance Imaging
Male
Memory Disorders
Middle Aged
Morphology
Neuropsychological Assessment
Neuropsychological Tests
Self-Report
Young Adult
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Summary:Background: Idiopathic descent of cerebellar tonsils into the cervical spine in Chiari malformation Type I (CMI) is typically associated with occipital headache. Accumulating evidence from experimental studies suggests cognitive effects of CMI. The aim of the current study was to examine the relationship between cognition and CMI using a battery of standardized neuropsychological and symptom inventory instruments. Method: Eighteen untreated adults with CMI, and 18 gender, age, and education matched healthy controls completed the Repeatable Battery for the Assessment of Neuropsychological Status (RBANS), and standardized measures of pain, mood, and disability. Morphometric measurements of key neural and osseous elements were also obtained from structural brain magnetic resonance images, for correlation with symptom outcomes. Results: CMI patients exhibited deficits in RBANS attention, immediate memory, delayed memory, and total score. After controlling for pain and associated affective disturbance, the significant group effect for RBANS attention remained. CMI patients also presented seven morphometric differences comprising the cerebellum and posterior cranial fossa compartment that differed from healthy controls, some of which were associated with self-reported pain and disability. Notably, group differences in tonsillar position were associated with self-reported pain, disability, and delayed memory. Conclusion: Adult CMI is associated with domain-specific cognitive change, detectable using a standard clinical instrument. The extent of cognitive impairment is independent of pain or affective symptomatology and may be related to the key pathognomonic feature of the condition. General Scientific Summary In this study, cognitive deficits in Chiari malformation Type I (CMI) were recognized using a routine clinical assessment. Learning and memory deficits were identified in CMI that were due to chronic pain and related symptoms. Additionally, attention deficits were found in CMI that were independent of pain and related symptoms. Furthermore, initial links between CMI brain structure, cognitive function, and symptomatology were established.
ISSN:0894-4105
1931-1559
DOI:10.1037/neu0000547