Type I Chiari malformation, RBANS performance, and brain morphology: Connecting the dots on cognition and macrolevel brain structure

Neuropsychology. 2019 Jul;33(5):725-738. doi: 10.1037/neu0000547. Epub 2019 May 16.

Abstract

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. (PsycINFO Database Record (c) 2019 APA, all rights reserved).

MeSH terms

  • Adolescent
  • Adult
  • Arnold-Chiari Malformation / complications*
  • Arnold-Chiari Malformation / pathology*
  • Cerebellum / diagnostic imaging
  • Cerebellum / pathology*
  • Cognitive Dysfunction / etiology*
  • Cognitive Dysfunction / physiopathology*
  • Cranial Fossa, Posterior / diagnostic imaging
  • Cranial Fossa, Posterior / pathology*
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Neuropsychological Tests
  • Young Adult