Cognitive and Psychological Functioning in Chiari Malformation Type I Before and After Surgical Decompression - A Prospective Cohort Study

Abstract

BACKGROUND Chiari Malformation Type I (CM-I) is defined as cerebellar tonsil displacement more than 5 mm below the foramen magnum. This displacement can alter cerebrospinal fluid flow at the cervicomedullary junction resulting in Valsalva-induced headaches and syringomyelia and compress the brainstem resulting in bulbar symptoms. However, little is known about cognitive and psychological changes in CM-I. OBJECTIVE To prospectively assess cognitive and psychological performance in CM-I and determine whether changes occur after surgical decompression. METHODS Blinded evaluators assessed symptomatic CM-I patients ages ≥18 with a battery of neuropsychological and psychological tests. Testing was conducted preoperatively and 6 to 18 mo postoperatively. Data were converted to Z-scores based on normative data, and t-tests were used to analyze pre-post changes. RESULTS A total of 26 patients were included, with 19 completing both pre- and post-op cognitive assessments. All patients had resolution of Valsalva-induced headaches and there was improvement in swallowing dysfunction (P < .0001), ataxia (P = .008), and sleep apnea (P = .021). Baseline performances in visual perception and construction (z = −1.11, P = .001) and visuospatial memory (z = −0.93, P = .002) were below average. Pre-post comparisons showed that CM-I patients had stable cognitive and psychological functioning after surgery, without significant changes from preoperative levels. CONCLUSION CM-I patients had below average performance in visuospatial and visuoconstructional abilities preoperatively. Prospective longitudinal data following surgery demonstrated improved neurologic status without any decline in cognition or psychological functioning. Routine pre- and postoperative formal neuropsychological assessment in CM-I patients help quantify cognitive and behavioral changes associated with surgical decompression.

Prevalence and treatment of typical and atypical headaches in patients with Chiari I malformation: A meta-analysis and literature review

“…No significant difference was observed between the two methods in pooled rates of improvement and reoperation, consistent with recent double-arm observational studies conducted by Seaman et al. (22) and Mozaffari et al. (21).…”

“…(38) performed IDD on 28 CIMs with typical headaches, and 57% of cases experienced complete relief before discharge, and typical headaches disappeared completely in all cases within six months, substantiating its strong and effective decompressive effect. In contrast, EDD provided a similar therapeutic effect to IDD against typical headaches (20)(21)(22)24,43) but lower rates of complications (6,21,44).…”

“…Six out of 24 included studies stratified patients according to their age (pediatric, adult) (13) and management methods (conservation, EDD, and IDD) (14,(20)(21)(22)(23), then quantified the incidence of typical and atypical headaches. This part of literature was expressed as "Author 1/Author 2" (Table 2).…”

A Novel Craniocervical Junction Compression Severity Index-Based Grading System for Multidirectional Quantification of the Biomechanics at Foramen Magnum of Chiari Malformation Type I

“…However, unquantifiable classifications by symptomatology might be avoided. On the other hand, the CVJCSI does not employ complicated methods such as hydrodynamical CSF 56 62 63 64 65 66 , biomechanical by DENSE MRI [67][68][69][70] , genomic 71 72 , eletroneurophysiological 9,73 , psychological [74][75][76] , due to unavailability and lack of reproducibility in the everyday busy clinical settings. 4) Combining type parameters can be complex but avoid the controversy of solo twodimensional morphological parameters caused by insufficient predictive value 11 12 .…”