Evidence map›Paper›PMID 42606755›Full record

ArticleCerebellum (London, England)2026

The CCAS-Scale in Chiari Malformation Type I: A Cerebellar-Focused Cognitive Assessment with Clinical Utility.

Natalie Amaral-Nieves, Belinda Shao, Mei E Tiemeyer, Lella Wirth, Shanzeh Sayied, Amanda Hasbrouck, Owen P Leary, Deus Cielo, Patricia L Zadnik Sullivan, Christine L Trask and 3 more

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Article in Cerebellum (London, England), 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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5 · Who and what money

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13 authors.

Natalie Amaral-NievesDepartment of Neurosurgery, Warren Alpert Medical School of Brown University, Providence, RI, USA. natalie_amaral-nieves@brown.edu.
Belinda ShaoDepartment of Neurosurgery, Warren Alpert Medical School of Brown University, Providence, RI, USA.
Mei E TiemeyerDepartment of Neurosurgery, Warren Alpert Medical School of Brown University, Providence, RI, USA.
Lella WirthDepartment of Neurosurgery, Warren Alpert Medical School of Brown University, Providence, RI, USA.
Shanzeh SayiedDepartment of Neurosurgery, Warren Alpert Medical School of Brown University, Providence, RI, USA.
Amanda HasbrouckDepartment of Neurosurgery, Warren Alpert Medical School of Brown University, Providence, RI, USA.
Owen P LearyDepartment of Neurosurgery, Warren Alpert Medical School of Brown University, Providence, RI, USA.
Deus CieloDepartment of Neurosurgery, Warren Alpert Medical School of Brown University, Providence, RI, USA.
Patricia L Zadnik SullivanDepartment of Neurosurgery, Warren Alpert Medical School of Brown University, Providence, RI, USA.
Christine L TraskPediatric Neuropsychology Program, Hasbro Children's Hospital, Providence, RI, USA.
Philip A AllenDepartment of Psychology, University of Akron, Akron, OH, USA.
Petra M KlingeDepartment of Neurosurgery, Warren Alpert Medical School of Brown University, Providence, RI, USA.
Konstantina SvokosDepartment of Neurosurgery, Warren Alpert Medical School of Brown University, Providence, RI, USA.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

BACKGROUND/

objectivesThe Cerebellar Cognitive-Affective Syndrome (CCAS) scale, introduced by Schmahmann, was prospectively administered to 73 symptomatic Chiari malformation type I (CM1) patients (17 pediatric) to assess cerebellar contribution to cognitive-affective dysfunction and evaluate the utility of a cerebellar-focused screening tool.

methodsCCAS was categorized as possible (1 failed test), probable (2 failed tests), or definite (≥ 3 failed tests). CCAS total and subtest scores were correlated with baseline symptoms (n = 73) and standard neuropsychological batteries (WAIS, PROMIS, D-KEFS, WRAT, and CVLT, n = 34) using Pearson product-moment correlation with Bonferroni correction. General-linear- model ANOVAs evaluated CCAS diagnostic categories and postoperative outcomes (Chicago-Chiari-Outcome Scale, n = 43).

resultsDefinite CCAS was identified in 44% of patients (53%pediatric, 41%adult), with Digit Span Forward showing the highest failure rates and no differences between surgical and non-surgical cohorts. Higher CCAS scores (less impairment) correlated with lower emotional distress (r=-0.34, p < 0.05) and fewer non-pain symptoms. In adults, CCAS scores correlated with broad intellectual, academic, and executive measures (WAIS, WRAT, D-KEFS) but not specific domains, i.e. verbal learning and memory (CVLT). Impairment in CCAS executive and memory domains predicted improved postoperative CCOS total (F = 4.40,p = 0.043), functional (F = 5.24,p = 0.028), and complication scores (F=6.42, p=0.0155).

conclusionsDefinite CCAS was present in nearly half of patients, supporting a cerebellar contribution to CM1-associated cognitive-affective symptoms. The CCAS scale predicted postoperative outcomes in non-pain domains and captured cognitive brain processing within a network that aligns with established neuropsychological instruments. These findings support CCAS as a valid complementary cognitive assessment tool, with the key distinction that it is designed to detect cognitive dysfunction originating from the cerebellum.

Indexed as

Arnold-Chiari MalformationCerebellar Cognitive Affective SyndromeCerebellumCognitionNeuropsychological TestsAdolescentAdultChildFemaleHumansMaleMiddle AgedProspective StudiesYoung AdultCCASCerebellar pathologyChiari malformationCognitive impairment

Identifiers

PMID42606755

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.