The Cerebellar Cognitive Affective/Schmahmann Syndrome Scale in Spinocerebellar Ataxias

Louisa P. Selvadurai, Susan L. Perlman, Tetsuo Ashizawa, George R. Wilmot, Chiadi U. Onyike, Liana S. Rosenthal, Vikram G. Shakkottai, Henry L. Paulson, Sub H. Subramony, Khalaf O. Bushara, Sheng Han Kuo, Cameron Dietiker, Michael D. Geschwind, Alexandra B. Nelson, Christopher M. Gomez, Puneet Opal, Theresa A. Zesiewicz, Trevor Hawkins, Talene A. Yacoubian, Peggy C. NopoulosSharon J. Sha, Peter E. Morrison, Karla P. Figueroa, Stefan M. Pulst, Jeremy D. Schmahmann

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

Abstract

The Cerebellar Cognitive Affective/Schmahmann Syndrome (CCAS) manifests as impaired executive control, linguistic processing, visual spatial function, and affect regulation. The CCAS has been described in the spinocerebellar ataxias (SCAs), but its prevalence is unknown. We analyzed results of the CCAS/Schmahmann Scale (CCAS-S), developed to detect and quantify CCAS, in two natural history studies of 309 individuals Symptomatic for SCA1, SCA2, SCA3, SCA6, SCA7, or SCA8, 26 individuals Pre-symptomatic for SCA1 or SCA3, and 37 Controls. We compared total raw scores, domain scores, and total fail scores between Symptomatic, Pre-symptomatic, and Control cohorts, and between SCA types. We calculated scale sensitivity and selectivity based on CCAS category designation among Symptomatic individuals and Controls, and correlated CCAS-S performance against age and education, and in Symptomatic patients, against genetic repeat length, onset age, disease duration, motor ataxia, depression, and fatigue. Definite CCAS was identified in 46% of the Symptomatic group. False positive rate among Controls was 5.4%. Symptomatic individuals had poorer global CCAS-S performance than Controls, accounting for age and education. The domains of semantic fluency, phonemic fluency, and category switching that tap executive function and linguistic processing consistently separated Symptomatic individuals from Controls. CCAS-S scores correlated most closely with motor ataxia. Controls were similar to Pre-symptomatic individuals whose nearness to symptom onset was unknown. The use of the CCAS-S identifies a high CCAS prevalence in a large cohort of SCA patients, underscoring the utility of the scale and the notion that the CCAS is the third cornerstone of clinical ataxiology.

Original languageEnglish (US)
Pages (from-to)1411-1425
Number of pages15
JournalCerebellum
Volume23
Issue number4
DOIs
StatePublished - Aug 2024

Keywords

  • Cerebellar cognitive affective syndrome
  • Cognition
  • Scale
  • Spinocerebellar ataxia

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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