The Cerebellar Cognitive Affective Syndrome: a Meta-analysis

Abstract

The cerebellar cognitive affective syndrome (CCAS) was first described by Schmahmann and Sherman in 1998. Despite their clear depiction of the syndrome, it is our experience that the CCAS has not yet found solid ground as a disease entity in routine clinical practice. This made us question the dimension of the CCAS in cerebellar patients. We performed a systematic review of the literature according to the PRISMA guidelines, in order to answer the question whether patients with acquired isolated cerebellar lesions perform significantly worse on neuropsychological testing compared to healthy controls. Studies were selected based on the predefined eligibility criteria and quality assessment. The systematic search resulted in ten studies, mainly observational cohorts consecutively including adult patients with isolated cerebellar lesions. Patients were compared to healthy controls, and neuropsychological investigation was done within one year of diagnosis. Meta-analysis of the twelve tests that were done in two or more studies showed that cerebellar patients perform significantly worse on Phonemic Fluency, Semantic Fluency, Stroop Test (naming, reading and interference), Block Design test and WMS-R visual memory. Cerebellar patients have significant and relevant deficits in the visuospatial, language and executive function domain. This meta-analysis therefore emphasizes the importance of the cerebellar cognitive affective syndrome as described by Schmahmann and Sherman.