Subtypes of cognitive impairment in cerebellar disease identified by cross-diagnostic cluster-analysis: results from a German multicenter study

J Neurol. 2024 Dec 21;272(1):83. doi: 10.1007/s00415-024-12831-1.

Abstract

Background: Cognitive and neuropsychiatric impairment, known as cerebellar cognitive affective syndrome (CCAS), may be present in cerebellar disorders. This study identified distinct CCAS subtypes in cerebellar patients using cluster analysis.

Methods: The German CCAS-Scale (G-CCAS-S), a brief screening test for CCAS, was assessed in 205 cerebellar patients and 200 healthy controls. K-means cluster analysis was applied to G-CCAS-S data to identify cognitive clusters in patients. Demographic and clinical variables were used to characterize the clusters. Multiple linear regression quantified their relative contribution to cognitive performance. The ability of the G-CCAS-S to correctly distinguish between patients and controls was compared across the clusters.

Results: Two clusters explained the variance of cognitive performance in patients' best. Cluster 1 (30%) exhibited severe impairment. Cluster 2 (70%) displayed milder dysfunction and overlapped substantially with that of healthy controls. Cluster 1 patients were on average older, less educated, showed more severe ataxia and more extracerebellar involvement than cluster 2 patients. The cluster assignment predicted cognitive performance even after adjusting for all other covariates. The G-CCAS-S demonstrated good discriminative ability for cluster 1, but not for cluster 2.

Conclusions: The variance of cognitive impairment in cerebellar disorders is best explained by one severely affected and one mildly affected cluster. Cognitive performance is not only predicted by demographic/clinical characteristics, but also by cluster assignment itself. This indicates that factors that have not been captured in this study likely have effects on cognitive cerebellar functions. Moreover, the CCAS-S appears to have a relative weakness in identifying patients with only mild cognitive deficits.

Study registration: The study has prospectively been registered at the German Clinical Study Register ( https://www.drks.de ; DRKS-ID: DRKS00016854).

Keywords: Cerebellar cognitive affective syndrome (CCAS); Cerebellar disorders; Cluster analysis; German CCAS-Scale; Subgroups of CCAS.

Publication types

  • Multicenter Study

MeSH terms

  • Adult
  • Aged
  • Cerebellar Diseases* / complications
  • Cerebellar Diseases* / diagnosis
  • Cerebellar Diseases* / physiopathology
  • Cluster Analysis
  • Cognitive Dysfunction* / diagnosis
  • Cognitive Dysfunction* / etiology
  • Cognitive Dysfunction* / physiopathology
  • Female
  • Germany / epidemiology
  • Humans
  • Male
  • Middle Aged
  • Neuropsychological Tests

Associated data

  • DRKS/DRKS00016854