TY - JOUR
T1 - Subtypes of cognitive impairment in cerebellar disease identified by cross-diagnostic cluster-analysis
T2 - results from a German multicenter study
AU - Liu, Qi
AU - Rubarth, Kerstin
AU - Faber, Jennifer
AU - Sulzer, Patricia
AU - Dogan, Imis
AU - Barkhoff, Miriam
AU - Minnerop, Martina
AU - Berlijn, Adam M.
AU - Elben, Saskia
AU - Jacobi, Heike
AU - Aktories, Julia Elisabeth
AU - Huvermann, Dana M.
AU - Erdlenbruch, Friedrich
AU - Van der Veen, Raquel
AU - Müller, Johanna
AU - Nio, Enzo
AU - Frank, Benedikt
AU - Köhrmann, Martin
AU - Wondzinski, Elke
AU - Siebler, Mario
AU - Reetz, Kathrin
AU - Konczak, Jürgen
AU - Konietschke, Frank
AU - Klockgether, Thomas
AU - Synofzik, Matthis
AU - Röske, Sandra
AU - Timmann, Dagmar
AU - Thieme, Andreas
N1 - Publisher Copyright:
© The Author(s) 2024.
PY - 2025/1
Y1 - 2025/1
N2 - 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).
AB - 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).
KW - Cerebellar cognitive affective syndrome (CCAS)
KW - Cerebellar disorders
KW - Cluster analysis
KW - German CCAS-Scale
KW - Subgroups of CCAS
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U2 - 10.1007/s00415-024-12831-1
DO - 10.1007/s00415-024-12831-1
M3 - Article
C2 - 39708269
AN - SCOPUS:85212779754
SN - 0340-5354
VL - 272
JO - Journal of Neurology
JF - Journal of Neurology
IS - 1
M1 - 83
ER -