Reumers Stacha F I, Maas Roderick P P W M, van den Brandt Veerle J M, Kocken Lotte A H, Wiegand Iris M, de Leeuw Frank-Erik, Schutter Dennis J L G, van de Warrenburg Bart P C, Kessels Roy P C
Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.
Donders Institute for Brain, Cognition and Behaviour, Radboud University, Nijmegen, The Netherlands.
J Int Neuropsychol Soc. 2025 Jul 14:1-11. doi: 10.1017/S1355617725101033.
The cerebellar cognitive affective syndrome (CCAS) scale has been developed to screen for possible cognitive and affective impairments in cerebellar patients, but previous studies stressed concerns regarding insufficient specificity of the scale. Also, direct comparisons of CCAS scale performance between cerebellar patients with and without CCAS are currently lacking. The aim of this study was to evaluate the validity of the CCAS scale in cerebellar patients.
In this study, cerebellar patients with CCAS ( = 49), without CCAS ( = 30), and healthy controls ( = 32) were included. The Dutch/Flemish version of the CCAS scale was evaluated in terms of validity and reliability using an extensive neuropsychological assessment as the gold standard for CCAS. Correlations were examined between the CCAS scale and possible confounding factors. Additionally, a correction for dysarthria was applied to timed neuropsychological tests to explore the influence of dysarthria on test outcomes.
Cerebellar patients with CCAS performed significantly worse on the CCAS scale compared to cerebellar controls. Sensitivity was acceptable, but specificity was insufficient due to high false-positive rates. Correlations were found between outcomes of the scale and both education and age. Although dysarthria did not affect the validity of the CCAS scale, it may influence timed neuropsychological test outcomes.
Evaluation of the CCAS scale revealed insufficient specificity. Our findings call for age- and education-dependent reference values, which may improve the validity and usability of the scale. Dysarthria might be a confounding factor in timed test items and should be considered to prevent misclassification.
小脑认知情感综合征（CCAS）量表已被开发用于筛查小脑疾病患者可能存在的认知和情感障碍，但先前的研究强调了对该量表特异性不足的担忧。此外，目前缺乏对有CCAS和无CCAS的小脑疾病患者之间CCAS量表表现的直接比较。本研究的目的是评估CCAS量表在小脑疾病患者中的有效性。
本研究纳入了患有CCAS的小脑疾病患者（n = 49）、未患有CCAS的小脑疾病患者（n = 30）和健康对照者（n = 32）。以广泛的神经心理学评估作为CCAS的金标准，对荷兰语/弗拉芒语版的CCAS量表进行了有效性和可靠性评估。检查了CCAS量表与可能的混杂因素之间的相关性。此外，对定时神经心理学测试进行了构音障碍校正，以探讨构音障碍对测试结果的影响。
与小脑疾病对照者相比，患有CCAS的小脑疾病患者在CCAS量表上的表现明显更差。敏感性尚可，但由于假阳性率高，特异性不足。发现量表结果与教育程度和年龄均存在相关性。虽然构音障碍不影响CCAS量表的有效性，但可能会影响定时神经心理学测试结果。
CCAS量表评估显示特异性不足。我们的研究结果呼吁建立依赖于年龄和教育程度的参考值，这可能会提高该量表的有效性和实用性。构音障碍可能是定时测试项目中的一个混杂因素，应予以考虑以防止错误分类。
