Thieme Andreas, Rubarth Kerstin, van der Veen Raquel, Müller Johanna, Faber Jennifer, Barkhoff Miriam, Minnerop Martina, Elben Saskia, Huvermann Dana, Erdlenbruch Friedrich, Berlijn Adam M, Sulzer Patricia, Reetz Kathrin, Dogan Imis, Jacobi Heike, Aktories Julia-Elisabeth, Batsikadze Giorgi, Liu Qi, Frank Benedikt, Köhrmann Martin, Wondzinski Elke, Siebler Mario, Konczak Jürgen, Synofzik Matthis, Klockgether Thomas, Konietschke Frank, Röske Sandra, Timmann Dagmar
Department of Neurology and Center for Translational Neuro- and Behavioral Sciences (C-TNBS), Essen University Hospital, University of Duisburg-Essen, Hufelandstraße 55, 45147, Essen, Germany.
Institute of Biometry and Clinical Epidemiology, Charité University Medicine Berlin, Corporate Member of Freie University Berlin, Berlin, Germany.
J Neurol. 2025 Apr 17;272(5):343. doi: 10.1007/s00415-025-13083-3.
Cerebellar disease may result in Cerebellar Cognitive Affective Syndrome (CCAS). The CCAS-Scale, designed to screen for CCAS, has been validated in English Hoche (Brain 141:248-270, 2018) and adapted to other languages.
Here, the German CCAS-Scale Thieme (Neurol Res Pract 2:39, 2020) was validated in 209 patients with cerebellar disorders and 232 healthy controls. Correction formulas for the outcome parameters [failed test items (range: 1-10) and sum raw score (range: 0-120)] were developed, controlling for age, education, and sex effects. Diagnostic accuracy and reliability were assessed.
Correction formulas improved selectivity in controls, reducing false positives (failed items: 40%; sum score: 13% vs. original method Hoche (Brain 141:248-270, 2018): 67%), while maintaining moderate sensitivity (failed items: 69%; sum score: 48% vs. original method Hoche (Brain 141:248-270, 2018): 87%). Word fluency tests differentiated best between patients and controls, while other items did not. Internal consistency (α = 0.71) was acceptable. Removal of word fluency tests worsened it. Retest and interrater reliability were high [intraclass correlation coefficients (ICC): 0.77-0.95]. However, these ICCs yielded a large minimal detectable change (MDC; 2.2-2.4 failed items, 9.5-11.4 raw score points) in patients, limiting the use of the CCAS-Scale in follow-up examinations.
The correction formulas improved diagnostic accuracy of the CCAS-Scale, particularly for the sum raw score. Therefore, we recommend using the corrected sum raw score for evaluation instead of the uncorrected number of failed items, proposed originally Hoche (Brain 141:248-270, 2018). Some test items, however, did not differentiate well between patients and controls and MDCs were large, highlighting the need for refined CCAS assessment instruments as progression or treatment outcomes.
小脑疾病可能导致小脑认知情感综合征（CCAS）。旨在筛查CCAS的CCAS量表已在英文版本中得到验证（Hoche，《大脑》141:248 - 270，2018年），并已改编为其他语言版本。
在此，德国版CCAS量表（Thieme，《神经研究实践》2:39，2020年）在209例小脑疾病患者和232名健康对照中进行了验证。针对年龄、教育程度和性别影响，制定了结果参数[测试项目未通过数（范围：1 - 10）和原始总分（范围：0 - 120）]的校正公式。评估了诊断准确性和可靠性。
校正公式提高了对照中的选择性，减少了假阳性（未通过项目：40%；总分：13%，而原始方法Hoche（《大脑》141:248 - 270，2018年）为67%），同时保持了中等敏感性（未通过项目：69%；总分：48%，而原始方法Hoche（《大脑》141:248 - 270，2018年）为87%）。词语流畅性测试在患者和对照之间的区分效果最佳，而其他项目则不然。内部一致性（α = 0.71）可接受。去除词语流畅性测试会使其变差。重测和评分者间信度较高[组内相关系数（ICC）：0.77 - 0.95]。然而，这些ICC在患者中产生了较大的最小可检测变化（MDC；2.2 - 2.4个未通过项目，9.5 - 11.4个原始得分点），限制了CCAS量表在随访检查中的应用。
校正公式提高了CCAS量表的诊断准确性，特别是对于原始总分。因此，我们建议使用校正后的原始总分进行评估，而不是最初Hoche（《大脑》141:248 - 270，2018年）提出的未校正的未通过项目数。然而，一些测试项目在患者和对照之间的区分效果不佳，且MDC较大。这凸显了需要更精细的CCAS评估工具来评估疾病进展或治疗结果。
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