Background <p>Cerebellar disease may result in <i>Cerebellar Cognitive Affective Syndrome (CCAS)</i>. The CCAS-Scale, designed to screen for CCAS, has been validated in English Hoche (Brain 141:248–270, 2018) and adapted to other languages.</p> Methods <p>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.</p> Results <p>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.</p> Conclusion <p>The correction formulas improved diagnostic accuracy of the CCAS-Scale, particularly for the sum raw score. Therefore, we recommend using the <i>corrected</i> sum raw score for evaluation instead of the <i>uncorrected</i> 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.</p>

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Optimizing selectivity of the Cerebellar Cognitive Affective Syndrome Scale by use of correction formulas, and validation of its German version

  • Andreas Thieme,
  • Kerstin Rubarth,
  • Raquel van der Veen,
  • Johanna Müller,
  • Jennifer Faber,
  • Miriam Barkhoff,
  • Martina Minnerop,
  • Saskia Elben,
  • Dana Huvermann,
  • Friedrich Erdlenbruch,
  • Adam M. Berlijn,
  • Patricia Sulzer,
  • Kathrin Reetz,
  • Imis Dogan,
  • Heike Jacobi,
  • Julia-Elisabeth Aktories,
  • Giorgi Batsikadze,
  • Qi Liu,
  • Benedikt Frank,
  • Martin Köhrmann,
  • Elke Wondzinski,
  • Mario Siebler,
  • Jürgen Konczak,
  • Matthis Synofzik,
  • Thomas Klockgether,
  • Frank Konietschke,
  • Sandra Röske,
  • Dagmar Timmann

摘要

Background

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.

Methods

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.

Results

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.

Conclusion

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.