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Open Access 01-12-2020 | Clinical trial protocol

Validation of a German version of the Cerebellar Cognitive Affective/ Schmahmann Syndrome Scale: preliminary version and study protocol

Authors: Andreas Thieme, Sandra Roeske, Jennifer Faber, Patricia Sulzer, Martina Minnerop, Saskia Elben, Heike Jacobi, Kathrin Reetz, Imis Dogan, Miriam Barkhoff, Juergen Konczak, Elke Wondzinski, Mario Siebler, Oliver Mueller, Ulrich Sure, Jeremy D. Schmahmann, Thomas Klockgether, Matthis Synofzik, Dagmar Timmann

Published in: Neurological Research and Practice | Issue 1/2020

Abstract

Background

Traditionally, cerebellar disorders including ataxias have been associated with deficits in motor control and motor learning. Since the 1980’s growing evidence has emerged that cerebellar diseases also impede cognitive and affective processes such as executive and linguistic functions, visuospatial abilities and regulation of emotion and affect. This combination of non-motor symptoms has been named Cerebellar Cognitive Affective/ Schmahmann Syndrome (CCAS). To date, diagnosis relies on non-standardized bedside cognitive examination and, if available, detailed neuropsychological test batteries. Recently, a short and easy applicable bedside test (CCAS Scale) has been developed to screen for CCAS. It has been validated in an US-American cohort of adults with cerebellar disorders and healthy controls. As yet, the CCAS Scale has only been available in American English. We present a German version of the scale and the study protocol of its ongoing validation in a German-speaking patient cohort.

Methods

A preliminary German version has been created from the original CCAS Scale using a standardized translation procedure. This version has been pre-tested in cerebellar patients and healthy controls including medical experts and laypersons to ensure that instructions are well understandable, and that no information has been lost or added during translation. This preliminary German version will be validated in a minimum of 65 patients with cerebellar disease and 65 matched healthy controls. We test whether selectivity and sensitivity of the German CCAS Scale is comparable to the original CCAS Scale using the same cut-off values for each of the test items, and the same pass/ fail criteria to determine the presence of CCAS. Furthermore, internal consistency, test-retest and interrater reliability will be evaluated. In addition, construct validity will be tested in a subset of patients and controls in whom detailed neuropsychological testing will be available. Secondary aims will be examination of possible correlations between clinical features (e.g. disease duration, clinical ataxia scores) and CCAS scores.

Perspective

The overall aim is to deliver a validated bedside test to screen for CCAS in German-speaking patients which can also be used in future natural history and therapeutic trials.

Study registration

The study is registered at the German Clinical Study Register (DRKS-ID: DRKS00016854).

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Title: Validation of a German version of the Cerebellar Cognitive Affective/ Schmahmann Syndrome Scale: preliminary version and study protocol
Authors: Andreas Thieme
Sandra Roeske
Jennifer Faber
Patricia Sulzer
Martina Minnerop
Saskia Elben
Heike Jacobi
Kathrin Reetz
Imis Dogan
Miriam Barkhoff
Juergen Konczak
Elke Wondzinski
Mario Siebler
Oliver Mueller
Ulrich Sure
Jeremy D. Schmahmann
Thomas Klockgether
Matthis Synofzik
Dagmar Timmann
Publication date: 01-12-2020
Publisher: BioMed Central
Published in: Neurological Research and Practice / Issue 1/2020
Electronic ISSN: 2524-3489
DOI: https://doi.org/10.1186/s42466-020-00071-3

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Validation of a German version of the Cerebellar Cognitive Affective/ Schmahmann Syndrome Scale: preliminary version and study protocol

Abstract

Background

Methods

Perspective

Study registration

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Methods

Perspective

Study registration

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