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

Neurological Research and Practice

Matthis Synofzik¹  Patricia Sulzer¹  Elke Wondzinski²  Mario Siebler²  Jeremy D. Schmahmann³  Saskia Elben⁴  Dagmar Timmann⁵  Andreas Thieme⁵  Heike Jacobi⁶  Ulrich Sure⁷  Miriam Barkhoff⁸  Sandra Roeske⁸  Jennifer Faber⁹  Thomas Klockgether⁹  Martina Minnerop^1,10  Kathrin Reetz^1,11  Imis Dogan^1,11  Oliver Mueller^1,12  Juergen Konczak^1,13 

[1] Department of Neurodegenerative Diseases, Hertie-Institute for Clinical Brain Research and Center of Neurology, Eberhard Karls University Tuebingen, Hoppe-Seyler-Str. 3, 72076, Tuebingen, Germany;German Center for Neurodegenerative Diseases (DZNE) Tuebingen, Helmholtz Association, Otfried-Mueller-Straße 23, 72076, Tuebingen, Germany;Department of Neurology and Neurorehabilitation, MediClin Fachklinik Rhein/ Ruhr, Auf der Roetsch 2, 45219, Essen, Germany;Department of Neurology, Ataxia Center, Cognitive Behavioral Neurology Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, 02114, Boston, MA, USA;Department of Neurology, Center for Movement Disorders and Neuromodulation, Medical Faculty, Heinrich-Heine University Duesseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany;Department of Neurology, Essen University Hospital, University of Duisburg-Essen, Hufelandstr. 55, 45147, Essen, Germany;Department of Neurology, Heidelberg University Hospital, Ruprecht-Karls University Heidelberg, Im Neuenheimer Feld 400, 69120, Heidelberg, Germany;Department of Neurosurgery, Essen University Hospital, University of Duisburg-Essen, Hufelandstr. 55, 45147, Essen, Germany;German Center for Neurodegenerative Diseases (DZNE) Bonn, Helmholtz Association, Venusberg-Campus 1, 53127, Bonn, Germany;German Center for Neurodegenerative Diseases (DZNE) Bonn, Helmholtz Association, Venusberg-Campus 1, 53127, Bonn, Germany;Department of Neurology, Bonn University Hospital, Rheinische Friedrich-Wilhelms University Bonn, Venusberg-Campus 1, 53127, Bonn, Germany;Institute of Neuroscience and Medicine (INM-1), Research Centre Juelich, Wilhelm-Johnen-Str., 52425, Juelich, Germany;Department of Neurology, Center for Movement Disorders and Neuromodulation, Medical Faculty, Heinrich-Heine University Duesseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany;Institute of Clinical Neuroscience and Medical Psychology, Medical Faculty, Heinrich-Heine University Duesseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany;Institute of Neuroscience and Medicine (INM-11), Research Centre Juelich, Wilhelm-Johnen-Str., 52425, Juelich, Germany;Department of Neurology, Aachen University Hospital, Rheinisch-Westfaelische Technische Hochschule Aachen (RWTH), Pauwelstr. 30, 52074, Aachen, Germany;JARA-BRAIN Institute, Molecular Neuroscience and Neuroimaging, Research Centre Juelich, Wilhelm-Johnen-Str., 52425, Juelich, Germany;Present Address: Department of Neurosurgery, Klinikum Dortmund, Muensterstr. 240, 44145, Dortmund, Germany;Department of Neurosurgery, Essen University Hospital, University of Duisburg-Essen, Hufelandstr. 55, 45147, Essen, Germany;School of Kinesiology, University of Minnesota, 400 Cooke Hall 1900 University Ave S E, 55455, Minneapolis, MN, USA;

关键词: Affect;    Cerebellum;    Bedside test;    Cognition;    Human;   

DOI  :  10.1186/s42466-020-00071-3

来源: Springer

【 摘 要 】

BackgroundTraditionally, 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.MethodsA 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.PerspectiveThe 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 registrationThe study is registered at the German Clinical Study Register (DRKS-ID: DRKS00016854).

【 授权许可】

CC BY