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000877976 1001_ $$0P:(DE-HGF)0$$aThieme, A.$$b0$$eCorresponding author
000877976 245__ $$aValidation of a German version of the Cerebellar Cognitive Affective/Schmahmann Syndrome Scale: preliminary version and study protocol
000877976 260__ $$a[London]$$bBioMed Central$$c2020
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000877976 520__ $$aBackgroundTraditionally, 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.
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000877976 7001_ $$0P:(DE-HGF)0$$aRöske, S.$$b1
000877976 7001_ $$0P:(DE-HGF)0$$aFaber, J.$$b2
000877976 7001_ $$0P:(DE-HGF)0$$aSulzer, P.$$b3
000877976 7001_ $$0P:(DE-Juel1)131622$$aMinnerop, Martina$$b4$$ufzj
000877976 7001_ $$0P:(DE-HGF)0$$aElben, S.$$b5
000877976 7001_ $$0P:(DE-HGF)0$$aJacobi, H.$$b6
000877976 7001_ $$0P:(DE-Juel1)177889$$aReetz, Kathrin$$b7$$ufzj
000877976 7001_ $$0P:(DE-HGF)0$$aDogan, I.$$b8
000877976 7001_ $$0P:(DE-HGF)0$$aBarkhoff, M.$$b9
000877976 7001_ $$0P:(DE-HGF)0$$aKonczak, J.$$b10
000877976 7001_ $$0P:(DE-HGF)0$$aWondzinski, E.$$b11
000877976 7001_ $$0P:(DE-HGF)0$$aSiebler , M.$$b12
000877976 7001_ $$0P:(DE-HGF)0$$aMüller, O.$$b13
000877976 7001_ $$0P:(DE-HGF)0$$aSure, U.$$b14
000877976 7001_ $$0P:(DE-HGF)0$$aSchmahmann, J. D.$$b15
000877976 7001_ $$0P:(DE-HGF)0$$aKlockgether, T.$$b16
000877976 7001_ $$0P:(DE-HGF)0$$aSynofzik, M.$$b17
000877976 7001_ $$0P:(DE-HGF)0$$aTimmann, D.$$b18
000877976 773__ $$0PERI:(DE-600)2947493-0$$a10.1186/s42466-020-00071-3$$p39$$tNeurological research and practice$$v2$$x2524-3489$$y2020
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