However, MMSE and MoCA are of limited use to screen for CCAS because cerebellar patients frequently perform within the normal range. Mini Mental State Examination (MMSE) or Montreal Cognitive Assessment (MoCA). Until recently, there has not been a validated bedside test that was able to reliably screen for CCAS in cerebellar patients – unlike well-established bedside tests for dementias or mild cognitive impairment (MCI), i.e. As yet, diagnosis of CCAS relies on non-standardized bedside cognitive examination and, if available, detailed neuropsychological test batteries. In recent years, advances in structural and functional brain imaging allowed for detailed mapping of cognitive functions in the posterolateral cerebellar hemisphere. Since its original description, evidence for the presence of CCAS has been accumulating in pediatric and adult patients suffering from different cerebellar diseases including various hereditary ataxias, cerebellar tumors, and cerebellar stroke. The core symptoms of CCAS are difficulties with executive, linguistic and visuospatial functions as well as problems with the regulation of emotion and affect. As early as 1998, Schmahmann and Sherman introduced the Cerebellar Cognitive Affective/ Schmahmann Syndrome (CCAS). During the last decades, there has been growing evidence that cerebellar disease is not only accompanied by motor disturbances but also by cognitive and affective symptoms (see for reviews). The study is registered at the German Clinical Study Register (DRKS-ID: DRKS00016854).Ĭerebellar disease results in well-known motor performance deficits, including ataxia of stance and gait, limb incoordination, dysarthria, and oculomotor abnormalities. 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. disease duration, clinical ataxia scores) and CCAS scores. Secondary aims will be examination of possible correlations between clinical features (e.g. In addition, construct validity will be tested in a subset of patients and controls in whom detailed neuropsychological testing will be available. Furthermore, internal consistency, test-retest and interrater reliability will be evaluated. 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. This preliminary German version will be validated in a minimum of 65 patients with cerebellar disease and 65 matched healthy controls. 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. MethodsĪ preliminary German version has been created from the original CCAS Scale using a standardized translation procedure. We present a German version of the scale and the study protocol of its ongoing validation in a German-speaking patient cohort. As yet, the CCAS Scale has only been available in American English. It has been validated in an US-American cohort of adults with cerebellar disorders and healthy controls. Recently, a short and easy applicable bedside test (CCAS Scale) has been developed to screen for CCAS. To date, diagnosis relies on non-standardized bedside cognitive examination and, if available, detailed neuropsychological test batteries. This combination of non-motor symptoms has been named Cerebellar Cognitive Affective/ Schmahmann Syndrome (CCAS). 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. Traditionally, cerebellar disorders including ataxias have been associated with deficits in motor control and motor learning.
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