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

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).

Wrist proprioceptive acuity: A comprehensive robot-aided assessment.

Proprioception is the sense of the body awareness. Proprioceptive deficits represent frequent consequences of several neurological conditions like stroke, Parkinson's disease and others. The assessment of such somatosensory function is crucial, although the available clinical tests are not sensitive enough. The human wrist is a crucial joint for many activities of daily living and to address the lack of its characterization in terms of proprioceptive acuity the authors in previous studies proposed a novel method that combined the use of a 3-DoF robot and a threshold haunting paradigm. Further experiments were performed to characterize the proprioceptive acuity of the dominant wrist for adduction, extension, pronation and supination by using a 2-alternative-forced-choice test. The acuity thresholds obtained from six subjects (mean values ± standard deviation of 1.65±0.39 for extension, 1.13±0.34 for adduction, 1.90±0.58 for pronation and 1.70±0.30 for supination) were finally combined with the ones harvested in the previous studies for flexion and abduction in order to build the first comprehensive database of human wrist proprioceptive acuity.

Two hands, one perception: how bimanual haptic information is combined by the brain.

Humans routinely use both of their hands to gather information about shape and texture of objects. Yet, the mechanisms of how the brain combines haptic information from the two hands to achieve a unified percept are unclear. This study systematically measured the haptic precision of humans exploring a virtual curved object contour with one or both hands to understand if the brain integrates haptic information from the two hemispheres. Bayesian perception theory predicts that redundant information from both hands should improve haptic estimates. Thus exploring an object with two hands should yield haptic precision that is superior to unimanual exploration. A bimanual robotic manipulandum passively moved the hands of 20 blindfolded, right-handed adult participants along virtual curved contours. Subjects indicated which contour was more "curved" (forced choice) between two stimuli of different curvature. Contours were explored uni- or bimanually at two orientations (toward or away from the body midline). Respective psychophysical discrimination thresholds were computed. First, subjects showed a tendency for one hand to be more sensitive than the other with most of the subjects exhibiting a left-hand bias. Second, bimanual thresholds were mostly within the range of the corresponding unimanual thresholds and were not predicted by a maximum-likelihood estimation (MLE) model. Third, bimanual curvature perception tended to be biased toward the motorically dominant hand, not toward the haptically more sensitive left hand. Two-handed exploration did not necessarily improve haptic sensitivity. We found no evidence that haptic information from both hands is integrated using a MLE mechanism. Rather, results are indicative of a process of "sensory selection", where information from the dominant right hand is used, although the left, nondominant hand may yield more precise haptic estimates.

Balance control in sitting and standing in children and young adults with benign cerebellar tumors.

Children and young adolescents with chronic surgical cerebellar lesions show persistent balance control problems during standing when lesions affect the deep cerebellar fastigial and adjacent interposed nuclei. The purpose of this study is to confirm that the same lesion sites are also associated with permanent signs of trunkal ataxia during sitting. A second aim is to demonstrate that examining the postural control of patients while sitting or standing on a foam cushion may constitute a simple clinical exam yielding results commensurate to a more involved dynamic posturography exam. Balance control was assessed in 16 patients after surgery of a benign cerebellar tumor in chronic state and healthy age- and gender-matched control subjects. Using an ultrasound-based kinematic recording system, trunkal and shoulder sway was measured during sitting and standing in different conditions. High-resolution MRI scans were acquired in the cerebellar patients. Voxel-wise statistical lesion symptom mapping was performed to compare lesioned areas between affected and unaffected patients in a given condition using χ² tests. During sitting, 56% of cerebellar patients exhibited trunkal sway outside the range of healthy controls, and 87.5% of cerebellar patients revealed abnormal sway patterns during standing. Abnormalities were most pronounced when visual information was absent, and somatosensory information became unreliable and/or when the base of support along the medio-lateral axis was minimized during tandem stance. Lesion symptom mapping revealed that pathological values in the behavior data were more likely in patients with surgical lesions involving the fastigial nuclei (NF) and adjacent interposed nuclei (NI). In patients with surgery <1-year lesions of the inferior cerebellar vermis also had an impact on balance function. Our results corroborate previous evidence that the extent of permanent damage to the deep cerebellar nuclei greatly impacts on the recovery on balance function.