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The cerebellar cognitive affective syndrome (CCAS) scale has been developed to screen for possible cognitive and affective impairments in cerebellar patients, but previous studies stressed concerns regarding insufficient specificity of the scale. Also, direct comparisons of CCAS scale performance between cerebellar patients with and without CCAS are currently lacking. The aim of this study was to evaluate the validity of the CCAS scale in cerebellar patients.
Method:
In this study, cerebellar patients with CCAS (n = 49), without CCAS (n = 30), and healthy controls (n = 32) were included. The Dutch/Flemish version of the CCAS scale was evaluated in terms of validity and reliability using an extensive neuropsychological assessment as the gold standard for CCAS. Correlations were examined between the CCAS scale and possible confounding factors. Additionally, a correction for dysarthria was applied to timed neuropsychological tests to explore the influence of dysarthria on test outcomes.
Results:
Cerebellar patients with CCAS performed significantly worse on the CCAS scale compared to cerebellar controls. Sensitivity was acceptable, but specificity was insufficient due to high false-positive rates. Correlations were found between outcomes of the scale and both education and age. Although dysarthria did not affect the validity of the CCAS scale, it may influence timed neuropsychological test outcomes.
Conclusions:
Evaluation of the CCAS scale revealed insufficient specificity. Our findings call for age- and education-dependent reference values, which may improve the validity and usability of the scale. Dysarthria might be a confounding factor in timed test items and should be considered to prevent misclassification.
This chapter presents an overview and practical approach to conceptualize manifestations of cerebellar lesions and outlines the principles that govern the cerebellar contribution to cognition and emotion as well as to sensorimotor function. Lesions of the cerebellum have been regarded as producing motor impairments. The cerebellar motor syndrome is characterized by wide-based and unsteady, or ataxic, gait; incoordination, or dysmetria, of the arms and legs; articulation impairment, or dysarthria; and eye movement abnormalities that disturb vision. The cerebellar cognitive affective syndrome (CCAS) results from lesions of the posterior lobe, characterized by clinically relevant deficits in executive function, visual spatial performance, linguistic processing, and dysregulation of affect. The connections of the cerebellum with brain circuits are implicated in psychiatric illness. Applying repetitive transcranial magnetic stimulation (TMS) to the limbic cerebellum in the vermis improves psychiatric disorders such as schizophrenia by upregulating cerebellar modulation of cerebrocerebellar circuits engaged in cognition and emotion.
This chapter presents a brief background on the rationale for how the cerebellum is engaged in non-motor functions. It discusses the relationship of the cerebellar motor syndrome to the higher order aspects of cerebellar dysfunction. The chapter analyzes the nature of the neuro-behavioral deficits and their relationship to cerebellar structure and vascular anatomy. It examines the clinical relevance of the cognitive and behavioral manifestations of cerebellar stroke in the diagnosis and management of patients with posterior circulation ischemia. In stroke neurology, the natural and appropriate tendency is to consider stroke syndromes as manifestations of occlusions of specific blood vessels. Bedside and office-based tests that the neurologist can administer to detect the cerebellar cognitive affective syndrome (CCAS) should focus on the domains known to be affected, namely, disorders of executive function, visuospatial cognition, language-based tests, and assessment of affect and other neuropsychiatric domains.
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