Many human lesion und functional brain imaging studies suggest involvement of the cerebellum in cognitive functions. However, negative and inconsistent findings are rarely discussed. It is still an open question as to which areas of cognition the cerebellum contributes, as well as how, and to what extent. Frequently cited earlier findings in one area of cognition have been challenged in more recent studies, that is the cerebellum may not be directly involved in attention. Furthermore, disorders in patients with acquired cerebellar disease are frequently mild and less severe compared to lesions of the corresponding areas of the cerebral cortex. Patients with cerebellar disease often perform within the normal range of neuropsychological test norms. This pattern is illustrated based on general intelligence and verbal working memory, which have been assessed by a large number of authors using comparable tests. Findings, however, appear to be more pronounced in individual cases with acute onset cerebellar disorders and in children, in particular with congenital disease. The review suggests that the inconsistencies in cognitive impairments may offer clues as to the nature of cerebellar cognitive involvement.

Dagmar Timmann

Irene Daum

How Consistent are Cognitive Impairments in Patients with Cerebellar Disorders?

John E. Desmond

Trends in Cerebellar Research

Verbal working memory (VWM) engages frontal and temporal/parietal circuits subserving the phonological loop, as well as, superior and inferior cerebellar regions which have projections from these neocortical areas. Different cerebro-cerebellar circuits may be engaged for integrating aurally- and visually-presented information for VWM. The present fMRI study investigated load (2, 4, or 6 letters) and modality (auditory and visual) dependent cerebro-cerebellar VWM activation using a Sternberg task. FMRI revealed modality-independent activations in left frontal (BA 6/9/44), insular, cingulate (BA 32), and bilateral inferior parietal/supramarginal (BA 40) regions, as well as in bilateral superior (HVI) and right inferior (HVIII) cerebellar regions. Visual presentation evoked prominent activations in right superior (HVI/CrusI) cerebellum, bilateral occipital (BA19) and left parietal (BA7/40) cortex while auditory presentation showed robust activations predominately in bilateral temporal regions (BA21/22). In the cerebellum, we noted a visual to auditory emphasis of function progressing from superior to inferior and from lateral to medial regions. These results extend our previous findings of fMRI activation in cerebro-cerebellar networks during VWM, and demonstrate both modality dependent commonalities and differences in activations with increasing memory load.

Matthew P. Kirschen

S. H. Annabel Chen

Modality Specific Cerebro-Cerebellar Activations in Verbal Working Memory: An fMRI Study

Over the last decade, studies have implicated the cerebellum not only in motor functioning, but also in cognition and social cognition. Although some aspects of cognition have been explored in the five most common forms of Spinocerebellar Ataxia (SCA), social cognition in these patients has rarely been examined. The present study provides a preliminary characterisation of the severity of cognitive and social cognitive impairments in patients with SCA2, SCA1 and SCA7 using an identical battery to the one previously used in SCA3 and SCA6 patients for comparison. The cognitive profiles of SCA1 and SCA7 patients were comparable to that of SCA6 patients; SCA1 patients had relatively intact profiles, while SCA7 patients demonstrated only some selective deficits. In contrast, SCA2 patients showed the greatest impairments, similarly to SCA3 patients. On tests of social cognition, SCA2 and SCA7 patients were impaired on a task of emotion attribution, whereas one SCA1 patient had a Theory of Mind deficit, which has also been documented in SCA3 and SCA6. We provide preliminary evidence that the neuropsychological profiles of SCA patients correspond well with the severity of pathological and clinical features. Moreover, these patients may also have social cognition impairments. Overall, we suggest that there is a degree of heterogeneity in the types of cognitive and social cognitive impairments in SCA patients.

N. Sokolovsky

A. Cook

H. Hunt

P. Giunti

L. Cipolotti

A Preliminary Characterisation of Cognition and Social Cognition in Spinocerebellar Ataxia Types 2, 1, and 7

This study sought evidence for a specific cerebellar contribution to cognition by characterising the cognitive phenotype of Spinocerebellar Ataxia Type 6 (SCA-6); an autosomal dominant genetic disease which causes a highly specific late-onset cerebellar degeneration. A comprehensive neuropsychological assessment was administered to 27 patients with genetically confirmed SCA-6. General intellectual ability, memory and executive function were examined using internationally standardised tests (Wechsler Adult Intelligence Scale-III, Wechsler Memory Scale-III, Delis and Kaplan Executive Function System, Brixton Spatial Anticipation test). The patient group showed no evidence of intellectual or memory decline. However, tests of executive function involving skills of cognitive flexibility, inhibition of response and verbal reasoning and abstraction demonstrated significant impairment at the group level with large effect sizes. The results demonstrate an executive deficit due to SCA-6 that can be conceptualised as parallel to the motor difficulties suffered by these patients: the data support a role for the cerebellum in the regulation and coordination of cognitive, as well as motor processes that is relevant to individual performance.

Freya E. Cooper

Manon Grube

Kelly J. Elsegood

John L. Welch

Thomas P. Kelly

Patrick F. Chinnery

Timothy D. Griffiths

The Contribution of the Cerebellum to Cognition in Spinocerebellar Ataxia Type 6

Neuroimaging studies report cerebellar activation during both motor and non-motor paradigms, and suggest a functional topography within the cerebellum. Sensorimotor tasks activate the anterior lobe, parts of lobule VI, and lobule VIII, whereas higher-level tasks activate lobules VI and VII in the posterior lobe. To determine whether these activation patterns are evident at a single-subject level, we conducted functional magnetic resonance imaging (fMRI) during five tasks investigating sensorimotor (finger tapping), language (verb generation), spatial (mental rotation), working memory (N-back), and emotional processing (viewing images from the International Affective Picture System). Finger tapping activated the ipsilateral anterior lobe (lobules IV-V) as well as lobules VI and VIII. Activation during verb generation was found in right lobules VII and VIIIA. Mental rotation activated left-lateralized clusters in lobules VII-VIIIA, VI-Crus I, and midline VIIAt. The N-back task showed bilateral activation in right lobules VI-Crus I and left lobules VIIB-VIIIA. Cerebellar activation was evident bilaterally in lobule VI while viewing arousing vs. neutral images. This fMRI study provides the first proof of principle demonstration that there is topographic organization of motor execution vs. cognitive/emotional domains within the cerebellum of a single individual, likely reflecting the anatomical specificity of cerebro-cerebellar circuits underlying different task domains. Inter-subject variability of motor and non-motor topography remains to be determined.

Catherine J. Stoodley

Eve M. Valera

Jeremy D. Schmahmann

An fMRI Study of Intra-Individual Functional Topography in the Human Cerebellum

Recent research has characterized the anatomical connectivity of the cortico-cerebellar system &#x2013; a large and important fibre system in the primate brain. Within this system, there are reciprocal projections between the prefrontal cortex and Crus II of the cerebellar cortex, which both play important roles in the acquisition and execution of cognitive skills. Here, we propose that this system also plays a particular role in sustaining skilled cognitive performance in patients with Relapsing-Remitting Multiple Sclerosis (RRMS), in whom advancing neuropathology causes increasingly inefficient information processing. We scanned RRMS patients and closely matched healthy subjects while they performed the Paced Auditory Serial Addition Test (PASAT), a demanding test of information processing speed, and a control task. This enabled us to localize differences between conditions that change as a function of group (group-by-condition interactions). Hemodynamic activity in some patient populations with CNS pathology are not well understood and may be atypical, so we avoided analysis strategies that rely exclusively on models of hemodynamic activity derived from the healthy brain, using instead an approach that combined a &#x2018;model-free&#x2019; analysis technique (Tensor Independent Component Analysis, TICA) that was relatively free of such assumptions, with a post-hoc &#x2018;model-based&#x2019; approach (General Linear Model, GLM). Our results showed group-by-condition interactions in cerebellar cortical Crus II. We suggest that this area may have in role maintaining performance in working memory tasks by compensating for inefficient data transfer associated with white matter lesions in MS.

E. Lesage

M. A. J. Apps

A. L. Hayter

C. F. Beckmann

D. Barnes

D. W. Langdon

N. Ramnani

Cerebellar Information Processing in Relapsing-Remitting Multiple Sclerosis (RRMS)

Impairment on verbal fluency tasks has been one of the more consistently reported neuropsychological findings after cerebellar lesions, but it has not been uniformly observed and the possible underlying cognitive basis has not been investigated. We tested twenty-two patients with chronic, unilateral cerebellar lesions (12 Left, 10 Right) and thirty controls on phonemic and semantic fluency tasks. We measured total words produced, words produced in the initial 15 seconds, errors and strategy switches. In the phonemic fluency task, the right cerebellar lesion (RC) group produced significantly fewer words compared to the left cerebellar lesion (LC) group and healthy controls, particularly over the first 15 seconds of the task with no increase in errors and significantly fewer switches over the entire task. In the semantic fluency task there was only a modest decrease in total words in the RC group compared to controls. RC lesions impair fluency with many of the same performance characteristics as left prefrontal lesions. This supports the hypotheses of a prefrontal-lateral cerebellar system for modulation of attention/executive or strategy demanding tasks.

Tom A. Schweizer

Michael P. Alexander

B. A. Susan Gillingham

Michael Cusimano

Donald T. Stuss

Lateralized Cerebellar Contributions to Word Generation: A Phonemic and Semantic Fluency Study

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Behavioural Neurology

Trends in Cerebellar Research

Trends in Cerebellar Research

Articles

How Consistent are Cognitive Impairments in Patients with Cerebellar Disorders?

Trends in Cerebellar Research

Modality Specific Cerebro-Cerebellar Activations in Verbal Working Memory: An fMRI Study

A Preliminary Characterisation of Cognition and Social Cognition in Spinocerebellar Ataxia Types 2, 1, and 7

The Contribution of the Cerebellum to Cognition in Spinocerebellar Ataxia Type 6

An fMRI Study of Intra-Individual Functional Topography in the Human Cerebellum

Cerebellar Information Processing in Relapsing-Remitting Multiple Sclerosis (RRMS)

Lateralized Cerebellar Contributions to Word Generation: A Phonemic and Semantic Fluency Study