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

Cooper, Freya E.; Grube, Manon; Elsegood, Kelly J.; Welch, John L.; Kelly, Thomas P.; Chinnery, Patrick F.; Griffiths, Timothy D.

doi:10.1155/2010/724861

Cited by 36 publications

(25 citation statements)

References 62 publications

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“…Thus, failure to find significant correlations for any of the tests in the present study could be due to insufficient variability, either behavioral or in gray matter, in the population under study. This is likely the reason why our correlation results for digit span were not as robust as those reported by Cooper et al (37). …”

Section: Discussioncontrasting

confidence: 76%

“…Previous SCA6 patient studies have demonstrated specific cognitive deficits in patients with this largely cerebellar pattern of degeneration, pointing to the role of the cerebellum in intellectual abilities (36,37). Cooper et al (2012) examined the correlation between cerebellar gray matter density and cognitive performance in a group of 15 SCA6 patients, demonstrating a relationship between verbal working memory and gray matter density in superior and inferior parts of the cerebellum.…”

Section: Discussionmentioning

confidence: 88%

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Distinct cerebellar regions related to motor and cognitive performance in SCA6 patients

et al. 2017

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Objective To demonstrate a correlation between anatomic regional changes in Spinocerebellar Ataxia type 6 (SCA6) patients and measures of cognitive performance on neuropsychological tests. Methods Neurocognitive testing was conducted on 24 SCA6 and 28 control subjects. For each cognitive test, SCA6 patients were compared against the controls using Student’s t-test. For the cerebellar patients, using voxel based morphometry, correlations between cerebellar gray matter volume at each voxel and performance on the neuropsychological exams were calculated using the Pearson correlation coefficient implemented in SPM8. Results Compared to controls, SCA6 patients exhibited significantly impaired performance on the following cognitive tests: Rey-Auditory Verbal Learning Test Trial V, Controlled Oral Word Association phonemic test and semantic-verb test, Rey-Osterrieth Complex Figure copy test as well as immediate and delayed visuo-spatial memory recall test, Trail Making Test (TMT) Part A and Part B, Stroop Color Task completion time, Stroop Color-Word Task score, and Grooved Pegboard Test (GPT) Dominant and Non-Dominant Hand time. Correlations of gray matter density with cognitive test performance were determined for all SCA6 subjects. Using a p-value threshold of 0.001 and family-wise small volume error correction, significant correlations were found for GPT Non-Dominant, GPT Dominant, TMT Part A, and TMT Part B. Conclusion Different regional patterns of cerebellar involvement were found for the motoric GPT task and the executive version of the TMT. The results for the GPT strongly indicated that the integrity of medial superior hemispheric regions was associated with motor task performance, whereas executive cognitive function was localized in distinctly different inferior regions. This is the first VBM study to differentiate cognitive and motor contributions of the cerebellum.

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Section: Discussioncontrasting

confidence: 76%

Section: Discussionmentioning

confidence: 88%

Distinct cerebellar regions related to motor and cognitive performance in SCA6 patients

et al. 2017

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“…One alternative explanation for the difference in performance between patients and controls could be based on cognitive demands and the recent implication of the cerebellum in cognition (26)(27)(28)(29). This appears unlikely, as the single-interval timing tasks are less demanding than those of beat-based timing, and as the cognitive deficits demonstrated in patients with cerebellar damage were specific to aspects of executive function or attention (27)(28)(29) and would not lead to the present dissociation.…”

Section: Discussionmentioning

confidence: 86%

“…This appears unlikely, as the single-interval timing tasks are less demanding than those of beat-based timing, and as the cognitive deficits demonstrated in patients with cerebellar damage were specific to aspects of executive function or attention (27)(28)(29) and would not lead to the present dissociation.…”

Section: Discussionmentioning

confidence: 89%

Dissociation of duration-based and beat-based auditory timing in cerebellar degeneration

Grube

Cooper

Chinnery

et al. 2010

Proc. Natl. Acad. Sci. U.S.A.

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175

183

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This work tests the hypothesis that the cerebellum is critical to the perception of the timing of sensory events. Auditory tasks were used to assess two types of timing in a group of patients with a stereotyped specific degeneration of the cerebellum: the analysis of single time intervals requiring absolute measurements of time, and the holistic analysis of rhythmic patterns based on relative measures of time using an underlying regular beat. The data support a specific role for the cerebellum only in the absolute timing of single subsecond intervals but not in the relative timing of rhythmic sequences with a regular beat. The findings support the existence of a stopwatch-like cerebellar timing mechanism for absolute intervals that is distinct from mechanisms for entrainment with a regular beat.T he relevance of the human cerebellum to the perception of time intervals and rhythmic sequences is controversial. Involvement of the cerebellum in perceptual timing (the perception of the timing of sensory events), in addition to its role in motor timing (the timed execution of movements), has been suggested by a number of studies (1-6). One distinction that we wish to address here, which has not been made clear in previous work, is between the absolute, duration-based timing of single subsecond intervals and the relative timing of subsecond intervals based on a regular beat. Functional imaging studies suggest neural activity in the human cerebellum during the perception of the absolute duration of single time intervals (7,8) as well as rhythmic patterns with a regular beat (9-13). However, previous lesion work to assess an obligatory cerebellar role in the perception of single time intervals has not yielded consistent results (4,(14)(15)(16)(17). Previous lesion work to assess any obligatory role of the cerebellum in the analysis of rhythmic sequences has assessed only deficits in related motor activity, such as tapping out a beat (4,14,18), that do not allow clear inference about perception.In this study, we test whether the cerebellum is a critical substrate for perceptual tasks that require the absolute, durationbased analysis of single time intervals as well as those that require the relative analysis of time intervals within rhythmic patterns based on a regular beat. Perceptual tests were conducted in the auditory domain, where accurate temporal encoding of sensory events is essential and entrainment with a beat is induced naturally. Tasks were administered to a group of 34 patients with a stereotyped cerebellar degeneration and a matched control group of 40 healthy individuals. Two absolute timing tasks tested the perception of single intervals for a variable and a fixed reference duration, respectively ( Fig. 1 A and B). Three relative timing tasks tested the beat-based analysis of rhythmic sequences, including the detection of the presence of a roughly regular beat (19), a deviation from an isochronous beat (20) and a distortion of a rhythmic pattern with a metrical beat (21) (Fig. 1 C-E). The data support a ce...

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“…They emphasize the role of the cerebellum in executive functions [2,3], working memory [4,5], language competence [6][7][8][9][10], dyslexia [11], visuospatial abilities [12,13].…”

Section: Introductionmentioning

confidence: 99%

“Cerebellar cognitive affective syndrome CCAS – a case report”

Milczarek¹,

Kwiatkowski²,

Bętkowska-Korpała³

et al. 2013

Arch Psych Psych

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Aim:The aim of the study was to describe a case of the patient with cerebellar cognitive affective syndrome CCAS,characterize the role of cerebellum in the regulation of cognitive functions and present the procedure of neuropsychological diagnosis useful in indicating the specific cognitive and emotional problems in patients with cerebellar damage. Case report:A 41- year old man with an ischemic cerebellar stroke of its right hemisphere manifested the neuropsychological symptoms typical for the frontal damage: euphoric mood, disorganized behavior, lack of criticism and mental plasticity, tendency to shorten the personal distance, problems with mistake correction. In neuropsychological diagnosis we used following methods: Raven Progressive Matrices Test, Mini Mental Stage Examination (MMSE), Trail Making Test, Wisconsin Card Sorting Test, Stroop Interference Test, Word Fluency Test, Auditory Verbal Learning Test by Łuria, Benton Visual Retention Test, Digit Span. Results:Analyzing the obtained results we observed the significant decrease of all executive functions: planning, abstract thinking, cognitive flexibility, adaptation to new situations as well as memory impairments and changes in emotional and behavioral state similar to frontal syndrome.The whole of impairments including the typical cerebellar symptoms (ataxia, dysarthria, dysmetria,hypotonia) create the cerebellar cognitive affective syndrome CCAS with leading role of dysexecutive syndrome. Conclusions:The cerebellum takes part in the regulation of cognitive functions. The cerebellar damages can imitate the emotional- cognitive problems of patients after frontal damages what additionally stress the functional link between these two brain structures. Patient’s with cerebellar damages should have neuropsychological and neuropsychiatric diagnosis and care.

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

Cited by 36 publications

References 62 publications

Distinct cerebellar regions related to motor and cognitive performance in SCA6 patients

Distinct cerebellar regions related to motor and cognitive performance in SCA6 patients

Dissociation of duration-based and beat-based auditory timing in cerebellar degeneration

“Cerebellar cognitive affective syndrome CCAS – a case report”

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