Cognitive impairment in spinocerebellar ataxia type 6

Suenaga, M.; Kawai, Yohei; Watanabe, Haruo; Atsuta, Naoki; Ito, Mizuki; Tanaka, Fumiaki; Katsuno, Masahisa; Fukatsu, Hiroshi; Naganawa, Shinji; Sobue, Gen

doi:10.1136/jnnp.2007.119883

Cited by 67 publications

(59 citation statements)

References 38 publications

(32 reference statements)

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“…Again, we think this unlikely, because the motor output in the behavior task was minimal, a single button press across a single joint. Table 3 The clusters activated by the contrast Hits>Early errors between the groups: control group and SCA group Cognitive and executive impairment are prominent in spinocerebellar patients with extracerebellar pathology (spinocerebellar ataxia 1, 2, and 3); however, mild cognitive and executive impairments were observed in both spinocerebellar ataxia 6 and 8-our sample- [60][61][62]. What is interesting is that more than other categories, these patients have trouble in theory of mind tasks, such as recognition and attribution of social and emotional responses [63].…”

Section: Discussionmentioning

confidence: 99%

The Neural Substrate of Predictive Motor Timing in Spinocerebellar Ataxia

et al. 2010

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The neural mechanisms involved in motor timing are subcortical, involving mainly cerebellum and basal ganglia. However, the role played by these structures in predictive motor timing is not well understood. Unlike motor timing, which is often tested using rhythm production tasks, predictive motor timing requires visuo-motor coordination in anticipation of a future event, and it is evident in behaviors such as catching a ball or shooting a moving target. We examined the role of the cerebellum and striatum in predictive motor timing in a target interception task in healthy (n = 12) individuals and in subjects (n = 9) with spinocerebellar ataxia types 6 and 8. The performance of the healthy subjects was better than that of the spinocerebellar ataxia. Successful performance in both groups was associated with increased activity in the cerebellum (right dentate nucleus, left uvula (lobule V), and lobule VI), thalamus, and in several cortical areas. The superior performance in the controls was related to activation in thalamus, putamen (lentiform nucleus) and cerebellum (right dentate nucleus and culmen-lobule IV), which were not activated either in the spinocerebellar subjects or within a subgroup of controls who performed poorly. Both the cerebellum and the basal ganglia are necessary for the predictive motor timing. The degeneration of the cerebellum associated with spinocerebellar types 6 and 8 appears to lead to quantitative rather than qualitative deficits in temporal processing. The lack of any areas with greater activity in the spinocerebellar group than in controls suggests that limited functional reorganization occurs in this condition.

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Section: Discussionmentioning

confidence: 99%

The Neural Substrate of Predictive Motor Timing in Spinocerebellar Ataxia

et al. 2010

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“…Globas et al failed to provide clear evidence for cognitive deficits in SCA6 patients [11]. However, we recently revealed significant impaired visual memory and verbal fluency in genetically confirmed SCA6 patients [12], suggesting that SCA6 patients have prefrontal dysfunction. Previous studies have indicated hypoperfusion restricted to the cerebellum [10], however these results may be attributable to the small sample size of the SCA6 patients used.…”

Section: Introductionmentioning

confidence: 96%

“…(Table 1) Thirteen genetically confirmed SCA6 patients from thirteen families and 21 control subjects were enrolled in this study (Table 1). These 13 SCA6 patients were from the same pool of 18 patients who were examined in our previous report [12]. All were native Japanese speakers.…”

Section: Introductionmentioning

confidence: 99%

Prefrontal hypoperfusion and cognitive dysfunction correlates in spinocerebellar ataxia type 6

Kawai¹,

Suenaga²,

Watanabe³

et al. 2008

Journal of the Neurological Sciences

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“…Globas et al [50] examined cognitive function in 12 patients with genetically confirmed SCA6, but found only mild deficits in fronto-executive tasks that were not significantly different from those in controls. However, Suenaga et al [51] examined cognitive function in 18 patients with genetically confirmed SCA 6, and showed that verbal fluency and immediate visual memory were markedly impaired. These cognitive dysfunctions did not correlate with CAG repeat length.…”

Section: Spinocerebellar Ataxia Typementioning

confidence: 99%

Cognitive Impairment in Spinocerebellar Degeneration

et al. 2009

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It has been reported that patients with spinocerebellar degenerations (SCDs) have cognitive dysfunction as well as limb and truncal ataxia, dysarthria and dysphagia. We review cognitive dysfunction in common types of SCD, including spinocerebellar ataxia types 1, 2, 3, 6, and 17, dentatorubral-pallidoluysian atrophy, Friedreich’s ataxia, and multiple system atrophy. There are few studies that address cognitive function in SCD. Although there are few comparison studies among the various SCDs, cognitive dysfunction may be more common and severe in spinocerebellar ataxia type 17 and dentatorubral-pallidoluysian atrophy. While cognitive dysfunction in SCD appears to represent frontal dysfunction, the mechanisms of cognitive dysfunction have not been directly clarified. Nevertheless, various lesions, including those in the cerebrocerebellar circuitry, cortico-striatal-thalamocortical circuitry, and the frontal lobe, may influence cognitive function to various degrees for each disease.

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Cognitive impairment in spinocerebellar ataxia type 6

Abstract: Our results demonstrate that specific cognitive deficits occur in patients with SCA6, independent of ataxic motor dysfunction. These deficits may reflect disruption of cortico-cerebellar circuits.

Cited by 67 publications

References 38 publications

The Neural Substrate of Predictive Motor Timing in Spinocerebellar Ataxia

The Neural Substrate of Predictive Motor Timing in Spinocerebellar Ataxia

Prefrontal hypoperfusion and cognitive dysfunction correlates in spinocerebellar ataxia type 6

Cognitive Impairment in Spinocerebellar Degeneration

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