Cognitive Impairments in Machado-Joseph Disease

Kawai, Yohei; Takeda, Akinori; Abe, Yuji; Washimi, Yukihiko; Tanaka, Fumiaki; Sobue, Gen

doi:10.1001/archneur.61.11.1757

Cited by 85 publications

(106 citation statements)

References 26 publications

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“…Zawacki et al [35] gave 6 individuals with SCA3 a battery of neuropsychological tests, and found relative impairments on timed verbal attention, verbal fluency and set-shifting. Kawai et al [36] examined 16 genetically confirmed SCA3 patients using neuropsychological tests, and found more extensive cognitive impairments, including those of verbal and visual memory, visuospatial and constructional abilities and verbal fluency. None of these impairments correlated with CAG repeat length or disease duration.…”

Section: Spinocerebellar Ataxia Type 3 (Machado-joseph Disease)mentioning

confidence: 99%

“…None of these impairments correlated with CAG repeat length or disease duration. It was also reported that depressive symptoms and apathy were common in SCA3 patients [13,35,36,37]. Recently, it was revealed that SCA3 patients were impaired on a Theory of Mind task, although they were not impaired on tasks requiring attribution of emotions or judgments of behavior in social situations [38].…”

Section: Spinocerebellar Ataxia Type 3 (Machado-joseph Disease)mentioning

confidence: 99%

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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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Section: Spinocerebellar Ataxia Type 3 (Machado-joseph Disease)mentioning

confidence: 99%

Section: Spinocerebellar Ataxia Type 3 (Machado-joseph Disease)mentioning

confidence: 99%

Cognitive Impairment in Spinocerebellar Degeneration

et al. 2009

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“…Although spinocerebellar ataxia type 3 (SCA3) is characterized by prominent cerebellar ataxia and atrophy [6], recent pathological and imaging studies on SCA3 have described damage not only confined to the cerebellum, but also including the gray matter of the sensory system, the cerebello-thalamocortical motor loop, the basal ganglia-thalamocortical motor loop, the ingestion-related and precerebellar brain stem system, etc. [7].…”

Section: Introductionmentioning

confidence: 99%

“…[7]. Several studies have reported cognitive and psychiatric deficits in patients with SCA3, mainly in the domains of verbal and visual memory, verbal attention, verbal fluency, executive functioning, as well as visuospatial and visuoconstructive abilities [6,8,9]. Nevertheless, none of these cognitive impairments was found to correlate with CAG repeat length in SCA3 [10].…”

Section: Introductionmentioning

confidence: 99%

“…Burk et al [11] reported that scores on digit span and verbal fluency tasks were weakly but significantly inversely correlated with scores for dysarthria. Kawai et al [6] reported a significant correlation between category fluency and ataxia severity but without further analysis of details of ataxia. Conversely, Klinke et al [12] found no correlation between ataxia and cognition in a sample of 15 in 2010 and Braga-Neto et al [8] did not address this issue in 2012.…”

Section: Introductionmentioning

confidence: 99%

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Cognitive Impairment in Native Chinese with Spinocerebellar Ataxia Type 3

Chen

Hou

et al. 2014

Eur Neurol

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Background: Previous studies have shown cognitive impairment in patients with spinocerebellar ataxia type 3 (SCA3). However, there is a lack of data on Chinese patients with SCA3. Method: We investigated 22 native Chinese with SCA3 and 18 controls matched for age, education as well as mental status. Cognitive assessments were carefully carried out to measure verbal fluency, memory, attention, executive function, visuospatial and visuoconstructive functions. Results: The most common impairments of cognition in native Chinese with SCA3 were disruption of phonemic verbal fluency and frontal executive dysfunction. Deficits in semantic fluency were detected in about 31.8% patients. Impaired visuospatial function and verbal memory were also found in native Chinese with SCA3. The degree of ataxia, CAG repeat length and education were found to correlate with cognitive performance. Multivariate binary logistic regression suggested that an oculomotor disorder and depression are predictors of cognitive impairment. Conclusion: Native Chinese with SCA3 had cognitive impairment of frontal executive function, temporal and parietal functions. An oculomotor disorder might be an index of cognitive dysfunction.

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Cerebello‐cerebral resting‐state functional connectivity in spinocerebellar ataxia type 3

Guo

Jiang

Liu

et al. 2022

Human Brain Mapping

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Spinocerebellar ataxia type 3 (SCA3) is a neurodegenerative disorder characterized by progressive motor and nonmotor deficits concomitant with degenerative pathophysiological changes within the cerebellum. The cerebellum is topographically organized into cerebello‐cerebral circuits that create distinct functional networks regulating movement, cognition, and affect. SCA3‐associated motor and nonmotor symptoms are possibly related not only to intracerebellar changes but also to disruption of the connectivity within these cerebello‐cerebral circuits. However, to date, no comprehensive investigation of cerebello‐cerebral connectivity in SCA3 has been conducted. The present study aimed to identify cerebello‐cerebral functional connectivity alterations and associations with downstream clinical phenotypes and upstream topographic markers of cerebellar neurodegeneration in patients with SCA3. This study included 45 patients with SCA3 and 49 healthy controls. Voxel‐based morphometry and resting‐state functional magnetic resonance imaging (MRI) were performed to characterize the cerebellar atrophy and to examine the cerebello‐cerebral functional connectivity patterns. Structural MRI confirmed widespread gray matter atrophy in the motor and cognitive cerebellum of patients with SCA3. We found reduced functional connectivity between the cerebellum and the cerebral cortical networks, including the somatomotor, frontoparietal, and default networks; however, increased connectivity was observed between the cerebellum and the dorsal attention network. These abnormal patterns correlated with the CAG repeat expansion and deficits in global cognition. Our results indicate the contribution of cerebello‐cerebral networks to the motor and cognitive impairments in patients with SCA3 and reveal that such alterations occur in association with cerebellar atrophy. These findings add important insights into our understanding of the role of the cerebellum in SCA3.

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Cognitive Impairments in Machado-Joseph Disease

Abstract: Machado-Joseph disease patients have verbal and visual memory deficits, visuospatial and constructional dysfunction, and verbal fluency deficits, all unrelated to CAG repeat length.

Cited by 85 publications

References 26 publications

Cognitive Impairment in Spinocerebellar Degeneration

Cognitive Impairment in Spinocerebellar Degeneration

Cognitive Impairment in Native Chinese with Spinocerebellar Ataxia Type 3

Cerebello‐cerebral resting‐state functional connectivity in spinocerebellar ataxia type 3

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