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.

Effects of Intensive Exercise on Cognitive Dysfunction in Patients With Pure Cerebellar Degeneration: A Single-Arm Pilot Study

“…Functional imaging studies have shown activation of cerebellar and cortical areas during the verbal fluency task [ 16 ]. In addition, a decrease in verbal fluency in SCD has been reported, supporting our finding [ 7 , 8 , 17 ]. Serial 7 is known to be a test of attention and working memory [ 18 ].…”

Effects of Intensive Exercise on Cognitive Dysfunction in Patients With Pure Cerebellar Degeneration: A Single-Arm Pilot Study

“…Functional imaging studies have shown activation of cerebellar and cortical areas during the verbal fluency task [ 16 ]. In addition, a decrease in verbal fluency in SCD has been reported, supporting our finding [ 7 , 8 , 17 ]. Serial 7 is known to be a test of attention and working memory [ 18 ].…”

Neurocognitive Changes in Spinocerebellar Ataxia Type 3: A Systematic Review with a Narrative Design

“…A previous review [33] and meta-analysis [5] on the CCAS in general showed that short cognitive screening instruments, such as the MMSE, are not sensitive for the detection of CCAS, but neuropsychological tests assessing a range of cognitive domains are able to distinguish CCAS patients from controls. Our review findings are in agreement with the previous review [33], as the TMT-B (Shifting) [20,23], DS Backward [22][23][24] (Working Memory), phonemic [17,19,20,[22][23][24] and semantic VF [17,23,24] (Language), TMT-A [20,24] (Attention/Processing Speed), and RAVLT [23,25] (word list; Episodic Memory) were impaired in patients with SCA3. Other tests that were found to be impaired in our review included the Stroop Color-Word Test [15,18,20,23] (Inhibition) and Wechsler Memory Scale subtests (i.e., VR [16] Episodic memory), in line with the results of the meta-analysis by Ahmadian and colleagues [5].…”

PET study of brain acetylcholinesterase in cerebellar degenerative disorders

“…Neuropsychological testing with FAB showed significant frontal lobe dysfunction in MSA‐C and SCA‐3 patients compared with healthy control subjects in this study. MSA patients do not typically present with dementia, but previous studies with neuropsychological examination have revealed frontal lobe dysfunction, such as executive dysfunction and apathy in patients with MSA,22 and in SCA‐3 patients23 in keeping with our results. Recently, MRI studies in MSA patients showed reduction in gray matter volume in orbitofrontal and midfrontal regions 24.…”

Effects of Intensive Exercise on Cognitive Dysfunction in Patients With Pure Cerebellar Degeneration: A Single-Arm Pilot Study

“…Functional imaging studies have shown activation of cerebellar and cortical areas during the verbal fluency task [ 16 ]. In addition, a decrease in verbal fluency in SCD has been reported, supporting our finding [ 7 , 8 , 17 ]. Serial 7 is known to be a test of attention and working memory [ 18 ].…”

Neurocognitive Changes in Spinocerebellar Ataxia Type 3: A Systematic Review with a Narrative Design

“…A previous review [33] and meta-analysis [5] on the CCAS in general showed that short cognitive screening instruments, such as the MMSE, are not sensitive for the detection of CCAS, but neuropsychological tests assessing a range of cognitive domains are able to distinguish CCAS patients from controls. Our review findings are in agreement with the previous review [33], as the TMT-B (Shifting) [20,23], DS Backward [22][23][24] (Working Memory), phonemic [17,19,20,[22][23][24] and semantic VF [17,23,24] (Language), TMT-A [20,24] (Attention/Processing Speed), and RAVLT [23,25] (word list; Episodic Memory) were impaired in patients with SCA3. Other tests that were found to be impaired in our review included the Stroop Color-Word Test [15,18,20,23] (Inhibition) and Wechsler Memory Scale subtests (i.e., VR [16] Episodic memory), in line with the results of the meta-analysis by Ahmadian and colleagues [5].…”

PET study of brain acetylcholinesterase in cerebellar degenerative disorders

“…Neuropsychological testing with FAB showed significant frontal lobe dysfunction in MSA‐C and SCA‐3 patients compared with healthy control subjects in this study. MSA patients do not typically present with dementia, but previous studies with neuropsychological examination have revealed frontal lobe dysfunction, such as executive dysfunction and apathy in patients with MSA,22 and in SCA‐3 patients23 in keeping with our results. Recently, MRI studies in MSA patients showed reduction in gray matter volume in orbitofrontal and midfrontal regions 24.…”

Effects of Intensive Exercise on Cognitive Dysfunction in Patients With Pure Cerebellar Degeneration: A Single-Arm Pilot Study

“…Functional imaging studies have shown activation of cerebellar and cortical areas during the verbal fluency task [ 16 ]. In addition, a decrease in verbal fluency in SCD has been reported, supporting our finding [ 7 , 8 , 17 ]. Serial 7 is known to be a test of attention and working memory [ 18 ].…”

Neurocognitive Changes in Spinocerebellar Ataxia Type 3: A Systematic Review with a Narrative Design

“…A previous review [33] and meta-analysis [5] on the CCAS in general showed that short cognitive screening instruments, such as the MMSE, are not sensitive for the detection of CCAS, but neuropsychological tests assessing a range of cognitive domains are able to distinguish CCAS patients from controls. Our review findings are in agreement with the previous review [33], as the TMT-B (Shifting) [20,23], DS Backward [22][23][24] (Working Memory), phonemic [17,19,20,[22][23][24] and semantic VF [17,23,24] (Language), TMT-A [20,24] (Attention/Processing Speed), and RAVLT [23,25] (word list; Episodic Memory) were impaired in patients with SCA3. Other tests that were found to be impaired in our review included the Stroop Color-Word Test [15,18,20,23] (Inhibition) and Wechsler Memory Scale subtests (i.e., VR [16] Episodic memory), in line with the results of the meta-analysis by Ahmadian and colleagues [5].…”

PET study of brain acetylcholinesterase in cerebellar degenerative disorders

“…Neuropsychological testing with FAB showed significant frontal lobe dysfunction in MSA‐C and SCA‐3 patients compared with healthy control subjects in this study. MSA patients do not typically present with dementia, but previous studies with neuropsychological examination have revealed frontal lobe dysfunction, such as executive dysfunction and apathy in patients with MSA,22 and in SCA‐3 patients23 in keeping with our results. Recently, MRI studies in MSA patients showed reduction in gray matter volume in orbitofrontal and midfrontal regions 24.…”