2009
DOI: 10.1590/s1980-57642009dn30300002
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Abstract: Spinocerebellar ataxias (SCAs) comprise a heterogeneous group of complex neurodegenerative diseases, characterized by the presence of progressive cerebellar ataxia, associated or otherwise with ophthalmoplegia, pyramidal signs, extrapyramidal features, pigmentary retinopathy, peripheral neuropathy, cognitive dysfunction and dementia.ObjectiveTo verify the presence of cognitive dysfunction among the main types of SCA described in the literature.Methodsthe review was conducted using the search system of the PUBM… Show more

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Cited by 17 publications
(15 citation statements)
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“…ID was associated to SCA 13 (due to KCNC3 defect) [55,56] and, according to the present review, to KCND3 defect (early-onset presentation).…”
Section: Discussionsupporting
confidence: 55%
See 1 more Smart Citation
“…ID was associated to SCA 13 (due to KCNC3 defect) [55,56] and, according to the present review, to KCND3 defect (early-onset presentation).…”
Section: Discussionsupporting
confidence: 55%
“…Cognitive decline and ID are non-motor symptoms described in several types of SCAs [ 55 , 56 ]. A recent work attempted to predict type and severity of cognitive issues in the expanded-polyglutamine tracts SCAs according to the underlying genetic diagnosis (i.e., SCA type 1, 2, 3, 6, 7, 8, and 17 and dentato pallidoluysian atrophy) [ 57 ].…”
Section: Discussionmentioning
confidence: 99%
“…Although the effects of cerebellar stimulation on cognitive functions have been abundantly studied in healthy controls [27], there is a lack of studies assessing the effect on cognition an emotions in patients with cerebellar ataxia. It is now clear that degenerative ataxias frequently have an impairment in several cognitive domains [95], which have been encompassed in the cerebellar cognitive affective syndrome, characterized by deficits in executive functions, linguistic processing, spatial cognition, and affect regulation [96,97].…”
Section: Discussionmentioning
confidence: 99%
“…As a member of the FGF homologous factor family, FGF14 is expressed in developing and mature nervous systems (1,2), in which it maintains normal nervous system function via its regulation of neuronal plasticity and excitability. Research has discovered that mutations in the FGF14 gene are responsible for the neurodegenerative condition spinocerebellar ataxia (SAC27) (3), and that FGF14 deficiency impairs the maturation of hippocampal dentate gyrus cells, which may result in schizophrenia (4,5).…”
Section: Introductionmentioning
confidence: 99%