Cognitive Impairment in Spinocerebellar Degeneration

Abstract

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.

Cognitive Impairment in Native Chinese with Spinocerebellar Ataxia Type 3

“…Furthermore, the impairment on not only phonemic but also semantic verbal fluency was also observed in the current study, which was different from the results of previous studies in an alphabetic system [8,10]. The semantic fluency task was associated with the involvement of left parietal (middle frontal gyrus) and temporal lobes (fusiform gyrus) [53].…”

“…Impaired executive function has been reported in previous studies [8,10]. In the current study, 100% of subjects with SCA3 displayed impairment in the SCWT Stroop III and significantly impaired SIE, which indicated the disruption of cognitive flexibility and inhibitory control [8].…”

“…Phonemic verbal fluency has been reported to be impaired in SCA3 in a series of studies [8,10,12]. It has been reported that greater activation was found in the left precentral and inferior frontal gyrus for phonemic fluency [53].…”

“…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]. Meanwhile, whether the cognitive dysfunction is correlated with ataxia is still unclear.…”

Cerebellar Cognitive Affective Syndrome and Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay: A Report of Two Male Sibs

“…As for the cognitive deficits that are reported in the various hereditary ataxias, it has to be underlined that these dysfunctions result from a disorganization of cerebrocerebellar circuitries at different levels, although a separate involvement of prefrontal areas cannot be excluded yet [18,19]. In the brothers we present, MRI scanning also showed mild global cerebral atrophy.…”