A Preliminary Characterisation of Cognition and Social Cognition in Spinocerebellar Ataxia Types 2, 1, and 7

Abstract

Over the last decade, studies have implicated the cerebellum not only in motor functioning, but also in cognition and social cognition. Although some aspects of cognition have been explored in the five most common forms of Spinocerebellar Ataxia (SCA), social cognition in these patients has rarely been examined. The present study provides a preliminary characterisation of the severity of cognitive and social cognitive impairments in patients with SCA2, SCA1 and SCA7 using an identical battery to the one previously used in SCA3 and SCA6 patients for comparison. The cognitive profiles of SCA1 and SCA7 patients were comparable to that of SCA6 patients; SCA1 patients had relatively intact profiles, while SCA7 patients demonstrated only some selective deficits. In contrast, SCA2 patients showed the greatest impairments, similarly to SCA3 patients. On tests of social cognition, SCA2 and SCA7 patients were impaired on a task of emotion attribution, whereas one SCA1 patient had a Theory of Mind deficit, which has also been documented in SCA3 and SCA6. We provide preliminary evidence that the neuropsychological profiles of SCA patients correspond well with the severity of pathological and clinical features. Moreover, these patients may also have social cognition impairments. Overall, we suggest that there is a degree of heterogeneity in the types of cognitive and social cognitive impairments in SCA patients.

The Cerebellum in Social Cognition

“…A patient with cerebellar atrophy exhibited deficient RMET performance (Parente et al, 2013). Absence of deficits in mental state attribution but altered attribution of emotions to a character of a short story were reported in eight patients with SCA types 1, 2, and 7 (Sokolovsky et al, 2010), with the opposite picture in 15 patients with SCA 3 and 6 (Garrard et al, 2008). This dissociation could reflect distinct pathophysiology in SCA subtypes.…”

Social cognition in neurodegenerative disorders: a systematic review

“…Finally, a few reports have suggested abnormal social cognitive function in patients with spinocerebellar degeneration 4 155 156. These preliminary findings have led to speculations regarding a possible role for the cerebellum in social cognition, but more detailed investigations are needed.…”

“…Initial research in social cognition focused on autistic spectrum disorders, frontal lobe injury and other disorders where difficulties in social skills represented a core feature. More recently, abnormalities in social cognition have been reported in a multiple neurological conditions ranging from Alzheimer's disease to spinocerebellar ataxia 3 4…”

Theory of Mind in Amnestic Mild Cognitive Impairment: An fMRI Study

“…In fact, after being confined only to motor planning and control for many years, the cerebellum is now considered to be involved in high-order cognitive functions (reasoning, language processing, working memory, ToM) due to its connections with the frontal lobes [89][90][91]. In particular, a malfunctioning of the frontal node of the ToM network was found in patients with progressive ataxia and cognitive dysfunction [92][93][94], as well as in patients with bilateral cerebellar lesions [95].…”