Affiliations: Department of Neuropsychology, National Hospital for
Neurology and Neurosurgery, Queen Square, London, UK | Department of Molecular Neuroscience, Institute of
Neurology, UCL, Queen Square, London, UK | Dipartimento Di Psicologia, Università Degli Studi
Di Palermo, Palermo, Italy
Note: [] Corresponding author: Prof. Lisa Cipolotti, Department of
Neuropsychology, Box 37, National Hospital for Neurology and Neurosurgery,
Queen Square, WC1N 3BG, London, UK. Tel.: +91 020 7829 8793; Fax: +91 080 7813
2516; E-mail: l.cipolotti@ion.ucl.ac.uk
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.
Keywords: Spinocerebellar ataxia (SCA), cognition, theory of mind, emotion
