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Abstract
Anatomical, physiological and functional neuroimaging studies suggest that the cerebellum
participates in the organization of higher order function, but there are very few
descriptions of clinically relevant cases that address this possibility. We performed
neurological examinations, bedside mental state tests, neuropsychological studies
and anatomical neuroimaging on 20 patients with diseases confined to the cerebellum,
and evaluated the nature and severity of the changes in neurological and mental function.
Behavioural changes were clinically prominent in patients with lesions involving the
posterior lobe of the cerebellum and the vermis, and in some cases they were the most
noticeable aspects of the presentation. These changes were characterized by: impairment
of executive functions such as planning, set-shifting, verbal fluency, abstract reasoning
and working memory; difficulties with spatial cognition including visual-spatial organization
and memory; personality change with blunting of affect or disinhibited and inappropriate
behaviour; and language deficits including agrammatism and dysprosodia. Lesions of
the anterior lobe of the cerebellum produced only minor changes in executive and visual-spatial
functions. We have called this newly defined clinical entity the 'cerebellar cognitive
affective syndrome'. The constellation of deficits is suggestive of disruption of
the cerebellar modulation of neural circuits that link prefrontal, posterior parietal,
superior temporal and limbic cortices with the cerebellum.