Abstract
Schmahmann’s syndrome represents a novel clinical condition consisting of a constellation of cognitive and affective deficits following cerebellar disease. The complex was first described in 1998 as cerebellar cognitive affective syndrome (CCAS) on the basis of a careful neurological examination, detailed bedside mental state tests, neuropsychological investigations and anatomical neuroimaging of a group of 20 patients with focal cerebellar disorders. The syndrome was characterized by four clusters of symptoms including: (a) impairment of executive functions such as planning, set-shifting, verbal fluency, abstract reasoning and working memory, (b) impaired visuo-spatial cognition, (c) personality changes with blunting of affect or abnormal behaviour, and (d) language deficits including agrammatism, wordfinding disturbances, disruption of language dynamics and dysprosodia. This complex of neurocognitive and behavioural-affective symptoms was ascribed to a functional disruption of the reciprocal pathways that connect the cerebellum with the limbic circuitry and the prefrontal, temporal and parietal association cortices. With the introduction of Schmahmann’s syndrome, clinical ataxiology has found its third cornerstone, the two others being the cerebellar motor syndrome (CMS) mainly delineated by the pioneer French and English neurologists of the 19th and early 20th century, and the vestibulo-cerebellar syndrome (VCS) consisting of ocular instability, deficits of oculomotor movements and ocular misalignment.
Highlights
A wealth of current evidence derived from detailed neuroanatomical investigations, functional neuroimaging studies and in-depth neuropsychological assessments of patients with cerebellar disorders has brought to the fore that the cerebellum is implicated in sensorimotor function and plays a cardinal role in the modulation of cognitive and affective processes [1]
The cerebellum has been considered as a pure motor controller for many decades and neurological examination of cerebellar disorders has long been exclusively directed to oculomotor disturbances, speech motor deficits, limbs incoordination and postural/gait difficulties [2]
We briefly review the anatomy of the cerebellum, summarize the cerebellar motor syndrome (CMS) and vestibulo-cerebellar syndrome (VCS), and subsequently report on the third milestone reached by J.D
Summary
A wealth of current evidence derived from detailed neuroanatomical investigations, functional neuroimaging studies and in-depth neuropsychological assessments of patients with cerebellar disorders has brought to the fore that the cerebellum is implicated in sensorimotor function and plays a cardinal role in the modulation of cognitive and affective processes [1]. Background A wealth of current evidence derived from detailed neuroanatomical investigations, functional neuroimaging studies and in-depth neuropsychological assessments of patients with cerebellar disorders has brought to the fore that the cerebellum is implicated in sensorimotor function and plays a cardinal role in the modulation of cognitive and affective processes [1].
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