Focusing Attention on Cognitive Impairment in Spinocerebellar Ataxia

Geschwind, Daniel H.

doi:10.1001/archneur.56.1.20

Cited by 35 publications

(7 citation statements)

References 18 publications

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“…Cognition is involved to varying degrees in the genetically defined SCAs [204]. These deficits conform to the pattern of the CCAS-deficits in executive function, linguistic processing, spatial cognition, verbal and visual memory, and changes in affect.…”

Section: Ccas In the Hereditary Ataxiasmentioning

confidence: 76%

The Cerebellar Cognitive Affective/Schmahmann Syndrome: a Task Force Paper

et al. 2019

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Sporadically advocated over the last two centuries, a cerebellar role in cognition and affect has been rigorously established in the past few decades. In the clinical domain, such progress is epitomized by the "cerebellar cognitive affective syndrome" ("CCAS") or "Schmahmann syndrome." Introduced in the late 1990s, CCAS reflects a constellation of cerebellar-induced sequelae, comprising deficits in executive function, visuospatial cognition, emotion-affect, and language, over and above speech. The CCAS thus offers excellent grounds to investigate the functional topography of the cerebellum, and, ultimately, illustrate the precise mechanisms by which the cerebellum modulates cognition and affect. The primary objective of this task force paper is thus to stimulate further research in this area. After providing an up-to-date overview of the fundamental findings on cerebellar neurocognition, the paper substantiates the concept of CCAS with recent evidence from different scientific angles, promotes awareness of the CCAS as a clinical entity, and examines our current insight into the therapeutic options available. The paper finally identifies topics of divergence and outstanding questions for further research.

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Section: Ccas In the Hereditary Ataxiasmentioning

confidence: 76%

The Cerebellar Cognitive Affective/Schmahmann Syndrome: a Task Force Paper

et al. 2019

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“…It is characterized by impairments in executive function, visual spatial processing, linguistic deficits, and affective dysregulation (Table 1). It can be prominent in developmental cerebellar disorders [63, 64] and following acute lesions such as stroke, hemorrhage, and cerebellitis [56], and it may be relatively subtle but still clinically relevant in late onset hereditary ataxias [58, 65]. …”

Section: Dysmetria Of Thought: a Unifying Hypothesis For The Cerebellmentioning

confidence: 99%

Consensus Paper: The Cerebellum's Role in Movement and Cognition

Koziol¹,

Budding²,

Andreasen³

et al. 2013

Cerebellum

919

586

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While the cerebellum's role in motor function is well recognized, the nature of its concurrent role in cognitive function remains considerably less clear. The current consensus paper gathers diverse views on a variety of important roles played by the cerebellum across a range of cognitive and emotional functions. This paper considers the cerebellum in relation to neurocognitive development, language function, working memory, executive function, and the development of cerebellar internal control models and reflects upon some of the ways in which better understanding the cerebellum's status as a “supervised learning machine” can enrich our ability to understand human function and adaptation. As all contributors agree that the cerebellum plays a role in cognition, there is also an agreement that this conclusion remains highly inferential. Many conclusions about the role of the cerebellum in cognition originate from applying known information about cerebellar contributions to the coordination and quality of movement. These inferences are based on the uniformity of the cerebellum's compositional infrastructure and its apparent modular organization. There is considerable support for this view, based upon observations of patients with pathology within the cerebellum.

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“…These genetically mediated disorders cause progressive motor disability, degrade quality of life, and in many instances, lead to early death. It is now apparent that cognition is involved to varying degrees in every gene-defined SCA [154]. These deficits conform to the pattern of the CCASexecutive function, linguistic processing, spatial cognition, verbal and visual memory and changes in affect.…”

Section: The Hereditary Ataxiasmentioning

confidence: 83%

The Cerebellum and Cognition

Nixon¹

2000

Journal of Psychophysiology

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A B S T R A C TWhat the cerebellum does to sensorimotor and vestibular control, it also does to cognition, emotion, and autonomic function. This hypothesis is based on the theories of dysmetria of thought and the universal cerebellar transform, which hold that the cerebellum maintains behavior around a homeostatic baseline, automatically, without conscious awareness, informed by implicit learning, and performed according to context. Functional topography within the cerebellum facilitates the modulation of distributed networks subserving multiple different functions. The sensorimotor cerebellum is represented in the anterior lobe with a second representation in lobule VIII, and lesions of these areas lead to the cerebellar motor syndrome of ataxia, dysmetria, dysarthria and impaired oculomotor control. The cognitive / limbic cerebellum is in the cerebellar posterior lobe, with current evidence pointing to three separate topographic representations, the nature of which remain to be determined. Posterior lobe lesions result in the cerebellar cognitive affective syndrome (CCAS), the hallmark features of which include deficits in executive function, visual spatial processing, linguistic skills and regulation of affect. The affective dyscontrol manifests in autism spectrum and psychosis spectrum disorders, and disorders of emotional control, attentional control, and social skill set. This report presents an overview of the rapidly growing field of the clinical cognitive neuroscience of the cerebellum. It commences with a brief historical background, then discusses tract tracing experiments in animal models and functional imaging observations in humans that subserve the cerebellar contribution to neurological function. Structure function correlation studies following focal cerebellar lesions in adults and children permit a finer appreciation of the functional topography and nature of the cerebellar motor syndrome, cerebellar vestibular syndrome, and the third cornerstone of clinical ataxiologythe cerebellar cognitive affective syndrome. The ability to detect the CCAS in real time in clinical neurology with a brief and validated scale should make it possible to develop a deeper understanding of the clinical consequences of cerebellar lesions in a wide range of neurological and neuropsychiatric disorders with a link to the cerebellum. What is less well known, or not appreciated at all, is that a half https://doi.Neuroscience Letters xxx (xxxx) xxx-xxx 0304-3940/

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Focusing Attention on Cognitive Impairment in Spinocerebellar Ataxia

Cited by 35 publications

References 18 publications

The Cerebellar Cognitive Affective/Schmahmann Syndrome: a Task Force Paper

The Cerebellar Cognitive Affective/Schmahmann Syndrome: a Task Force Paper

Consensus Paper: The Cerebellum's Role in Movement and Cognition

The Cerebellum and Cognition

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