American University
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The cerebellar cognitive affective/Schmahmann syndrome : A task force paper

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journal contribution
posted on 2023-08-05, 12:49 authored by Georgios P.D. Argyropoulos, Kim van Dun, Michael Adamaszek, Maria Leggio, Mario Ubaldo Manto, Marcella Masciullo, Marco Molinari, Catherine StoodleyCatherine Stoodley, Frank J. van Overwalle, Richard B. Ivry, Jeremy Dan Schmahmann

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.





Cerebellum, Volume 19, Issue 1, 1 February 2020, Pages 102-125.


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