2019 Sep 14. doi: 10.1007/s12311-019-01068-8. [Epub ahead of print]

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

Author information

1
Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK. georgios.argyropoulos@ndcn.ox.ac.uk.
2
Rehabilitation Research Center REVAL, UHasselt, Hasselt, Belgium.
3
Clinical and Cognitive Neurorehabilitation, Center of Neurology and Neurorehabilitation, Klinik Bavaria Kreischa, An der Wolfsschlucht 1-2, 01703, Kreischa, Germany.
4
Department of Psychology, Sapienza University of Rome, Rome, Italy.
5
Ataxia Laboratory, IRCCS Fondazione Santa Lucia, Rome, Italy.
6
Department of Neurology, CHU-Charleroi, 6000, Charleroi, Belgium.
7
Department of Neurosciences, University of Mons, 7000, Mons, Belgium.
8
SPInal REhabilitation Lab (SPIRE), IRCCS Fondazione Santa Lucia, Via Ardeatina 306, 00179, Rome, Italy.
9
Neuro-Robot Rehabilitation Lab, IRCCS Fondazione Santa Lucia, Via Ardeatina 306, 00179, Rome, Italy.
10
Department of Psychology, American University, Washington, DC, 20016, USA.
11
Department of Psychology, Vrije Universiteit Brussel, Brussels, Belgium.
12
Department of Psychology, University of California, Berkeley, CA, USA.
13
Ataxia Unit, Cognitive Behavioral Neurology Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Department of Neurology Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.

Abstract

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.

KEYWORDS:

Affect; Cerebellar cognitive affective syndrome; Cerebellum; Cognition; Emotion; Schmahmann syndrome

PMID:
 
31522332
 
DOI:
 
10.1007/s12311-019-01068-8