Action perception recruits the cerebellum and is impaired in patients with spinocerebellar ataxia

Using a combination of neuroimaging and behavioural studies, Abdelgabar et al. show that the cerebellum helps us perceive the actions of others. Disorders such as spinocerebellar ataxia type 6, which disrupt cerebellar functioning, impair our ability to perceive the kinematics of other people’s actions, with potential implications for social cognition.

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Action perception recruits the cerebellum and is impaired in spinocerebellar ataxia patients

10.1101/480293 ◽

2018 ◽

Cited By ~ 1

Author(s):

Abdel R. Abdelgabar ◽

Judith Suttrup ◽

Robin Broersen ◽

Ritu Bhandari ◽

Samuel Picard ◽

...

Keyword(s):

Social Cognition ◽

Spinocerebellar Ataxia ◽

Neurological Disorders ◽

Healthy Subjects ◽

Spinocerebellar Ataxia Type ◽

Action Perception ◽

Spinocerebellar Ataxia Type 6 ◽

Human Cerebellum ◽

Cerebellar Disorders

AbstractOur cerebellum has been proposed to generate prediction signals that may help us plan and execute our motor programs. However, to what extent our cerebellum is also actively involved in perceiving the action of others remains to be elucidated. Using fMRI, we show here that observing goal-directed hand actions of others bilaterally recruits cerebellar Lobules VI, VIIb and VIIIa. Moreover, whereas healthy subjects (n=31) were found to be able to discriminate subtle differences in the kinematics of observed limb movements of others, patients suffering from spinocerebellar ataxia type 6 (SCA6; n=21) were severely impaired in performing such tasks. Our data suggest that the human cerebellum is actively involved in perceiving the kinematics of the hand actions of others and that SCA6 patients’ deficits include a difficulty in perceiving the actions of other individuals. This finding alerts us to the fact that cerebellar disorders can alter social cognition. Given that impairments in social cognition have been reported to be one of the most debilitating consequences of neurological disorders, this finding may be relevant to improving the quality of life of patients and their families.

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Frequency analysis of autosomal dominant cerebellar ataxias in Japanese patients and clinical characterization of spinocerebellar ataxia type 6

Clinical Genetics ◽

10.1034/j.1399-0004.1998.531530104.x ◽

1998 ◽

Vol 53 (1) ◽

pp. 13-19 ◽

Cited By ~ 51

Author(s):

Hidetaka Watanabe ◽

Fumiaki Tanaka ◽

Michiyo Matsumoto ◽

Manabu Doyu ◽

Tetsuo Ando ◽

...

Keyword(s):

Spinocerebellar Ataxia ◽

Frequency Analysis ◽

Autosomal Dominant ◽

Japanese Patients ◽

Spinocerebellar Ataxia Type ◽

Spinocerebellar Ataxia Type 6 ◽

Cerebellar Ataxias

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The neurobiological underpinning of the social cognition impairments in patients with spinocerebellar ataxia type 2

Cortex ◽

10.1016/j.cortex.2020.12.027 ◽

2021 ◽

Author(s):

Silvia Clausi ◽

Giusy Olivito ◽

Libera Siciliano ◽

Michela Lupo ◽

Marco Bozzali ◽

...

Keyword(s):

Social Cognition ◽

Spinocerebellar Ataxia ◽

Spinocerebellar Ataxia Type ◽

Spinocerebellar Ataxia Type 2 ◽

The Social

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Decreased metabotropic glutamate receptor type 1 availability in a patient with spinocerebellar ataxia type 6: A 11C-ITMM PET study

Journal of the Neurological Sciences ◽

10.1016/j.jns.2015.05.041 ◽

2015 ◽

Vol 355 (1-2) ◽

pp. 202-205 ◽

Cited By ~ 4

Author(s):

Kenji Ishibashi ◽

Yoshiharu Miura ◽

Kinya Ishikawa ◽

Kenji Ishii ◽

Kiichi Ishiwata

Keyword(s):

Spinocerebellar Ataxia ◽

Glutamate Receptor ◽

Metabotropic Glutamate Receptor ◽

Receptor Type ◽

Spinocerebellar Ataxia Type ◽

Metabotropic Glutamate ◽

Spinocerebellar Ataxia Type 6

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Bovine CACNA1A gene and comparative analysis of the CAG repeats associated to human spinocerebellar ataxia type-6

Gene ◽

10.1016/j.gene.2006.06.003 ◽

2006 ◽

Vol 380 (1) ◽

pp. 54-61 ◽

Cited By ~ 2

Author(s):

Eva Andrés-Mateos ◽

Jesús Cruces ◽

Jaime Renart ◽

Luisa M. Solís-Garrido ◽

Rocío Serantes ◽

...

Keyword(s):

Comparative Analysis ◽

Spinocerebellar Ataxia ◽

Cag Repeats ◽

Spinocerebellar Ataxia Type ◽

Spinocerebellar Ataxia Type 6 ◽

Cacna1a Gene

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Spinocerebellar ataxia type 6 knockin mice develop a progressive neuronal dysfunction with age-dependent accumulation of mutant CaV2.1 channels

Proceedings of the National Academy of Sciences ◽

10.1073/pnas.0804350105 ◽

2008 ◽

Vol 105 (33) ◽

pp. 11987-11992 ◽

Cited By ~ 99

Author(s):

K. Watase ◽

C. F. Barrett ◽

T. Miyazaki ◽

T. Ishiguro ◽

K. Ishikawa ◽

...

Keyword(s):

Spinocerebellar Ataxia ◽

Spinocerebellar Ataxia Type ◽

Neuronal Dysfunction ◽

Spinocerebellar Ataxia Type 6 ◽

Age Dependent

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Transient cerebellar alterations during development prior to obvious motor phenotype in a mouse model of spinocerebellar ataxia type 6

The Journal of Physiology ◽

10.1113/jp273184 ◽

2016 ◽

Vol 595 (3) ◽

pp. 949-966 ◽

Cited By ~ 13

Author(s):

Sriram Jayabal ◽

Lovisa Ljungberg ◽

Alanna J. Watt

Keyword(s):

Mouse Model ◽

Spinocerebellar Ataxia ◽

Spinocerebellar Ataxia Type ◽

Spinocerebellar Ataxia Type 6 ◽

Motor Phenotype

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Frequency analysis of autosomal dominant cerebellar ataxias in Japanese patients and clinical characterization of spinocerebellar ataxia type 6

Clinical Genetics ◽

10.1111/j.1399-0004.1998.tb02575.x ◽

2008 ◽

Vol 53 (1) ◽

pp. 13-19

Author(s):

Hidetaka Watanabe ◽

Fumiaki Tanaka ◽

Michiyo Matsumoto ◽

Manabu Doyu ◽

Tetsuo Ando ◽

...

Keyword(s):

Spinocerebellar Ataxia ◽

Frequency Analysis ◽

Autosomal Dominant ◽

Japanese Patients ◽

Spinocerebellar Ataxia Type ◽

Spinocerebellar Ataxia Type 6 ◽

Cerebellar Ataxias

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Spinocerebellar ataxia type 6 in Brazil

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2008000500015 ◽

2008 ◽

Vol 66 (3b) ◽

pp. 691-694 ◽

Cited By ~ 13

Author(s):

Hélio A.G. Teive ◽

Renato Puppi Munhoz ◽

Salmo Raskin ◽

Lineu César Werneck

Keyword(s):

Cerebellar Ataxia ◽

Spinocerebellar Ataxia ◽

Late Onset ◽

Cag Repeat ◽

Autosomal Dominant Cerebellar Ataxia ◽

Spinocerebellar Ataxia Type ◽

Spinocerebellar Ataxia Type 6 ◽

Voltage Dependent ◽

Pure Cerebellar Ataxia ◽

Japanese Ancestry

Spinocerebellar ataxia type 6 (SCA 6) is an autosomal dominant cerebellar ataxia caused by CAG repeat expansion in the SCA6 gene, a alpha 1A voltage-dependent calcium channel subunit gene on chromosome 19p13. SCA-6 is characterized predominantly by slowly progressive pure cerebellar ataxia with late onset. We report three index patients, with pure, late onset, cerebellar ataxia, belonging to three different Brazilian families, all of them with Japanese ancestry, from Hokkaido island of Japan.

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