Cerebellar Dysfunction in Rodent Models With Dystonia, Tremor, and Ataxia

Authors

Meike E van der Heijden
Roy V Sillitoe

Publication Date

1-1-2023

Journal

Dystonia

DOI

10.3389/dyst.2023.11515

PMID

38105800

PMCID

PMC10722573

PubMedCentral® Posted Date

12-15-2023

PubMedCentral® Full Text Version

Author MSS

Published Open-Access

yes

Keywords

dystonia, tremor, cerebellum, rodent, ataxia

Abstract

Dystonia is a movement disorder characterized by involuntary co- or over-contractions of the muscles, which results in abnormal postures and movements. These symptoms arise from the pathophysiology of a brain-wide dystonia network. There is mounting evidence suggesting that the cerebellum is a central node in this network. For example, manipulations that target the cerebellum cause dystonic symptoms in mice, and cerebellar neuromodulation reduces these symptoms. Although numerous findings provide insight into dystonia pathophysiology, they also raise further questions. Namely, how does cerebellar pathophysiology cause the diverse motor abnormalities in dystonia, tremor, and ataxia? Here, we describe recent work in rodents showing that distinct cerebellar circuit abnormalities could define different disorders and we discuss potential mechanisms that determine the behavioral presentation of cerebellar diseases.