Cerebellar dysfunction in rodent models with dystonia, tremor, and ataxia

Meike E. van der Heijden1,2* and Roy V. Sillitoe1,2,3,4,5*

• 1Department of Pathology & Immunology, Baylor College of Medicine, Houston, TX, United States
• 2Jan and Dan Duncan Neurological Research Institute at Texas Children’s Hospital, Houston, TX, United States
• 3Department of Neuroscience, Baylor College of Medicine, Houston, TX, United States
• 4Program in Developmental Biology, Baylor College of Medicine, Houston, TX, United States
• 5Development, Disease Models & Therapeutics Graduate Program, Baylor College of Medicine, Houston, TX, United States

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.

For the full article visit https://www.frontierspartnerships.org/articles/10.3389/dyst.2023.11515/full