Dystonia is the third most common movement disorder. It causes debilitating twisting postures that are accompanied by repetitive and sometimes intermittent co- or over-contractions of agonist and antagonist muscles. Historically diagnosed as a basal ganglia disorder, dystonia is increasingly considered a network disorder involving various brain regions including the cerebellum. In certain etiologies of dystonia, aberrant motor activity is generated in the cerebellum and the abnormal signals then propagate through a "dystonia circuit" that includes the thalamus, basal ganglia, and cerebral cortex. Importantly, it has been reported that non-motor defects can accompany the motor symptoms; while their severity is not always correlated, it is hypothesized that common pathways may nevertheless be disrupted. In particular, circadian dysfunction and disordered sleep are common non-motor patient complaints in dystonia. Given recent evidence suggesting that the cerebellum contains a circadian oscillator, displays sleep-stage-specific neuronal activity, and sends robust long-range projections to several subcortical regions involved in circadian rhythm regulation, disordered sleep in dystonia may result from cerebellum-mediated dysfunction of the dystonia circuit. Here, we review the evidence linking dystonia, cerebellar network dysfunction, and cerebellar involvement in sleep. Together, these ideas may form the basis for the development of improved pharmacological and surgical interventions that could take advantage of cerebellar circuitry to restore normal motor function as well as non-motor (sleep) behaviors in dystonia.
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http://dx.doi.org/10.3389/dyst.2022.10691 | DOI Listing |
Parkinsonism Relat Disord
January 2025
Institute of Systems Motor Science, University Medical Center Schleswig-Holstein, University of Lübeck, Lübeck, Germany; Center for Brain, Behavior, and Metabolism, University of Lübeck, Lübeck, Germany; Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, United States; Center of Rare Diseases, University Medical Center Schleswig-Holstein, Lübeck, Germany. Electronic address:
Background: Functional neurological movement disorders are common and disabling. Little is known about their coexistence with other non-functional movement disorders and their impact on the general disease burden.
Objectives: Investigating frequency and characteristics of functional movement disorders in GCH1-positive dopa-responsive dystonia patients.
Parkinsonism Relat Disord
January 2025
Human Sensorimotor Control Laboratory, School of Kinesiology, University of Minnesota, USA; Center for Clinical Movement Science, University of Minnesota, USA.
Introduction: Cervical dystonia (CD) is characterized by involuntary neck muscle spasms that lead to abnormal head movements or postures. It is associated with somatosensory (tactile and proprioceptive) dysfunction. Here we tested whether vibro-tactile stimulation (VTS) of the cervical muscles constitutes a non-invasive form of neuromodulation of the somatosensory system that can provide temporary symptom relief for people with CD.
View Article and Find Full Text PDFPLoS Biol
January 2025
Carney Institute for Brain Science, Department of Cognitive & Psychological Sciences, Brown University, Providence, Rhode Island, United States of America.
The basal ganglia (BG) play a key role in decision-making, preventing impulsive actions in some contexts while facilitating fast adaptations in others. The specific contributions of different BG structures to this nuanced behavior remain unclear, particularly under varying situations of noisy and conflicting information that necessitate ongoing adjustments in the balance between speed and accuracy. Theoretical accounts suggest that dynamic regulation of the amount of evidence required to commit to a decision (a dynamic "decision boundary") may be necessary to meet these competing demands.
View Article and Find Full Text PDFStereotact Funct Neurosurg
January 2025
Introduction: Functional thalamic surgery is known for alleviating isolated focal hand dystonia; however, the optimal target site in the thalamus is not determined. This study aimed to identify effective sites for thalamic deep brain stimulation (DBS) in treating this condition.
Methods: Four patients presenting with focal hand dystonia underwent thalamic DBS.
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