Pathophysiology of idiopathic dystonia: findings from genetic animal models

  title={Pathophysiology of idiopathic dystonia: findings from genetic animal models},
  author={Angelika Richter and Wolfgang L{\"o}scher},
  journal={Progress in Neurobiology},
Alternative Approaches to Modeling Hereditary Dystonias
It was found that partial selective pharmacological block of the sodium pumps in the cerebellum and basal ganglia of mice recapitulates all of the salient features of DYT12, including dystonia and parkinsonism induced by stress.
Dystonia and the cerebellum: A new field of interest in movement disorders?
The functional neuroanatomy of dystonia
Experimental therapeutics for dystonia
Rodent models for dystonia research: Characteristics, evaluation, and utility
A recent workshop addressed four main issues affecting those who conduct dystonia research with animal models, including the different ways in which dystonic disorders can be modeled in rodents, key features that constitute a useful model, methods used in the evaluation of these models, and recommendations for future research.


Neuropathological studies in a mutant hamster model of paroxysmal dystonia
The lack of neuropathological alterations related to dySTONic movements in the present study in dtsz hamsters is comparable to most cases of human hereditary or idiopathic dystonia, which show dystonic movementsIn the absence of morphological alterations.
Striatal [18F]dopa uptake in familial idiopathic dystonia.
To determine whether there is impaired function of the nigro-striatal dopaminergic terminals in ITD, 11 subjects with generalized ITD and a positive family history and three presumed carriers of the ITD gene were studied, suggesting that [18F]dopa PET is unsuitable as a screening tool for ITD.
Advances in the Understanding of Neural Mechanisms in Movement Disorders
Primate models of a spectrum of movement disorders are developed and the neural circuitry which is responsible for mediating them are elucidated and the underlying characteristic changes in neural activity in each of the affected basal ganglia nuclei are defined.
Neuropharmacological correlates of the motor syndrome of the genetically dystonic (dt) rat.
The behavioral syndrome of the dt rat is consistent with a cerebellar defect, and it has been suggested that the cerebellum is important for the continuing calibration of coordinated motor behavior.
A dystonia-like syndrome after neuropeptide (MSH/ACTH) stimulation of the rat locus ceruleus.
  • Y. Jacquet
  • Biology, Psychology
    Advances in neurology
  • 1988
Experimental results indicated that peptidergic stimulation of the LC resulted in a NE-mediated inhibition of cerebellar Purkinje cells located at terminals of the ceruleo-cerebellar pathway.
Cerebellectomy Eliminates the Motor Syndrome of the Genetically Dystonic Rat
The results of this study provide the first evidence that the abnormalities detected in the cerebellum of the dt rat are causally related to the motor syndrome and suggest that abnormal cerebellar output may contribute to the expression of motor signs in some human dystonias.
The functional anatomy of basal ganglia disorders
SSRIs and movement disorders: is serotonin the culprit?
While 5HT may be involved in movement disorders associated with use of the SSRIs, some of the adverse effects of these drugs could be due to direct activation of sigma 2 receptors in the rubro‐cerebellar circuit.