News

  • August
    31st

    The anatomical basis of dystonia: Current view using neuroimaging.

    Lehéricy S, Tijssen MA, Vidailhet M, Kaji R, Meunier S. - August 31, 2013

    This review will consider the knowledge that neuroimaging studies have provided to the understanding of the anatomy of dystonia. Major advances have occurred in the use of neuroimaging for dystonia in the past 2 decades. At present, the most developed imaging approaches include whole-brain or region-specific studies of structural or diffusion changes, functional imaging using fMRI or positron emission tomography (PET), and metabolic imaging using fluorodeoxyglucose PET. These techniques have provided evidence that regions other than the basal ganglia are involved in dystonia.

  • August
    31st

    Emerging concepts in the physiological basis of dystonia.

    Quartarone A, Hallett M. - August 31, 2013

    Work over the past 2 decades has led to substantial changes in our understanding of dystonia pathophysiology. Three general abnormalities appear to underlie the pathophysiological substrate. The first is a loss of inhibition. This makes sense considering that it may be responsible for the excess of movement and for the overflow phenomena seen in  dystonia. A second abnormality is sensory dysfunction which is related to the mild sensory complaints in patients with focal dystonias and may be responsible for some of the motor dysfunction.

  • August
    31st

    Emerging common molecular pathways for primary dystonia.

    Ledoux MS, Dauer WT, Warner TT. - August 31, 2013

    The dystonias are a group of hyperkinetic movement disorders whose principal cause is neuron dysfunction at 1 or more interconnected nodes of the motor system. The study of genes and proteins that cause familial dystonia provides critical information about the cellular pathways involved in this dysfunction, which disrupts the motor pathways at the systems level. In recent years study of the increasing number of DYT genes has implicated a number of cell functions that appear to be involved in the pathogenesis of dystonia.

  • August
    31st

    Animal models for dystonia.

    Wilson BK, Hess EJ. - August 31, 2013

    Symptomatic animal models have clinical features consistent with human disorders and are often used to identify the anatomical and physiological processes involved in the expression of symptoms and to experimentally demonstrate causality where it would be infeasible in the patient population. Rodent and primate models of dystonia have identified basal ganglia abnormalities, including alterations in striatal GABAergic (ie, transmitting or secreting γ-aminobutyric acid) and dopaminergic transmission.

  • August
    31st

    Engineering animal models of dystonia.

    Oleas J, Yokoi F, Deandrade MP, Pisani A, Li Y. - August 31, 2013

    Dystonia is a neurological disorder characterized by abnormal involuntary movements that are prolonged and often cause twisting and turning. Several genetically modified worms, fruit flies, and rodents have been generated as models of genetic dystonias, in particular DYT1, DYT11, and DYT12 dystonias. Although these models do not show overt dystonic symptoms, the rodent models exhibit motor deficits in specialized behavioral tasks, such as the rotarod and beam-walking tests.

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