4.7 Article

Cervical dystonia: a neural integrator disorder

Journal

BRAIN
Volume 139, Issue -, Pages 2590-2599

Publisher

OXFORD UNIV PRESS
DOI: 10.1093/brain/aww141

Keywords

integrator; cerebellum; midbrain; tremor; nystagmus

Funding

  1. Dystonia Coalition [NIH U54 TR001456 07]
  2. Dystonia Medical Research Foundation

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Dysfunction of the oculomotor neural integrator leads to gaze-induced nystagmus. Shaikh et al. briefly recapitulate the basic principles behind neural integration, before developing the idea of an analogous neural integrator for head movements and the putative role of such an integrator in cervical dystonia.Dysfunction of the oculomotor neural integrator leads to gaze-induced nystagmus. Shaikh et al. briefly recapitulate the basic principles behind neural integration, before developing the idea of an analogous neural integrator for head movements and the putative role of such an integrator in cervical dystonia.Ocular motor neural integrators ensure that eyes are held steady in straight-ahead and eccentric positions of gaze. Abnormal function of the ocular motor neural integrator leads to centripetal drifts of the eyes with consequent gaze-evoked nystagmus. In 2002 a neural integrator, analogous to that in the ocular motor system, was proposed for the control of head movements. Recently, a counterpart of gaze-evoked eye nystagmus was identified for head movements; in which the head could not be held steady in eccentric positions on the trunk. These findings lead to a novel pathophysiological explanation in cervical dystonia, which proposed that the abnormalities of head movements stem from a malfunctioning head neural integrator, either intrinsically or as a result of impaired cerebellar, basal ganglia, or peripheral feedback. Here we briefly recapitulate the history of the neural integrator for eye movements, then further develop the idea of a neural integrator for head movements, and finally discuss its putative role in cervical dystonia. We hypothesize that changing the activity in an impaired head neural integrator, by modulating feedback, could treat dystonia.

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