Dystonia is a neurological disorder characterized by sustained or repetitive involuntary muscle contractions and abnormal postures. In the present article, we will introduce our recent electrophysiological studies in hyperkinetic transgenic mice generated as a model of DYT1 dystonia and in a human cervical dystonia patient, and discuss the pathophysiology of dystonia on the basis of these electrophysiological findings. Recording of neuronal activity in the awake state of DYT1 dystonia model mice revealed reduced spontaneous activity with bursts and pauses in both internal (GPi) and external (GPe) segments of the globus pallidus. Electrical stimulation of the primary motor cortex evoked responses composed of excitation and subsequent long-lasting inhibition, the latter of which was never observed in normal mice. In addition, somatotopic arrangements were disorganized in the GPi and GPe of dystonia model mice. In a human cervical dystonia patient, electrical stimulation of the primary motor cortex evoked similar long-lasting inhibition in the GPi and GPe. Thus, reduced GPi output may cause increased thalamic and cortical activity, resulting in the involuntary movements observed in dystonia.
Keywords: dystonia, globus pallidus, extracellular recording, stereotactic surgery, movement disorders
Citation: Nambu A, Chiken S, Shashidharan P, Nishibayashi H, Ogura M, Kakishita K, Tanaka S, Tachibana Y, Kita H and Itakura T (2011) Reduced pallidal output causes dystonia. Front. Syst. Neurosci. 5:89. doi: 10.3389/fnsys.2011.00089
Received: 21 April 2011;
Accepted: 18 October 2011;
Published online: 28 November 2011.
Edited by:Charles J. Wilson, University of Texas at San Antonio, USA
Copyright: © 2011 Nambu, Chiken, Shashidharan, Nishibayashi, Ogura, Kakishita, Tanaka, Tachibana, Kita and Itakura. This is an open-access article subject to a non-exclusive license between the authors and Frontiers Media SA, which permits use, distribution and reproduction in other forums, provided the original authors and source are credited and other Frontiers conditions are complied with.
*Correspondence: Atsushi Nambu, Division of System Neurophysiology, National Institute for Physiological Sciences, 38 Nishigonaka, Myodaiji, Okazaki 444-8585, Japan. e-mail: firstname.lastname@example.org