期刊
FRONTIERS IN SYSTEMS NEUROSCIENCE
卷 5, 期 -, 页码 -出版社
FRONTIERS MEDIA SA
DOI: 10.3389/fnsys.2011.00089
关键词
dystonia; globus pallidus; extracellular recording; stereotactic surgery; movement disorders
资金
- Ministry of Education, Culture, Sports, Science, and Technology of Japan (MEXT) [18300135, 19500354]
- The Uehara Memorial Foundation
- Takeda Science Foundation
- United States-Japan Brain Research Cooperative Program
- National Institute of Neurological Disorders and Stroke-National Institutes of Health Grant [NS-043038)]
- Wakayama Foundation for the Promotion of Medicine
- Uehara Memorial Foundation
- NIH [NS-47085, NS-57236]
- [18650089]
- NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE [R01NS043038] Funding Source: NIH RePORTER
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.
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