The Anatomical Basis for Dystonia: The Motor Network Model

Tremor and Other Hyperkinetic Movements

article

H. A. Jinnah¹  Vladimir Neychev²  Ellen J. Hess³ 

[1] Departments of Neurology, Emory University;Department of Surgery, University Multiprofile Hospital for Active Treatment ‘‘Alexandrovska’’, Medical University of Sofia;Departments of Pharmacology and Neurology, Emory University

关键词: Dystonia;    cervical dystonia;    torticollis;    blepharospasm;    focal hand dystonia;    laryngeal dystonia;    spasmodic dysphonia;    neuroanatomy;    neuroimaging;   

DOI  :  10.5334/tohm.383

学科分类：社会科学、人文和艺术（综合）

来源: Ubiquity Press

【 摘 要 】

Background: The dystonias include a clinically and etiologically very diverse group of disorders. There are both degenerative and non-degenerative subtypes resulting from genetic or acquired causes. Traditionally, all dystonias have been viewed as disorders of the basal ganglia. However, there has been increasing appreciation for involvement of other brain regions including the cerebellum, thalamus, midbrain, and cortex. Much of the early evidence for these other brain regions has come from studies of animals, but multiple recent studies have been done with humans, in an effort to confirm or refute involvement of these other regions. The purpose of this article is to review the new evidence from animals and humans regarding the motor network model, and to address the issues important to translational neuroscience. Methods: The English literature was reviewed for articles relating to the neuroanatomical basis for various types of dystonia in both animals and humans. Results: There is evidence from both animals and humans that multiple brain regions play an important role in various types of dystonia. The most direct evidence for specific brain regions comes from animal studies using pharmacological, lesion, or genetic methods. In these studies, experimental manipulations of specific brain regions provide direct evidence for involvement of the basal ganglia, cerebellum, thalamus and other regions. Additional evidence also comes from human studies using neuropathological, neuroimaging, non-invasive brain stimulation, and surgical interventions. In these studies, the evidence is less conclusive, because discriminating the regions that cause dystonia from those that reflect secondary responses to abnormal movements is more challenging. Discussion: Overall, the evidence from both animals and humans suggests that different regions may play important roles in different subtypes of dystonia. The evidence so far provides strong support for the motor network model. There are obvious challenges, but also advantages, of attempting to translate knowledge gained from animals into a more complete understanding of human dystonia and novel therapeutic strategies.

【 授权许可】

CC BY