Convergent mechanisms in etiologically-diverse dystonias.

Autor: Thompson VB; Emory University School of Medicine, Department of Pharmacology, Woodruff Memorial Research Building, Suite 6000, 101 Woodruff Circle, Atlanta, GA 30322, USA., Jinnah HA, Hess EJ
Jazyk: angličtina
Zdroj: Expert opinion on therapeutic targets [Expert Opin Ther Targets] 2011 Dec; Vol. 15 (12), pp. 1387-403. Date of Electronic Publication: 2011 Dec 03.
DOI: 10.1517/14728222.2011.641533
Abstrakt: Introduction: Dystonia is a neurological disorder associated with twisting motions and abnormal postures, which compromise normal movements and can be both painful and debilitating. It can affect a single body part (focal), several contiguous regions (segmental), or the entire body (generalized), and can arise as a result of numerous causes, both genetic and acquired. Despite the diversity of causes and manifestations, shared clinical features suggest that common mechanisms of pathogenesis may underlie many dystonias.
Areas Covered: Shared themes in etiologically-diverse dystonias exist at several biological levels. At the cellular level, abnormalities in the dopaminergic system, mitochondrial function and calcium regulation are often present. At the anatomical level, the basal ganglia and the cerebellum are frequently implicated. Global CNS dysfunction, specifically aberrant neuronal plasticity, inhibition and sensorimotor integration, are also observed in a number of dystonias. Using clinical data and data from animal models, this article seeks to highlight shared pathways that may be critical in understanding mechanisms and identifying novel therapeutic strategies in dystonia.
Expert Opinion: Identifying shared features of pathogenesis can provide insight into the biological processes that underlie etiologically diverse dystonias, and can suggest novel targets for therapeutic intervention that may be effective in a broad group of affected individuals.
Databáze: MEDLINE