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Titolo:
PATHOPHYSIOLOGY OF IDIOPATHIC DYSTONIA - FINDINGS FROM GENETIC ANIMAL-MODELS
Autore:
RICHTER A; LOSCHER W;
Indirizzi:
HANNOVER SCH VET MED,DEPT PHARMACOL TOXICOL & PHARM,BUNTEWEG 17 D-30559 HANNOVER GERMANY
Titolo Testata:
Progress in neurobiology
fascicolo: 6, volume: 54, anno: 1998,
pagine: 633 - 677
SICI:
0301-0082(1998)54:6<633:POID-F>2.0.ZU;2-L
Fonte:
ISI
Lingua:
ENG
Soggetto:
MUTANT HAMSTER MODEL; DOPA-RESPONSIVE DYSTONIA; WRIGGLE-MOUSE-SAGAMI; INVOLUNTARY MOVEMENT-DISORDERS; POSITRON EMISSION TOMOGRAPHY; EXERTS ANTIDYSTONIC ACTIVITY; BASAL GANGLIA DISORDERS; GAMMA-AMINOBUTYRIC ACID; NON-JEWISH FAMILY; TORSION DYSTONIA;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
255
Recensione:
Indirizzi per estratti:
Citazione:
A. Richter e W. Loscher, "PATHOPHYSIOLOGY OF IDIOPATHIC DYSTONIA - FINDINGS FROM GENETIC ANIMAL-MODELS", Progress in neurobiology, 54(6), 1998, pp. 633-677

Abstract

Dystonia is a common movement disorder which is thought to represent a disease of the basal ganglia. However, the pathogenesis of the idiopathic dystonias, i.e. the neuroanatomic and neurochemical basis, is still a mystery. Research in dystonia is complicated by the existence ofvarious phenotypic and genotypic subtypes of idiopathic dystonia, probably related to heterogeneous dysfunctions. In neurological diseases in which no obvious neuronal degeneration can be found, such as in idiopathic dystonia, the identification of a primary defect is difficult,because of the large number of chemically distinct, but functionally interrelated, neurotransmitter systems in the brain. The variable response to pharmacological agents in patients with idiopathic dystonia supports the notion that the underlying biochemical dysfunctions vary inthe subtypes of idiopathic dystonia. Hence, in basic research it is important to clearly define the involved type of dystonia. Animal models of dystonias were described as limited. However, over the last years, there has been considerable progress in the evaluation of animal models for different types of dystonia. Apart from animal models of symptomatic dystonia, genetic animal models with inherited dystonia which occurs in the absence of pathomorphological alterations in brain and spinal cord are described. This review will focus mainly on genetic animal models of different idiopathic dystonias and pathophysiological findings. In particular, in the case of the mutant dystonic (dr) rat, a model of generalized dystonia, and in the case of the genetically dystonic hamster (dt(sz)), a model of paroxysmal dystonic choreoathetosis has been used, as these show great promise in contributing to the identification of underlying mechanisms in idiopathic dystonias, although even a proper animal model will probably never be equivalent to a humandisease. Several pathophysiological findings from animal models are in line with clinical observations in dystonic patients, indicating abnormalities not only in the basal ganglia and thalamic nuclei, but alsoin the cerebellum and brainstem. Through clinical studies and neurochemical data several similarities were found in the genetic animal models, although the current data indicates different defects in dystonic animals which is consistent with the notion that dystonia is a heterogenous disorder. Different supraspinal dysfunctions appear to lead to manifestation of dystonic movements and postures. In addition to increasing our understanding of the pathophysiology of idiopathic dystonia, animal models may help to improve therapeutic strategies for this movement disorder. (C) 1998 Elsevier Science Ltd.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 02/04/20 alle ore 21:43:01