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Clinical and Research Advances in Huntington's Disease

  • M. SuttonBrown (a1) and O. Suchowersky (a2)

Abstract

Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder characterized by abnormalities of movement and dementia. No curative treatment is available and HD results in gradually increasing disability. Characterization of the genetic abnormality has dramatically increased our understanding of the underlying mechanisms of the disease process, and has resulted in the development of a number of genetic models. These research tools are forming the basis of advanced work into the diagnosis, pathophysiology, and potential treatment of the disease. Clinically, the availability of genetic testing has eased confirmation of diagnosis in symptomatic individuals. Presymptomatic testing allows at-risk individuals to make informed choices but requires supportive care from physicians. Current clinical treatment is focused on symptom control. Advances in research have resulted in the development of potential neuroprotective strategies which are undergoing clinical testing.

RÉSUMÉ

La maladie de Huntington (MH) est une maladie neurodénétrice dominante autosomique caractésépar des mouvements anormaux et une dénce. Il n’existe aucun traitement curatif de cette maladie qui conduit àne invaliditérogressive. La caractésation de l’anomalie gétique a accru significativement notre comprénsion des ménismes sous-jacents et a menéu déloppement de modès gétiques. Ces outils de recherche constituent la base du travail actuel sur le diagnostic, la physiopathologie et les avenues de traitement de la maladie. Au point de vue clinique, la disponibilitéu test gétique a facilitéa confirmation du diagnostic chez les individus symptomatiques. Le test prémptomatique permet aux individus àisque de faire des choix éairémais demande du soutien de la part des mécins. Le traitement actuel de la maladie vise le contrôdes symptô. Les progrède la recherche ont permis de délopper des stratées neuroprotectrices potentielles qui font préntement l’objet d’essais thépeutiques.

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References

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