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Clinical diagnosis and management in early Huntington's disease: a review

Authors Schiefer J, Werner CJ, Reetz K

Received 1 November 2014

Accepted for publication 20 January 2015

Published 25 March 2015 Volume 2015:5 Pages 37—50

DOI https://doi.org/10.2147/DNND.S49135

Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 4

Editor who approved publication: Prof. Dr. Thomas Müller

Johannes Schiefer,1,* Cornelius J Werner,1,* Kathrin Reetz1,2
1Euregional Huntington Center, 2Jülich Aachen Research Alliance (JARA) – Translational Brain Medicine, Department of Neurology, RWTH Aachen University, Aachen, Germany

*These authors contributed equally to this work


Abstract: This review focuses on clinical diagnosis and both pharmacological and nonpharmacological therapeutic options in early stages of the autosomal dominant inherited neurodegenerative Huntington's disease (HD). The available literature has been reviewed for motor, cognitive, and psychiatric alterations, which are the three major symptom domains of this devastating progressive disease. From a clinical point of view, one has to be aware that the HD phenotype can vary highly across individuals and during the course of the disease. Also, symptoms in juvenile HD can differ substantially from those with adult-onset of HD. Although there is no cure of HD and management is limited, motor and psychiatric symptoms often respond to pharmacotherapy, and nonpharmacological approaches as well as supportive care are essential. International treatment recommendations based on study results, critical statements, and expert opinions have been included. This review is restricted to symptomatic and supportive approaches since all attempts to establish a cure for the disease or modifying therapies have failed so far.

Keywords: Neurodegeneration, clinical picture, early symptoms, therapy, treatment

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