Journal
NEUROLOGIC CLINICS
Volume 33, Issue 1, Pages 101-+Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.ncl.2014.09.003
Keywords
Huntington; Chorea; Huntingtin; Polyglutamine; Striatum; Pathogenesis; Neurodegeneration; Treatment
Categories
Ask authors/readers for more resources
Huntington disease (HD) is an autosomal dominant inherited neurodegenerative disease characterized by progressive motor, behavioral, and cognitive decline, culminating in death. It is caused by an expanded CAG repeat in the huntingtin gene. Even years before symptoms become overt, mutation carriers show subtle but progressive striatal and cerebral white matter atrophy by volumetric MRI. Although there is currently no direct treatment of HD, management options are available for several symptoms. A better understanding of HD pathogenesis, and more sophisticated clinical trials using newer biomarkers, may lead to meaningful treatments. This article reviews the current knowledge of HD pathogenesis and treatment.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available