Wilson diseaseHuntington's disease (HD) is a monogenic, autosomal dominant, neurodegenerative condition. The importance of motor impairment is evident in that it correlates with functional limitation in HD, predicts need for nursing home placement and falls risk, and defines clinical diagnosis. The ...
The archetypal hereditary cause of chorea is Huntington's disease (HD). However, this condition often manifests as a mixed movement disorder, and some individuals with the Westphal variant may not display chorea at all. Moreover, since gene-specific testing has become available, we now know ...
Huntington's disease is caused by a single dominant allele and results in progressive mental and neurological damage. The disease usually becomes symptomatic when a person is between 30 and 50 years old and the patient usually dies within 15 years of diagnosis. Approximately 1 in 25,000 Caucasian...
Huntington Disease and Other ChoreasAuthor links open overlay panelFrancisco Cardoso MD, PhDShow more Add to Mendeley Share Cite https://doi.org/10.1016/j.ncl.2009.04.001Get rights and content Under an Elsevier user license open accessPrevious article in issue Next article in issue...
Huntington disease and other diseases of polyglutamine expansion are each caused by a different protein bearing an excessively long polyglutamine sequence and are associated with neuronal death. Although these diseases affect largely different brain regions, they all share a number of characteristics, and...
Targeting aggregation in the development of therapeutics for the treatment of Huntington’’’s disease and other polyglutamine repeat diseases. Steffan, J.S,L.M Thompson. Expert Opinion on Therapeutic Patents . 2003Steffan JS, Thompson LM. Targeting aggregation in the development of therapeutics for...
Tetrabenazine for treatment of chorea associated with Huntington’s disease. Expert Opin Orphan Drugs. 2013;1:423–36.Tetrabenazine for treatment of chorea associated with Huntington’s disease and other potential indications[J] . Joohi Jimenez-Shahed,Joseph Jankovic.Expert Opinion on Orphan Drugs . ...
A hallmark of neurodegenerative diseases like Huntington's is the progressive death of nerve cells in the brain. The cells don't die quickly, though. They first start to disconnect from each other because their neurites—long finger-like extensions that
Huntington’s disease (HD) is an autosomal dominant neurodegenerative disease resulting in motor impairment and death in patients. Recently, several studies have demonstrated insulin or insulin-like growth factor (IGF) treatment in models of HD, resultin
Polyglutamine diseases are inherited, fatal neurodegenerative disorders caused by genomic expansion of exonic cytosine–adenine–guanine (CAG) trinucleotide repeats. These diseases include Huntington's disease, spinal and bulbar muscular atrophy, dentatorubral–pallidoluysian atrophy, and spinocerebellar ataxia...