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Mary Kugler, MSN, RN,BC
Guide to Rare/Orphan Diseases
The following are summaries of some of the research articles about Huntington's disease published in 2001-2002.
Dementia in Huntington's disease
Before developing dementia, people with Huntington's disease may have problems with attention and the ability to process information. In one study, researchers found that people with the gene for Huntington's disease generally had normal performance on cognitive tests compared to people who did not have the gene. However, having problems with the cognitive tests correlated to the beginning of the disease process in those people with the Huntington's gene.
[Brandt, J. (2002). Dementia in Huntington's disease. Neurobiol Aging 23(1S), p. S8.]
Research on treatments for Huntington's disease: creatine, amantadine, and tauroursodeoxycholic acid (TUDCA)
Creatine
A study done with mice bred to have Huntington's disease reported success in improving the mice's symptoms with the use of creatine. However, two studies of people with Huntington's disease did not report success in improving symptoms, although the treatment did no harm, either.
[Kieburtz, K. (2001). Placebo-controlled trial of creatine in Huntington's disease. Neurology 56 (Supplement 3).
Verbessem, P., Hespel, P., & Dom, R. (2002). Oral creatine supplementation in patients with Huntington's disease. Neurology 58(Supplement 7).]
Amantadine (Symmetrel)
In a study of amantadine (Symmetrel), 22 people with Huntington's disease received either the drug or a sugar pill (placebo). People receiving amantadine had a 25% decrease on average in purposeless movements of the face, arms, and legs (called chorea) versus a 5% decrease for those receiving the placebo.
[Verhagen, L., Morris, M., Farmer, C., Gillespie, M., Wu, J., & Chase, T. N. (2001). A double-blind, placebo-controlled crossover study of the effect of amantadine on chorea in Huntington's disease. Neurology 56(Supplement 3).]
Tauroursodeoxycholic acid (TUDCA)
Researchers gave mice bred to have Huntington's disease tauroursodeoxycholic acid (TUDCA), a bile acid. The TUDCA protected the mice's nerve cells from the effects of Huntington's disease. Normally, Huntington's causes the gradual death of nerve cells. The researchers suggest that TUDCA could be developed as a preventive treatment for the effects of Huntington's disease.
[Keene, C. D., Rodrigues, C. M., Eich, T., Chhabra, M. S., Steer, C. J., & Low, W. C. (2002). Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease. Proc Natl Acad Sci 99(16), pp. 10671-10676.]
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