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Seven working groups have been established by members
of the HSG with expertise and interest in specific areas of research
(clinical correlates, surgical approaches, neuropsychology, behavioral
aspects, animal models, clinical trials initiatives and genetic correlates).
The purpose of these working groups is to develop the expanding knowledge
base in HD necessary to address key clinical research issues that are
of the greatest therapeutic relevance, so as to develop appropriate
research methods and generate pilot data for the clinical trials of
the next decade. |
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Clinical Correlates Working Group The Clinical Correlates Working Group is analyzing the expanding Unified Huntington's Disease Rating Scale (UHDRS) database. Analyses are focused on the clinical correlates associated with early and late onset of HD and on factors that may be predictive of the rate of clinical progression of the disease. The group is also investigating the relationship among the various components of the UHDRS (motor, cognitive, behavioral and functional). A report regarding the predictors of functional decline was published in Neurology (54:452-458, 2000) |
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Surgical Approaches Working Group The participants in this working group are interested in a variety of surgical approaches for the treatment of HD. The current experimental focus is transplantation of tissue to the area of the brain called the striatum of HD patients. The group is maintaining a registry of surgical procedures for HD and is tracking the data from these experiments in collaboration with other organizations including the Network of European Centers for Transplantation and Restoration (NECTAR) and the American Society for Neuronal Transplantation (ASENT). This working group formulated a position paper (October 1996) addressing experimental surgical treatments in HD. This paper, which sets forth the standards for surgical research in HD, has been distributed to the HD organizations in the United States and Canada and to HSG members. |
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Neuropsychology Working Group The Neuropsychology Working Group was established with the inception of the HSG and consists of clinical and research neuropsychologists from HSG sites. This group helped develop the cognitive section of the UHDRS and a Supplemental Cognitive Battery which was used in the INTRO-HD and CARE-HD trials. The Supplemental Cognitive Battery was developed to assess specific cognitive impairments known to be associated with HD that may also be sensitive measures for detecting the onset of HD and relevant changes resulting from therapeutic interventions. |
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Behavioral Aspects of HD Working Group The Behavioral Aspects of HD Working Group is interested in the behavioral manifestations and complications of HD, including depression, irritability, and aggression. This working group is currently analyzing the behavioral elements of the UHDRS database and is planning therapeutic trials to evaluate symptomatic treatments for aggression in HD. |
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Animal Models Working Group The HSG Animal Models Working Group is examining the effects of promising interventions in experimental animal models of HD. The recent development of transgenic animal models provides an important opportunity to test promising therapies aimed at slowing the progression of illness in these models. The working group is evaluating proposals to test therapeutic agents and working with scientists from the HDF (Hereditary Disease Foundation) and HDSA (Huntington's Disease Society of America) to assist investigators in securing transgenic animals for study. This approach holds great promise for rapid screening of promising therapeutic agents. |
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Genetic Correlates Working Group This working group is interested in defining the genetic correlates of HD with specific attention to the genetic factors that account for the age of onset of HD and the pattern of clinical features and course. A study is underway to perform genetic linkage analysis in affected HD sibling pairs to help identify modifying genes important in the onset and progression of HD. |
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Clinical Trials Initiatives Working Group
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