This subproject is one of many research subprojects utilizing theresources provided by a Center grant funded by NIH/NCRR. The subproject andinvestigator (PI) may have received primary funding from another NIH source,and thus could be represented in other CRISP entries. The institution listed isfor the Center, which is not necessarily the institution for the investigator.COHORT is an observational trial designed to collect prospective data from individuals who are part of a Huntington's disease (HD) family. Subjects will either be clinically diagnosed with HD, be a positive gene carrier, or be at-risk for inheritance/have an unknown gene status. Family members who have no HD family history (spouses) and/or gene negative status are also eligible to participate. For individuals under age 18, only those with clinically diagnosed features of HD in conjunction with a positive gene test will be eligible to participate. There is no limit on the number of eligible subjects.Study visits occur annually. Baseline procedures include: Unified Huntington's Disease Rating Scale 99 (UHDRS 99), Mini-Mental State Examination (MMSE), physical and neurological examinations, medical and concomitant medication history. All subjects 18 years of age and older will have a single blood specimen collected to obtain the subject's genotype/CAG repeat number. Optional procedures available at baseline for subjects 18 years of age and older include the completion of a Family History Questionnaire (FHQ) and the collection of blood and urine samples in a specimen repository for future research in HD. Annual study visits include: UHDRS 99; MMSE; medical examination; changes in concomitant medications and previous medical history. If the subject agreed to participate in the FHQ at baseline, the subject will be asked to provide information about new deaths and onsets of HD. If the subject agreed to participate in the optional blood and urine samples for repository storage, these samples will be collected.
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