This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. The purpose of this project is to validate clinical outcome measures in individuals with Friedreich's Ataxia (FRDA). To date, there is a lack of reliable and sensitive mesures which is the limiting factor in the initiation of therapeutic trials. The study will follow approximately 200 individuals at various stages of illness using multiple clinical assessment tools including a newly devised FRDA rating scale (FARS), the 9-hole peg test, and a timed 25-foot walk. A measure of contrast letter acuity and quantative measures of speech will also be performed. All the measures are non-invasive and many are assessments performed during a detailed neurological examination. A health related quality of life will also be administered to determine how patients'conditions impact their quality of life and how this changes over time. Participants will be followed annually over a period of three years. They will be allowed to participate in all of these studies or only a portion if they choose.
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