Huntington's disease in Tasmania

This compilation of papers is the product of five years of research and clinical involvement in Huntington' s Disease (HD) in Tasmania. In 1949, Dr Charles Brothers published some details of a large, Tasmanian HD pedigree. Others performed calculations on his figures and stated that the region had one of the highest per capita rates in the world. Later, the Department of Psychiatry, of the University of Melbourne, took an interest in Tasmanian HD families. However, no further information was published until that which appears herein. In 1986, the author commenced research and clinical practice in HD. First, the functions of HD Registers (HDRs) were examined. This led to the establishment and maintained of an HDR. This, in turn called for the establishment of an HD Advisory Committee. These events have been described. Pure research was subsequently conducted on the prevalence of HD in Tasmania, the age of onset, the age of death and the duration of the disease, the fertility of affected individuals and their unaffected siblings, and sundry features of the total HD population and of the large HD family earlier described by Dr Brothers. The above findings were used as authorative data in a review of the social justice issues of HD in Tasmania. This review favourably influenced government policy and funding. Concurrently, a study of early detection techniques using neuropsychological testing, neurological examination and Quantitative (Q)EEG has been running for two years. Final data is not yet available and thus results can not be presented, but a single case study of an individual examined with QEEG before and after the clinical onset of HD is described. The appendix contains two sets of data. The first is details of the large Tasmanian which at January 1, 1990, extended over 43 meters of paper. The second is complete details of the neurophysiological results of the single case mentioned above.