ABSTRACT

More than a century ago George Huntington (1850-1916) described a family with neurodegenerative symptoms in several generations and this disease became known as Huntington’s disease (HD; Huntington, 1872). During the last two decades advances in molecular and genetic technology have generated new and far-reaching insights into the hereditary characteristics of the disease. Because the gene is known, each child of an affected parent has a 50% risk of having inherited the disease. The diagnosis of HD may have profound consequences for offspring: children at risk subsequently have to deal with the knowledge of having a severely ill parent with irreversible and progressive motor, neurological and personality impairments and they will be confronted with personal consequences for future and family planning (Harper, 1996).