Huntington's disease in popular culture: a brief historical perspective.

In Ruth Rendell’s 1988 London mystery House of Stairs, the narrator suggests a startling sea change in the world of Huntington’s disease (HD). “The newspapers and television and magazines have been full of Huntington’s lately,” she observes. “Huntington’s has become a fashionable disease, displacing multiple sclerosis and even schizophrenia in the public’s curiosity.” [1]. She refers, no doubt, to the publicity surrounding the discovery of a genetic marker for HD in 1983 and the beginning of presymptomatic genetic testing three years later [2]. The start of the Human Genome Project in 1990 and the identification of the expanded Huntingtin gene in 1993 drew further attention to HD [3]. Considering that many people get their medical information from popular culture, it is useful to consider how HD has been portrayed on television and also in novels such as House of Stairs [4]. Why did the disease suddenly start to appear at this time when it was largely absent before? What aspects were emphasized? How accurate were fictional and dramatic portrayals? What societal attitudes toward the disease were revealed? How did presentations of HD differ from those of other neurological, psychiatric, and/or genetic disorders? Did representations change over time as biomedical knowledge of HD advanced? Prior to the 1980s, characters with HD or at risk rarely appeared in fiction or in television dramas. When they did, they were minor figures, portrayed inaccurately, like Tony Polar in Valley of the Dolls (1966) [5]. Or they were patients in a single episode of a medical drama, as in Marcus Welby, M.D. (1970) [6]. But starting in the 1980s, persons at risk began to