Joseph Hartman and his wife, Marilyn, smile stiffly as they sit in the Ben Franklin room of the University of Pennsylvania’s student union. He is gaunt and pale, with a long face and cropped, graying hair. She has the delicate frame of a songbird and is prim and neat in glasses and a green and white floral-print dress. They might be posing for a picture--a modern version of American Gothic--except that Joseph Hartman’s body is in constant motion.
Two dozen physicians and biologists politely but intently inspect Hartman as his case is presented by his neurologist. Hartman (not his real name) first sought medical help 13 years ago, complaining of irritability, lack of sleep, the jitters. He soon became unable to work and began to lose his short-term memory.
Only after many years did the chorea begin. The word comes from the Greek for dance, and that is exactly what Hartman is doing now. His hands dart and float in the air as if manipulated by an inebriated puppeteer. His head has a looseness that recalls those toy puppies whose heads once bobbed in the rear windows of American sedans. He watches his own limbs as if from a distance, occasionally reasserting control long enough to force a hand to grasp at the fabric of his pant leg or scratch the nape of his neck. Inevitably, his body rebels and resumes its dance. It is a dance of death.
This is Huntington’s disease: a devastating inherited condition that often waits until midlife to strike. A rare genetic flaw, present in one of every 10,000 people, selectively destroys two small regions of the brain--the putamen and the caudate nucleus--that help control movement. Eventually the muscles cannot be controlled at all; many Huntington’s patients die because they choke on food they can no longer swallow.
The presentation of Hartman’s case is the opening act in a two- day workshop aimed at generating research on how the gene kills brain cells. Hartman and his wife respond to questions. Do you like sweets? asks the woman seated next to Hartman’s wife. The woman could not present a more dramatic contrast to Joseph Hartman. Her blue eyes are clear and probing; she holds her shoulders square and steady. Indeed, Nancy Wexler, a psychologist and president of the Hereditary Disease Foundation, which organized the session, is as elegant and sharp as the tailoring of her cream suit.
