The HOPES Timeline (Non-Flash, Part 7)

A Brief History of Huntington's Disease

Scenes 30-35: Notable People and Places

Philippus Aureolus Theophrastus Paracelsus Bombastus von Honenheim

1493-1541

Paracelsus was a notable alchemist and reformer during the Renaissance period. He introduced the name chorea sancti viti (Latin for "St. Vitus´ dance") to describe a peculiar disease characterized by writhing, sporadic movements. Most likely due to the mass hysteria and religious superstition of the time, this "dancing mania" had reached epidemic proportions in Europe. It is now thought that many of the sufferers may have experienced epileptic seizures or ergot poisoning. Near the end of Paracelsus´s lifetime, the spread of the disease began to slow, the symptoms became milder, and Paracelsus termed this new form "chorea naturalis," or chorea due to natural causes.

Thomas Sydenham

1624-1689

Thomas Sydenham was an English physician who is considered one of the most important revivers of Hippocrates´ views. He stressed careful observation and bedside attendance, and he remarked keenly on many symptoms commonly associated with HD. He noted, for instance, "The hand cannot be steady for an instant. It passes from one position to another, however the patient may strive to the contrary." He believed that these movements were caused by "some humor falling on the nerves, and such irritation causes the spasm." Today, however, Sydenham chorea refers to chorea that is associated with rheumatic fever, even though Thomas Sydenham was an English physician who is considered one of the most important revivers of Hippocrates´ views. He stressed careful observation and bedside attendance, and he remarked keenly on many symptoms commonly associated with HD. He noted, for instance, "The hand cannot be steady for an instant. It passes from one position to another, however the patient may strive to the contrary." He believed that these movements were caused by "some humor falling on the nerves, and such irritation causes the spasm." Today, however, Sydenham chorea refers to chorea that is associated with rheumatic fever, even though Sydenham never explicitly made that link.

George Huntington

1850-1916

George Huntington, an American physician, was only twenty-two years old when he submitted his famous paper "On Chorea" (1872) to The Medical and Surgical Reporter. Much of the paper drew from the written observations of his father and grandfather, both physicians who had noticed the involuntary shaking of some patients. The paper gained Huntington instant notability because, in the words of Sir William Osler, "In the history of medicine there are few instances in which a disease has been more accurately more graphically, or more briefly described." Huntington was able to explicitly point to genetic inheritance as the mode of transmission, and he noticed that the first symptoms usually appear at an adult age and that they are usually accompanied by mental decline as well. It is due to these significant observations and conclusions that "Huntington´s disease" bears George Huntington´s name.

Woody Guthrie

1912-1967

Woody Guthrie was one of the most famous Americans with HD. Born in Okemah, Oklahoma, Guthrie gained fame in the 1930s and 1940s as a folk singer and radio entertainer. He was known for putting political and social commentary in the lyrics of his music, and he often celebrated the plight of the American laborer. In his songs, Guthrie includes references to many of the 20th century´s most historic events, including the Great Depression, the "Dust Bowl" migration, World War II, and the Cold War. His most famous songs include "This Land Is Your Land," "Grand Coulee Dam," and "I Ain´t Got No Home."

Guthrie´s mental state began to deteriorate in the early 1950s. His memory declined, and his behavior became unpredictable. He left his wife, Marjorie, and his home in New York to marry a woman twenty years his junior in California. However, due to his mental state, Guthrie was eventually forced to return to New York, where he was placed in one hospital after another. HD

The Wexler Family

The Wexler family is inextricably tied to the history of Huntington´s disease research. In 1968, Leonore Wexler was diagnosed with HD, which inspired her two daughters, Nancy and Alice, and her husband, Milton, to become involved in the search for a cure for HD.

Milton Wexler, a prominent psychologist, is responsible for bringing world renown researchers together to focus on HD research. He founded the Hereditary Disease Foundation, which funds HD research and sponsors workshops for scientists to share ideas.

Nancy Wexler has played a pivotal role in the scientific research of HD. She pioneered the fieldwork in Lake Maracaibo, Venezuela that led to the discovery of the Huntington gene (see Lake Maracaibo, Venezuela) and has since helped other researchers map genes responsible for Alzheimer´s disease, kidney cancer, manic depression, and other disorders. She served as the Hereditary Disease Foundation´s president, and is currently a Professor of Neuropsychology at Columbia University.

Alice Wexler, a teacher, writer, and historian, chronicled her family´s journey in the insightful book Mapping Fate: A Memoir of Family, Risk, and Genetic Research.

Huntington´s disease in Lake Maracaibo, Venezuela

In the early 1950s, Dr. Amerigo Negrette first diagnosed Huntington´s disease in Lake Maracaibo, Venezuela. Working as a rural physician, Negrette was perplexed by the fact that many townspeople often appeared drunk, staggering and weaving at all hours of the day. He learned from locals that these people were not drunk, but instead suffered from a disease referred to as el mal de San Vito, or the sickness of Saint Vitus. After visiting many people with the sickness, Negrette diagnosed the disease as HD. He discovered that HD ran deep in the community; people with the illness were interrelated and had common ancestry. In 1963, he published a book entitled Corea de Huntington: Estudio de una Sola Familia a Traves de Varias Genereaciones describing HD in his community. The world learned of this tragic occurrence when Negrette´s work was presented at the 1972 Centennial Symposium.

In 1981, Dr. Nancy Wexler led a team of scientists to study HD in Lake Maracaibo. Their original goal was to find an HD homozygote (an individual who has inherited two copies of the HD allele), but the team also ended up collecting blood samples from as many HD sufferers as they could find and test. These samples played a key role in the discovery of a genetic marker for HD in 1983 and led to the creation of a community pedigree, the largest of its kind in the world.

Click here to return to the main HOPES Timeline page.

Last Modified: 05/22/2009

An educational product of HOPES, not to be used in place of medical care. For more information about HOPES, click on the Logo. To contact HOPES with comments or questions, click here.