Huntington’s disease is a hereditary illness which causes degeneration of neurones, affecting motor, cognitive and behavioural functions, resulting in dementia and generally death. Huntington’s disease leads to abnormal and incoherent movements, difficulty in balance, a form of dormancy, in some way, lethargy.

 Huntington’s disease owes its name to George Huntington, an American physician who was the first to describe it in scientific terms in 1872. This disease was once called Saint Vitus’ dance.

 Huntington’s disease is therefore a genetic, neurodegenerative and hereditary affliction. This disease attacks the brain, through the destruction of specific cells, namely the caudate nucleus, the putamen and, in time, when the disease has progressed, the cerebral cortex. As for the meninges, the membranes that surround the brain and protect it, they become thicker.

In the context of current scientific research there is no veritable medical treatment that is able to halt Huntington’s chorea or slow it down, although numerous studies have been carried out. At the moment, hope of treating Huntington’s disease lies in implantation of foetal brain cells into the brain of the sufferer in the hope that these fœtal brain cells will develop and take the place of the dead cells. Studies into medicine capable of curbing neurone degeneration through their molecules are also under way. The gene responsible for Huntington’s disease has been found on the fourth chromosome, and more specifically on the end part of the short arm of this chromosome, at the G8 locus.

This disease generally appears between the ages of 30 and 45. It materialises in its first phase, through mental disorders akin to a state of depression, which are often accompanied by suicidal tendencies, or moments of euphoria. Next come the involuntary movements, not controlled by the sufferer, which manifest themselves as jerky and sudden gestures. Speaking becomes very difficult. The afflicted person suffers from insomnia and headaches, and has difficulty in eating and swallowing their saliva. He or she is constantly tired. Huntington’s disease will evolve irreparably into dementia through a diminishing of mental faculties and total psychological and physical disability.

Current medical treatment for Huntington’s disease consists in administering neuroleptics to the sufferer. Neurone grafting seems to bring great hope to the treatment of Huntington’s disease. Clinical trials are moreover being carried out at the moment by French researchers, led by Dr Anne Bachoud-Lévi, who have grafted onto the brains of five patients suffering from Huntington’s disease, neuronal cells that have come from foetuses, and which have been removed after an abortion. This team of researchers have observed a remission of symptoms related to motivity for up to four or five years and an increase of intellectual function.

Scientific progress in Huntington’s disease is made every day. It is important to remain hopeful for all people currently suffering from this degenerating and destructive illness.