Approximately one in 15,000 people have a defect in their HTT gene and the outcome of that defect is ultimately life-ending.

It’s called Huntington’s disease, or HD, and the average age of symptom manifestation occurs between the ages of 35 and 44. Symptoms include involuntary movements that are referred to as chorea, increasing dementia, and, finally, death.

If that sounds bleak, you’re right–it is bleak. But hope for HD may be on the horizon.

The molecule xyloketal B is being studied and has shown a potential for protecting neurons from deteriorating in early HD.

As the disease progresses, neurons that control voluntary movement gradually die off, causing the patient to twitch and jerk. Right now, there is one medication on the market, Xenazine, which is specifically targeted to treat chorea–but to date, there is no treatment to prevent those neurons from dying off.

Many times, people with HD are misdiagnosed. The early symptoms can include clumsiness, mood swings, and odd behavior. So, of course, psychiatric issues rather than HD are suspected.

The late folk singer Woody Guthrie, was misdiagnosed as an alcoholic (slurred speech is common with HD), and was hospitalized in a series of psychiatric hospitals. He died in 1967 before much was known about the disease. His mother Nora, died from HD complications in 1930 where she’d been hospitalized in the Oklahoma Hospital for the Insane because of her dementia. Fortunately, medical science has come a long way since then.

Beginning in 1993, genetic testing became available so that people could determine whether they wanted to have a child who, if the HTT deficiency is passed down, would have a 50/50 chance of developing HD.

Guthrie’s son Arlo, who is also a folk singer, chose not to be tested, and fortunately has not shown signs of the inherited disorder.

Xyloketal B is interesting because it has the potential to actually protect the neurons.

If that can be achieved, progression of Huntington’s can be slowed, giving patients a better quality of life and paving the way for new treatments.

What would finding a cure to Huntington’s disease mean to you? Share your thoughts with Patient Worthy!