What is Huntington's Disease
Huntington´s Disease (HD) is a rare, hereditary, neurodegenerative illness that affects people of all races worldwide. The disease was named after Dr. George Huntington, a physician from Long Island, United States of America, who first published a description of “hereditary chorea” in 1872. It was due to his essay, entitled On Chorea, that the disorder became known as Huntington´s Chorea. The term chorea is the Greek word for “dance” and refers to the involuntary movements which are seen in many individuals with HD. Since Chorea is not the only symptom, the disorder has in later years been called Huntington´s Disease.
For a long time, little was known or published about HD. In the last 30 years, however, we have learnt a lot about the cause of HD and its effects, as well as the therapies and techniques for managing its symptoms. The gene that causes HD was discovered in 1993 and since then important scientific advances have been made. To date, there are no drugs to slow or stop the progression of Huntington’s Disease, but scientists are getting closer each day to finding a disease modifying treatment.
HD causes gradual degeneration of brain cells which in turn can result in physical, cognitive and emotional symptoms, which typically become apparent between the ages of 35 and 55, but can also appear much earlier (Juvenile-Onset HD) or later (late onset HD).
In most cases, individuals affected by HD can maintain their independence for several years after the first symptoms appear. A knowledgeable physician can prescribe treatment to minimize the impact. Allied health professionals, such as social workers, occupational and physical therapists, speech therapists and nutritionists, can all play a useful role in maximizing abilities and prolonging independence.
Huntington´s Disease is a hereditary disorder. Each child of a parent who has HD has a 50% chance of inheriting it. HD occurs in all races. Males and females are equally at risk. Carriers of the HD gene will eventually develop the disease should they live long enough.
Globally, there is estimated to be between 3.6 and 5.7 people with HD for every 100,000 of population.
Symptoms
Huntington’s Disease (HD) is characterized by three main types of symptoms: impairments in movements, cognitive abilities, and mood.
Movement Impairments: People with HD may experience involuntary movements, which are often the first most noticeable symptoms. These movements, medically referred to as ‘chorea’, can disrupt walking, talking, and balance.
Cognitive Issues: HD also affects the brain, leading to difficulties in concentration, memory loss, and becoming overly fixated on certain thoughts or tasks.
Mood Changes: Perhaps the most challenging for families are the mood changes. Individuals with HD can experience anxiety, depression, or unexplained anger, sometimes resulting in drastic changes in behavior.
HD symptom onset usually begins in middle age between the ages of 30-40 and individuals with HD typically die 15-20 years after onset of symptoms.
It’s important to remember that HD affects everyone differently, and symptoms and disease progression can vary widely from person to person. While there is currently no cure for HD, treatments are available to manage symptoms and improve comfort. Supportive care, including therapy and counseling, are vital for both patients and their families.