October 2, 2025, 8:09 pm | Read time: 5 minutes
Huntington’s disease, also known as Huntington’s chorea and formerly called “Saint Vitus’ dance,” is a rare, hereditary disorder of the central nervous system. It is characterized by jerky movements and outbursts of anger. Those affected often remain unaware of their condition for a long time—to outsiders, it may seem as if the person is gesticulating in an exaggerated manner. Over time, the cognitive abilities of those affected also deteriorate.
Overview
Cause
The cause of Huntington’s disease is a genetic mutation. Specifically, an expansion of the nucleotides cytosine, adenine, and guanine (CAG)—known as the Huntington gene. In a healthy person, this gene contains several repeat sequences of the bases cytosine, adenine, and guanine. If the nucleotide expansion (CAG count) is 36 or more, Huntington’s symptoms are likely to occur. With a CAG count of 40 or more, the individual will eventually develop symptoms of Huntington’s disease.
Probability of Inheriting the Disease
Men and women are equally likely to develop the disease. Since the Huntington gene is not located on a sex chromosome, it is sufficient for one genetic trait to be altered: Offspring of a mother or father with this genetic alteration have a fifty percent chance of inheriting the gene from the affected parent and then developing the disease.1
Frequency and Symptom Onset
According to the German Huntington Association, about 8,000 to 12,000 people in Germany live with Huntington’s disease.2 Each year, several hundred new cases occur. It is estimated that about 30,000 people in Germany may carry the Huntington gene. The disease can manifest at any age and may occur more frequently in certain age phases. Most often, the first symptoms appear between the ages of 35 and 50. Occurrence in childhood or old age is possible but relatively rare.3
Discovery of the Disease and Development of the Term
Records of the disease date back to the Middle Ages, when it was referred to as “Saint Vitus’ dance.” The disease was named so because people would call upon Saint Vitus for help during episodes of dance mania. The American physician George Huntington first described the disease in 1872 and discovered its hereditary nature. For a long time, Huntington’s disease was called Huntington’s chorea—the Greek root means “to dance.”
Possible Early Symptoms
The following symptoms may occur:
- Jerky, involuntary movements
- Movement disorders
- Behavioral changes
- Personality changes
- Depression
- Delusions
- Decline in intellectual abilities
- Outbursts of anger
- Mistrust
- Compulsive behavior
Course of the Disease
The disease ultimately affects the entire body and severely limits activities such as eating, speaking, walking, swallowing, and dressing. Huntington’s chorea progresses over many years. The rate at which a person’s condition changes varies for each individual. In most cases, those affected die from the failure of vital bodily functions associated with the disease.4
Early Stage
Initially, the symptoms of Huntington’s chorea are rather nonspecific; they manifest in a noticeable, individual behavior pattern. Some affected individuals may feel aggressive, irritable, or uninhibited. Others may become anxious, fall into depression, and tend to have outbursts of anger or self-harm.
At first, people with Huntington’s chorea manage to integrate their symptoms, such as excessive or noticeable movements, into their daily lives. Many affected individuals are often unaware that they have a movement disorder. To outsiders, it may seem as if they are merely gesticulating in an exaggerated manner.
The cause of the twitching movements is likely the rapid and brief contraction of muscles. All body parts can be affected. A typical symptom of Huntington’s disease is sudden grimacing. Those affected appear “remote-controlled” in their movements, almost as if they are being manipulated by invisible strings, like a marionette.
Mental Changes
Changes in a person’s mental state can begin either before or simultaneously with unusual movements, making them initially unnoticeable. It can also happen that affected individuals suddenly lose interest in usual activities and react more impulsively and irritably.
Also of interest: Causes, Symptoms, and Course of Parkinson’s
Later Stages
As the disease progresses, the destruction it leaves behind becomes more noticeable. Depending on the severity, the tongue and throat muscles may also be affected, causing speech to become choppy. Affected individuals can only produce sounds explosively. At the same time, the risk of choking increases. If food, for example, enters the airways, the development of pneumonia is not ruled out.
Further Course
As the disease progresses, affected individuals lose more and more control over their bodies. They may behave irresponsibly or wander aimlessly. Many affected individuals develop dementia 15 years after the onset of Huntington’s chorea.
End Stage
In this stage, individuals have already lost their ability to think rationally. They may fall into severe depression, develop an obsessive-compulsive disorder, or be at risk of suicide. Additionally, dementia is pronounced, and individuals are bedridden, relying on the help of others.
Where Did the Term “Saint Vitus’ Dance” Come From?
The disease was known in the Middle Ages and received its name due to its symptoms and a legend: According to Christian folklore, Saint Vitus was called upon to heal the disease. It is said that he succeeded in doing so during his lifetime with an afflicted child.
Rett Syndrome: When Parents Suddenly Don’t Recognize Their Child
Eye Cancer in Children – a Noticeable Sign Indicates Illness
Diagnosis
If there is a suspicion of the disease, a medical history is taken by a doctor, which includes a detailed documentation of family medical history. The individual’s symptoms are also analyzed and observed. A blood test can determine whether one carries the genetic defect or not.
Therapeutic Measures
Huntington’s disease is currently incurable and inevitably leads to death. Certain medications and supportive measures, such as occupational or physical therapy, can at least alleviate the symptoms and discomfort of the disease. Speech therapy can be helpful for speech or swallowing difficulties, and psychotherapy can also provide support. In the end, however, the disease remains an internal battle with an invisible opponent.