What is the course of the disease?

Chorea Huntington is a chronically progressive neurodegenerative disease. This means that it usually progresses slowly but continuously, destroying the nerves and ultimately leading to the patient’s death. The disease is characterized by mental abnormalities and movement disorders.

In the initial stage, unwanted movements (hyperkinesia) usually occur more frequently. Hypokinesia then develops in the course of the disease. Literally translated, this means “less movement”, meaning a lack of movement, as is also typical for Parkinson’s syndrome.

As the disease progresses, the patient is increasingly in need of care. Progressive dementia initially leads to speech deprivation and orientation disorders. Food intake is usually made more difficult by a swallowing disorder, and patients lose weight. On average, patients die 10 – 15 years after the onset of the disease. If the disease starts late, the course of the disease is often somewhat delayed.

Is there a cure?

There is currently no cure for Huntington’s disease. Since 1993 the cause of the disease is known to be a defective gene on chromosome 4, but unfortunately there is no way to treat the gene defect or its consequences. Therefore, it is not possible to stop the course of the disease at this point in time.

Of course, intensive research is being conducted into new therapeutic approaches. The genetic basis of the disease is well known in the meantime. Therefore, the only hope for patients and their families is that research will eventually lead to an important breakthrough.

Which drugs help?

The cause of Huntington’s disease is a gene mutation. Unfortunately, there are currently no drugs that treat this cause or cure the disease. One can try to treat the different symptoms with medication.

Neuroleptics are often used to treat the classic movement disorders. Antidepressants help with depressive moods. Ultimately, these drugs cannot stop the course of the disease. One only tries to control the symptoms a little better with the drugs.

What does the final stage look like?

Usually the final stage is reached 10-15 years after the onset of the disease. Patients are bedridden and need care around the clock. Due to the swallowing disorder, which develops as the disease progresses, many are very emaciated (medical: cachectic).

In addition, there is a permanent risk of life-threatening pneumonia (aspiration pneumonia) if food is swallowed. If the patient is no longer able to swallow at all, an artificial diet must be considered. Psychological abnormalities also increase in the course of the disease. In the end, the dementia is advanced, the patients lose the ability to communicate and are disoriented.

Differential diagnoses

A similar symptomatology, consisting of movement disorders and intellectual decline, can occur in the course of Creutzfeld-Jakob disease, in later stages of syphilis and after inflammation of the brain.