Synonyms in a broader sense
English: Huntington’s disease, Chorea major. – St. Vitus Dance (vulg.) – Huntington’s disease
Definiton
Hereditary disease that leads to the destruction of brain cells in certain brain regions of the unconscious holding and supporting motor functions. The disease usually occurs between the ages of 35 and 50 and manifests itself in
- Movement disorders such as unintentional, lightning-like, flinging movements of the limbs
- Grimace
- Reduction of intellectual capacity and
- Personality decline.
What is the life expectancy for Huntington’s disease?
Compared to the normal population, life expectancy is significantly reduced in patients with Huntington’s disease. The overall life expectancy varies greatly from individual to individual. On the one hand, this depends on the age of the disease and on the course of the disease.
Usually the first symptoms appear between the age of 30 and 40. Almost half of those affected die within the first 10 years of the disease. After the 15th year of illness only 25% are still alive.
In 10% of the cases, however, a disease duration of more than 20 years has also occurred. In general, women have on average a slightly longer duration of illness than men. The earlier the illness occurs, the more serious is the course of the disease. The life expectancy of patients with Huntington’s disease is therefore on average between 40 and 50 years, although if the disease starts late, an age of around 60 years may well be reached.
Epidemiology:
The frequency of Huntington’s disease is 5 – 10/100. 000, the inheritance is autosomal dominant. This means that children of affected persons have a 50% risk of contracting the disease themselves.
Symptoms:
Those affected suffer from muscle slackness and, at the same time, sweeping, lightning-like flinging movements of the limbs, which are intensified during emotional tension and hardly ever occur during sleep. The reason for this is the loss of necessary inhibitory impulses on the execution of movements. The disturbed coordination of movement further manifests itself in grimaces, swallowing disorders and speech difficulties.
Huntington’s disease is progressive, and as the disease progresses, patients have difficulty walking and eye movement coordination and become unable to hold stool and urine. In addition, personality changes such as tantrums and attention disorders as well as delusions in the context of psychoses occur in the chorea. The decline in intellectual performance leads to progressive dementia (acquired intelligence reduction, see there). Huntington’s disease is fatal within 15-20 years of diagnosis, often as a result of secondary diseases caused by the patient’s poor general condition.
What are the first signs?
The first signs of Huntington’s disease are usually noticed between the ages of 30 and 40. Often psychological symptoms precede the movement disorders characteristic of the disease by years. Typical psychological abnormalities are depressive moods and reduced drive.
Sometimes the incipient cognitive deficits manifest themselves in the form of concentration and memory disorders. These symptoms can easily be confused with depression in the early stages. The fact that the illness often leads to impulsive and hurtful behaviour towards other people is also a burden for the relatives.
Patients are sometimes unable to process visual information, e.g. facial expressions, correctly and thus cannot react appropriately to the emotions of others. The movement disorders are initially characterized by hyperkinesia (Greek hyper – over, kinesis – movement). This is understood to mean increased unwanted movements.
The muscle tone – the state of tension of the musculature – is reduced. This lack of control over their own body is perceived by patients as very stressful. Particularly in the early phase there are isolated attempts at suicide.
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