What are the symptoms of Huntington's disease?

Huntington's disease is characterised by a triad of symptoms: motor, psychiatric and cognitive. Symptoms, as well as their intensity and presentation, vary greatly from one patient to another. Clinical manifestations of the disease generally appear between the ages of 30 and 50, but the disease can appear at any age, from childhood onwards, for example before the age of 20 in 10% of cases. Motor symptoms are the most visible and often the first to be identified, but cognitive or psychiatric problems may already be present. In early forms, before the age of 20, the disease manifests itself mainly as behavioural and learning disorders. On average, Huntington's disease progresses over a period of 13 to 15 years after the onset of clinical symptoms, although this figure varies widely from patient to patient, towards severe dementia, an inability to walk and swallow, and eventually death.
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MOTOR SYMPTOMS

The best-known and most visible motor symptom is chorea. These are sudden, jerky and involuntary abnormal movements of one or more parts of the body. As with all involuntary movements, external factors such as stress or strong emotions can aggravate choreic movements. Other motor symptoms include problems with posture, walking and balance. Typical features include a jerky gait, a grimacing face and an inability to move the eyes voluntarily without blinking or moving the head forward. In more advanced forms, there are also significant problems with chewing and swallowing. These problems worsen as the disease progresses, culminating in the inability to perform certain movements, such as dressing, washing, getting around on one’s own, eating and even communicating, all of which have a major impact on people’s independence.

COGNITIVE SYMPTOMS

Huntington’s disease affects a wide range of cognitive functions, particularly the executive functions. Patients have difficulty reasoning, planning, carrying out complex tasks or several tasks at the same time, and organising their daily activities. Problems with language and visual perception may appear, and there is often a slowdown in information processing, resulting in a longer reaction time to external stimulation. This cognitive decline generally leads to severe dementia in the advanced stages of the disease.

PSYCHIATRIC AND BEHAVIOURAL SYMPTOMS

Psychiatric and behavioural disorders are very common in Huntington’s disease and often precede the motor symptoms. Anxiety, depression and apathy are the most common. Psychotic states with delusions (in 10% of cases), hallucinations or obsessive disorders (more than 15% of cases), similar to schizophrenia, may occur.

OTHER SYMPTOMS

Other symptoms may affect patients, such as sleep disorders, circadian rhythm disorders (biological processes that take place over 24 hours, such as sleep-wake rhythms, body temperature or hormone production), or weight loss.