Chorea is a neurological manifestation resulting in involuntary and uncontrollable movements. This can be of hereditary origin (Huntington’s chorea) or occur following a bacterial infection with certain streptococci (Sydenham’s chorea). Causes, symptoms, treatments, etc. Know everything.

Chorea is a neurological disease that results in involuntary, repetitive, jerky and rapid movements, which manifest in one part of the body, before suddenly moving to another part. These sudden and irregular movements can affect any part of the body at any time of the day, except during sleep. This term is commonly used to refer to two diseases: Sydenham’s chorea and Huntington’s chorea. Both are relatively rare, and Sydenham’s chorea has almost disappeared thanks to antibiotic treatments.

Causes of Sydenham’s chorea

Also known as St. Vitus dance or rheumatic chorea, Sydenham’s chorea is an infectious disease of the central nervous system, which follows bacterial infection with certain streptococci. It is a complication of rheumatic fever, an inflammatory disease that mainly affects children between the ages of 5 and 15, and is manifested by fever, joint pain and a rash.

Causes of Huntington’s chorea

Huntington’s chorea is an inherited neurodegenerative disease which, by destroying certain neurons, affects motor, cognitive and behavioral functions. These disorders lead to a progressive loss of autonomy, until the grabatisation. Huntington’s chorea is caused by a mutation in the gene encoding the huntingtin protein. If this gene was identified in the 90s, Huntington’s disease remains incurable to this day. According to Inserm, it affects 18 people in France: around 000 already have symptoms and around 6 are carriers of the mutated gene but still asymptomatic. You should know that any person carrying the gene will develop the disease sooner or later. The disease affects both women and men, and usually begins between the ages of 000 and 12. On average, death occurs twenty years after the onset of symptoms.

Sydenham’s chorea and Huntington’s chorea have in common the involuntary (so-called choreic) movements that they cause. Other symptoms are specific to each disease.

Symptoms of Huntington’s chorea

Abrupt and involuntary movements are the most characteristic symptom of chorea. All the muscles are gradually affected. Stress and concentration would be contributing factors. Other symptoms such as dystonia (involuntary contractions of muscles), abnormal postures or slow movements may also occur. Daily actions such as eating, dressing, chewing, swallowing or even talking are becoming more and more difficult. Over time, the patient loses his autonomy. At the same time, language, vision and memory disorders may appear. Other signs can testify to Huntington’s disease: anxiety, depression, irritability or even psychotic states.

Symptoms of Sydenham’s chorea

In addition to the involuntary, sudden, and irregular movements that are characteristic of chorea, Sydenham’s chorea mainly manifests as fever, restlessness, headache, joint pain, and rash. Fatigue, swallowing disorders and a decrease in muscle strength have also been described.

The treatment of Sydenham’s chorea is based on the administration of antibiotics which eliminate the streptococcus that causes the disease. However, to date, there is no curative treatment for Huntington’s disease.

However, certain measures can improve the patient’s quality of life. This is the case with neuroleptics which are given to reduce choreic movements and anti-depressants which relieve depression. In addition to this drug treatment, physical rehabilitation through physiotherapy helps maintain the muscles while reducing balance disorders.

Speech therapy sessions may also be indicated to improve swallowing and language disorders. Prenatal diagnosis can be done to look for a mutation in the huntingtin gene in the fetus during pregnancy. This genetic test can only be performed if one of the two parents carries the gene. If the result is positive, the couple can decide to terminate the pregnancy. This genetic abnormality can also be looked for in people having recourse to in vitro fertilization. In fact, healthy embryos can be implanted in the mother’s uterus.