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Chorea (Huntington’s Disease) : Definition, Causes, Symptoms, Diagnosis and Treatment

Tuesday, August 14th 2012. | Brain and Nerves, Disease, Physical Health

Chorea Definition

The chorea is a syndrome, that is to say a set of symptoms, characterized by involuntary movements, irregular and rather large amplitude. There are several causes for chorea: vascular, neoplastic, endocrine, toxic or drug.

This syndrome is characteristic of both diseases: Sydenham’s chorea and Huntington’s chorea. Sydenham’s chorea or St. Vitus dance has almost disappeared with the advent of antibiotics that fight effectively against streptococcus involved. Huntington’s is it, a hereditary neurodegenerative disease : some neurons are destroyed leading to the onset of chorea. The mutation is dominant, meaning that a person carries the gene will inevitably develop the disease. This usually occurs between 30 and 45.

Huntington's disease

Chorea (Huntington’s disease) (img thanks to tqtqtq.edublogs.org)

Chorea Causes

Huntington’s disease is due to a fault (a repeat of DNA) on chromosome 4. This repetition of genetic information results in the production of an abnormal protein called huntingtin,. Mutant huntingtin aggregates in clusters in the nuclei of certain neurons in the brain, particularly the striatum, a brain structure involved in controlling movement. 

These neurons degenerate and eventually die, explaining the appearance of abnormal movements. Then huntingtin accumulates in the nuclei of neurons in the cerebral cortex also causing neuronal degeneration with consequent cognitive impairment (memory, attention …) and psychiatric disorders.

Chorea Symptoms

Different signs can appear early in the disease. For example, people are finding more clumsy than before and feel more nervous and anxious. Sometimes they have difficulty writing because their hands are jerking movements. These early mild symptoms and do not prevent subtle to live normally. Then, when the disease sets in, the person has gross motor difficulties obvious: it makes involuntary movements, irregular and illogical. These increase in cases of stress or physical exertion. Coordination necessary for walking and balance is done with more and more difficult. People with walking unsteadily. In addition, swallowing disorders and joints may accompany the motor signs.

Huntington’s disease is also characterized by mental disorders, including personality changes. In most cases, people with depressionThe environment is often a great irritability accompanied by an unusual aggressiveness. 

Other signs: cognitive impairment manifested, among other things, memory loss and an inability to concentrate.

What is the evolution of Huntington’s disease?

Huntington’s disease worsens progressively and inexorably.There is no universal chronology: the speed of this aggravation that comes with new attacks depends on the individual. At an advanced stage, involuntary movements are less frequent but members tend to stiffen. The people have become unable to perform most activities of daily living, then in need of assistance. The disease progresses for 20 years on average (there are milder forms in which life expectancy is longer), since the beginning of symptoms to death which usually occurs because of swallowing complications and disability.

Huntington's disease

Chorea Brain (img thanks to carewellunanihospital.blogspot.com)

 

Chorea Diagnosis

The diagnosis can be established from clinical signs – involuntary movements and psycho-behavioral disorders – and the existence of family history.

Neurological and psychological tests used to confirm the damage and a review of brain imaging (CT or magnetic resonance imaging – view a decrease in volume (atrophy) of certain areas of it (caudate and putamen) involved in the movement. Only genetic analysis from a blood test can confirm the diagnosis of Huntington’s disease.

Can we know if one has the gene mutated?

You can tell if someone is free from clinical signs of disease carrier. Generally, children with one parent has died or of Huntington’s are offered to perform a genetic test. The risk of disease transmission is 50%: when is a parent carrying the disease, there is a risk of transmitting the disease two to each of his children. They can therefore, as adults, go into genetic counseling in order to take stock of their desire to know if they are themselves carriers. Information that can be invaluable. Indeed, if a positive test, revealing the mutation on chromosome 14, and in case of desire for children, a pre-implantation diagnosis (PGD) is possible. PGD is, after in vitro fertilization, test embryos and implant only those that are healthy.

Chorea Treatment

There is not, to date, cure for Huntington’s disease. However some drugs, such as neuroleptics (which have an effect on twitching of muscles) and antidepressants (which have an effect on depression), to ease symptoms and help to slow the physical and intellectual impairments. In addition, physical therapy allows the solicitation of muscles and limit balance disorders. Speech therapy helps her to improve joint disorders and swallowing.

How to live with Huntington’s disease?

Huntington’s disease, further degradation of motor disorders, is characterized by behavioral disorders and aggressive sometimes painful for the environment that can also be supported psychologically. Furthermore, the person with Huntington’s disease tend to lose weight because of disorders of mastication and swallowing, extreme vigilance on nutrition is therefore essential. When the disease becomes too heavy, placement in a specialized unit may be considered.

What specialty concerned?

Neurology a

Neurology is a specialty devoted to the study of anatomy and functioning of the central nervous system (brain and spinal cord) or peripheral (nerves and roots) and treatment of diseases that can touch it. The neurological diagnosis is based on a careful history and examination of different reflexes and nervous system functions. It also calls for further investigations: imaging (MRI or CT), electroencephalogram (EEG), Electroneuromyography (EMG), lumbar puncture, neuropsychological tests.

Neurology supports the movement disorders – paralysis of cerebral origin or related to a spinal cord or peripheral nerves, or to a muscle disease (myopathy), movement disorders (tremors, tics, such as torticollis ), cramps – and sensitivity: tingling, numbness, insensibility, some balance problems but also neuralgic pains, migraines, headaches.

Epilepsy, disorders of consciousness and sleep disorders (insomnia, mental confusion) and disruption of brain function (memory, language, comprehension, drawing, calculating, reasoning …) are also the responsibility of the neurologist.

 

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