Today is the International Day of a disease as unknown as it is famous. And although it sounds absurd to put together two such antagonistic terms, the reality is that everything depends on whether we give it one name or the other, and if we are of a certain age.
We are talking about a neurological disorder (Huntington’s disease) characterized by irregular and involuntary movements of muscle groups in various parts of the body.
A little history of the disease
If you are an older person and you moved around a lot as a child, it is very easy that you have heard your grandfather say that, “child, be still, it seems that you have the dance of San Vito”.
And that name was given by Thomas Sydenham, known as the British Hippocrates, in memory of a Christian boy who was tortured in the year 303.
Saint Vitus, born in Sicily, was just over 7 years old when, after converting to Christianity thanks to his nurse, he began to perform miraculous cures.
But, as the story goes, when he cured the son of the Emperor Diocletian of epileptic seizures, he threatened him with death if he did not renounce his God.
And given the child’s refusal, he had him killed in boiling oil. And they tell of his convulsions during his martyrdom were the ones that gave way to this legend.
Today, San Vito is considered the patron saint of epileptics, and in many parts of dancers and actors. He is also the protector against storms and hurricanes.
Some 4,000 people in Spain suffer from it
The Huntington’s disease It is a disease suffered by some 4,000 people in Spain, which was first described by the American doctor George Huntington in 1872. It is a rare hereditary pathology that causes the progressive degradation of nerve cells in the brain.
This November 13 is World Huntington’s Disease Day, which is also known as korea, which has its origins in the Greek word choreiawhich means dance in Spanish.
And it is that, one of the most common symptoms are the movement disorderswhich can be involuntary and jerky movement problems, difficulty speaking or swallowing (), slow eye movements, or muscle problems including body stiffness or muscle contractures.
This disease, which mainly affects the functional capacities of people, especially in the cognitive and psychiatric functionsIt usually appears between the ages of 30 and 50. If it occurs in youth, which is rare, it is called juvenile Huntington’s disease. In this case, the symptoms usually progress more rapidly, which incapacitates the patient in a few years.
Causes of Huntington’s disease
The most common cause is hereditary. As explained by , “HD does not skip any generation, but the symptoms of the disease can; for example, if the person carrying the disease dies before they begin to appear, which makes it more difficult to follow the story familiar”.
A person diagnosed with the disease has a 50% chance of inheriting it to their descendants.
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“If you have a 50% risk of inheriting the gene and decides not to pass the predictive test, statistically, their children have a 25% risk each. If a person does not inherit the altered HD gene, they will not develop the disease and cannot pass it on to the next generation.”
This implies that, although the decision to have children while carrying the gene is a strictly “personal” matter, a test before birth (prenatal) is recommended to rule out that the baby is also a carrier of Huntington’s disease. It can be done in two different ways:
- Amniocentesis. This is a test that is performed during pregnancy, through the extraction of amniotic fluid, to diagnose certain genetic disorders, such as Huntington’s disease.
- Umbilical cord sample analysis (cordocentesis). A blood sample is taken for further analysis.
Huntington’s disease symptoms
As explained by specialists from the prestigious Mayo Clinic, the symptoms of this disease can be classified into 3 areas since it can cause motor, cognitive and psychiatric disorders.
And knowing that the initial symptoms vary greatly from one person to another, we include here those that could be considered the most dominant:
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Difficulty learning and communicating.
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Lack of impulse control.
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Insomnia.
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Tendency to social isolation.
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Irritability and sadness.
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Suicidal thoughts.
As for the disorders caused by the disease we can find:
Movement disorders (involuntary or voluntary)
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Involuntary jerking or writhing movements
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Muscle problems, such as stiffness or muscle contractures
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slow or unusual eye movements
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Altered gait, posture and balance
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Difficulty speaking or swallowing
The Dr. Rosario Luquin Piudoa specialist from the Neurology Department of the , points out that “despite being involuntary movements, the subject tends to incorporate them into his repertoire of habitual movements in an attempt to hide them.”
Cognitive disorders
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Difficulty organizing or focusing
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Lack of flexibility or a tendency to get stuck on a thought or action
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Lack of impulse control, which can result in outbursts and sexual promiscuity
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Lack of awareness of one’s own behaviors and aptitudes
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Slow to process thoughts or find words
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Difficulty learning new information
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Psychiatric disorders such as depression
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Feeling irritable, sad, or listless
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Social isolation
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Insomnia
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Fatigue and loss of energy
Psychiatric disorders:
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(OCD), which is characterized by recurring and repetitive thoughts.
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