Huntington disease was discovered by George Huntington; he wrote an article about a disorder that is passed on through generations.
This disorder was formerly known as chorea. Chorea is a practical term to describe the common characteristics of this disease. It is usually characterized by a twist, wriggle, and spin in a rigid, irrepressible manner. Other eloquent names developed as years passed by. Inherited chorea put emphasis on how the disorder is transferred from one generation to another. Long-term progressive chorea emphasizes on the process of how symptoms deteriorate after a while. Today, people usually call this disorder as HD or Huntington disease.
Huntington disease recently became the focus of attention among scientists. Unfortunately, they have only identified a little information about this disease. They had no choice but to just observe the persistence of this condition to pass on through generations.
What are the Symptoms of Huntington Disease?
Huntington disease commonly results in cognitive, psychiatric, and movement disorders with a wide variety of manifestations. Which manifestations emerge first depends significantly among the concerned persons. Throughout the course of this disorder, some people may have a dominant effect on the person’s abilities.
Movement dysfunctions are linked to this disease and can include both voluntary and involuntary movements of the patient such as the following:
• Chorea or instinctive writhing movements
• Dystonia or instinctively maintained contracture of muscles
• Muscle firmness
• Sluggish and inept fine actions
• Sluggish and irregular movements of the eyes
• Unsteady posture, balance, and gait
• Difficulties in producing speech
• Dysphagia or difficulty in swallowing
Patients with Huntington disease can be greatly affected in their abilities to work, execute daily actions, stay independent, and communicate.
Cognitive disorders are usually linked with this disease which include the following:
• Difficulty in prioritizing, organizing, and planning responsibilities
• Incapacity to establish a conversation or a task
• Inflexibility or getting fixed on a behavior and thought
• Lack of impulse power which can result in flare-ups
• Sexual promiscuity
Psychiatric impairments are also linked with Huntington disease. The most usual psychiatric impairment is depression. Depression is anticipated to occur due to an injury to the brain. Manifestations of this disorder can involve the following:
• Thoughts of unhappiness or sadness
• Lack of interest in daily activities
• Social abandonment
• Extreme fatigue and loss of energy
• Guilt feelings
Huntington’s disease also has a juvenile type like diabetes mellitus. In this condition, the commencement of this disease progresses in younger persons. Symptoms of this type of Huntington disease vary from the adult ones. The dysfunctions associated with this disease involve the following:
• Physical skills and academic information are lost
• Quick, noteworthy drop in academic standing
• Behavioral dilemmas
Can a Person Outgrow Huntington Disease?
The progression of this disease and the age of its onset differ from one person to another. The adult onset of HD may progress in a person. These people may have rigid movements termed as akinesia. It is more common in adults than young people.
Some people build up manifestations of this disease at a very young age. This condition is termed as Juvenile Huntington disease. People with this disorder can have it before they reach 20 years old. A typical sign of HD in younger persons is a quick drop in his academic performance. Manifestations can also involve a slight change in a person’s handwriting and subtle difficulties in movements, such as rigidity, tremors, sluggishness, and myoclonus.
As you observe patients with this disorder, you will notice that the symptoms that they exhibit are similar to Parkinson’s disease. These people as well can have mental retardations and seizures. The progression of HD depends on its onset. It develops quickly in juvenile cases, and bereavement results within ten years. So this type of disease can’t be outgrown by a patient; instead, it can get worse as time passes by.
Research was made about this disease, and they have proven that it can be inherited from the patient’s father. The method of sperm production has a great significance for this information since sperm cells are replicated millions of times. The tendency is there would be a possible mix-up of genetic components within the cells. A few elderly people can have senile chorea. They can have involuntary movements but don’t show signs of dementia. Gene mutations can also occur which can lead to the incidence of this condition.
How to Care for an Individual Who has Huntington Disease?
The initial process in caring for this patient is diagnosing Huntington disease by referring the patient to a neurologist. Monitoring this patient is necessary to measure the person’s capabilities. Dilemmas may come about when these types of persons try to articulate multifaceted insights in words that they cannot enunciate properly. It may be useful to reiterate words back to them. This is to boost his confidence that some insights are comprehended.
What are the Complications of Huntingtons Disease?
The patient’s efficiency to perform tasks gradually worsens as the disease progresses subsequent to the onset of this disease. The duration and progression of the disease differ with every patient. Frequently, it will take 10-30 years after the onset of the disease to cause the death of a patient. The medical depression linked with Huntingtons disease can augment the chances of suicide. Research recommends that a higher chance of suicide happens sooner than a diagnosis is performed. In-between junctures of the disease, a person has started to become dependent on people around him.
In due course, Huntingtons disease patients require assistance with all tasks of daily living. In the late phase of the disease, the patient may probably be flat on his back and unable to utter a single word. Nevertheless, the patient’s perception of his interactions and surroundings stays integral for a long moment in time. Patients with this disorder can eventually die because of various conditions, such as injuries linked to falls, infections or pneumonia, and dysphagia or difficulty swallowing.
What are the Diagnostic Tests for Huntingtons Disease?
The diagnosis of this disease is based mainly on the patient’s answers to the queries of a physician, a complete physical examination, and an evaluation of his family’s medical records of psychiatric disorders.
Neurologic examinations will be done by a neurologist. He will ask the patient some questions and perform fairly simple exams to assess the patient’s muscle strength, muscle tone, reflexes, vision and eye movements, tactile sensations, auditory abilities, coordination, balance, mood, and mental standing. Psychiatric assessments will be performed by a psychiatrist to rule out numerous psychiatric disorders that could give way to the patient’s diagnosis. This series of tests includes the assessment of behavioral patterns, emotional stability, judgment quality, and signs of substance abuse.
Brain imagery and function assessments are also recommended to these patients. These may include a brain-imaging examination to divulge structural damage at specific locations in the brain cavity. These tests are utilized to discount the occurrence of other conditions that can cause manifestations to come about. These imaging procedures can involve the use of MRI and CT scans. An EEG, or electroencephalogram, is used as well to document the brain’s electrical activities. Definite irregular patterns in the activities in the brain can point out problems possibly contributing to the incidence of seizures.
Genetic testing and counseling are suggested for patients with an evident diagnosis of Huntingtons disease to identify the faulty gene. Genetic testing can also confirm the occurrence of the disease. The information from this test will be very valuable for the members of the family. This will make them aware of their risks. The results of this test can’t be the basis of a patient’s treatment regimen. It just confirms that the patient has the disease.
What are the Treatments for Huntingtons Disease?
There is no specific treatment that can alter the development of this disorder. Medications can only be a palliative treatment to these patients. These medications can only lessen the effects of some symptoms. A multifaceted approach can aid a person to become accustomed to certain alterations in his capabilities. The drug prescription can evolve over the stages of this disorder. Consequently, the treatment goals will also be altered due to a change of treatment regimen. These plans and goals are frequently updated and reviewed.
There are specific drugs that are prescribed to manage movement dysfunctions. Tetrabenazine was approved by various sectors of the society to control the involuntary jerking movements manifested by this disease. This drug has side effects, such as sleeplessness, nausea, drowsiness, and restlessness. Antipsychotic medications are also given to suppress involuntary movements. These medications are useful in managing chorea. Antidepressants are given to decrease the depressive state of the patient.
Psychotherapy is also part of the multifaceted approach for Huntington disease. This is performed by a psychotherapist. These professionals can offer counseling therapies to assist the person to control his behavioral crisis, coping approaches, and facilitating effective communication among other members of society. Speech therapy can help the patient to improve his skills to communicate efficiently or teach the patient how to use devices to express his thoughts more clearly.
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