What is Huntington’s disease?
The first healthcare expert who examined and researched Huntington was Dr. George Huntington in the late 1800s. He wrote the now-famous article “On Chorea,” in which he went into great depth on the East Hampton family with this strange ailment. Huntington’s chorea was the term given to the disorder throughout time, but as not all Huntington’s disease patients have chorea, the name was modified to Huntington’s disease.
An uncommon hereditary disease called Huntington’s disease is mainly acquired from a parent and impacts the brain. According to estimates, three to seven out of every 100,000 persons have Huntington’s disease. Additionally, when progressed, issues from it might cause death. Even though there is no cure, more research is being done to develop one. Additionally, some therapies may improve a person’s quality of life and provide confidence.
What is Chorea?
One of several recognized involuntary movements in Huntington’s disease is represented as chorea but is not fatal. Other involuntary movements include more typical ones like tremors and tics. Because the representation of chorea varies from person to person, it might be challenging to recognize chorea to the untrained eye. Rheumatic fever, pregnancy, Huntington’s disease, and adverse responses to drugs used to treat gastrointestinal and behavioral health issues are among the causes.
- Neurological illnesses are associated with at least two additional chorea-related motions.
- The hands and feet often involve athetosis, a constant stream of sluggish, twisted, or squirming-like movements. Among other doctor-recommended treatments and therapies, Tetrabenazine may be prescribed to help treat these movement symptoms as they may closely mirror the symptoms of Tardive Dyskinesia.
- Ballimus often entails more forceful motions, including frantic arm- or leg-flinging. Typically, the motions affect just one side of the body (hemiballism).
Occurrence of chorea
Accurate figures on how many individuals experience chorea are unknown. Generally, chorea is a sign of another illness. The number of Americans with Huntington’s disease is about 30,000. According to medical professionals, 200,000 more individuals are at risk of Huntington’s disease since one or both of their parents have the hereditary disorder. The most typical sign of Huntington’s disease is chorea.
Rheumatic fever causes Sydenham chorea in roughly 4,000 children in the US each year. Untreated strep throat may lead to the dangerous consequence of rheumatic fever. Rheumatic fever is more likely to affect girls than boys. Generally, it affects children between the ages of 5 and 15.
Individuals of all ages can experience chorea. Moreover, the risk of chorea can be increased in specific groups of individuals, such as
People with a family history of Huntington’s disease:
The risk will be 50% that you will acquire Huntington’s disease if one of your parents has it. Typically, Huntington’s disease symptoms appear between the ages of 40 and 50. The most prevalent kind of chorea that is hereditary is Huntington’s disease.
Children who have had a rheumatic fever:
Rheumatic fever may lead to Sydenham chorea in children and adolescents, a side effect of untreated strep throat.
People with other medical conditions:
Chorea may be due to autoimmune conditions (like lupus), hormonal issues (like hyperthyroidism), and metabolic issues (like hypoglycemia). There is a vast list of other illnesses that may result in chorea.
Symptoms of Chorea
Regardless of the underlying cause of the movement condition, chorea symptoms mostly remain the same. The most typical chorea symptoms include:
Involuntary muscle movements:
Dance-like motions, often known as fidgety movements, typically include the hands, feet, and face. They may affect how you move, swallow, and speak. The motions might be smooth or a little erratic. They can make you seem to sing, play the piano, or even writhe in agony. When you’re upset or nervous, the motions often worsen and stop when you sleep.
One may attempt to shake someone’s hand, but instead of keeping your hand steady, you may repeatedly grab and release your fingers. Since it appears that you are milking a cow, doctors refer to this condition as a milkmaid’s grasp.
Jack-in-the-box tongue and Speech problems:
Your tongue can glide in and out of your mouth as you attempt to stick it out. Such a chorea symptom is also known as a jack-in-the-box tongue or a harlequin tongue.
Slurred speech may occur in both children and adults when the rhythm of the muscles that produce speech deteriorates.
Headaches and seizures:
Children who suffer from chorea after rheumatic fever may have headaches and convulsions. Some kids may struggle with emotional and behavioral problems.
Symptoms of Huntington’s disease
Huntington’s disease often introduces movement, cognitive, and psychological impairments, and its indications and symptoms may range considerably. It substantially differs from person to person, and symptoms manifest initially. While specific symptoms may be more prominent or have a more significant impact on functionality than others, this might alter during the illness.
Individuals’ capacity to work, carry out everyday activities, interact, and maintain independence may be more negatively impacted by impairments in voluntary movements than by limitations in involuntary movements.
Huntington’s disease-related movement abnormalities may affect both voluntary and involuntary motions, resulting in symptoms like:
- Writing movements and involuntary jerking
- Swallowing issues, and speech difficulty
- Unusual eye movements
- Issues with balance, posture, and gait
- Muscle rigidness and muscle contracture
The following are the cognitive issues related to Hungtington’s disease
- Lack of focus and difficulty in managing daily activities and tasks
- Inability to get stuck on actions and behaviour
- Sexual promiscuity or lack of impulse control
- Inability to learn new things and slowness in processing thoughts
Depression is the mental condition most frequently linked to Huntington’s disease, but this isn’t only a reaction to the Huntington’s disease diagnosis. However, brain damage and subsequent alterations in brain function seem to be the source of depression. Some warning signs and symptoms include:
- Suicidal thoughts
- Sadness, apathy, or feelings of irritability
- Lack of social interests
- Loss of energy and fatigue
One of the main signs of Huntington’s disease is chorea. While it is often linked to the illness, there are other potential reasons, like pregnancy, specific drugs, and other related diseases like hyperthyroidism.
The progression of Huntington’s disease occurs gradually. It eventually results in a person losing their physical and mental abilities. After symptoms start, individuals with Huntington’s disease may survive for 15 to 20 years.