Huntington's disease is also known as Huntington's chorea, is an inherited disorder that results in the death of brain cells, a general lack of coordination and as the disease advance turns into uncoordinated jerky body movements. Loss of physical abilities leads to many problems associated with walking, running and doing basic day to day work.
Symptoms generally appear between the ages of 30 to 50 years. It can be inherited from the parents or may be acquired during lifetime due to mutations.
Huntington's disease is an Autosomal dominant disorder that means if one parent is affected there is 50% chance of transferring the disease to progeny. This disease gradually damages the cells of the brain. There is no cure for this disease till now, but we can treat some symptoms.
Some interesting facts -
- This disease progress very slowly over a 10-20 year period and affected individuals cannot walk, talk and think properly. It is usually occurring between the ages of 30 and 50, it generally affects the basal ganglia which control all the body movements of the body, and brain cortex which helps in thinking and perception.
- The most common symptom of the disease is involuntary jerky movements of the limbs and facial muscles. Due to functional damage of brain cortex, there are mood swings, depression, irritability, slurred speech. As the disease progress symptoms become severe like difficulty swallowing, unsteady gait, loss of balance, impaired reasoning and memory problems, Death can occur in severe cases of pneumonia, heart failure, and other complications.
- Diagnosis includes taking the history of the patient, detail clinical examination. Brain scans and EEG for recording the electrical activity of the brain, the identification of the gene that causes Huntington's disease by genetic testing. Prenatal testing in the case of children, there is no cure for this disease but by medications, we can overcome some severe symptoms of the disease.
- At present, about 30,000 Americans are affected by Huntington's disease and about 200,000 are at risk of inheriting the disease, it is now considered the most common hereditary disease associated with the brain.
- If we take the example of the whole world, Huntington's disease is affecting about 1 individual in every 15000 individuals. Huntington's disease can be found in every country of the world and pass from one generation to other.
- Earlier signs of the disease are mood swings, irritability, apathetic, passive, depressed or angry, these symptoms can be overcome by medication or become severe as the disease progress.
- A few individuals develop this disease at the age of 55, in these peoples it is very difficult to diagnose the disease and person may not display the severity of symptoms that are generally present in individuals with Huntington's disease.
- There is a disorder related to Huntington's disease called senile cholera.
- Most drugs that are used for this disease have side effects like fatigue, restlessness or hyperexcitability.