What is Huntington’s Disease (HD)

Huntington's  Disease  is  a  genetically  transmitted neurological illness caused by a genetic stutter, a stretch of DNA that is repeated over and over at one end of a gene on chromosome four. A progressive disorder of the brain, Huntington's disease affects how people think, feel, and move.

 

There are two types of HD: adult-onset and juvenile. The adult-onset form—the most common—usually strikes people in their thirties and forties. The symptoms of HD vary from person to person. Early symptoms can include psychological changes such as depression or irritability; cognitive changes such as difficulty learning new things, remembering, and making decisions; and changes in motor ability such as coordination difficulties and the onset of small involuntary movements.

 

As the illness progresses and more areas of the brain are affected, the HD-afflicted individual’s psychological, cognitive, and motor functions worsen. Involuntary jerking, twisting, and twitching movements, referred to as chorea, become more pronounced. There is a decline in the individual’s ability to walk, talk, and swallow. The cognitive functions of thinking and reasoning decline and psychological changes can run the gamut of mental illnesses. The average individual with HD lives for fifteen to twenty years after the onset of symptoms.

 

Juvenile Huntington’s begins in childhood or adolescence and is less common than the adult-onset form. Like adult-onset HD, juvenile HD also causes motor, cognitive, and emotional changes. Motor symptoms such as clumsiness, slurred speech, rigidity, or drooling begin to manifest and the cognitive abilities of thinking and reasoning decline. Psychological changes may translate into behavioral difficulties. Thirty to fifty percent of children will have seizures. This form of HD progresses more quickly than the adult-onset type and affected children can live from ten to fifteen years after the onset of symptoms.

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An  estimated  30,000 people  in  the  U.S. have Huntington's disease. Those numbers are expected to increase since approximately 200,000 Americans are at risk of inheriting the disease from an affected parent. At risk individuals are the offspring of HD carriers who have not been tested for HD  either because they are too young or because  they have made a personal decision not to be tested. In the category of rare diseases, this population is one of the largest still waiting to have its clinical needs met. The HTT gene (or HD gene) is usually inherited from a parent in an autosomal dominant pattern, meaning that one copy in each cell is sufficient to cause the disease. Because the gene for Huntington’s is a dominant gene, there is 50% chance that each child conceived by a Huntington’s-gene-carrying parent could inherit the disease. Conversely, there is a 50% chance that each child could receive two normal recessive genes in which case the illness would not be expressed. In rare cases, Huntington’s has arisen in individuals whose parents do not have the condition, and there are individuals who have the HTT gene but do not develop the disorder. Despite being disorder free, these individuals still carry the HTT gene and   are at risk of having children who will develop the disorder.

HD does not discriminate. It affects both women and men and all ethnic groups. People from two to 80+ years of age can develop HD.  Individuals afflicted with HD  slowly decline over a period of 10-25 years.  Eventually, they become totally dependent on others for their care before dying from complications, such as heart failure or aspiration pneumonia. 

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Articles on this site are written after careful research by the author of Twisting Legacy. Even so, the author is not an expert on any of the subjects. Please do your own research if you have questions about the authenticity of the information in the articles on this website.  

An  estimated  30,000 people  in  the  U.S. have Huntington's disease. Those numbers are expected to increase since approximately 200,000 Americans are at risk of inheriting the disease from an affected parent. At risk individuals are the offspring of HD carriers who have not been tested for HD  either because they are too young or because  they have made a personal decision not to be tested. In the category of rare diseases, this population is one of the largest still waiting to have its clinical needs met. The HTT gene is usually inherited from a parent in an autosomal dominant pattern, meaning that one copy in each cell is sufficient to cause the disease. Because the gene for Huntington’s is a dominant gene, there is 50% chance that each child conceived by a Huntington’s-gene-carrying parent could inherit the disease. Conversely, there is 50% chance that each child could receive two normal recessive genes in which case the illness would not be expressed. In rare cases, Huntington’s has arisen in individuals whose parents do not have the condition, and there are individuals who have the HTT gene but do not develop the disorder. Despite being disorder free, these individuals still carry the HTT gene and   are at risk of having children who will develop the disorder.

 

HD does not discriminate. It affects both women and men and all ethnic groups. People from two to 80+ years of age can develop HD.  Individuals afflicted with HD  slowly decline over a period of 10-25 years.  Eventually, they become totally dependent on others for their care before dying from complications, such as heart failure or aspiration pneumonia.

Articles on this site are written after careful research by the author of Twisting Legacy. Even so, the author is not an expert on any of the subjects. Please do your own research if you have questions about the authenticity of the information in the articles on this website.