Huntington's disease (HD), known also as Huntington’s Chorea, is a progressive and ultimately fatal neuro-degenerational disease. HD is a devastatingly destructive disease where certain neurons (brain cells) degenerate because of genetic programming. Only those with the gene will develop the disease as well as potentially transmit it to offspring (see link in References). Progressively, activities of daily living are unable to be performed by the patient; eventually, function declines to the point of dependency. Clinical observations include involuntary movements, cognitive and emotional deterioration with increasing dependence. Huntington’s is incurable and profoundly affects everyone involved because of its devastating disease course.
Age of Onset
The age of onset and disease progression varies from person to person; however, the established mean age of onset is between 35 to 44 years, according to the Genetic and Rare Diseases Information Center (GARD). While onset is generalized to this middle-age range, rare onset of devastating HD has occurred in children as young as 2 years of age. Anyone with a family history of HD, who may or may not be exhibiting some symptoms, should seek immediate medical care.
Behavioral Symptoms
Behavioral signs and symptoms may include lack of coordination with involuntary jerky movements (chorea) and fidgety behavior with decreasing motor control. There is also a decreasing ability to walk, talk, drive, chew, eat or swallow. Also, rapid eye movements may occur.
Emotional Symptoms
Emotional signs and symptoms may include irritability or aggressive outbursts, anxiety, mood swings and apathy. Additionally, the symptoms of depression and dementia present themselves.
Cognitive Symptoms
Cognitive signs and symptoms may include impaired ability to concentrate or to reason with poor judgment capacity; reduced problem-solving ability; and, difficulty in thought organization. Also, the patient may have trouble learning new things. There will also be decreased attention span, as well as diminished short-term memory ability.
