A profile of
Huntington's disease is an inherited degenerative neurological disease that leads to dementia. It is a disorder of the basal ganglia causing progressive motor incoordination, abnormal involuntary movements (chorea), and intellectual decline.
Clinical features and Symptoms:
Huntington's disease usually causes movement, cognitive and psychiatric disorders with a wide spectrum of signs and symptoms. Which symptoms appear first varies greatly among affected people. During the course of the disease, some disorders appear to be more dominant or have a greater effect on functional ability. The disease begins insidiously with restlessness, choreiform (dancelike) movements, and personality changes (irritability, depression, and occasionally psychosis). The symptoms gradually worsen, with severe involuntary, large-amplitude motions, grimacing, poor balance, and very poor fine-motor coordination. Abnormalities of eye movement also occur early in the disease and worsen with its progression; speech and swallowing are always affected. A detailed list of symptoms is outlined below. Movement disorders
The movement disorders associated with Huntington's disease can include both involuntary movements and impairments in voluntary movements:
Involuntary jerking or writhing movements (chorea)
Involuntary, sustained contracture of muscles (dystonia)
Slow, uncoordinated fine movements
Slow or abnormal eye movements
Impaired gait, posture and balance
Difficulty with the physical production of speech
Cognitive impairments often associated with Huntington's disease include: •
Difficulty planning, organizing and prioritizing tasks
Inability to start a task or conversation
Lack of flexibility, or the tendency to get stuck on a thought, behavior or action (perseveration)
Lack of impulse control that can result in outbursts, acting without thinking and sexual promiscuity
Problems with spatial perception that can result in falls, clumsiness or accidents
Lack of awareness of one's own behaviours and abilities
Difficulty focusing on a task for long periods
Slowness in processing thoughts or "finding" words
Difficulty in learning new information
The most common psychiatric disorder associated with Huntington's disease is depression. This isn't simply a reaction to receiving a diagnosis of Huntington's disease. Instead, depression appears to occur because of injury to the brain and subsequent changes in brain function.
After the onset of Huntington's disease, a person's functional abilities gradually worsen over time. The rate of disease progression and duration varies. The time from disease onset to death is often about 10 to 30 years. Juvenile onset usually results in death in fewer than 15 years. Death frequently results from secondary causes such as pneumonia( or other infections), injuries related to falls and complications due to inability to swallow. About 10% of those affected have a rigid form of the disease in which choreiform movements are less prominent, and stiffness and
slowness of movement and abnormal posturing (dystonia) are the dominant features. This variety is more likely to occur when the disease begins before the age of 20 years. Therapy is merely supportive: no medications significantly affect the course of the disease or functional capacity of the sufferer. Depression or psychosis, however, can be temporarily alleviated by antidepressant and antipsychotic medications. A progressive intellectual decline is usually observed, but even in the late stages of the disease, patients can often recognize family members and interact with other people to a limited extent.
The gene for Huntington's disease has been localized at the end of the short arm of...
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