Huntingtons disease profile

Topics: Huntington's disease, Basal ganglia, Trinucleotide repeat disorder Pages: 6 (1045 words) Published: October 1, 2013
A profile of

HUNTINGTON'S
DISEASE

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)



Muscle rigidity



Slow, uncoordinated fine movements



Slow or abnormal eye movements



Impaired gait, posture and balance



Difficulty with the physical production of speech



Difficulty swallowing

Cognitive disorders
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

Psychiatric disorders
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.

Gene locus:
The gene for Huntington's disease has been localized at the end of the short arm of...


Bibliography: 1. Huntington. (n.d.). Huntington. Retrieved June 1, 2013, from
http://www.huntingtonsociety.ca
2. Huntington 's disease. (n.d.). Mayo Clinic. Retrieved June 1, 2013, from
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3. Huntingtons disease. (n.d.). omim.org. Retrieved May 30, 2013, from
omim.org/entry/143100
4. Young, A. (2012). huntingtons disease. Access Science , .. Retrieved
May 30, 2013, from
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ngton 's-disease/325700
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