A dominant genetic disorder in which a protein is produced abnormally, leading to the breakdown in the parts of the brain that control movement.
An autosomal dominant inherited disorder, usually beginning in middle age, featuring insidious progression of mental abnormalities associated with frontal lobe damage (emotional, personality, and intellectual) and motor abnormalities associated with basal ganglion damage (hence the alternate name, Huntington's chorea). Although there are exceptions, most patients with this disorder deteriorate to a vegetative state in 10-15 years. Pathological examination of the brain reveals extensive degeneration of the striatum, and to a lesser extent, frontal cortex. Molecular biology reveals abnormal expansion of the triplet repeat at the 3' end for the coding region of a newly characterized gene; its function is not yet understood.
An inherited, degenerative brain disease affecting the mind and body, characterized by intellectual decline and involuntary movement of limbs.
formerly Huntington's chorea, hereditary disease of the central nervous system, beginning usually in middle age and characterized by involuntary jerky movements, personality changes, and progressive mental deterioration. It is caused by a mutated gene on chromosome 4. The child of a person with Huntington's has a 50% chance of inheriting the gene, which inevitably leads to the disease Hydrolysis
trinucleotide repeat, autosomal dominant condition characterised by progressive dementia and the development of a movement disorder, most commonly chorea
A hereditary, progressive, neurodegenerative disorder primarily characterized by the development of emotional, behavioral, and psychiatric abnormalities; gradual deterioration of thought processing and acquired intellectual abilities (dementia); and movement abnormalities, including involuntary, rapid, irregular jerky movements (chorea) of the face, arms, legs, or trunk. HD may be inherited as an autosomal dominant trait or, less commonly, appear to occur randomly for unknown reasons (sporadically). The disorder results from abnormally long sequences or "repeats" of certain coded instructions (i.e., unstable expanded CAG repeats) within a gene (located on chromosome p16.3). Progressive nervous system dysfunction associated with HD results from loss of neurons in certain areas of the brain, including the basal ganglia and cerebral cortex.
A progressive neurological disorder which manifests as dementia, depression, and involuntary movements. It is caused by a gene mutation on the short arm of chromosome 4 (the HD gene).
A genetic disease of the central nervous system that produces progressive dementia and involuntary movements. It usually starts between ages 30 and 50 and slowly progresses to death. Since it is a dominant disease, a child of a parent with Huntington's disease has a 50-50 chance of inheriting it.
hereditary disease; develops in adulthood and ends in dementia
a degenerative hereditary disorder caused by a faulty gene for a protein called huntington. The disease causes degeneration in many regions of the brain and spinal cord and patients eventually develop severe dementia.
a disease characterized by involuntary jerking or writhing of the limbs and face and mental deterioration; usually results in death within 15 years.
an inherited disease of the CNS with a usual onset between the ages of 25 and 55 (35 is the average age); degeneration in the brain causes bizarre, involuntary dance-like movements and mental deterioration, ending in dementia; death usually results in 10 to 15 years
An inherited adult-onset disease of the brain characterised by dementia and involuntary movements. The disease is progressive and there is currently no known cure
A disease with involuntary jerky movements particularly affecting the shoulders, hips and face, and accompanied by a progressive dementia.
A degenerative brain disorder that usually appears in mid-life. Its symptoms, which include involuntary movement of the face and limbs, mood swings, and forgetfulness, get worse as the disease progresses. It is generally fatal within 20 years.
an adult onset disease characterized by progressive mental and physical deterioration; it is caused by an inherited dominant gene mutation.
a progressive genetic disorder involving the degeneration of nerve cells in the brain. It is characterised by abnormal movements of the body and mental decline.
A progressive hereditary disorder that involves degeneration of the basal ganglia and that results in jerky limb movements, facial twitches, and uncontrolled writhing of the body.
An inherited brain disease affecting the body that is characterized by mood changes, intellectual decline, and involuntary movement of limbs.
An inherited sex-linked nervous disease that develops in adulthood and leads to a state of deteriorated mentality.
A hereditary neurological disorder characterized by movement, cognitive, and psychiatric symptoms.
Huntington's disease (HD), also known as Huntington disease and previously as Huntington's chorea and chorea maior, is a rare inherited neurological disorder affecting up to 8 people per 100,000. It takes its name from the Ohio physician George Huntington who described it precisely in 1872 in his first medical paper. HD has been heavily researched in the last few decades and it is one of the first inherited genetic disorders for which an accurate test can be performed.