Chorea progressive dementia

Huntington s disease CAG 11-30 unaffected 36-121 affected Chorea, progressive dementia, death... 

This dominantly inherited disorder is characterized by progressive chorea and dementia that usually... 

HD is an autosomal dominant, late-onset neurodegenerative disorder with an incidence of about 1 in 10,000 in most populations of European origin. The disease is progressive and characterized by frequent involuntary, rapid movements (chorea) and dementia with a median survival time of 15 to 18 years after the onset of symptoms. The mean age of onset is in the decade between 35 and 44 years, but approximately 25% of patients first display symptoms after the age of 50, and about 10% of patients have juvenile HD with the age of onset before 20 years. In the first few years of the disease, symptoms include mood disturbances, cognitive deficits, clumsiness, and impairment of voluntary move-ment. The next stage of the disease is associated with... 

Huntington disease (HD) is the prototypic disease caused by expansion of unstable GAG repeat. It primarily affects striatal neurons. It is a mid-life onset disorder characterized by unvoluntary movements (chorea), personality changes and dementia that progress to death within 10-20 years of onset. There are currently no treatment to delay or prevent appearance of the symptoms in the patients. Other diseases in this class include spinocerebellar ataxias (SCA) 1, 2, 3 (also known as Machado-Joseph disease, MJD), 6, and 7, DRPLA, and spinobulbar muscular atrophy (SMA, also known as Kennedy s disease) (Zoghbi and Orr 2000). 

Huntington s disease, an autosomal dominant disorder, has a mean age-of-onset of 43-48 years. Symptoms appear gradually and worsen over a period of about 15 years until death occurs. Mood disturbance, impaired memory, and hyperrefiexia are often the first signs, followed by abnormal gait, chorea (loss of motor control), dystonia, dementia, and dysphagia. Cases of juvenile onset (<10 years old) are more severe and most frequently occur when the defective allele is inherited paternaily. About 25% of cases have late onset, slower progression, and milder symptoms. 

Huntington s chorea is a rare, dominantly inherited, progressive disease characterised by chorea (brief involuntary jerky muscle contractions) and dementia. It has an insidious onset and usually occurs between 30 and 50 years of age. Symptoms include uncontrolled movements, personality disorders, severe depression and anxiety. 

Huntington s disease (HD) is an autosomal dominant disorder characterized by progressive chorea, dementia. 

An inherited disease of the CNS that usually has its onset between ages 25 and 55. Degeneration in the cerebral cortex and basal gangha causes chronic progressive chorea and mental deterioration, ending in dementia. 

Consider rarer dementias if unusual (particularly neurological) symptoms frontotemporal, dementia in Parkinson s disease, Huntingdon s disease (family histoiy, chorea), Creutzfeldt Jakob Disease, AIDs-related encephalopathy, neurosyphilis, progressive supranuclear palsy. 

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