Huntington’s Disease
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Huntington’s disease is a movement disorder characterized by chorea and dementia. It is inherited in an autosomal dominant manner and occurs throughout the world, in all ethnic groups, with a prevalence rate of about 5 per 100,000. The gene responsible for the disease has been located on the short arm of chromosome No. 4. At 4p16.3 there is an expanded and unstable CAG trinucleotide repeat. Symptoms do not usually develop until after 30 years of age, by which time the patient has usually had children, and so the disease continues from one generation to the next. The cause of Huntington’s disease is unknown. Online Trusted Pharmacy
Clinical Findings
Clinical onset is usually between 30 and 50 years of age. The disease is progressive and usually leads to a fatal outcome within 15–20 years. The initial symptoms may consist of either abnormal movements or intellectual changes, but ultimately both occur. The earliest mental changes are often behavioral, with irritability, moodiness, antisocial behavior, or a psychiatric disturbance, but a more obvious dementia subsequently develops. The dyskinesia may initially be no more than an apparent fidgetiness or restlessness, but eventually choreiform movements and some dystonic posturing occur. Progressive rigidity and akinesia (rather than chorea) sometimes occur in association with dementia, especially in cases with childhood onset. CT scanning usually demonstrates cerebral atrophy and atrophy of the caudate nucleus in established cases. MRI and positron emission tomography (PET) have shown reduced glucose utilization in an anatomically normal caudate nucleus. Buy Cheap Antibiotics pills
Chorea developing with no family history of choreoathetosis should not be attributed to Huntington’s disease, at least not until other causes of chorea have been excluded clinically and by appropriate laboratory studies. In younger patients, self-limiting Sydenham’s chorea develops after group A streptococcal infections on rare occasions. If a patient presents solely with progressive intellectual failure, it may not be possible to distinguish Huntington’s disease from other causes of dementia unless there is a characteristic family history or a dyskinesia develops. Canadian Prescriptions Drugs Read the rest of this entry »