Diseases Treatment

What do doctors call this condition?

Huntington’s chorea, hereditary chorea, chronic progressive chorea, adult chorea
What is this condition?

Huntington’s disease is a hereditary disease in which degeneration of the cerebral cortex and basal ganglia cause chronic progressive chorea (involuntary movements) and mental deterioration, ending in dementia. Huntington’s disease usually strikes persons between ages 25 and 55 (the average age is 35); however, 2% of cases occur in children, and 5% as late as age 60. Death usually results 10 to 15 years after onset, from suicide, congestive heart failure, or pneumonia.
What causes it?

The cause of Huntington’s disease is unknown. Because this disease is transmitted as a genetic trait common to men and women, either sex can transmit and inherit it. Each child of a parent with this disease has a 50% chance of inheriting it; however, the child who doesn’t inherit it can’t pass it on to his or her own children.
What are its symptoms?

Onset is insidious. The person eventually becomes totally dependent, emotionally and physically, through loss of musculoskeletal control. Gradually, the individual develops progressively severe choreic movements. Such movements are rapid, often violent, and purposeless. Initially, they appear on one side and are more prominent in the face and arms than in the legs. They progress from mild fidgeting to grimacing; tongue smacking; indistinct speech; slow, writhing movements (especially of the hands) related to emotional state; and contracted neck muscles.

Ultimately, the person with Huntington’s disease develops dementia, although the dementia doesn’t always progress at the same rate as the chorea. Dementia can be mild at first but eventually severely disrupts the personality. Such personality changes include obstinacy, carelessness, untidiness, moodiness, apathy, inappropriate behavior, loss of memory and concentration, and sometimes paranoia.
How is it diagnosed?

Huntington’s disease can be detected by positron emission tomography and DNA analysis. Diagnosis is based on a characteristic clinical history: progressive chorea and dementia, onset in early middle age (35 to 40), and confirmation of a genetic link. Computed tomography scan (commonly called a CAT scan) and magnetic resonance imaging (commonly called an MRI) demonstrate brain atrophy. Molecular genetics may detect the gene for Huntington’s disease in people at risk while they’re still symptom-free.
How is it treated?

Because a cure for Huntington’s disease has not yet been found, treatment is supportive, protective, and symptomatic. Tranquilizers, as well as anti psychotics such as Thorazine, Haldol, and Tofranil, help control choreic movements. They also relieve discomfort and depression, making the person easier to manage. However, tranquilizers increase rigidity and can’t stop mental deterioration. Institutionalization is often necessary because of mental deterioration.

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