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Huntington’s disease

Steve Smith discusses Huntington’s disease
© University of East Anglia

The symptoms of Huntington’s disease (HD) usually manifest in midlife- between the ages of 35-50, though it can occur earlier or later in life than this.

Over many years a movement disorder (chorea- uncoordinated involuntary dance-like jerking movements), begins insidiously and gradually worsens, along with increasing problems with thinking and planning, and managing impulses and moods. Mental health problems such as depression and anxiety are common and some experience psychosis, with effects such as hallucinations and / or delusions.

Eventually control of all muscle movement including those muscles associated with breathing and swallowing are affected and death is often the result of aspiration pneumonia (inhaling fluids and foods) or choking.

HD is inherited as a dominant genetic trait. This means that the offspring of a person who has HD is at a 50% risk of developing the condition. So, within one household a person with early effects of HD may be caring for a parent in the advanced stages and for children who are at 50% risk of developing the disease in later years.

There is no current treatment that slows or halts disease progression but researchers are increasingly hopeful that a breakthrough will change that situation within a decade or so. Meanwhile, many treatments are effective in managing effects such as movement and mental health problems.

Life expectancy for people with HD has increased significantly over recent decades due to interventions to avoid choking and aid swallowing and breathing, and good nursing including pressure care, avoiding development of pressure ulcers.

© University of East Anglia
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