Huntington disease (HD) is a hereditary neurological disorder.
The disease causes certain areas of the brain to atrophy,
or break down, faster than normal. The death of cells
in a certain part of the brain results in a triad of clinical
features that include changes in an individual’s
motor (choreic movement), cognitive (thinking or reasoning)
and emotional (personality changes or depression) abilities.
Symptoms usually appear between the ages of 30 and 45.
Although rare, symptoms of Huntington disease can appear
as early as childhood as well as very late in life. The
average duration of the disease is 15 to 25 years.
In the early stages of HD, perhaps even before a diagnosis
is made, there can be some changes in reasoning, movement,
and emotions. Often the affected person either does
not notice or denies the changes. In some cases, a family
member, employer, or health care professional may be
the first to notice that something is different or “wrong.”
In an early stage, a person can go about doing all of
their usual daily tasks and is able to be active and
maintain employment. Some of the most common early symptoms
of Huntington disease include clumsiness, forgetfulness
and depression. Of course, these are problems that anyone
can experience, which makes it difficult to determine
if these problems are indeed caused by the onset of
Huntington disease.
As the disease progresses, the person typically begins
to have trouble accomplishing daily tasks, especially
employment. They can perform most of their usual roles,
just not in the way they used to. They may be depressed,
somewhat easy to anger, and resentful of suggestions
that they are not performing well, but they continue
to be independent and active.
In the later disease stages, a person can usually no
longer manage employment or full household tasks and
requires assistance and supervision in handling financial
affairs. Activities of daily life, such as dressing
and going to the bathroom, are impaired but can be managed
with minimal help.
In the advanced stages of the disease, people are typically
no longer independent in function, but they are capable
of being supported within the family home or by minimal
care in an extended care facility. Throughout the advanced
stages symptoms typically continue to worsen until the
person is no longer able to complete normal activities
of daily living. Some affected persons will eventually
no longer be able to function independently and may
require continued professional health care for complete
support with the activities of daily living. Death usually
occurs from an associated condition, such as pneumonia
or choking.
Remember that this is just a general list of symptoms.
Every person affected with Huntington disease is different.
Some people will have jerky movements, some will not.
Some will lose the power of speech; some will be able
to speak. Many symptoms depend on the individual and
on the availability of help and encouragement from family,
friends, and health care professionals.
There are no treatments which will stop or reverse the
disease at the present time. It is possible to treat
some of the effects, such as depression and jerking
movements, with various medications. Improvements in
general health, such as good nutrition, will bring about
improvements in the condition of the person and in their
enjoyment of life. Many scientists are working on finding
treatments and a cure for Huntington disease.
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