In addition to the physical symptoms of HD, there are
often subtle cognitive signs as well.
Initial stages
People with early Huntington's may find they have
difficulty organizing routine matters or coping
effectively with new situations. Difficulty recalling
information may make them appear forgetful. Work
activities may become more time-consuming, and
decision-making and attention to detail may be
impaired.
Early emotional symptoms may be equally subtle.
Individuals at this stage may experience more periods
of depression, apathy, irritability, or impulsiveness, or
perhaps changes in personality.
At this stage, people with Huntington's can function
quite well at work and home.
Intermediate stages
As the disease progresses, the symptoms become
worse. The initial motor symptoms will gradually
develop into more obvious involuntary movements
such as jerking and twitching of the head, neck, arms
and legs. These movements may interfere with
walking, speaking and swallowing.
People at this stage of Huntington's often look as if
they're drunk: they stagger when they walk and their
speech is slurred. They have increasing difficulty
working or managing a household, but can still deal
with most activities of daily living.
Advanced stages
The advanced stages of Huntington's typically involve
fewer involuntary movements and more rigidity. People
in these stages of HD can no longer manage the activities
usually require professional nursing. Difficulties with
swallowing, communication and weight loss are common.
Death usually occurs 15 to 20 years after the onset of
the disease. People don't die from Huntington's itself,
but from complications such as choking, heart failure,
or infection.
Juvenile HD
About 10 per cent of Huntington's cases are considered
"juvenile" - the symptoms appear in childhood or
adolescence.
The symptoms of juvenile HD are different from the
adult disease. Children with HD move slowly and
stiffly, they have increasing difficulty learning, and
they can have convulsions or epileptic seizures.
Some children have severe behavioral problems.
The earlier the disease onset, the more likely the child
is to be very rigid and appear very different from the
majority of adult cases. This often makes it difficult to
diagnose. Children with HD are more likely to have an
affected father than an affected mother.
Diagnosis, testing, and treatment
Huntington's is usually diagnosed using neurological
and psychological tests. Sometimes doctors use brain
scans to see whether the caudate and putamen are
working properly, or they use a recently devised
genetic test to confirm diagnosis.
The genetic test uses a blood sample to reveal whether
or not an individual has the gene that causes HD.
Currently, there are no treatments that slow down or
stop HD. Some drugs will reduce symptoms such as
depression, anxiety, and involuntary movements. These
drugs may have side effects so not every Huntington's
patient can use them.
Neurologists, psychologists, genetic counselors and
social workers play an important role in helping
individuals and families deal with HD.