Huntington's disease is a progressive,
neurodegenerative disease causing uncontrolled
physical Huntington disease mental deterioration.
Huntingtons disease results from degeneration of brain
cells. Specifically affected early in the disease are
cells of the basal ganglia, a structure deep in the
center of the brain where movement is coordinated.
Causes
HD is inherited in an autosomal dominant manner. The
gene responsible for the disease has been located on
chromosome number 4. The HD gene is a dominant gene,
meaning that only one copy of it is needed to develop
the disease. HD affects both males and females. The
gene may be inherited from either parent, who will
also be affected by the disease.
Symptoms
The earliest signs and symptoms of Huntington's
disease often include depression, irritability,
anxiety and apathy. The person with HD may become
impulsive, aggressive or socially withdrawn.
As the disease progresses, you may develop:
- Forgetfulness and disorganization - Common early
symptoms
- Poor judgment
- Severe problems with balance and coordination
- Dementia
- Difficulty shifting your gaze without moving your head
- Inability to swallow
- Problems with abstract thought, reasoning, or
problem solving
- Spatial disorientation
- Difficulties with sequencing, organizing, or
planning
- Inability to learn new things
- Memory loss
Treatment
There is at present no known means of altering the
disease process or the fatal outcome. The choreic
movements can be controlled by the use of neuroleptic
agents, including dopamine receptor blockers such as
haloperidol and perphenazine, and presynaptic dopamine
depletors such as reserpine.
