Huntington's Disease
Huntington's Disease (HD), also known as Huntington's Chorea is a genetic
degenerative disease affecting the nerves in the brain. It is described as an
autosomal dominant hereditary progressive neurological disease. It has a
prevalence of affecting 5-10 individuals per 100,000. Life expectancy of those
with HD is estimated at approximately 15-20 years after symptoms begin.
Huntington's Disease Pathophysiology:
• Although it affects the entire brain, the basal ganglia is most affected.
• This area plays a key role in movement, coordination and posture.
• It is caused by a mutation of the HTT gene.
• If a parent has the gene you have a 50% chance of developing the
disease.
Clinical Presentations:
• Rigidity
• Chorea
• Dystonia
• Difficulty concentrating and memory lapses
• Depression, anxiety and mania
• Psychosis
• Speech difficulty
• Gait abnormality
Neurological Conditions Page 1
Huntington's Disease (HD), also known as Huntington's Chorea is a genetic
degenerative disease affecting the nerves in the brain. It is described as an
autosomal dominant hereditary progressive neurological disease. It has a
prevalence of affecting 5-10 individuals per 100,000. Life expectancy of those
with HD is estimated at approximately 15-20 years after symptoms begin.
Huntington's Disease Pathophysiology:
• Although it affects the entire brain, the basal ganglia is most affected.
• This area plays a key role in movement, coordination and posture.
• It is caused by a mutation of the HTT gene.
• If a parent has the gene you have a 50% chance of developing the
disease.
Clinical Presentations:
• Rigidity
• Chorea
• Dystonia
• Difficulty concentrating and memory lapses
• Depression, anxiety and mania
• Psychosis
• Speech difficulty
• Gait abnormality
Neurological Conditions Page 1
