From the Guidelines
Huntington's disease can present at any age, with symptoms typically appearing between 30 and 50 years old, but can occur as early as age 2 or as late as age 80. The symptoms of Huntington's disease include involuntary jerking or writhing movements (chorea), muscle problems such as rigidity and contractures, slow or abnormal eye movements, impaired gait, posture and balance, difficulty with speech and swallowing, and cognitive decline including difficulty organizing, prioritizing or focusing on tasks 1. Some key points to consider about Huntington's disease include:
- The disease is hereditary, with children of affected parents having a 50% chance of inheriting the condition 1
- The disease causes progressive degeneration of nerve cells in the brain, particularly in the basal ganglia and cerebral cortex, due to a genetic mutation that creates an abnormal version of the huntingtin protein 1
- Psychiatric symptoms are also common, including depression, anxiety, obsessive-compulsive disorder, irritability, and personality changes 1
- In juvenile cases, symptoms often include stiffness, slowness, difficulty at school, seizures, and rapid decline in mental abilities 2
- The average age of onset is between 35 to 45 years and symptom progression until death within 15 to 20 years of onset 1
From the Research
Age of Presentation
Huntington's disease (HD) can present at any age, but most typically manifests between the ages of 35 and 45 years 3. However, the disease can also present in younger individuals, under the age of 30 years, or in older individuals, over the age of 70 years 4.
Symptoms of Huntington's Disease
The symptoms of HD include:
- Involuntary movements, such as chorea 5, 3
- Psychiatric symptoms, such as cognitive dysfunction and behavioral impairment 5, 3, 6
- Cognitive dysfunction, including decline in cognitive function and dementia 5, 6, 7
- Motor symptoms, including dystonia, eye movement abnormalities, and bradykinesia 4
- Functional decline, including loss of ability to perform daily activities 5, 6
Clinical Features
The clinical features of HD can vary depending on the age of presentation. For example:
- Younger-onset HD patients (under 30 years) may have more dystonia, eye movement abnormalities, and a greater rate of motor progression 4
- Later-onset HD patients (over 70 years) may have more chorea and a slower rate of motor progression 4
- Both younger-onset and later-onset HD patients can have significant cognitive and psychiatric symptoms 5, 3, 6, 7