What is the typical sequence of symptom development in Huntington disease?

What is the typical sequence of symptom development in Huntington disease?

• A. Behavioral changes → chorea → dementia
• B. Dementia → chorea → behavioral changes
• C. Seizures → chorea
• D. Chorea only

Answer: (A)

In Huntington disease, the typical progression starts with psychiatric or behavioral changes, then choreiform movements, and later cognitive decline leading to dementia. This pattern fits the disease’s pathology: degeneration of the caudate and putamen disrupts frontostriatal circuits that regulate behavior and mood first, followed by motor circuit disruption that produces chorea, and finally more widespread neurodegeneration affecting cognition. Seizures aren’t characteristic, and “chorea only” ignores the eventual cognitive decline that commonly accompanies the illness. So the sequence is behavioral changes → chorea → dementia.

In Huntington disease, the typical progression starts with psychiatric or behavioral changes, then choreiform movements, and later cognitive decline leading to dementia. This pattern fits the disease’s pathology: degeneration of the caudate and putamen disrupts frontostriatal circuits that regulate behavior and mood first, followed by motor circuit disruption that produces chorea, and finally more widespread neurodegeneration affecting cognition. Seizures aren’t characteristic, and “chorea only” ignores the eventual cognitive decline that commonly accompanies the illness. So the sequence is behavioral changes → chorea → dementia.