Which condition is characterized by chorea, dementia, and caudate degeneration?

Prepare for USMLE Step 1 Pathology Exam with comprehensive quizzes, flashcards, and detailed explanations. Enhance your understanding and be exam-ready!

Huntington's disease is characterized by a triad of chorea, dementia, and degeneration of the caudate nucleus. This genetic condition is caused by an abnormal expansion of CAG repeat sequences in the HTT gene, leading to the progressive neurodegeneration primarily affecting the basal ganglia.

Chorea refers to the involuntary, irregular, and non-repetitive movements that patients exhibit; these movements can be quite dramatic and are a hallmark symptom of the disorder. As the disease progresses, cognitive decline occurs, resulting in dementia that affects executive function and behavioral changes as well. The degeneration of the caudate nucleus is particularly noteworthy; it leads to the characteristic movement disorders and cognitive impairment seen in patients.

Other conditions, while they may present with some overlapping symptoms, do not encompass the same triad. For example, Parkinson's disease primarily features tremor, rigidity, and bradykinesia rather than chorea. Alzheimer's disease is primarily known for memory loss and cognitive decline without the prominent movement disorders seen in Huntington's. Multiple sclerosis can cause a variety of neurological symptoms including cognitive dysfunction but does not typically cause the specific combination of chorea, dementia, and caudate degeneration. Thus, Huntington's disease stands out as the

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