American Board of Psychiatry and Neurology (ABPN) Practice Exam

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Which condition is known for causing a risk of dementia in affected individuals?

  1. Huntington's disease

  2. Trisomy 21

  3. Fragile X syndrome

  4. Down's syndrome

The correct answer is: Trisomy 21

Down's syndrome, also known as Trisomy 21, is associated with an increased risk of dementia, particularly Alzheimer's disease. Individuals with Down's syndrome have an extra copy of chromosome 21, which leads to overproduction of amyloid precursor protein (APP). This overproduction can contribute to the development of amyloid plaques typically seen in Alzheimer's disease. As individuals with Down's syndrome age, they show a higher prevalence of cognitive decline and dementia, often beginning in middle age. Around 50% of adults with Down's syndrome will develop Alzheimer's symptoms by the time they reach their 60s. The connection between Down's syndrome and dementia highlights the importance of monitoring cognitive health in these individuals as they age. While Huntington's disease and Fragile X syndrome can also impact cognitive abilities and cause various degrees of functional decline, the specific and increased association of Down's syndrome with early-onset dementia sets it apart in this context.