[Alzheimer-type dementia and Down syndrome]

Area of Science:

• Neurology
• Genetics
• Gerontology

Context:

• Down syndrome (DS) is associated with a significantly higher incidence and earlier onset of dementia compared to the general population.
• Patients with DS exhibit neuropathological hallmarks mirroring those found in dementia of Alzheimer's type (DAT).

Purpose:

• To review the existing literature on dementia of Alzheimer's type (DAT) in individuals with Down syndrome (DS).
• To discuss the clinical presentation and differential diagnoses of dementia in the context of DS.
• To emphasize the importance of excluding treatable causes of dementia prior to diagnosing DAT in DS patients.

Summary:

• Down syndrome (DS) presents a unique demographic for studying dementia, with earlier and more frequent onset.
• Neuropathological similarities exist between dementia in DS and typical dementia of Alzheimer's type (DAT).
• Accurate diagnosis requires differentiating DAT from other potential causes of cognitive decline in DS, particularly treatable conditions.

Impact:

• This review provides a comprehensive overview for clinicians and researchers regarding dementia in Down syndrome (DS).
• Highlights the critical need for differential diagnosis to ensure appropriate patient management and treatment strategies.
• Contributes to a better understanding of Alzheimer's-like pathology in a genetically defined population.