Progress in clinical neurosciences: Frontotemporal dementia-pick's disease

Area of Science:

• Neurology
• Neuroscience
• Genetics

Background:

• Frontotemporal dementia (FTD), clinically referred to as Pick's disease, is a prevalent yet frequently underdiagnosed neurodegenerative condition affecting the presenium.
• It accounts for an estimated 6-12% of all dementia cases.
• FTD encompasses distinct clinical syndromes: FTD-behavioural variant, Primary Progressive Aphasia (PPA), and Corticobasal Degeneration/Progressive Supranuclear Palsy (CBD/PSP).

Purpose of the Study:

• To describe the diagnostic features and clinical course of different FTD presentations.
• To emphasize the overlap in disease evolution among FTD subtypes.
• To discuss the neuropathological and genetic aspects of FTD.

Main Methods:

• Review of clinical presentations, diagnostic features, and disease progression.
• Analysis of neuropathological findings, including tau-negative and tau-positive inclusions.
• Examination of genetic factors, including tau mutations and tau-negative families.

Main Results:

• Neuropathology varies from tau-negative, ubiquitin-positive inclusions (ALS-type) to tau-positive Pick bodies, and changes seen in PSP and CBD.
• Tau-negative FTD cases are often linked to behavioural presentations and semantic dementia.
• Tau-positive FTD cases are more frequently associated with PPA and the CBD/PSP syndrome.

Conclusions:

• Despite distinct associations, the significant overlap between tau-negative and tau-positive FTD subtypes precludes definitive separation of the disease.
• Accurate diagnosis and understanding of FTD subtypes are crucial for patient management.
• A glossary is provided to aid in navigating the complex terminology associated with FTD.