Table 1 Examples of primary and secondary tauopathies
From: The therapeutic landscape of tauopathies: challenges and prospects
Tauopathy type | Name | Tau isoforms | Pathology | Localization | Presentation |
|---|---|---|---|---|---|
Primary | Progressive supranuclear palsy (PSP) | 4R | NFTs, tau deposits in astrocytes | Midbrain, basal ganglia, diencephalon | Supranuclear vertical ophthalmoplegia, pseudobulbar palsy, and dementia |
Corticobasal degeneration (CBD) | 4R | NFTs, coiled bodies, argyrophilic threads, astrocytic plaques | Primary motor cortex, basal ganglia, white matter | Progressive, asymmetric apraxia and akinetic-rigid syndrome | |
Frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17) | 4R, 3R or 3R/4R | Neuronal and glial tau deposits | Frontal and/or temporal lobe | Language-related dementia syndromes termed primary progressive aphasia with preserved memory | |
Pick’s disease (PiD) | 3R | Pick bodies | Frontal lobe, medial temporal lobe, basal ganglia | Broad range of personality changes prior to cognitive decline | |
Chronic traumatic encephalopathy (CTE)—sometimes classified as a secondary tauopathy | 3R/4R | NFT’s and glial tangles | Frontal and temporal cortices, hippocampus | Personality and behavioral changes, memory loss, and speech and gait difficulty with repetitive trauma history | |
Argyrophilic grain disease (AGD) | 4R | Argyrophilic grains | Entorhinal cortex, hippocampus, amygdala | Cognitive decline, seizures, personality changes | |
Primary age-related tauopathy (PART) | 3R/4R | NFTs | Medial temporal mode, basal forebrain, brain stem | MCI or amnestic decline | |
Secondary | Alzheimer’s disease (AD) | 3R/4R | NFTs | Medial temporal lobe, temporal cortex, neocortex | Cognitive decline, changes in behavior, mood swings, language difficulties |
Down’s syndrome | 3R/4R | NFTs | Brain stem, cerebellum, frontal, and temporal lobes | In adults—similar to AD patients |