Table 2.
Spatiotemporal pattern of accumulation of tauopathies.
| Tauopathy | Spatiotemporal pattern of neuronal tau accumulation | Main clinical phenotypes | ||
|---|---|---|---|---|
| Early or pre-clinical | Mild stage | Late-stage | ||
| Pick's disease | Limbic system Neocortical frontotemporal cortex |
Basal ganglia Brainstem Noradrenergic/serotonergic nuclei Dentate nucleus Sensory tract of the spinal cord |
Pre-motor cortex Pre-cerebellar nuclei Primary visual cortex |
bvFTD nfaPPA |
| Progressive supranuclear palsy | Globus pallidus Substantia nigra Subthalamic nucleus |
Brainstem, cerebellum (dentate nucleus), posterior frontal lobe | Association cortices | PSP-RS PSP-P PSP-CBS bvFTD nfaPPA |
| Corticobasal degeneration | Anterior frontal cortex Basal ganglia |
Posterior frontal and parietal cortices | Higher tau burden in same regions as early/mild stage Midbrain and pons |
CBS bvFTD nfaPPA RS |
| Chronic traumatic encephalopathy | Depth of sulci in frontal lobe Nucleus basalis Meynert Locus coeruleus |
Superficial cortical layers Nucleus basalis Meynert Locus coeruleus |
Amygdala Hippocampus Temporal, parietal and insular cortices Diencephalon Brainstem Spinal cord |
Cognitive dysfunction Emotional dysregulation Behavior change Motor disturbance |
| Globular glial tauopathies | (Unknown temporal pattern) Frontal and temporal lobes Motor cortex Corticospinal tracts |
bvFTD bvFTD + MND MND PSP-RS CBS |
||
| Primary age-related tauopathy | Transentorhinal region | Limbic structures (entorhinal and hippocampus) |
Rarely neocortex is affected | Amnestic mild cognitive changes |
| Argyrophilic grain disease | Ambient gyrus and CA1 of hippocampus | Amygdala Medial temporal lobe |
Insular cortex Anterior cingulum Nucleus accumbens Septal nuclei Hypothalamus Gyri recti |
Amnestic mild cognitive changes Behavior and psychiatric symptoms bvFTD |
| Alzheimer's disease | Transentorhinal region | Limbic structures (entorhinal and hippocampus) | Isocortical | From amnestic cognitive changes to dementia |
bvFTD, behavioral variant of frontotemporal dementia; nfaPPA, non-fluent-agrammatic variant of primary progressive aphasia; CBS, corticobasal syndrome; RS, Richardson syndrome; PSP, progressive supranuclear palsy; MND, motor neuron disease.