| Neuronal tau inclusions | Pre-tangles, neurofibrillary tangles, and Pick body-like inclusions in cortical neurons, though less prominent than glial pathology |
| White matter degeneration | Severe myelin loss and axonal damage, particularly in subcortical white matter and corticospinal tracts, correlating with the burden of GOIs |
| Neuronal loss and gliosis | Cortical neuronal depletion with reactive astrocytosis, most severe in frontotemporal regions (Type I) or motor cortex (Type II) |
| Coexisting pathologies | Some cases show co-occurrence with aging-related tauopathy (PART) or AGD |
| Databases | OMIMOrphanetClinicalTrialsPubMed |
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