| Frontotemporal atrophy | Particularly affecting the frontal and anterior temporal lobes |
| Neuronal loss and gliosis | In affected cortical regions and subcortical structures |
| Substantia nigra degeneration | Loss of dopaminergic neurons |
| Basal ganglia involvement | Variable involvement of caudate nucleus, putamen, and globus pallidus |
| Databases | OMIMOrphanetClinicalTrialsPubMed |