Early onset (3rd or 4th decade), PSP phenotype, vertical supranuclear gaze palsy |
Symmetric fronto-temporal atrophy |
MAPT |
Tau |
Late onset (5th or 6th decade), CBS phenotype, FTD-MND overlap, language involvement, apraxia, dyscalculia, visuospatial impairment, episodic memory involvement, hallucination |
Asymmetric fronto-temporal atrophy, more posterior involvement (temporo-parietal, parieto-occipital), significant white matter hyperintensities |
PGRN |
TDP43 |
FTD-MND overlap, early cognitive, and/or behavioral symptoms, psychosis, hallucination, chorea (Huntington's disease phenocopy), positive family history of MND or FTD |
Symmetric fronto-temporal and cerebellar atrophy |
C9orf72 |
TDP43, Ubiquitin |