Table 5.
Clinical and radiological clues for familial FTD with Parkinsonism.
Clinical clues | Radiological clues | Targeted gene | Suspected pathology |
---|---|---|---|
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 |