Table 1.

Clinical, genetic, and pathological heterogeneity in FTD

Clinical subtypes	Genetic mutations	Pathological subtypes
• Behavioral variant FTD	• TAU (coding for the microtubule associated protein tau)	• Pick’s disease
• Non-fluent primary progressive aphasia	• PRGN (coding for progranulin)	• Tau-positive FTDP-17
• semantic dementia	• VCP (coding for the valosin-containing protein)	• TDP-43 positive FTD with or without motor neuron disease
• FTD with Parkinsonism	• CHMP2B (coding for the charged multivesicular protein 2B)	• FUS positive
• FTD with motor neuron disease	• TDP (coding for the tar-DNA binding protein 43)	• Progressive supranuclear palsy
• FTD with inclusion body myopathy and Paget’s disease of the bone	• FUS (coding for the fused in sarcoma protein)	• Corticobasal degeneration
• Progressive supranuclear palsy	• Unidentified gene on chromosome 9	• Ubiquitin-positive (tau/TDP43/FUS-negative) FTD
• Corticobasal degeneration