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Table 1 Clinical phenotypes, pathological and molecular genetic spectrum in FTLD[13, 19, 22, 35, 72, 77, 78]

From: Clinic, neuropathology and molecular genetics of frontotemporal dementia: a mini-review

Core clinical feature Praxiological obstacle Logopathy Extrapyramidal symptoms + praxiological obstacle Dyskinesia + praxiological obstacle
Clinical syndrome Behavioral variant Frontotemporal dementia (bvFTD) Primary progressive aphasia(PPA) Corticobasal degeneration (CBD) progressive supranuclear palsy (PSP) FTD-MND/ALS
   nonfluent/agrammatic variant PPA (nfvPPA) Semantics variation (svPPA) logopenic variant PPA (lvPPA)    
Brain morphologically affected parts Prefrontal lobe and temporal lobe Left posterior frontal lobe, insula The front/ventral temporal lobe left posterior superior temporal lobe and medial parietal lobe Frontal and temporal lobe, Basal ganglia Basal ganglia and brainstem Cortex and motor neuron
Biochemistry and Neuropathology FTLD-Tau (Pick type, 3R-tau) FTLD-TDP43 FTLD-Tau more than FTLD-TDP43, AD like pathological visible Most belongs to FTLD-TDP43; AD like pathological rare AD-like pathological common; FTLD-TDP43 visible FTLD-Tau (CBD type,4R-tau) common FTLD-Tau (PSP type, 4R-tau) common FTLD-TDP43, FTLD-FUS
Causative and Associated Genes C9ORF72 PGRN, C9ORF72   PGRN MAPT C9ORF72
PGRN C9ORF72 PGRN   MAPT PGRN FUS
MAPT MAPT MAPT PGRN C9ORF72 C9ORF72 VCP
VCP VCP VCP   VCP VCP
  CHMP2B CHMP2B CHMP2B   CHMP2B CHMP2B  
  1. Note: 3R tau: three microtubulebinding repeats; 4R tau:four microtubule-binding repeats.