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

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

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	VCP
	CHMP2B	CHMP2B	CHMP2B		CHMP2B	CHMP2B

Note: 3R tau: three microtubulebinding repeats; 4R tau:four microtubule-binding repeats.

ISSN: 2047-9158