Table 1.

Nomenclature for Frontotemporal Lobar Degeneration (Mackenzie et al ⁴⁹ ).

2010 Recommendation
Major Molecular Class	Recognized Subtype a	Associated Gene
FTLD-tau	PiD	MAPT
	CBD
	PSP
	AGD
	MSTD
	NFT-dementia
	WMT-GGI
	Unclassifiable
FTLD-TDP	Types 1-4	GRN
	Unclassifiable	VCP
		9p
		(TARDBP) b
FTLD-UPS	FTD-3	CHMP2B
FTLD-FUS	aFTLD-U	(FUS) c
	NIFID
	BIBD
FTLD-ni

Abbreviations: aFTLD-U, atypical frontotemporal lobar degeneration with ubiquitinated inclusions; AGD, argyrophilic grain disease; BIBD, basophilic inclusion body disease; CBD, corticobasal degeneration; CHMP2B, charged multivescicular body protein 2B; FTD-3, frontotemporal dementia linked to chromosome 3; FTLD, frontotemporal lobar degeneration; FUS, fused in sarcoma; GRN, progranulin gene; IFs, intermediate filaments; MAPT, microtubule-associated protein tau; MSTD, multiple system tauopathy with dementia; NFT-dementia, neurofibrillary tangle predominant dementia; ni, no inclusions; NIFID, neuronal intermediate filament inclusion disease; PiD, Pick’s disease; PSP, progressive supranuclear palsy; TARDBP, transactive response DNA binding protein; TDP, TAR DNA-binding protein 43; UPS, ubiquitin proteasome system; VCP, valosin containing protein; WMT-GGI, white matter tauopathy with globular glial inclusions; 9p, genetic locus on chromosome 9p linked to familial amyotrophic lateral sclerosis and frontotemporal dementia.

^a Indicates the characteristic pattern of pathology, not the clinical syndrome. Note that FTDP-17 is not listed as a pathological subtype because cases with different MAPT mutations do not have a consistent pattern of pathology. These cases would all be FTLD-tau, but further subtyping would vary.

^b Rare case reports of patients with clinical FTD and TDP-43 pathology associated with TARDBP genetic variants.

^c One patient reported with a FUS mutation and FTD/ALS clinical phenotype but no description of pathology.