Skip to main content
. Author manuscript; available in PMC: 2009 Oct 14.
Published in final edited form as: J Neuropathol Exp Neurol. 2008 Jul;67(7):635–648. doi: 10.1097/NEN.0b013e31817d751c

TABLE 3.

Frontotemporal Lobar Degeneration: Pathologic Subtypes, 2008

Tauopathies Ubiquitinopathies
Pick disease FTLD-U/TDP-43 proteinopathy
Corticobasal degeneration FTLD-MND
Progressive supranuclear palsy FTDP-17 with PGRN mutations
FTDP-17 with MAPT mutations FTLD-U with VCP mutations
Sporadic MSTD FTLD-U with CHMP2B mutations
Tauopathies, unclassifiable FTLD-MND linked to chromosome 9p
Atypical FTLD-U (TDP-43 negative)

CHMP2B, charged multivesicular body protein 2B; FTDP, familial tauopathy with dementia linked to chromosome 17; FTLD, frontotemporal lobar degeneration; FTLD-U, FTLD with ubiquitinated inclusions; MND, motor neuron disease; MSTD, multiple system tauopathy with presenile dementia; PGRN, progranulin; TDP-43, TAR-DNA binding protein-43; VCP, valosin-containing protein.