TABLE 3–2.

Primary Proteinopathies Associated With Frontotemporal Lobar Degeneration Spectrum Disorders^a

Tauopathies (approximately 40–45% of cases) ♦ Pick disease ♦ Progressive supranuclear palsy (PSP) ♦ Corticobasal degeneration (CBD) ♦ Argyrophilic grain disease (AGD) ♦ Globular glial tauopathy (GGT) ♦ Age-related tau astrogliopathy (ARTAG) ♦ Primary age-related tauopathy (PART) Transactive response DNA-binding protein 43 (TDP-43) proteinopathies (approximately 40–45% of cases) ♦ Subtypes (TDP types A-D +/− U) are based on the relative abundance of different types of TDP+ neuronal inclusions and their laminar distribution within the cerebral neocortex ♦ Limbic-predominant age-related TDP-43 encephalopathy (LATE) FET protein familyb and other disorders (approximately 5–10% of cases) ♦ Basophilic inclusion body disease (BIBD) ♦ Neuronal intermediate filament inclusion body disease (NIFID) ♦ Atypical frontotemporal lobar degeneration with ubiquitin-positive inclusions (aFTLD-U) ♦ Frontotemporal lobar degeneration with inclusions labeled with markers of the ubiquitin/proteasome system ♦ Hereditary diffuse leukoencephalopathy with spheroids (HDLS) ♦ Others

^aData from Neumann M, Mackenzie IRA, Neuropathol Appl Neurobiol.⁴⁵

^bFUS, EWSR1, TAF15.