Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations

Determinants of survival in behavioral variant frontotemporal dementia

“…The pathologic subtypes were tau positive pathology (frontotemporal lobar degeneration [FTLD]-tau) (n ϭ 16; 57.1%), pathology with TDP-43 positive/ tau-negative inclusions (FTLD-TDP/FTLD-UPS) (n ϭ 11; 39.4%), and lacking distinctive histology (n ϭ 1; 3.5%). 14 The median survival for the whole group from symptom onset was 9.0 years (95% confidence interval [CI[ 8.0 -10.0) and from diagnosis was 5.4 years (95% CI 4.6 -6.2).…”

Imaging correlates of pathology in corticobasal syndrome

“…16 Using published criteria, 17 cases were diagnosed as having FTLD-TDP type 1 pathology if there were neuronal inclusions and short dystrophic neurites in layer II of neocortex and inclusions in the dentate gyrus of the hippocampus; type 2 if there were large and thick neurites throughout neocortex with absent-sparse inclusions; and type 3 if there were inclusions in neocortex or dentate granule cells of hippocampus and absent-sparse neurites. Pathologic diagnoses of PSP 18 and CBD 19 were made according to published diagnostic criteria.…”

Recent biomarker approaches in the diagnosis of frontotemporal lobar degeneration/Neurochemische Ansätze in der Diagnose der Frontotemporalen Lobärdegeneration

“…Historically, FTLD patients were subclassifi ed as tau-positive cases (FTLD-tau) and those with tau-negative, ubiquitin-positive inclusions (FTLD-U). In most FTLD-U cases, the ubiquitinated protein is TAR DNA-binding protein-43 (TDP-43) [7] and the term FTLD-TDP was recently introduced for this subgroup [8] , whereas in approximately 10 % of FTLD-U cases FUS (fused in sarcoma) protein pathology could be found [9,10] , and the term FTLD-FUS was introduced for this FTLD subtype [11] . TDP-43 and FUS pathology are also found in patients with ALS.…”