Clinicopathological correlates in frontotemporal dementia.
The term frontotemporal dementia (FTD) encompasses a range of clinical syndromes that are believed not to map reliably onto the spectrum of recognized pathologies. This study reexamines the relationships between clinical and pathological subtypes of FTD in a large series from two centers (n = 61). Clinical subtypes defined were behavioral variant FTD (n = 26), language variants (semantic dementia, n = 9; and progressive nonfluent aphasia, n = 8), and motor variants (corticobasal degeneration, n = 9; and motor neuron disease, n = 9), although most cases presented with a combination of behavioral and language problems. Unexpectedly, some behavioral cases (n = 5) had marked amnesia at presentation. The pathological subtypes were those with tau-immunopositive inclusions (with Pick bodies, n = 20; or without, n = 11), those with ubiquitin immunopositive inclusions (n = 16), and those lacking distinctive histology (n = 14). Behavioral symptoms and semantic dementia were associated with a range of pathologies. In contrast, other clinical phenotypes had relatively uniform underlying pathologies: motor neuron disease predicted ubiquitinated inclusions, parkinsonism and apraxia predicted corticobasal pathology, and nonfluent aphasia predicted Pick bodies. Therefore, the pathological substrate can be predicted in a significant proportion of FTD patients, which has important implications for studies targeting mechanistic treatments.
Hodges
John R
JR
Prince of Wales Medical Research Institute, the University of New South Wales, Randwick, New South Wales, Australia. john.hodges@mrc-cbu.cam.ac.uk
Davies
R Rhys
RR
Xuereb
John H
JH
Casey
Barney
B
Broe
Melissa
M
Bak
Thomas H
TH
Kril
Jillian J
JJ
Halliday
Glenda M
GM
D000368:Aged; D000369:Aged, 80 and over; D001921:Brain / Q000473:pathology*; D003704:Dementia / Q000175:diagnosis / Q000473:pathology* / Q000523:psychology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D015203:Reproducibility of Results; D012189:Retrospective Studies; D016875:tau Proteins / Q000032:analysis
D016875:tau Proteins