Looking for Measures of Disease Severity in the Frontotemporal Dementia Continuum.
Frontotemporal dementia (FTD) is characterized by executive dysfunctions, behavioral disturbances, language deficits and extrapyramidal symptoms. Frontotemporal lobar degeneration-modified Clinical Dementia Rating Scale (FTLD modified-CDR) has been proposed to measure disease severity in behavioral variant FTD (bvFTD). No tools of global disease severity are available in the other FTLD phenotypes [primary progressive aphasias (PPAs), progressive supranuclear palsy (PSP), and corticobasal syndrome (CBS)]. This would be strategic as outcome measures in clinical trials. To this aim, we evaluated the association between brain volume (voxel based morphometry) and available clinical scales in FTD. In 176 FTD patients (64 bvFTD, 40 PPAs, 32 PSP, 40 CBS), instrumental activities of daily living (ADLs), FTLD-modified CDR, Mini-Mental State Examination (MMSE), Frontal Behavioral Inventory (FBI), and Neuropsychiatry Inventory (NPI) were administered and MRI performed. Whole-brain linear correlation between each clinical rating scale and brain volume was performed. In bvFTD and PPAs, FTLD-modified CDR was associated with regional brain volume, thereby providing evidence for validity of the FTLD-modified CDR. In PSP, none of the clinical indicators were associated with regional brain volume. In CBS, ADLs and MMSE correlated with frontotemporal lower volume. Considering monogenic disease, FTLD-modified CDR was the best measure. In FTD continuum, different measures able to correlate with brain damage should be considered for the different clinical phenotypes or genetic traits.
Centre for Ageing Brain and Neurodegenerative Disorders, Neurology Unit, University of Brescia, Italy.
Enrico
E
Premi
Centre for Ageing Brain and Neurodegenerative Disorders, Neurology Unit, University of Brescia, Italy.
Vera
V
Gualeni
Centre for Ageing Brain and Neurodegenerative Disorders, Neurology Unit, University of Brescia, Italy.
Paolo
P
Costa
Centre for Ageing Brain and Neurodegenerative Disorders, Neurology Unit, University of Brescia, Italy.
Maura
M
Cosseddu
Neuroradiology Unit, University of Brescia, Italy.
Roberto
R
Gasparotti
Centre for Ageing Brain and Neurodegenerative Disorders, Neurology Unit, University of Brescia, Italy.
Alessandro
A
Padovani
Centre for Ageing Brain and Neurodegenerative Disorders, Neurology Unit, University of Brescia, Italy.
Barbara
B
Borroni
D000203:Activities of Daily Living / Q000523:psychology; D000368:Aged; D001921:Brain / Q000000981:diagnostic imaging; D005260:Female; D057180:Frontotemporal Dementia / Q000145:classification / Q000175:diagnosis* / Q000000981:diagnostic imaging; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D059906:Neuroimaging; D009483:Neuropsychological Tests; D012720:Severity of Illness Index
Activities of daily living; FTLD-modified CDR; Frontal Behavioral Inventory; Mini-Mental State Examination; Neuropsychiatry Inventory; frontotemporal dementia continuum; voxel-based morphometry