Clinico-pathological correlations in Frontotemporal dementia: an update on the Cambridge series and review of the literature
Journal Title: Acta Neuropsychologica - Year 2011, Vol 9, Issue 2
Abstract
The pathological basis of frontotemporal dementia is complex. Few studies have followed large groups prospectively to examine clinico-pathological correlations. Improved prediction is important for planning therapy with the advent of disease-modifying therapies.From a total of 250 brain donors recruited into the Cambridge Brain Bank between 1990 and 2010, 150 had a diagnosis of an FTD variant in life, of whom 132 had sufficient data for inclusion in this study, which examines the correlations between in vivo clinical diagnosis and pathology postmortem.Overall 50 patients had FTLD-U, 41 a form of FTLD tau, 33 Alzheimer’s disease (AD) and 8 other pathologies. There were numerous clear correlations. Of the 26 with semantic dementia (SD), 19 (73%) had FLTD-U. Patients with progressive nonfluent aphasia (n=26) had mainly tau deposition (43%) or AD (46%). In mixed aphasia (n=8), AD was present in 75%. Patients with FTD complicated by motor neurone disease (FTD-MND) showed FTLD-U in 100%. In behavioural variant FTD (n=40), the pathology was unpredictable and split equally between FTLD-tau and FTLD-U. In corticobasal syndrome (CBS n=19), the majority (58%) had AD pathology and the remainder FTLD-tau.The underlying pathology in patients diagnosed clinically with FTD is heterogeneous. Clear predictions can be made in SD, FTD-MND (which are FTLD-U related) and to a lesser extent PNFA (which is largely tau related or secondary to AD) but not in bvFTD or CBS. Alzheimer’s disease is surprisingly common in patients with progressive nonfluent or mixed aphasia and CBS.
Authors and Affiliations
John Hodges
Keywords
Related Articles
- EP ID EP55371
- DOI -
- Views 115
- Downloads 0
REPETITIVE TRANSCRANIAL MAGNETIC STIMULATION (RTMS): A USEFUL TOOL ALSO FOR PATIENTS SUFFERING FROM DYSLEXIA?
[b]SUMMARY[/b]Wanzek & Vaughn (2008) have reported that improve-ment of reading fluency depends on the amount of time in reading inten/ention. For that reason it can be assumed that brain stimulation - psychologi...
Cerebral plasticity in post-stroke rehabilitation
The plastic changes described in this study enable regeneration and compensation of damage to the nervous system. They can also explain the capacity for adaptation, modification, and organization, as well as the strength...
Occupational activity and subjective perception of the quality of life in persons with motor disabilities in Poland
Background: The aim of the study was to verify the correlation between the undertaken occupational activity and the subjective perception of quality of life for individuals with motor disabilities, manifested as declared...
AN EVALUATION OF VISUAL SPATIAL ORIENTATION IN PRESCHOOL CHILDREN
Current research on spatial orientation indicates that, so far, we do not fully understand how spatial orientation develops in onto-genesis. Similarly, one encounters difficulties when searching for norms concerning orie...
Portrait of a scholar: Jason Walter Brown
This biography presents a half century of the commitment of Prof. Jason W. Brown, M.D., to the development of neurology, neuropsychology and philosophy of mind. For over 30 years clinical professor of neurology at New Yo...