Frontotemporal dementia: Clinicopathological correlations
Article first published online: 22 MAY 2006
Copyright © 2006 American Neurological Association
Annals of Neurology
Volume 59, Issue 6, pages 952–962, June 2006
How to Cite
Forman, M. S., Farmer, J., Johnson, J. K., Clark, C. M., Arnold, S. E., Coslett, H. B., Chatterjee, A., Hurtig, H. I., Karlawish, J. H., Rosen, H. J., Van Deerlin, V., Lee, V. M.-Y., Miller, B. L., Trojanowski, J. Q. and Grossman, M. (2006), Frontotemporal dementia: Clinicopathological correlations. Ann Neurol., 59: 952–962. doi: 10.1002/ana.20873
- Issue published online: 22 MAY 2006
- Article first published online: 22 MAY 2006
- Manuscript Accepted: 24 MAR 2006
- Manuscript Revised: 20 MAR 2006
- Manuscript Received: 11 NOV 2005
- NIH. Grant Numbers: P01 AG17586, P01 AG09215, P01 AG19724, P30 AG10124, R01 AG15116, R01 AG 022538, K08 AG20073
- National Institute on Neurological Disorders and Stroke. Grant Numbers: R01 NS44266, K08 NS41408
Frontotemporal lobar degeneration (FTLD) is characterized by impairments in social, behavioral, and/or language function, but postmortem studies indicate that multiple neuropathological entities lead to FTLD. This study assessed whether specific clinical features predict the underlying pathology.
A clinicopathological correlation was performed on 90 consecutive patients with a pathological diagnosis of frontotemporal dementia and was compared with an additional 24 cases accrued during the same time period with a clinical diagnosis of FTLD, but with pathology not typically associated with frontotemporal dementia.
Postmortem examination showed multiple pathologies including tauopathies (46%), FTLD with ubiquitin-positive inclusions (29%), and Alzheimer's disease (17%). The pathological groups manifested some distinct demographic, clinical, and neuropsychological features, although these attributes showed only a statistical association with the underlying pathology. FTLD with ubiquitin-positive inclusions was more likely to present with both social and language dysfunction, and motor neuron disease was more likely to emerge in these patients. Tauopathies were more commonly associated with an extrapyramidal disorder. Alzheimer's disease was associated with relatively greater deficits in memory and executive function.
Clinical and neuropsychological features contribute to delineating the spectrum of pathology underlying a patient diagnosed with FTLD, but biomarkers are needed that, together with the clinical phenotype, can predict the underlying neuropathology. Ann Neurol 2006;59:952-962