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March 2013

Elderly Individuals With FTLD

Author Affiliations

Author Affiliations: Department of Neuropathology, Walton Centre for Neurology and Neurosurgery, Liverpool (Drs Baborie, Keir, and Larner), Institute of Neuroscience, Medical School (Dr Griffiths), and Institute for Ageing and Health, Campus for Ageing and Vitality (Drs J aros, McKeith, Burn, and Perry), Newcastle University; Department of Neuropathology/Cellular Pathology, Royal Victoria Infirmary (Drs Jaros and Perry), Newcastle upon Tyne; Mental Health and Neurodegeneration Research Group, Greater Manchester Neurosciences Centre, University of Manchester, Salford (Dr Mann), England; and Department of Internal Medicine, Texas Tech University Health Sciences Center, Lubbock (Dr Momeni).

JAMA Neurol. 2013;70(3):412-413. doi:10.1001/jamaneurol.2013.1649

In reply

We thank Dr Jellinger for describing 2 additional cases of frontotemporal lobar degeneration (FTLD) in elderly individuals with tauopathy (FTLD-tau) with similar clinical signs and symptoms and neuropathological features to those we have described in our earlier study.1 Both female patients presented with cognitive symptoms, including memory loss, and showed hippocampal sclerosis. However, in patient 1, it is not mentioned whether TDP-43 changes were present. To what extent Braak stage IV could be compatible with all clinical changes mentioned is not certain, but it probably could account for the amnestic symptoms; however, whether these could also explain the frontal lobe signs is uncertain given that under Braak stage IV, tau pathology within frontal lobes would be sparse. We and others1-3 have found that when hippocampal sclerosis is present in FTLD, there is nearly always an accompanying TDP-43 (or FUS) pathology; hippocampal sclerosis is much less common in FTLD-tau.

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