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Neuropathol Appl Neurobiol. 2013 Feb;39(2):166-78. doi: 10.1111/j.1365-2990.2012.01272.x.

Neuropathology of the hippocampus in FTLD-Tau with Pick bodies: a study of the BrainNet Europe Consortium.

Author information

1
Institute of Neurology, Medical University of Vienna, Austria,Semmelweis University Neuropathology and Prion Disease Reference Center (Former Department of Neuropathology, National Institute of Psychiatry and Neurology), Budapest, Hungary,Netherlands Brain Bank and VU University Medical Center, Amsterdam,Erasmus MC, Rotterdam, The Netherlands,Clinical Neuropathology Department, King's College Hospital,King's College London, MRC Centre for Neurodegeneration Research, Department of Clinical Neuroscience, Institute of Psychiatry, London,Department of Neuropathology, John Radcliffe Hospital, Oxford,Department of Pathology, University of Edinburgh, Western General Hospital, Edinburgh, UK,Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy,Institut de Neuropatologia, Hospital Universitari de Bellvitge, Universitat de Bacelona, Hospitalet de Llobregat, Barcelona, Spain,Centre for Neuropathology and Prion Research, Ludwig-Maximilians-University, and Neurobiobank Munich/BrainNet Germany, Munich, Germany,Department of Geriatrics, Karolinska Institutet, HuddingeRudbeck Laboratory, Department of Genetics and Pathology, Uppsala University, Uppsala, SwedenDepartment of Neuroscience and Neurology, Kuopio University, Kuopio, Finland.

Abstract

AIMS:

Frontotemporal lobar degeneration with Pick bodies (Pick's disease) is characterized by the presence of tau immunoreactive spherical structures in the cytoplasm of neurones. In view of confusion about the molecular pathology of Pick's disease, we aimed to evaluate the spectrum of tau pathology and concomitant neurodegeneration-associated protein depositions in the characteristically affected hippocampus.

METHODS:

We evaluated immunoreactivity (IR) for tau (AT8, 3R, 4R), α-synuclein, TDP43, p62, and ubiquitin in the hippocampus, entorhinal and temporal cortex in 66 archival cases diagnosed neuropathologically as Pick's disease.

RESULTS:

Mean age at death was 68.2 years (range 49-96). Fifty-two (79%) brains showed 3R immunoreactive spherical inclusions in the granule cells of the dentate gyrus. These typical cases presented mainly with the behavioural variant of frontotemporal dementia, followed by progressive aphasia, mixed syndromes or early memory disturbance. α-Synuclein IR was seen only in occasional spherical tau-positive inclusions, TDP-43 IR was absent, and 4R IR was present only as neurofibrillary tangles in pyramidal neurones. Aβ IR was observed in 16 cases; however, the overall level of Alzheimer's disease-related alterations was mainly low or intermediate (n = 3). Furthermore, we identified six cases with unclassifiable tauopathy.

CONCLUSIONS:

(i) Pick's disease may occur also in elderly patients and is characterized by a relatively uniform pathology with 3R tau inclusions particularly in the granule cells of dentate gyrus; (ii) even minor deviation from these morphological criteria suggests a different disorder; and (iii) immunohistological revision of archival cases expands the spectrum of tauopathies that require further classification.

KEYWORDS:

Pick's disease; TDP-43; Tau; alpha-synuclein; frontotemporal lobar degeneration; unclassifiable tauopathy

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