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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration</title><author><name sortKey="Cairns, Nigel J" sort="Cairns, Nigel J" uniqKey="Cairns N" first="Nigel J." last="Cairns">Nigel J. Cairns</name></author></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">17579875</idno><idno type="pmc">2827877</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2827877</idno><idno type="RBID">PMC:2827877</idno><idno type="doi">10.1007/s00401-007-0237-2</idno><date when="2007">2007</date><idno type="wicri:Area/Pmc/Corpus">001415</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">001415</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration</title><author><name sortKey="Cairns, Nigel J" sort="Cairns, Nigel J" uniqKey="Cairns N" first="Nigel J." last="Cairns">Nigel J. Cairns</name></author></analytic><series><title level="j">Acta Neuropathologica</title><idno type="ISSN">0001-6322</idno><idno type="eISSN">1432-0533</idno><imprint><date when="2007">2007</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p id="P1">The aim of this study was to improve the neuropathologic recognition and provide criteria for the pathological diagnosis in the neurodegenerative diseases grouped as frontotemporal lobar degeneration (FTLD); revised criteria are proposed. Recent advances in molecular genetics, biochemistry, and neuropathology of FTLD prompted the Midwest Consortium for Frontotemporal Lobar Degeneration and experts at other centers to review and revise the existing neuropathologic diagnostic criteria for FTLD. The proposed criteria for FTLD are based on existing criteria, which include the tauopathies [FTLD with Pick bodies, corticobasal degeneration, progressive supranuclear palsy, sporadic multiple system tauopathy with dementia, argyrophilic grain disease, neurofibrillary tangle dementia, and FTD with <italic>microtubule-associated tau (MAPT)</italic> gene mutation, also called FTD with parkinsonism linked to chromosome 17 (FTDP-17)]. The proposed criteria take into account new disease entities and include the novel molecular pathology, TDP-43 proteinopathy, now recognized to be the most frequent histological finding in FTLD. TDP-43 is a major component of the pathologic inclusions of most sporadic and familial cases of FTLD with ubiquitin-positive, tau-negative inclusions (FTLD-U) with or without motor neuron disease (MND). Molecular genetic studies of familial cases of FTLD-U have shown that mutations in the <italic>progranulin</italic> (<italic>PGRN</italic>) gene are a major genetic cause of FTLD-U. Mutations in <italic>valosin-containing protein</italic> (<italic>VCP</italic>) gene are present in rare familial forms of FTD, and some families with FTD and/or MND have been linked to chromosome 9p, and both are types of FTLD-U. Thus, familial TDP-43 proteinopathy is associated with defects in multiple genes, and molecular genetics is required in these cases to correctly identify the causative gene defect. In addition to genetic heterogeneity amongst the TDP-43 proteinopathies, there is also neuropathologic heterogeneity and there is a close relationship between genotype and FTLD-U sub-type. In addition to these recent significant advances in the neuropathology of FTLD-U, novel FTLD entities have been further characterized, including neuronal intermediate filament inclusion disease. The proposed criteria incorporate up-to-date neuropathology of FTLD in the light of recent immunohistochemical, biochemical, and genetic advances. These criteria will be of value to the practicing neuropathologist and provide a foundation for clinical, clinico-pathologic, mechanistic studies and in vivo models of pathogenesis of FTLD.</p></div></front></TEI><pmc article-type="research-article" xml:lang="EN"><pmc-comment>The publisher of this article does not allow downloading of the full text in XML form.</pmc-comment>
<pmc-dir>properties manuscript</pmc-dir>
<front><journal-meta><journal-id journal-id-type="nlm-journal-id">0412041</journal-id><journal-id journal-id-type="pubmed-jr-id">133</journal-id><journal-id journal-id-type="nlm-ta">Acta Neuropathol</journal-id><journal-title>Acta Neuropathologica</journal-title><issn pub-type="ppub">0001-6322</issn><issn pub-type="epub">1432-0533</issn></journal-meta><article-meta><article-id pub-id-type="pmid">17579875</article-id><article-id pub-id-type="pmc">2827877</article-id><article-id pub-id-type="doi">10.1007/s00401-007-0237-2</article-id><article-id pub-id-type="manuscript">NIHMS176475</article-id><article-categories><subj-group subj-group-type="heading"><subject>Article</subject></subj-group></article-categories><title-group><article-title>Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration</article-title></title-group><contrib-group><contrib contrib-type="author"><name><surname>Cairns</surname><given-names>Nigel J.</given-names></name></contrib><aff id="A1">Department of Neurology, Washington University School of Medicine, Campus Box 8118, 660 South Euclid Avenue, St Louis, MO 63110, USA</aff><aff id="A2">Department of Pathology and Immunology, Washington University School of Medicine, Campus Box 8118, 660 South Euclid Avenue, St Louis, MO 63110, USA</aff><aff id="A3">Alzheimer's Disease Research Center, Washington University School of Medicine, Campus Box 8118, 660 South Euclid Avenue, St Louis, MO 63110, USA</aff></contrib-group><contrib-group><contrib contrib-type="author"><name><surname>Bigio</surname><given-names>Eileen H.</given-names></name></contrib><aff id="A4">Department of Pathology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA</aff><aff id="A5">Cognitive Neurology and Alzheimer Disease Center, Northwestern University Feinberg School of Medicine, Chicago, IL, USA</aff></contrib-group><contrib-group><contrib contrib-type="author"><name><surname>Mackenzie</surname><given-names>Ian R. A.</given-names></name></contrib><aff id="A6">Department of Pathology and Laboratory Medicine, Vancouver General Hospital, Vancouver, BC, Canada</aff></contrib-group><contrib-group><contrib contrib-type="author"><name><surname>Neumann</surname><given-names>Manuela</given-names></name></contrib><aff id="A7">Center for Neuropathology and Prion Research, Ludwig-Maximilians University, Munich, Germany</aff></contrib-group><contrib-group><contrib contrib-type="author"><name><surname>Lee</surname><given-names>Virginia M. -Y.</given-names></name></contrib><aff id="A8">Department of Pathology and Laboratory Medicine, Center for Neurodegenerative Disease Research, University of Pennsylvania School of Medicine, Philadelphia, PA, USA</aff></contrib-group><contrib-group><contrib contrib-type="author"><name><surname>Hatanpaa</surname><given-names>Kimmo J.</given-names></name></contrib><contrib contrib-type="author"><name><surname>White</surname><given-names>Charles L.</given-names><suffix>III</suffix></name></contrib><aff id="A9">Neuropathology Laboratory, Department of Pathology, University of Texas Southwestern Medical School, Dallas, TX, USA</aff></contrib-group><contrib-group><contrib contrib-type="author"><name><surname>Schneider</surname><given-names>Julie A.</given-names></name></contrib><aff id="A10">Rush Alzheimer's Disease Center, Rush University Medical School, Chicago, IL, USA</aff></contrib-group><contrib-group><contrib contrib-type="author"><name><surname>Grinberg</surname><given-names>Lea Tenenholz</given-names></name></contrib><aff id="A11">Department of Pathology and Instituto Israelita de Ensino e Pesquisa Albert Einstein, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, Brazil</aff></contrib-group><contrib-group><contrib contrib-type="author"><name><surname>Halliday</surname><given-names>Glenda</given-names></name></contrib><aff id="A12">Prince of Wales Medical Research Institute, Sydney, NSW, Australia</aff></contrib-group><contrib-group><contrib contrib-type="author"><name><surname>Duyckaerts</surname><given-names>Charles</given-names></name></contrib><aff id="A13">Laboratoire de Neuropathologie Escourolle, Hôpital de La Salpêtrière, Paris, France</aff></contrib-group><contrib-group><contrib contrib-type="author"><name><surname>Lowe</surname><given-names>James S.</given-names></name></contrib><aff id="A14">Department of Neuropathology, Queen's Medical Centre, Nottingham University Hospitals NHS Trust, Nottingham, UK</aff></contrib-group><contrib-group><contrib contrib-type="author"><name><surname>Holm</surname><given-names>Ida E.</given-names></name></contrib><aff id="A15">Department of Pathology, Aalborg Hospital, Aarhus University Hospital, Aalborg, Denmark</aff></contrib-group><contrib-group><contrib contrib-type="author"><name><surname>Tolnay</surname><given-names>Markus</given-names></name></contrib><aff id="A16">Department of Neuropathology, Institute of Pathology, University Hospital Basel, Basel, Switzerland</aff></contrib-group><contrib-group><contrib contrib-type="author"><name><surname>Okamoto</surname><given-names>Koichi</given-names></name></contrib><aff id="A17">Department of Neurology, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan</aff></contrib-group><contrib-group><contrib contrib-type="author"><name><surname>Yokoo</surname><given-names>Hideaki</given-names></name></contrib><aff id="A18">Department of Human Pathology, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan</aff></contrib-group><contrib-group><contrib contrib-type="author"><name><surname>Murayama</surname><given-names>Shigeo</given-names></name></contrib><aff id="A19">Geriatric Neuroscience (Neuropathology), Tokyo Metropolitan institute of Gerontology, Itabshiku, Tokyo, Japan</aff></contrib-group><contrib-group><contrib contrib-type="author"><name><surname>Woulfe</surname><given-names>John</given-names></name></contrib><aff id="A20">Department of Pathology, Ottawa Hospital and University of Ottawa, Ottawa, ON, Canada</aff></contrib-group><contrib-group><contrib contrib-type="author"><name><surname>Munoz</surname><given-names>David G.</given-names></name></contrib><aff id="A21">Department of Pathology, Saint Michael's Hospital and University of Toronto, Toronto, ON, Canada</aff></contrib-group><contrib-group><contrib contrib-type="author"><name><surname>Dickson</surname><given-names>Dennis W.</given-names></name></contrib><aff id="A22">Neuropathology Laboratory, Mayo Clinic College of Medicine, Jacksonville, FL, USA</aff></contrib-group><contrib-group><contrib contrib-type="author"><name><surname>Ince</surname><given-names>Paul G.</given-names></name></contrib><aff id="A23">Neuropathology Group, Academic Unit of Pathology, University of Sheffield Medical School, Sheffield, UK</aff></contrib-group><contrib-group><contrib contrib-type="author"><name><surname>Trojanowski</surname><given-names>John Q.</given-names></name></contrib><aff id="A24">Department of Pathology and Laboratory Medicine, Center for Neurodegenerative Disease Research, University of Pennsylvania School of Medicine, Philadelphia, PA, USA</aff><aff id="A25">Institute on Aging, University of Pennsylvania School of Medicine, Philadelphia, PA, USA</aff></contrib-group><contrib-group><contrib contrib-type="author"><name><surname>Mann</surname><given-names>David M. A.</given-names></name></contrib><aff id="A26">Clinical Neuroscience Research Group, School of Translational Medicine, Greater Manchester Neurosciences Centre, University of Manchester, Salford, UK</aff></contrib-group><author-notes><corresp id="CR1"><email>cairns@wustl.edu</email></corresp></author-notes><pub-date pub-type="nihms-submitted"><day>18</day><month>2</month><year>2010</year></pub-date><pub-date pub-type="epub"><day>20</day><month>6</month><year>2007</year></pub-date><pub-date pub-type="ppub"><month>7</month><year>2007</year></pub-date><pub-date pub-type="pmc-release"><day>24</day><month>2</month><year>2010</year></pub-date><volume>114</volume><issue>1</issue><fpage>5</fpage><lpage>22</lpage><permissions><copyright-statement>© Springer-Verlag 2007</copyright-statement><copyright-year>2007</copyright-year></permissions><abstract><p id="P1">The aim of this study was to improve the neuropathologic recognition and provide criteria for the pathological diagnosis in the neurodegenerative diseases grouped as frontotemporal lobar degeneration (FTLD); revised criteria are proposed. Recent advances in molecular genetics, biochemistry, and neuropathology of FTLD prompted the Midwest Consortium for Frontotemporal Lobar Degeneration and experts at other centers to review and revise the existing neuropathologic diagnostic criteria for FTLD. The proposed criteria for FTLD are based on existing criteria, which include the tauopathies [FTLD with Pick bodies, corticobasal degeneration, progressive supranuclear palsy, sporadic multiple system tauopathy with dementia, argyrophilic grain disease, neurofibrillary tangle dementia, and FTD with <italic>microtubule-associated tau (MAPT)</italic> gene mutation, also called FTD with parkinsonism linked to chromosome 17 (FTDP-17)]. The proposed criteria take into account new disease entities and include the novel molecular pathology, TDP-43 proteinopathy, now recognized to be the most frequent histological finding in FTLD. TDP-43 is a major component of the pathologic inclusions of most sporadic and familial cases of FTLD with ubiquitin-positive, tau-negative inclusions (FTLD-U) with or without motor neuron disease (MND). Molecular genetic studies of familial cases of FTLD-U have shown that mutations in the <italic>progranulin</italic> (<italic>PGRN</italic>) gene are a major genetic cause of FTLD-U. Mutations in <italic>valosin-containing protein</italic> (<italic>VCP</italic>) gene are present in rare familial forms of FTD, and some families with FTD and/or MND have been linked to chromosome 9p, and both are types of FTLD-U. Thus, familial TDP-43 proteinopathy is associated with defects in multiple genes, and molecular genetics is required in these cases to correctly identify the causative gene defect. In addition to genetic heterogeneity amongst the TDP-43 proteinopathies, there is also neuropathologic heterogeneity and there is a close relationship between genotype and FTLD-U sub-type. In addition to these recent significant advances in the neuropathology of FTLD-U, novel FTLD entities have been further characterized, including neuronal intermediate filament inclusion disease. The proposed criteria incorporate up-to-date neuropathology of FTLD in the light of recent immunohistochemical, biochemical, and genetic advances. These criteria will be of value to the practicing neuropathologist and provide a foundation for clinical, clinico-pathologic, mechanistic studies and in vivo models of pathogenesis of FTLD.</p></abstract><kwd-group><kwd>Frontotemporal dementia</kwd><kwd>Semantic dementia</kwd><kwd>Progressive non-Xuent aphasia</kwd><kwd>Frontotemporal lobar degeneration</kwd><kwd>Motor neuron disease</kwd><kwd>Tauopathy</kwd><kwd>Ubiquitin</kwd><kwd>TDP-43 proteinopathy</kwd><kwd>Progranulin</kwd><kwd>Valosin-containing protein</kwd><kwd>Charged multivesicular body protein 2B</kwd><kwd>Neuronal intermediate filament inclusion disease</kwd><kwd>Neuropathologic diagnosis</kwd></kwd-group><contract-num rid="AG1">U01 AG016976-09
||AG</contract-num><contract-num rid="AG1">P50 AG005681-25
||AG</contract-num><contract-num rid="NS1">P30 NS057105-04
||NS</contract-num><contract-num rid="AG1">P30 AG013854-14
||AG</contract-num><contract-num rid="AG1">P30 AG010124-16
||AG</contract-num><contract-num rid="AG1">P01 AG017586-09
||AG</contract-num><contract-num rid="AG1">P01 AG003991-26
||AG</contract-num><contract-sponsor id="AG1">National Institute on Aging : NIA</contract-sponsor><contract-sponsor id="NS1">National Institute of Neurological Disorders and Stroke : NINDS</contract-sponsor></article-meta></front></pmc></record>Pour manipuler ce document sous Unix (Dilib)
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