Asymmetric pathology in primary progressive aphasia with progranulin mutations and TDP inclusions

Neurology
Garam KimChangiz Geula

Abstract

To investigate quantitative regional distribution and hemispheric asymmetry of TDP-43 (TAR DNA-binding protein 43) inclusions, neurons, and activated microglia in primary progressive aphasia (PPA) with progranulin (GRN) mutations, and to determine concordance between distribution of pathology, clinical phenotype, and known atrophy patterns. Antibodies to phospho-TDP-43, NeuN (neuronal nuclei), and HLA-DR were used to visualize inclusions, neurons, and activated microglia in paraffin-embedded tissue sections from 4 participants with PPA: 2 of the agrammatic and 2 of the logopenic subtype. Unbiased stereological counting techniques were used for quantitation of immunoreactive profiles in language- and memory-related cortical areas bilaterally. Patterns of pathology across cortical areas and hemispheres were compared and their relationships with known patterns of atrophy investigated. Numerical densities of TDP-43 inclusions, and less so of activated microglia, were greater in language-related areas compared with memory-related areas. In language areas, neuronal density displayed a pattern opposite to inclusions and activated microglia. Densities of inclusions and microglia were greater (p < 0.05), and densities of neurons were lo...Continue Reading

References

Feb 13, 2007·Journal of Neuroinflammation·Zeshan AhmedDennis W Dickson
Dec 23, 2009·The Journal of Experimental Medicine·Fangfang YinAihao Ding
Feb 10, 2010·Archives of Neurology·Chang-En YuVivianna M Van Deerlin
Aug 13, 2010·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Ya-Fei XuLeonard Petrucelli
Jan 13, 2011·The American Journal of Pathology·Fiona PickfordMichael Hutton
Feb 18, 2011·Neurology·M L Gorno-TempiniM Grossman
Mar 4, 2011·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Emily RogalskiM-Marsel Mesulam
Apr 13, 2011·Archives of Neurology·Alice S Chen-PlotkinVivianna M Van Deerlin
Jun 7, 2011·Acta Neuropathologica·Ian R A MackenzieVirginia M Y Lee
Oct 28, 2011·Molecular Neurodegeneration·Ya-Fei XuLeonard Petrucelli
Feb 22, 2012·Brain : a Journal of Neurology·Ging-Yuek R HsiungIan R A Mackenzie
Apr 24, 2012·Brain : a Journal of Neurology·Tamar GefenM-Marsel Mesulam
Apr 25, 2012·Brain : a Journal of Neurology·M-Marsel MesulamSandra Weintraub
Apr 28, 2012·Acta Neuropathologica·Ashley CannonJada Lewis
Aug 21, 2012·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·S SpinaB Ghetti
Oct 9, 2012·The Journal of Clinical Investigation·Lauren Herl MartensRobert V Farese
Oct 24, 2012·Journal of Leukocyte Biology·Jinlong JianChuanju Liu
Feb 28, 2014·Brain : a Journal of Neurology·M-Marsel MesulamEileen H Bigio
Aug 29, 2014·Neurology·Emily RogalskiM-Marsel Mesulam
Sep 3, 2014·Nature Reviews. Neurology·M-Marsel MesulamSandra Weintraub

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Citations

Jun 1, 2018·Continuum : Lifelong Learning in Neurology·Murray Grossman, David J Irwin
Mar 9, 2019·Brain : a Journal of Neurology·Henri J M M MutsaertsUNKNOWN GENetic Frontotemporal dementia Initiative (GENFI)
Mar 22, 2019·Cognitive and Behavioral Neurology : Official Journal of the Society for Behavioral and Cognitive Neurology·Marek-Marsel MesulamSandra Weintraub
Mar 10, 2019·Annals of Neurology·Lucia A A GianniniDavid J Irwin
Dec 12, 2017·Brain : a Journal of Neurology·David J IrwinMurray Grossman
Jan 18, 2020·Journal of Neuropathology and Experimental Neurology·Sanjana ShellikeriYana Yunusova
May 11, 2020·Journal of Neuropathology and Experimental Neurology·Pouya JamshidiChangiz Geula
Jun 23, 2020·Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration·Abigail LicataJanine Diehl-Schmid
Feb 13, 2019·Journal of Neuropathology and Experimental Neurology·Garam KimChangiz Geula
May 20, 2018·Nature Reviews. Neurology·Sara Van MosseveldeChristine Van Broeckhoven
May 14, 2021·Neurology·Dario SaracinoUNKNOWN French Research Network on FTD/FTD-ALS
Apr 29, 2020·Dementia and Geriatric Cognitive Disorders·Ione O C WoollacottJonathan D Rohrer

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