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Stages of pTDP‐43 pathology in amyotrophic lateral sclerosis

Annals of Neurology · 2013 · Vol. 74(1) · pp. 20–38

Johannes Brettschneider(Institute for Neurodegenerative Disorders)Kelly Del Tredici(Universität Ulm)Jon B. Toledo(Institute for Neurodegenerative Disorders)John Robinson(Institute for Neurodegenerative Disorders)David J. Irwin(University of Pennsylvania)Murray Grossman(University of Pennsylvania)EunRan Suh(University of Pennsylvania)Vivianna M. Van Deerlin(Institute for Neurodegenerative Disorders)Elisabeth McCarty Wood(University of Pennsylvania)Young Min Baek(Institute for Neurodegenerative Disorders)Linda K. Kwong(University of Pennsylvania)Edward B. Lee(University of Pennsylvania)Lauren Elman(University of Pennsylvania)Leo McCluskey(University of Pennsylvania)Lubin Fang(Universität Ulm)Simone Feldengut(Universität Ulm)Albert C. Ludolph(Universität Ulm)Virginia M.‐Y. Lee(University of Pennsylvania)Heiko Braak(Universität Ulm)John Q. Trojanowski✉(Institute for Neurodegenerative Disorders)

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Abstract

pTDP-43 pathology in ALS possibly disseminates in a sequential pattern that permits recognition of 4 neuropathological stages consistent with the hypothesis that pTDP-43 pathology is propagated along axonal pathways. Moreover, the finding that pTDP-43 pathology develops in the prefrontal cortex as part of an ongoing disease process could account for the development of executive cognitive deficits in ALS.

Amyotrophic Lateral Sclerosis Research Parkinson's Disease Mechanisms and Treatments Neurological diseases and metabolismReticular formationPathologyBrainstemAnatomyBiologyNeuroscienceCentral nervous systemMedicine

MeSH terms

Autophagy-Related ProteinsC9orf72 ProteinAdultAgedAged, 80 and overAmyotrophic Lateral SclerosisBrainCross-Sectional StudiesDNA-Binding ProteinsFemaleGenetic TestingHumansMaleMiddle AgedMutation

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Limbic-predominant age-related TDP-43 encephalopathy (LATE): consensus working group report