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@ARTICLE{JecmenicaLukic:164981,
      author       = {Jecmenica Lukic, Milica and Respondek, Gesine and Kurz,
                      Carolin and Compta, Yaroslau and Gelpi, Ellen and Ferguson,
                      Leslie W and Rajput, Alex and Troakes, Claire and van
                      Swieten, John C and Giese, Armin and Roeber, Sigrun and
                      Herms, Jochen and Arzberger, Thomas and Höglinger, Günter
                      and Giese, Armin and Troakes, Claire and Gelpi, Ellen and
                      Respondek, Gesine and Höglinger, Günter U and van Swieten,
                      John and Arzberger, Thomas and Compta, Yaroslau and
                      Molina-Porcel, L. and Aldecoa, I. and Tolosa, E. and Martí,
                      M. J. and Valldeoriola, F. and Pagonabarraga, J. and Calopa,
                      M. and Bayès, A. and Hernandez, I. and Aguilar, M. and
                      Genis, D.},
      collaboration = {group, MDS-endorsed PSP study},
      title        = {{L}ong-duration progressive supranuclear palsy: clinical
                      course and pathological underpinnings.},
      journal      = {Annals of neurology},
      volume       = {92},
      number       = {4},
      issn         = {0364-5134},
      address      = {Hoboken, NJ},
      publisher    = {Wiley-Blackwell},
      reportid     = {DZNE-2022-01385},
      pages        = {637-649},
      year         = {2022},
      abstract     = {To identify the clinical characteristics of the subgroup of
                      benign progressive supranuclear palsy with particularly long
                      disease duration; to define neuropathological determinants
                      underlying variability in disease duration in progressive
                      supranuclear palsy.Clinical and pathological features were
                      compared among 186 autopsy-confirmed cases with progressive
                      supranuclear palsy with ≥10 years and shorter survival
                      times.45 cases $(24.2\%)$ had a disease duration of ≥10
                      years. The absence of ocular motor abnormalities within the
                      first 3 years from disease onset was the only significant
                      independent clinical predictor of longer survival.
                      Histopathologically, the neurodegeneration parameters in
                      each survival group were paralleled anatomically by the
                      distribution of neuronal cytoplasmic inclusions, whereas the
                      tufted astrocytes displayed anatomically an opposite
                      severity pattern. Most interestingly, we found significantly
                      less coiled bodies in those who survive longer, in contrast
                      to patients with less favorable course.A considerable
                      proportion of patients had a more 'benign' disease course
                      with ≥10 years survival. They had a distinct pattern and
                      evolution of core symptoms compared to patients with short
                      survival. The inverted anatomical patterns of astrocytic tau
                      distribution suggest distinct implications of these cell
                      types in trans-cellular propagation. The tempo of disease
                      progression appeared to be determined mostly by
                      oligodendroglial tau, where high degree of oligodendroglial
                      tau pathology might affect neuronal integrity and function
                      on top of neuronal tau pathology. The relative contribution
                      of glial tau should be further explored in cellular and
                      animal models. This article is protected by copyright. All
                      rights reserved.},
      keywords     = {Astrocytes: metabolism / Autopsy / Disease Progression /
                      Humans / Neurons: metabolism / Supranuclear Palsy,
                      Progressive: pathology / tau Proteins: metabolism},
      cin          = {AG Höglinger 2 / Neuropathology / Brainbank / AG Herms},
      ddc          = {610},
      cid          = {I:(DE-2719)1111015 / I:(DE-2719)1140013 /
                      I:(DE-2719)1110001},
      pnm          = {353 - Clinical and Health Care Research (POF4-353) / 352 -
                      Disease Mechanisms (POF4-352)},
      pid          = {G:(DE-HGF)POF4-353 / G:(DE-HGF)POF4-352},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:35872640},
      doi          = {10.1002/ana.26455},
      url          = {https://pub.dzne.de/record/164981},
}