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@ARTICLE{JecmenicaLukic:153339,
      author       = {Jecmenica Lukic, Milica and Kurz, Carolin and Respondek,
                      Gesine and Grau-Rivera, Oriol and Compta, Yaroslau and
                      Gelpi, Ellen and Troakes, Claire and Barcelona Brain Bank
                      collaborative group, the MDS-endorsed PSP study group and
                      van Swieten, John C and Giese, Armin and Roeber, Sigrun and
                      Arzberger, Thomas and Höglinger, Günter and Grau-Rivera,
                      O. and Compta, Y. 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.
                      and Fernandez, M. and Munoz-Garcia, C. and Antonell, A. and
                      Gelpi, E.},
      title        = {{C}opathology in {P}rogressive {S}upranuclear {P}alsy:
                      {D}oes {I}t {M}atter?},
      journal      = {Movement disorders},
      volume       = {35},
      number       = {6},
      issn         = {1531-8257},
      address      = {New York, NY},
      publisher    = {Wiley},
      reportid     = {DZNE-2020-01336},
      pages        = {984 - 993},
      year         = {2020},
      abstract     = {The influence of concomitant brain pathologies on the
                      progression rate in PSP is unclear.To analyze the frequency
                      and severity of copathologies and their impact on the
                      progression in PSP.We analyzed clinic-pathological features
                      of 101 PSP patients. Diagnoses and stages of copathologies
                      were established according to standardized criteria,
                      including Alzheimer's disease-related pathology,
                      argyrophilic grains, Lewy-related pathology, transactive
                      response DNA-binding protein 43 pathology, fused in sarcoma
                      pathology, cerebral amyloid angiopathy, and small vessel
                      disease. Demographic data and major clinical milestones
                      (frequency and latency to onset) were extracted from
                      patients' files.Only $8\%$ of 101 patients presented with
                      pure PSP pathology without any copathology. Alzheimer's
                      disease-related pathology was the most frequent $(84\%),$
                      followed by argyrophilic grains $(58\%),$ both occurring as
                      single copathology or in combination with other
                      proteinopathies or cerebrovascular disease. Lewy-related and
                      transactive response DNA-binding protein 43 copathology
                      occurred rarely $(8\%$ and $6\%,$ respectively). Fused in
                      sarcoma-positive cases were not found. While being common,
                      copathology was mostly mild in severity, with the exception
                      of frequently widespread argyrophilic grains. Small vessel
                      disease was also frequent $(65\%).$ Cerebral amyloid
                      angiopathy occurred only in the presence of Alzheimer's
                      disease-related changes $(25\%).$ The copathologies did not
                      have major impact on prevalence and time frame of major
                      disease milestones.In PSP, concomitant neurodegenerative
                      proteinopathies or cerebrovascular diseases are frequent,
                      but generally mild in severity. Our data confirmed that four
                      repeat tau is still the most relevant target for PSP,
                      whereas the impact of copathologies on progression rate
                      appears to be of less importance. This is relevant
                      information for the development of disease-modifying
                      therapies. © 2020 The Authors. Movement Disorders published
                      by Wiley Periodicals, Inc. on behalf of International
                      Parkinson and Movement Disorder Society.},
      keywords     = {Alzheimer Disease: epidemiology / Brain: metabolism /
                      Humans / Movement Disorders / Supranuclear Palsy,
                      Progressive: epidemiology / tau Proteins: metabolism},
      cin          = {AG Höglinger 2 / AG Höglinger 1 / München Pre 2020 /
                      Neuropathology / Brainbank},
      ddc          = {610},
      cid          = {I:(DE-2719)1111015 / I:(DE-2719)1110002 /
                      I:(DE-2719)6000016 / I:(DE-2719)1140013},
      pnm          = {344 - Clinical and Health Care Research (POF3-344)},
      pid          = {G:(DE-HGF)POF3-344},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:32125724},
      doi          = {10.1002/mds.28011},
      url          = {https://pub.dzne.de/record/153339},
}