@article{LombardiMayerSemleretal.2021,
  author    = {Lombardi, Jolina and Mayer, Benjamin and Semler, Elisa and Anderl-Straub, Sarah and Uttner, Ingo and Kassubek, Jan and Diehl-Schmid, Janine and Danek, Adrian and Levin, Johannes and Fassbender, Klaus and Fliessbach, Klaus and Schneider, Anja and Huppertz, Hans-J{\"u}rgen and Jahn, Holger and Volk, Alexander and Kornhuber, Johannes and Landwehrmeyer, Bernhard and Lauer, Martin and Prudlo, Johannes and Wiltfang, Jens and Schroeter, Matthias L. and Ludolph, Albert and Otto, Markus},
  title     = {Quantifying progression in primary progressive aphasia with structural neuroimaging},
  series = {Alzheimer's \& Dementia},
  volume    = {17},
  journal   = {Alzheimer's \& Dementia},
  number    = {10},
  doi       = {10.1002/alz.12323},
  url       = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-262605},
  pages     = {1595 -- 1609},
  year      = {2021},
  abstract  = {Introduction The term primary progressive aphasia (PPA) sums up the non-fluent (nfv), the semantic (sv), and the logopenic (lv) variant. Up to now, there is only limited data available concerning magnetic resonance imaging volumetry to monitor disease progression. Methods Structural brain imaging and an extensive assessment were applied at baseline and up to 4-year(s) follow-up in 269 participants. With automated atlas-based volumetry 56 brain regions were assessed. Atrophy progression served to calculate sample sizes for therapeutic trials. Results At baseline highest atrophy appeared in parts of the left frontal lobe for nfvPPA (-17\%) and of the left temporal lobe for svPPA (-34\%) and lvPPA (-24\%). Severest progression within 1-year follow-up occurred in the basal ganglia in nfvPPA (-7\%), in the hippocampus/amygdala in svPPA (-9\%), and in (medial) temporal regions in lvPPA (-6\%). Conclusion PPA presents as a left-dominant, mostly gray matter sensitive disease with considerable atrophy at baseline that proceeds variant-specific.},
  language  = {en}
}
@article{SchroeterPawelkeBiseniusetal.2018,
  author    = {Schroeter, Matthias L. and Pawelke, Sarah and Bisenius, Sandrine and Kynast, Jana and Schuemberg, Katharina and Polyakova, Maryna and Anderl-Straub, Sarah and Danek, Adrian and Fassbender, Klaus and Jahn, Holger and Jessen, Frank and Kornhuber, Johannes and Lauer, Martin and Prudlo, Johannes and Schneider, Anja and Uttner, Ingo and Th{\"o}ne-Otto, Angelika and Otto, Markus and Diehl-Schmid, Janine},
  title     = {A Modified Reading the Mind in the Eyes Test Predicts Behavioral Variant Frontotemporal Dementia Better Than Executive Function Tests},
  series = {Frontiers in Aging Neuroscience},
  volume    = {10},
  journal   = {Frontiers in Aging Neuroscience},
  organization    = {FTLD Study Group Germany},
  doi       = {10.3389/fnagi.2018.00011},
  url       = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-234254},
  year      = {2018},
  abstract  = {Behavioral variant frontotemporal dementia (bvFTD) is characterized by deep alterations in behavior and personality. Although revised diagnostic criteria agree for executive dysfunction as most characteristic, impairments in social cognition are also suggested. The study aimed at identifying those neuropsychological and behavioral parameters best discriminating between bvFTD and healthy controls. Eighty six patients were diagnosed with possible or probable bvFTD according to Rascovsky et al. (2011) and compared with 43 healthy age-matched controls. Neuropsychological performance was assessed with a modified Reading the Mind in the Eyes Test (RMET), Stroop task, Trail Making Test (TMT), Hamasch-Five-Point Test (H5PT), and semantic and phonemic verbal fluency tasks. Behavior was assessed with the Apathy Evaluation Scale, Frontal Systems Behavioral Scale, and Bayer Activities of Daily Living Scale. Each test's discriminatory power was investigated by Receiver Operating Characteristic curves calculating the area under the curve (AUC). bvFTD patients performed significantly worse than healthy controls in all neuropsychological tests. Discriminatory power (AUC) was highest in behavioral questionnaires, high in verbal fluency tasks and the RMET, and lower in executive function tests such as the Stroop task, TMT and H5PT. As fluency tasks depend on several cognitive functions, not only executive functions, results suggest that the RMET discriminated better between bvFTD and control subjects than other executive tests. Social cognition should be incorporated into diagnostic criteria for bvFTD in the future, such as in the International Classification of Diseases (ICD)-11, as already suggested in the Diagnostic and Statistical Manual for Mental Disorders (DSM)-5.},
  language  = {en}
}
@article{SemlerAnderlStraubUttneretal.2018,
  author    = {Semler, Elisa and Anderl-Straub, Sarah and Uttner, Ingo and Diehl-Schmid, Janine and Danek, Adrian and Einsiedler, Beate and Fassbender, Klaus and Fliessbach, Klaus and Huppertz, Hans-J{\"u}rgen and Jahn, Holger and Kornhuber, Johannes and Landwehrmeyer, Bernhard and Lauer, Martin and Muche, Rainer and Prudlo, Johannes and Schneider, Anja and Schroeter, Matthias L. and Ludolph, Albert C. and Otto, Markus},
  title     = {A language-based sum score for the course and therapeutic intervention in primary progressive aphasia},
  series = {Alzheimer's Research \& Therapy},
  volume    = {10},
  journal   = {Alzheimer's Research \& Therapy},
  organization    = {FLTD consortium},
  doi       = {10.1186/s13195-018-0345-3},
  url       = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-236277},
  year      = {2018},
  abstract  = {Background With upcoming therapeutic interventions for patients with primary progressive aphasia (PPA), instruments for the follow-up of patients are needed to describe disease progression and to evaluate potential therapeutic effects. So far, volumetric brain changes have been proposed as clinical endpoints in the literature, but cognitive scores are still lacking. This study followed disease progression predominantly in language-based performance within 1 year and defined a PPA sum score which can be used in therapeutic interventions. Methods We assessed 28 patients with nonfluent variant PPA, 17 with semantic variant PPA, 13 with logopenic variant PPA, and 28 healthy controls in detail for 1 year. The most informative neuropsychological assessments were combined to a sum score, and associations between brain atrophy were investigated followed by a sample size calculation for clinical trials. Results Significant absolute changes up to 20\% in cognitive tests were found after 1 year. Semantic and phonemic word fluency, Boston Naming Test, Digit Span, Token Test, AAT Written language, and Cookie Test were identified as the best markers for disease progression. These tasks provide the basis of a new PPA sum score. Assuming a therapeutic effect of 50\% reduction in cognitive decline for sample size calculations, a number of 56 cases is needed to find a significant treatment effect. Correlations between cognitive decline and atrophy showed a correlation up to r = 0.7 between the sum score and frontal structures, namely the superior and inferior frontal gyrus, as well as with left-sided subcortical structures. Conclusion Our findings support the high performance of the proposed sum score in the follow-up of PPA and recommend it as an outcome measure in intervention studies.},
  language  = {en}
}