1. A language-based sum score for the course and therapeutic intervention in primary progressive aphasia
- Author
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Semler, Elisa, Anderl-Straub, Sarah, Kornhuber, Johannes, Landwehrmeyer, Bernhard, Lauer, Martin, Muche, Rainer, Prudlo, Johannes, Schneider, Anja, Schroeter, Matthias L, Ludolph, Albert C, Otto, Markus, consortium, FTLD, Uttner, Ingo, Bisenius, Sandrine, Feneberg, Emily, Faber, Jennifer, Hammer, Anke, Haefner, Sibylle, Kasper, Elisabeth, Kurzwelly, Delia, Levin, Johannes, Lornsen, Finn, Luley, Maxine, Diehl-Schmid, Janine, Maler, Manuel, Müller, Hans-Peter, Oberstein, Timo, Pellkofer, Hannah, Prix, Catharina, Riederer, Isabelle, Roßmeier, Carola, Schomburg, Robert, Roeske, Sandra, Schönecker, Sonja, Danek, Adrian, Spitzer, Philipp, Spottke, Annika, Stuke, Katharina, Teipel, Stefan, von Arnim, Christine, Wilken, Petra, Wiltfang, Jens, Einsiedler, Beate, Fassbender, Klaus, Fließbach, Klaus, Huppertz, Hans-Jürgen, Jahn, Holger, and FTLD consortium
- Subjects
Male ,therapy [Aphasia, Primary Progressive] ,cerebrospinal fluid [Amyloid beta-Peptides] ,Audiology ,Neuropsychological Tests ,lcsh:RC346-429 ,Primary progressive aphasia ,0302 clinical medicine ,pathology [Brain] ,Assessment of cognitive disorders/dementia ,Medizinische Fakultät ,Memory span ,Cognitive decline ,Language ,pathology [Atrophy] ,Volumetric MRI ,05 social sciences ,Neuropsychology ,Brain ,Cognition ,Middle Aged ,amyloid beta-protein (1-42) ,Magnetic Resonance Imaging ,3. Good health ,Cognitive test ,Boston Naming Test ,Neurology ,therapy [Cognition Disorders] ,Disease Progression ,Female ,medicine.symptom ,Frontotemporal dementia ,medicine.medical_specialty ,Cognitive Neuroscience ,diagnostic imaging [Aphasia, Primary Progressive] ,tau Proteins ,050105 experimental psychology ,Statistics, Nonparametric ,lcsh:RC321-571 ,03 medical and health sciences ,Aphasia ,medicine ,Humans ,0501 psychology and cognitive sciences ,cerebrospinal fluid [Peptide Fragments] ,Cognitive neuropsychology in dementia ,ddc:610 ,cerebrospinal fluid [Aphasia, Primary Progressive] ,lcsh:Neurosciences. Biological psychiatry. Neuropsychiatry ,lcsh:Neurology. Diseases of the nervous system ,etiology [Atrophy] ,Aged ,Amyloid beta-Peptides ,business.industry ,Research ,complications [Aphasia, Primary Progressive] ,etiology [Cognition Disorders] ,medicine.disease ,Peptide Fragments ,Aphasia, Primary Progressive ,cerebrospinal fluid [tau Proteins] ,Neurology (clinical) ,Atrophy ,business ,Cognition Disorders ,030217 neurology & neurosurgery ,Follow-Up Studies - 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.
- Published
- 2018
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