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000153339 0247_ $$2doi$$a10.1002/mds.28011
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000153339 0247_ $$2ISSN$$a0885-3185
000153339 0247_ $$2ISSN$$a1531-8257
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000153339 037__ $$aDZNE-2020-01336
000153339 041__ $$aEnglish
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000153339 1001_ $$0P:(DE-2719)2812616$$aJecmenica Lukic, Milica$$b0$$udzne
000153339 245__ $$aCopathology in Progressive Supranuclear Palsy: Does It Matter?
000153339 260__ $$aNew York, NY$$bWiley$$c2020
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000153339 520__ $$aThe 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.
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000153339 650_2 $$2MeSH$$aAlzheimer Disease: epidemiology
000153339 650_2 $$2MeSH$$aBrain: metabolism
000153339 650_2 $$2MeSH$$aHumans
000153339 650_2 $$2MeSH$$aMovement Disorders
000153339 650_2 $$2MeSH$$aSupranuclear Palsy, Progressive: epidemiology
000153339 650_2 $$2MeSH$$atau Proteins: metabolism
000153339 7001_ $$0P:(DE-2719)9000176$$aKurz, Carolin$$b1$$udzne
000153339 7001_ $$0P:(DE-2719)2811600$$aRespondek, Gesine$$b2$$udzne
000153339 7001_ $$aGrau-Rivera, Oriol$$b3
000153339 7001_ $$aCompta, Yaroslau$$b4
000153339 7001_ $$aGelpi, Ellen$$b5
000153339 7001_ $$aTroakes, Claire$$b6
000153339 7001_ $$0P:(DE-HGF)0$$aBarcelona Brain Bank collaborative group, the MDS-endorsed PSP study group$$b7
000153339 7001_ $$avan Swieten, John C$$b8
000153339 7001_ $$0P:(DE-HGF)0$$aGiese, Armin$$b9
000153339 7001_ $$aRoeber, Sigrun$$b10
000153339 7001_ $$0P:(DE-2719)2811333$$aArzberger, Thomas$$b11$$udzne
000153339 7001_ $$0P:(DE-2719)2811373$$aHöglinger, Günter$$b12$$eLast author$$udzne
000153339 7001_ $$aGrau-Rivera, O.$$b13
000153339 7001_ $$aCompta, Y.$$b14
000153339 7001_ $$aTolosa, E.$$b15
000153339 7001_ $$aMartí, M. J.$$b16
000153339 7001_ $$aValldeoriola, F.$$b17
000153339 7001_ $$aPagonabarraga, J.$$b18
000153339 7001_ $$aCalopa, M.$$b19
000153339 7001_ $$aBayès, A.$$b20
000153339 7001_ $$0P:(DE-HGF)0$$aHernandez, I.$$b21
000153339 7001_ $$aAguilar, M.$$b22
000153339 7001_ $$aGenis, D.$$b23
000153339 7001_ $$aFernandez, M.$$b24
000153339 7001_ $$aMunoz-Garcia, C.$$b25
000153339 7001_ $$aAntonell, A.$$b26
000153339 7001_ $$aGelpi, E.$$b27
000153339 773__ $$0PERI:(DE-600)2041249-6$$a10.1002/mds.28011$$gVol. 35, no. 6, p. 984 - 993$$n6$$p984 - 993$$tMovement disorders$$v35$$x1531-8257$$y2020
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