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000164981 041__ $$aEnglish
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000164981 1001_ $$0P:(DE-2719)2812616$$aJecmenica Lukic, Milica$$b0$$eFirst author$$udzne
000164981 245__ $$aLong-duration progressive supranuclear palsy: clinical course and pathological underpinnings.
000164981 260__ $$aHoboken, NJ$$bWiley-Blackwell$$c2022
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000164981 520__ $$aTo identify the clinical characteristics of the subgroup of benign progressive supranuclear palsy with particularly long disease duration; to define neuropathological determinants underlying variability in disease duration in progressive supranuclear palsy.Clinical and pathological features were compared among 186 autopsy-confirmed cases with progressive supranuclear palsy with ≥10 years and shorter survival times.45 cases (24.2%) had a disease duration of ≥10 years. The absence of ocular motor abnormalities within the first 3 years from disease onset was the only significant independent clinical predictor of longer survival. Histopathologically, the neurodegeneration parameters in each survival group were paralleled anatomically by the distribution of neuronal cytoplasmic inclusions, whereas the tufted astrocytes displayed anatomically an opposite severity pattern. Most interestingly, we found significantly less coiled bodies in those who survive longer, in contrast to patients with less favorable course.A considerable proportion of patients had a more 'benign' disease course with ≥10 years survival. They had a distinct pattern and evolution of core symptoms compared to patients with short survival. The inverted anatomical patterns of astrocytic tau distribution suggest distinct implications of these cell types in trans-cellular propagation. The tempo of disease progression appeared to be determined mostly by oligodendroglial tau, where high degree of oligodendroglial tau pathology might affect neuronal integrity and function on top of neuronal tau pathology. The relative contribution of glial tau should be further explored in cellular and animal models. This article is protected by copyright. All rights reserved.
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000164981 650_2 $$2MeSH$$aAstrocytes: metabolism
000164981 650_2 $$2MeSH$$aAutopsy
000164981 650_2 $$2MeSH$$aDisease Progression
000164981 650_2 $$2MeSH$$aHumans
000164981 650_2 $$2MeSH$$aNeurons: metabolism
000164981 650_2 $$2MeSH$$aSupranuclear Palsy, Progressive: pathology
000164981 650_2 $$2MeSH$$atau Proteins: metabolism
000164981 7001_ $$0P:(DE-2719)2811600$$aRespondek, Gesine$$b1$$udzne
000164981 7001_ $$0P:(DE-2719)9000176$$aKurz, Carolin$$b2$$udzne
000164981 7001_ $$aCompta, Yaroslau$$b3
000164981 7001_ $$aGelpi, Ellen$$b4
000164981 7001_ $$aFerguson, Leslie W$$b5
000164981 7001_ $$aRajput, Alex$$b6
000164981 7001_ $$aTroakes, Claire$$b7
000164981 7001_ $$agroup, MDS-endorsed PSP study$$b8$$eCollaboration Author
000164981 7001_ $$avan Swieten, John C$$b9
000164981 7001_ $$0P:(DE-2719)9000906$$aGiese, Armin$$b10$$udzne
000164981 7001_ $$aRoeber, Sigrun$$b11
000164981 7001_ $$0P:(DE-2719)2810441$$aHerms, Jochen$$b12$$udzne
000164981 7001_ $$0P:(DE-2719)2811333$$aArzberger, Thomas$$b13$$udzne
000164981 7001_ $$0P:(DE-2719)2811373$$aHöglinger, Günter$$b14$$eLast author$$udzne
000164981 7001_ $$0P:(DE-2719)9000906$$aGiese, Armin$$b15$$udzne
000164981 7001_ $$aTroakes, Claire$$b16
000164981 7001_ $$aGelpi, Ellen$$b17
000164981 7001_ $$0P:(DE-2719)2811600$$aRespondek, Gesine$$b18$$udzne
000164981 7001_ $$0P:(DE-2719)2811373$$aHöglinger, Günter U$$b19$$udzne
000164981 7001_ $$avan Swieten, John$$b20
000164981 7001_ $$0P:(DE-2719)2811333$$aArzberger, Thomas$$b21$$udzne
000164981 7001_ $$aCompta, Yaroslau$$b22
000164981 7001_ $$aMolina-Porcel, L.$$b23
000164981 7001_ $$aAldecoa, I.$$b24
000164981 7001_ $$aTolosa, E.$$b25
000164981 7001_ $$aMartí, M. J.$$b26
000164981 7001_ $$aValldeoriola, F.$$b27
000164981 7001_ $$aPagonabarraga, J.$$b28
000164981 7001_ $$aCalopa, M.$$b29
000164981 7001_ $$aBayès, A.$$b30
000164981 7001_ $$0P:(DE-HGF)0$$aHernandez, I.$$b31
000164981 7001_ $$aAguilar, M.$$b32
000164981 7001_ $$aGenis, D.$$b33
000164981 773__ $$0PERI:(DE-600)2037912-2$$a10.1002/ana.26455$$gp. ana.26455$$n4$$p637-649$$tAnnals of neurology$$v92$$x0364-5134$$y2022
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