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000141523 041__ $$aEnglish
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000141523 1001_ $$0P:(DE-2719)2811945$$aJiang, Xueyan$$b0$$eFirst author
000141523 245__ $$aCharacterization of Cerebellar Atrophy and Resting State Functional Connectivity Patterns in Sporadic Adult-Onset Ataxia of Unknown Etiology (SAOA).
000141523 260__ $$aLondon$$bDunitz$$c2019
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000141523 520__ $$aSporadic adult-onset ataxia of unknown etiology (SAOA) is a non-genetic neurodegenerative disorder of the cerebellum of unknown cause which manifests with progressive ataxia without severe autonomic failure. Although SAOA is associated with cerebellar degeneration, little is known about the specific cerebellar atrophy pattern in SAOA. Thirty-seven SAOA patients and 49 healthy controls (HCs) were included at two centers. We investigated the structural and functional characteristics of SAOA brains using voxel-based morphometry (VBM) and resting-state functional imaging (rs-fMRI). In order to examine the functional consequence of structural cerebellar alterations, the amplitude of low-frequency fluctuation (ALFF) and degree centrality (DC) were analyzed, and then assessed their relation with disease severity, disease duration, and age of onset within these regions. Group differences were investigated using two-sample t tests, controlling for age, gender, site, and the total intracranial volume. The VBM analysis revealed a significant, mostly bilateral reduction of local gray matter (GM) volume in lobules I-V, V, VI, IX, X, and vermis VIII a/b in SAOA patients, compared with HCs. The GM volume loss in these regions was significantly associated with disease severity, disease duration, and age of onset. The disease-related atrophy regions did not show any functional alternations compared with HCs but were functionally characterized by high ALFF and poor DC compared with intact cerebellar regions. Our data revealed volume reduction in SAOA in cerebellar regions that are known to be involved in motor and somatosensory processing, corresponding with the clinical phenotype of SAOA. Our data suggest that the atrophy occurs in those cerebellar regions which are characterized by high ALFF and poor DC. Further studies have to show if these findings are specific for SAOA, and if they can be used to predict disease progression.
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000141523 542__ $$2Crossref$$i2019-08-17$$uhttp://www.springer.com/tdm
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000141523 650_2 $$2MeSH$$aAdult
000141523 650_2 $$2MeSH$$aAged
000141523 650_2 $$2MeSH$$aAtrophy: diagnostic imaging
000141523 650_2 $$2MeSH$$aAtrophy: physiopathology
000141523 650_2 $$2MeSH$$aCerebellar Ataxia: diagnostic imaging
000141523 650_2 $$2MeSH$$aCerebellar Ataxia: physiopathology
000141523 650_2 $$2MeSH$$aCerebellum: diagnostic imaging
000141523 650_2 $$2MeSH$$aCerebellum: physiopathology
000141523 650_2 $$2MeSH$$aFemale
000141523 650_2 $$2MeSH$$aHumans
000141523 650_2 $$2MeSH$$aMagnetic Resonance Imaging: methods
000141523 650_2 $$2MeSH$$aMale
000141523 650_2 $$2MeSH$$aMiddle Aged
000141523 650_2 $$2MeSH$$aNerve Net: diagnostic imaging
000141523 650_2 $$2MeSH$$aNerve Net: physiopathology
000141523 650_2 $$2MeSH$$aRest: physiology
000141523 693__ $$0EXP:(DE-2719)DELCODE-20140101$$5EXP:(DE-2719)DELCODE-20140101$$eLongitudinal Cognitive Impairment and Dementia Study$$x0
000141523 7001_ $$0P:(DE-2719)2811327$$aFaber, J.$$b1
000141523 7001_ $$0P:(DE-2719)2811662$$aGiordano, I.$$b2
000141523 7001_ $$0P:(DE-2719)2810317$$aMachts, J.$$b3
000141523 7001_ $$0P:(DE-2719)9000373$$aKindler, Ch$$b4
000141523 7001_ $$0P:(DE-HGF)0$$aDudesek, A.$$b5
000141523 7001_ $$0P:(DE-2719)2810706$$aSpeck, O.$$b6
000141523 7001_ $$0P:(DE-HGF)0$$aKamm, Ch$$b7
000141523 7001_ $$0P:(DE-2719)2000005$$aDüzel, E.$$b8
000141523 7001_ $$0P:(DE-2719)2000032$$aJessen, F.$$b9
000141523 7001_ $$0P:(DE-2719)2811324$$aSpottke, A.$$b10
000141523 7001_ $$0P:(DE-2719)2000035$$aVielhaber, St$$b11
000141523 7001_ $$0P:(DE-2719)2810726$$aBoecker, H.$$b12
000141523 7001_ $$0P:(DE-2719)2810314$$aKlockgether, T.$$b13
000141523 7001_ $$0P:(DE-2719)2811377$$aScheef, L.$$b14
000141523 77318 $$2Crossref$$3journal-article$$a10.1007/s12311-019-01072-y$$b : Springer Science and Business Media LLC, 2019-08-17$$n5$$p873-881$$tThe Cerebellum$$v18$$x1473-4222$$y2019
000141523 773__ $$0PERI:(DE-600)2071266-2$$a10.1007/s12311-019-01072-y$$gVol. 18, no. 5, p. 873 - 881$$n5$$p873-881$$q18:5<873 - 881$$tThe Cerebellum$$v18$$x1473-4222$$y2019
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