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000285346 037__ $$aDZNE-2026-00212
000285346 041__ $$aEnglish
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000285346 1001_ $$aMaas, Roderick P P W M$$b0
000285346 245__ $$aCognitive impairment in SCA3: A multi-center cohort study with demographic, imaging, and biomarker correlates.
000285346 260__ $$a[Amsterdam]$$bElsevier$$c2026
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000285346 520__ $$aCognitive deficits are common in spinocerebellar ataxia type 3 (SCA3), but their neurobiological correlates remain largely unknown.To investigate cognitive performance in a large international cohort of SCA3 mutation carriers covering the entire disease course and to explore associations with posterior cerebellar volumes, basal ganglia and thalamus volumes, and plasma neurofilament light chain (NfL) concentration.The Montreal Cognitive Assessment (MoCA) was used to evaluate cognitive impairment in this prospective, observational cohort study involving 13 ataxia referral centers. Standardized motor assessments, brain MR imaging, and peripheral blood biosampling were also performed.MoCA data were collected from 61 pre-ataxic SCA3 mutation carriers, 231 ataxic SCA3 patients, and 111 healthy controls. After adjustments for educational level and age, there were significant differences in MoCA total score, as well as visuospatial/executive, attention, language, and abstraction subscores, between healthy controls and ataxic, but not pre-ataxic individuals. MoCA scores declined with ataxia severity, especially in patients with a lower educational level. Patients with a MoCA score < 26 had lower pallidal volumes and higher plasma NfL concentrations than those with a score ≥ 26. However, only the interaction term between ataxia severity and educational level was independently associated with cognitive performance in multivariable regression analyses containing demographic, clinical, volumetric, and biochemical parameters.Cognitive deficits in SCA3 generally appear after clinical ataxia onset and progress in parallel with ataxia severity, especially in patients with a lower cognitive reserve. Other measured biochemical and imaging parameters did not have a significant additional contribution.
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000285346 650_7 $$2Other$$aCerebellum
000285346 650_7 $$2Other$$aCognition
000285346 650_7 $$2Other$$aMachado-Joseph disease
000285346 650_7 $$2Other$$aSpinocerebellar ataxia type 3
000285346 650_7 $$2NLM Chemicals$$aBiomarkers
000285346 650_7 $$2NLM Chemicals$$aNeurofilament Proteins
000285346 650_7 $$2NLM Chemicals$$aneurofilament protein L
000285346 650_2 $$2MeSH$$aHumans
000285346 650_2 $$2MeSH$$aMale
000285346 650_2 $$2MeSH$$aFemale
000285346 650_2 $$2MeSH$$aMiddle Aged
000285346 650_2 $$2MeSH$$aCognitive Dysfunction: diagnostic imaging
000285346 650_2 $$2MeSH$$aCognitive Dysfunction: etiology
000285346 650_2 $$2MeSH$$aCognitive Dysfunction: blood
000285346 650_2 $$2MeSH$$aCognitive Dysfunction: psychology
000285346 650_2 $$2MeSH$$aAdult
000285346 650_2 $$2MeSH$$aBiomarkers: blood
000285346 650_2 $$2MeSH$$aMagnetic Resonance Imaging
000285346 650_2 $$2MeSH$$aMachado-Joseph Disease: complications
000285346 650_2 $$2MeSH$$aMachado-Joseph Disease: diagnostic imaging
000285346 650_2 $$2MeSH$$aMachado-Joseph Disease: psychology
000285346 650_2 $$2MeSH$$aMachado-Joseph Disease: genetics
000285346 650_2 $$2MeSH$$aMachado-Joseph Disease: blood
000285346 650_2 $$2MeSH$$aCohort Studies
000285346 650_2 $$2MeSH$$aNeurofilament Proteins: blood
000285346 650_2 $$2MeSH$$aAged
000285346 650_2 $$2MeSH$$aProspective Studies
000285346 650_2 $$2MeSH$$aMental Status and Dementia Tests
000285346 693__ $$0EXP:(DE-2719)ESMI-20140101$$5EXP:(DE-2719)ESMI-20140101$$eEuropean Spinocerebellar Ataxia Type 3/Machado-Joseph Disease Initiative$$x0
000285346 7001_ $$aGarcia-Moreno, Hector$$b1
000285346 7001_ $$0P:(DE-2719)2811327$$aFaber, Jennifer$$b2$$udzne
000285346 7001_ $$aGonzalez, Carlos$$b3
000285346 7001_ $$0P:(DE-2719)2810795$$aSchöls, Ludger$$b4$$udzne
000285346 7001_ $$ade Vries, Jeroen J$$b5
000285346 7001_ $$aBushara, Khalaf$$b6
000285346 7001_ $$aReetz, Kathrin$$b7
000285346 7001_ $$aOnyike, Chiadi U$$b8
000285346 7001_ $$aJacobi, Heike$$b9
000285346 7001_ $$aErdlenbruch, Friedrich$$b10
000285346 7001_ $$aInfante, Jon$$b11
000285346 7001_ $$aSantana, Magda M$$b12
000285346 7001_ $$aHübener-Schmid, Jeannette$$b13
000285346 7001_ $$ade Almeida, Luís Pereira$$b14
000285346 7001_ $$aLima, Manuela$$b15
000285346 7001_ $$aGiunti, Paola$$b16
000285346 7001_ $$0P:(DE-2719)2810314$$aKlockgether, Thomas$$b17$$udzne
000285346 7001_ $$agroup, ESMI study$$b18$$eCollaboration Author
000285346 7001_ $$avan de Warrenburg, Bart P C$$b19
000285346 773__ $$0PERI:(DE-600)1471408-5$$a10.1016/j.nbd.2026.107301$$gVol. 220, p. 107301 -$$p107301$$tNeurobiology of disease$$v220$$x0969-9961$$y2026
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