{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,15]],"date-time":"2026-04-15T17:15:08Z","timestamp":1776273308411,"version":"3.50.1"},"reference-count":61,"publisher":"MDPI AG","issue":"16","license":[{"start":{"date-parts":[[2024,8,19]],"date-time":"2024-08-19T00:00:00Z","timestamp":1724025600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background\/Objectives: Cognitive impairment in spinocerebellar ataxia patients has been reported since the early-disease stage. We aimed to assess cognitive differences in SCA1 and SCA2 patients. Methods: We performed neuropsychological (NPS) and neurophysiological (auditory event-related potentials, aERPs) assessments in 16 SCA1 and 18 SCA2 consecutive patients. Furthermore, clinical information (age at onset, disease duration, motor disability) was collected. Results: NPS tests yielded scores in the normal range in both groups but with lower scores in the Frontal Assessment Battery (p &lt; 0.05) and Visual Analogue Test for Anosognosia for motor impairment (p &lt; 0.05) in SCA1, and the Trail Making Test (p &lt; 0.01), Raven\u2019s progressive matrices (p &lt; 0.01), Stroop (p &lt; 0.05), and emotion attribution tests (p &lt; 0.05) in SCA2. aERPs showed lower N100 amplitude (p &lt; 0.01) and prolonged N200 latency (p &lt; 0.01) in SCA1 compared with SCA2. Clinically, SCA2 had more severe motor disability than SCA1 in the Assessment and Rating of Ataxia Scale. Conclusions: SCA2 showed more significant difficulties in attentional, visuospatial, and emotional function, and greater motor impairment. In contrast, SCA1 showed less cognitive flexibility\/phasic ability, probably affected by a more severe degree of dysarthria. The same group revealed less neural activity during nonconscious attentional processing (N100-N200 data), suggesting greater involvement of sensory pathways in discriminating auditory stimuli. NFS did not correlate with NPS findings, implying an independent relationship. However, the specific role of the cerebellum and cerebellar symptoms in NPS test results deserves more focus.<\/jats:p>","DOI":"10.3390\/jcm13164880","type":"journal-article","created":{"date-parts":[[2024,8,19]],"date-time":"2024-08-19T06:41:31Z","timestamp":1724049691000},"page":"4880","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Cognition in Patients with Spinocerebellar Ataxia 1 (SCA1) and 2 (SCA2): A Neurophysiological and Neuropsychological Approach"],"prefix":"10.3390","volume":"13","author":[{"given":"Fabiana","family":"Colucci","sequence":"first","affiliation":[{"name":"Clinical Neurology Unit, Department of Neuroscience and Rehabilitation, University of Ferrara, 44124 Ferrara, Italy"},{"name":"Parkinson and Movement Disorders Unit, Department of Clinical Neurosciences, Fondazione IRCCS, Istituto Neurologico Carlo Besta, 20133 Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sara","family":"Stefanelli","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Neuroscience, Azienda Ospedaliero-Universitaria S. Anna, 44124 Ferrara, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0218-5251","authenticated-orcid":false,"given":"Elena","family":"Contaldi","sequence":"additional","affiliation":[{"name":"Centro Parkinson e Parkinsonismi, ASST Gaetano Pini-CTO, 20122 Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5248-791X","authenticated-orcid":false,"given":"Andrea","family":"Gozzi","sequence":"additional","affiliation":[{"name":"Clinical Neurology Unit, Department of Neuroscience and Rehabilitation, University of Ferrara, 44124 Ferrara, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alessia","family":"Marchetti","sequence":"additional","affiliation":[{"name":"Clinical Neurology Unit, Department of Neuroscience and Rehabilitation, University of Ferrara, 44124 Ferrara, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maura","family":"Pugliatti","sequence":"additional","affiliation":[{"name":"Clinical Neurology Unit, Department of Neuroscience and Rehabilitation, University of Ferrara, 44124 Ferrara, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6192-9137","authenticated-orcid":false,"given":"Michele","family":"Laudisi","sequence":"additional","affiliation":[{"name":"Clinical Neurology Unit, Department of Neuroscience and Rehabilitation, University of Ferrara, 44124 Ferrara, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0710-5334","authenticated-orcid":false,"given":"Pietro","family":"Antenucci","sequence":"additional","affiliation":[{"name":"Clinical Neurology Unit, Department of Neuroscience and Rehabilitation, University of Ferrara, 44124 Ferrara, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jay Guido","family":"Capone","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Neuroscience, Azienda Ospedaliero-Universitaria S. Anna, 44124 Ferrara, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniela","family":"Gragnaniello","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Neuroscience, Azienda Ospedaliero-Universitaria S. Anna, 44124 Ferrara, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6434-5433","authenticated-orcid":false,"given":"Mariachiara","family":"Sensi","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Neuroscience, Azienda Ospedaliero-Universitaria S. 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