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Type: Artigo de periódico
Title: Widespread Neuronal Damage And Cognitive Dysfunction In Spinocerebellar Ataxia Type 3.
Author: Lopes, Tátila Martins
D'Abreu, Anelyssa
França, Marcondes Cavalcante
Yasuda, Clarissa Lin
Betting, Luiz Eduardo
Samara, Adriana Bastos
Castellano, Gabriela
Somazz, Júlio César
Balthazar, Marcio Luiz Figueredo
Lopes-Cendes, Iscia
Cendes, Fernando
Abstract: Previous studies demonstrated cognitive impairments in spinocerebellar ataxia type 3 (SCA3/MJD); however, there is no consensus about the cognitive domains affected and the correlation with structural brain abnormalities. We investigated the neuropsychological profile and 3T-MRI findings, including high-resolution T1-images, diffusion tensor imaging and magnetic resonance spectroscopy of 32 patients with SCA3/MJD and 32 age-, gender- and educational level-matched healthy controls. We reviewed patients' clinical history and CAG repeat length, and performed assessment and rating of ataxia (SARA)-Brazilian version and the neuropsychiatric inventory. Patients presented worse performance in episodic and working memory and Beck inventories (depression and anxiety). SCA3/MJD patients had a reduction of gray matter volume (GM) in the cerebellum, putamen, cingulum, precentral and parietal lobe. A positive correlation was identified between the cognitive findings and GM of temporal, frontal, parietal, culmen and insula. We observed positive correlation between the brainstem's fractional anisotropy and digit span-forward. The following cerebellar metabolite groups (measured relative to creatine) were reduced in patients: N-acetyl-aspartate (NAA), NAA + N-acetyl-aspartate-glutamate and glutamate + glutamine (Glx). We found a positive correlation between Corsi's block-tapping task forward with Glx; semantic verbal fluency with phosphorylcholine and glycerophosphorylcholine; digits span-forward with NAA. The cognitive impairments in SCA3/MJD are associated not only with cerebellar and brainstem abnormalities, but also with neuroimaging evidence of diffuse neuronal and axonal dysfunction, particularly in temporal, frontal, parietal and insular areas.
Subject: Brain
Cognition Disorders
Diffusion Tensor Imaging
Image Interpretation, Computer-assisted
Machado-joseph Disease
Middle Aged
Neuropsychological Tests
Citation: Journal Of Neurology. v. 260, n. 9, p. 2370-9, 2013-Sep.
Rights: fechado
Identifier DOI: 10.1007/s00415-013-6998-8
Date Issue: 2013
Appears in Collections:Unicamp - Artigos e Outros Documentos

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