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Type: Artigo de periódico
Title: Widespread neuronal damage and cognitive dysfunction in spinocerebellar ataxia type 3
Author: Lopes, TM
D'Abreu, A
Franca, MC
Yasuda, CL
Betting, LE
Samara, AB
Castellano, G
Somazz, JC
Balthazar, MLF
Lopes-Cendes, I
Cendes, F
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: Spinocerebellar ataxia type 3
Cognitive deficits
Voxel-based morphometry
Diffusion tensor imaging
Magnetic resonance spectroscopy
Country: Alemanha
Editor: Springer Heidelberg
Citation: Journal Of Neurology. Springer Heidelberg, v. 260, n. 9, n. 2370, n. 2379, 2013.
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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