Corticospinal tract involvement in spinocerebellar ataxia type 3: a diffusion tensor imaging study
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FUNCTIONAL NEURORADIOLOGY
Corticospinal tract involvement in spinocerebellar ataxia type 3: a diffusion tensor imaging study Bruno Shigueo Yonekura Inada 1,2 & Thiago Junqueira Ribeiro Rezende 3 & Fernando Vieira Pereira 1 & Lucas Ávila Lessa Garcia 4 & Antônio José da Rocha 5 & Pedro Braga Neto 6,7 & Orlando Graziani Povoas Barsottini 1 & Marcondes Cavalcante França Jr 3 & José Luiz Pedroso 1 Received: 18 June 2020 / Accepted: 16 August 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Purpose The aim of this study was to evaluate the integrity of the corticospinal tracts (CST) in patients with SCA3 and age- and gender-matched healthy control subjects using diffusion tensor imaging (DTI). We also looked at the clinical correlates of such diffusivity abnormalities. Methods We assessed 2 cohorts from different Brazilian centers: cohort 1 (n = 29) scanned in a 1.5 T magnet and cohort 2 (n = 91) scanned in a 3.0 T magnet. We used Pearson’s coefficients to assess the correlation of CST DTI parameters and ataxia severity (expressed by SARA scores). Results Two different results were obtained. Cohort 1 showed no significant between-group differences in DTI parameters. Cohort 2 showed significant between-group differences in the FA values in the bilateral precentral gyri (p < 0.001), bilateral superior corona radiata (p < 0.001), bilateral posterior limb of the internal capsule (p < 0.001), bilateral cerebral peduncle (p < 0.001), and bilateral basis pontis (p < 0.001). There was moderate correlation between CST diffusivity parameters and SARA scores in cohort 2 (Pearson correlation coefficient: 0.40–0.59). Conclusion DTI particularly at 3 T is able to uncover and quantify CST damage in SCA3. Moreover, CST microstructural damage may contribute with ataxia severity in the disease. Keywords Spinocerebellar ataxia type 3 . Machado-Joseph disease . Cerebellar ataxia . Corticospinal tract . Diffusion tensor imaging . Retrograde degeneration
Introduction Spinocerebellar ataxia type 3 (SCA3) or Machado-Joseph disease (MJD) is the most common autosomal dominant spinocerebellar ataxia (SCA) worldwide and is caused by a CAG trinucleotide expansion located in exon 10 of ATXN3 [1]. This is a neurodegenerative disorder that typically begins
* Bruno Shigueo Yonekura Inada [email protected] 1
Division of General Neurology and Ataxia Unit, Department of Neurology, Federal University of São Paulo (UNIFESP), São Paulo, Brazil
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Department of Neuroradiology, Hospital Beneficência Portuguesa, São Paulo, Brazil
3
Department of Neurology, State University of Campinas (UNICAMP), São Paulo, Brazil
in the 3rd or 4th decade of life. The clinical spectrum of SCA3 includes variable degrees of cerebellar ataxia, ophthalmoplegia, nystagmus, pyramidal and extra-pyramidal signs, and also non-motor symptoms [2–5]. Several clinical, imaging, and pathological studies in SCA3 have demonstrated widespread neurodegeneration, with involvement of the brainstem, basal ganglia, spinal cord, peripheral nerves,
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Departmen
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