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Cervical Spinal Cord Degeneration in Spinocerebellar Ataxia Type 7

Cervical Spinal Cord Degeneration in Spinocerebellar Ataxia Type 7 Published July 1, 2021 as 10.3174/ajnr.A7202 ORIGINAL RESEARCH SPINE Cervical Spinal Cord Degeneration in Spinocerebellar Ataxia Type 7 C.R. Hernandez-Castillo, R. Diaz, T.J.R. Rezende, I. Adanyeguh, I.H. Harding, F. Mochel, and J. Fernandez-Ruiz ABSTRACT BACKGROUND AND PURPOSE: Spinocerebellar ataxia type 7 is an autosomal dominant neurodegenerative disease caused by a cytosine-adenine-guanine (CAG) repeat expansion. Clinically, spinocerebellar ataxia type 7 is characterized by progressive cerebellar ataxia, pyramidal signs, and macular degeneration. In vivo MR imaging studies have shown extensive gray matter degeneration in the cerebellum and, to a lesser extent, in a range of cortical cerebral areas. The purpose of this study was to evaluate the impact of the disease in the spinal cord and its relationship with the patient's impairment. MATERIALS AND METHODS: Using a semiautomated procedure applied to MR imaging data, we analyzed spinal cord area and ec- centricity in a cohort of 48 patients with spinocerebellar ataxia type 7 and compared them with matched healthy controls. The motor impairment in the patient group was evaluated using the Scale for Assessment and Rating of Ataxia. RESULTS: Our analysis showed a significantly smaller cord area (t ¼ 9.04, P , .001, d ¼ 1.31) and greater eccentricity (t¼–2.25, P http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Neuroradiology American Journal of Neuroradiology

Cervical Spinal Cord Degeneration in Spinocerebellar Ataxia Type 7

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Publisher
American Journal of Neuroradiology
Copyright
© 2021 by American Journal of Neuroradiology
ISSN
0195-6108
eISSN
1936-959X
DOI
10.3174/ajnr.A7202
Publisher site
See Article on Publisher Site

Abstract

Published July 1, 2021 as 10.3174/ajnr.A7202 ORIGINAL RESEARCH SPINE Cervical Spinal Cord Degeneration in Spinocerebellar Ataxia Type 7 C.R. Hernandez-Castillo, R. Diaz, T.J.R. Rezende, I. Adanyeguh, I.H. Harding, F. Mochel, and J. Fernandez-Ruiz ABSTRACT BACKGROUND AND PURPOSE: Spinocerebellar ataxia type 7 is an autosomal dominant neurodegenerative disease caused by a cytosine-adenine-guanine (CAG) repeat expansion. Clinically, spinocerebellar ataxia type 7 is characterized by progressive cerebellar ataxia, pyramidal signs, and macular degeneration. In vivo MR imaging studies have shown extensive gray matter degeneration in the cerebellum and, to a lesser extent, in a range of cortical cerebral areas. The purpose of this study was to evaluate the impact of the disease in the spinal cord and its relationship with the patient's impairment. MATERIALS AND METHODS: Using a semiautomated procedure applied to MR imaging data, we analyzed spinal cord area and ec- centricity in a cohort of 48 patients with spinocerebellar ataxia type 7 and compared them with matched healthy controls. The motor impairment in the patient group was evaluated using the Scale for Assessment and Rating of Ataxia. RESULTS: Our analysis showed a significantly smaller cord area (t ¼ 9.04, P , .001, d ¼ 1.31) and greater eccentricity (t¼–2.25, P

Journal

American Journal of NeuroradiologyAmerican Journal of Neuroradiology

Published: Jul 1, 2021

References