Alex Tiburtino Meira1, Walter Oleschko Arruda2, Sergio Eiji Ono3, Gustavo Leite Franklin2, Arnolfo de Carvalho Neto4, Salmo Raskin5, Tetsuo Ashizawa6, Carlos Henrique F Camargo7, Hélio A G Teive8. 1. Movement Disorders Unit, Neurology Service, Department of Internal Medicine, Hospital de Clínicas, Federal University of Paraná, Curitiba, Paraná, Brazil. Electronic address: alex.meira@maisunifacisa.com.br. 2. Movement Disorders Unit, Neurology Service, Department of Internal Medicine, Hospital de Clínicas, Federal University of Paraná, Curitiba, Paraná, Brazil. 3. DAPI, Advanced Diagnostic Imaging Center, Curitiba, Paraná, Brazil. 4. DAPI, Advanced Diagnostic Imaging Center, Curitiba, Paraná, Brazil; Neurological Disorders Unit, Postgraduate Program in Internal Medicine, Hospital de Clínicas, Federal University of Paraná, Curitiba, Paraná, Brazil. 5. Genetika Counseling Center and Laboratory, Curitiba, Paraná, Brazil. 6. Program of Neuroscience, Houston Methodist Research Institute, Houston, TX, USA. 7. Neurological Disorders Unit, Postgraduate Program in Internal Medicine, Hospital de Clínicas, Federal University of Paraná, Curitiba, Paraná, Brazil. 8. Movement Disorders Unit, Neurology Service, Department of Internal Medicine, Hospital de Clínicas, Federal University of Paraná, Curitiba, Paraná, Brazil; Neurological Disorders Unit, Postgraduate Program in Internal Medicine, Hospital de Clínicas, Federal University of Paraná, Curitiba, Paraná, Brazil.
Abstract
INTRODUCTION: There is a dearth of studies of spinocerebellar ataxias (SCAs) and diffusion tensor magnetic resonance imaging (DTI). OBJECTIVE: To analyze changes observed in DTI parameters and correlate these to clinical findings in SCA3 and SCA10 patients. METHODS: SCA3 (n = 19) and SCA10 (n = 18) patients were compared with a similar number of controls and assessed clinically and with the scale for the assessment and rating of ataxia (SARA) before undergoing the same MRI protocol. TRACULA (TRActs Constrained by UnderLying Anatomy) software was used to analyze the DTI metrics FA, AD, RD and MD. RESULTS: More white matter fiber tracts with changes in diffusivity were found in SCA3 patients than in SCA10 patients. There was a reduction in AD in altered fiber tracts in SCA3 and a greater increase in RD in SCA10. In the SCA3 patients, FA was reduced in the corticospinal tract (CST) and inferior longitudinal fasciculus (ILF), but this was not observed in the SCA10 patients. SARA score was correlated with DTI findings in SCA3 but not in SCA10. CONCLUSION: Changes were observed in DTI for both SCA3 and SCA10 but were more widespread in SCA3. Our finding of myelin-sheath changes in SCA10 and secondary axonal changes in SCA3 may reflect the more rapid, aggressive clinical course of SCA3.
INTRODUCTION: There is a dearth of studies of spinocerebellar ataxias (SCAs) and diffusion tensor magnetic resonance imaging (DTI). OBJECTIVE: To analyze changes observed in DTI parameters and correlate these to clinical findings in SCA3 and SCA10patients. METHODS:SCA3 (n = 19) and SCA10 (n = 18) patients were compared with a similar number of controls and assessed clinically and with the scale for the assessment and rating of ataxia (SARA) before undergoing the same MRI protocol. TRACULA (TRActs Constrained by UnderLying Anatomy) software was used to analyze the DTI metrics FA, AD, RD and MD. RESULTS: More white matter fiber tracts with changes in diffusivity were found in SCA3patients than in SCA10patients. There was a reduction in AD in altered fiber tracts in SCA3 and a greater increase in RD in SCA10. In the SCA3patients, FA was reduced in the corticospinal tract (CST) and inferior longitudinal fasciculus (ILF), but this was not observed in the SCA10patients. SARA score was correlated with DTI findings in SCA3 but not in SCA10. CONCLUSION: Changes were observed in DTI for both SCA3 and SCA10 but were more widespread in SCA3. Our finding of myelin-sheath changes in SCA10 and secondary axonal changes in SCA3 may reflect the more rapid, aggressive clinical course of SCA3.
Authors: Sarah M Brooker; Chandrakanth Reddy Edamakanti; Sara M Akasha; Sheng-Han Kuo; Puneet Opal Journal: Ann Clin Transl Neurol Date: 2021-05-21 Impact factor: 4.511
Authors: Yuka Koike; Karen R Jansen-West; Rana Hanna Al-Shaikh; Yari Carlomagno; Yuping Song; Judith A Dunmore; Mark S LeDoux; Joseph H Friedman; Ashley B Pena; Ryan J Uitti; Jacek Zaremba; Jay A van Gerpen; Ronald F Pfeiffer; Venka Veerappan; Ikuko Aiba; Rina Hashimoto; Samuel S Giles; Jaimin S Shah; Philip W Tipton; Josephine F Huang; Klaas J Wierenga; Jan Aasly; John D Fryer; Leonard Petrucelli; Zbigniew K Wszolek; Mercedes Prudencio Journal: Parkinsonism Relat Disord Date: 2021-07-17 Impact factor: 4.891