Jennifer Faber1,2, Ilaria Giordano1,2, Xueyan Jiang1, Christine Kindler1,2, Annika Spottke1,2, Julio Acosta-Cabronero3, Peter J Nestor4,5, Judith Machts6,7, Emrah Düzel6,7, Stefan Vielhaber6,7, Oliver Speck6,8,9, Ales Dudesek10, Christoph Kamm10, Lukas Scheef1,11, Thomas Klockgether1,2. 1. Clinical Research, German Center for Neurodegenerative Diseases, Bonn, Germany. 2. Department of Neurology, University Hospital Bonn, Germany. 3. Tenoke Ltd., Cambridge, United Kingdom. 4. Queensland Brain Institute, University of Queensland, Brisbane, Australia. 5. Neuroscience and Cognitive Health Program, Mater Hospital, South Brisbane, Australia. 6. German Center for Neurodegenerative Diseases, Magdeburg, Germany. 7. Department of Neurology, Otto-von-Guericke University, Magdeburg, Germany. 8. Department of Biomedical Magnetic Resonance, Faculty for Natural Sciences, Otto-von-Guericke University, Magdeburg, Germany. 9. Center for Behavioral Brain Sciences, Magdeburg, Germany. 10. Department of Neurology, University of Rostock, Rostock, Germany. 11. Department of Radiology, University of Bonn, Bonn, Germany.
Abstract
BACKGROUND: Sporadic degenerative ataxia patients fall into 2 major groups: multiple system atrophy with predominant cerebellar ataxia (MSA-C) and sporadic adult-onset ataxia (SAOA). Both groups have cerebellar volume loss, but little is known about the differential involvement of gray and white matter in MSA-C when compared with SAOA. OBJECTIVES: The objective of this study was to identify structural differences of brain gray and white matter between both patient groups. METHODS: We used magnetic resonance imaging to acquire T1-weighted images and diffusion tensor images from 12 MSA-C patients, 31 SAOA patients, and 55 healthy controls. Magnetic resonance imaging data were analyzed with voxel-based-morphometry, tract-based spatial statistics, and tractography-based regional diffusion tensor images analysis. RESULTS: Whole-brain and cerebellar-focused voxel-based-morphometry analysis showed gray matter volume loss in both patient groups when compared with healthy controls, specifically in the cerebellar areas subserving sensorimotor functions. When compared with controls, the SAOA and MSA-C patients showed white matter loss in the cerebellum, whereas brainstem white matter was reduced only in the MSA-C patients. The tract-based spatial statistics revealed reduced fractional anisotropy within the pons and cerebellum in the MSA-C patients both in comparison with the SAOA patients and healthy controls. In addition, tractography-based regional analysis showed reduced fractional anisotropy along the corticospinal tracts in MSA-C, but not SAOA. CONCLUSION: Although in our cohort extent and distribution of gray and white matter loss were similar between the MSA-C and SAOA patients, magnetic resonance imaging data showed prominent microstructural white matter involvement in the MSA-C patients that was not present in the SAOA patients. Our findings highlight the significance of microstructural white matter changes in the differentiation between both conditions.
BACKGROUND: Sporadic degenerative ataxiapatients fall into 2 major groups: multiple system atrophy with predominant cerebellar ataxia (MSA-C) and sporadic adult-onset ataxia (SAOA). Both groups have cerebellar volume loss, but little is known about the differential involvement of gray and white matter in MSA-C when compared with SAOA. OBJECTIVES: The objective of this study was to identify structural differences of brain gray and white matter between both patient groups. METHODS: We used magnetic resonance imaging to acquire T1-weighted images and diffusion tensor images from 12 MSA-C patients, 31 SAOA patients, and 55 healthy controls. Magnetic resonance imaging data were analyzed with voxel-based-morphometry, tract-based spatial statistics, and tractography-based regional diffusion tensor images analysis. RESULTS: Whole-brain and cerebellar-focused voxel-based-morphometry analysis showed gray matter volume loss in both patient groups when compared with healthy controls, specifically in the cerebellar areas subserving sensorimotor functions. When compared with controls, the SAOA and MSA-C patients showed white matter loss in the cerebellum, whereas brainstem white matter was reduced only in the MSA-C patients. The tract-based spatial statistics revealed reduced fractional anisotropy within the pons and cerebellum in the MSA-C patients both in comparison with the SAOA patients and healthy controls. In addition, tractography-based regional analysis showed reduced fractional anisotropy along the corticospinal tracts in MSA-C, but not SAOA. CONCLUSION: Although in our cohort extent and distribution of gray and white matter loss were similar between the MSA-C and SAOA patients, magnetic resonance imaging data showed prominent microstructural white matter involvement in the MSA-C patients that was not present in the SAOA patients. Our findings highlight the significance of microstructural white matter changes in the differentiation between both conditions.
Authors: S Ponticorvo; R Manara; M C Russillo; R Erro; M Picillo; G Di Salle; F Di Salle; P Barone; F Esposito; M T Pellecchia Journal: Sci Rep Date: 2021-11-04 Impact factor: 4.379
Authors: Leslie J Roberts; Michael McVeigh; Linda Seiderer; Ian H Harding; Louise A Corben; Martin Delatycki; David J Szmulewicz Journal: Neurol Genet Date: 2022-09-28