Y Liu1,2, Z Ye3,4, J Hu1, Z Xiao5, F Zhang1, X Yang1, W Chen3,4,6, Y Fu3,4, D Cao7,8. 1. From the Departments of Radiology (Y.L., J.H., F.Z., X.Y., D.C.). 2. Department of Medical Imaging Technology (Y.L.), College of Medical Technology and Engineering. 3. Neurology and Institute of Neurology (Z.Y., W.C., Y.F.). 4. Department of Neurology and Institute of Neurology (Z.Y., W.C., Y.F.). 5. Department of Biomedical Sciences (Z.X.), University of Pennsylvania, Philadelphia, Pennsylvania. 6. Fujian Key Laboratory of Molecular Neurology (W.C.), Fujian Medical University, Fuzhou, China. 7. From the Departments of Radiology (Y.L., J.H., F.Z., X.Y., D.C.) dairongcao@163.com. 8. Key Laboratory of Radiation Biology of Fujian Higher Education Institutions (D.C.), First Affiliated Hospital, Fujian Medical University, Fuzhou, China.
Abstract
BACKGROUND AND PURPOSE: In spastic paraplegia type 5, spinal cord atrophy and white matter signal abnormalities in the brain are the main MR imaging alterations. However, the specific mechanism remains unclear. We explored the microstructural changes occurring in spastic paraplegia type 5 and assessed the relation between MR imaging and clinical data. MATERIALS AND METHODS: Seventeen patients with spastic paraplegia type 5 and 17 healthy controls were scanned with DTI and T1 mapping on a 3T MR imaging scanner. Fractional anisotropy, mean diffusivity, radial diffusivity, axial diffusivity, and T1 values were obtained using Tract-Based Spatial Statistics and the Spinal Cord Toolbox. Neurofilament light and myelin basic protein in the CSF were measured. The differences in MR imaging and biochemical data between patients with spastic paraplegia type 5 and healthy controls were compared using the Student t test. RESULTS: A widespread reduction of fractional anisotropy values and an elevation of mean diffusivity, T1, and radial diffusivity values were found in most cervical, T4, and T5 spinal cords; corona radiata; optic radiations; and internal capsules in spastic paraplegia type 5. A variation in axial diffusivity values was shown only in C2, C6, and the corona radiata but not in the gray matter. The levels of neurofilament light and myelin basic protein were higher in those with spastic paraplegia type 5 than in healthy controls (myelin basic protein, 3507 [SD, 2291] versus 127 [SD, 219] pg/mL; neurofilament light, 617 [SD, 207] versus 265 [SD, 187] pg/mL; P < .001). No correlation was found between the clinical data and MR imaging-derived measures. CONCLUSIONS: Multiparametric MR imaging and biochemical indicators demonstrated that demyelination (mainly) and axonal loss led to the white matter integrity loss without gray matter injury in spastic paraplegia type 5.
BACKGROUND AND PURPOSE: In spastic paraplegia type 5, spinal cord atrophy and white matter signal abnormalities in the brain are the main MR imaging alterations. However, the specific mechanism remains unclear. We explored the microstructural changes occurring in spastic paraplegia type 5 and assessed the relation between MR imaging and clinical data. MATERIALS AND METHODS: Seventeen patients with spastic paraplegia type 5 and 17 healthy controls were scanned with DTI and T1 mapping on a 3T MR imaging scanner. Fractional anisotropy, mean diffusivity, radial diffusivity, axial diffusivity, and T1 values were obtained using Tract-Based Spatial Statistics and the Spinal Cord Toolbox. Neurofilament light and myelin basic protein in the CSF were measured. The differences in MR imaging and biochemical data between patients with spastic paraplegia type 5 and healthy controls were compared using the Student t test. RESULTS: A widespread reduction of fractional anisotropy values and an elevation of mean diffusivity, T1, and radial diffusivity values were found in most cervical, T4, and T5 spinal cords; corona radiata; optic radiations; and internal capsules in spastic paraplegia type 5. A variation in axial diffusivity values was shown only in C2, C6, and the corona radiata but not in the gray matter. The levels of neurofilament light and myelin basic protein were higher in those with spastic paraplegia type 5 than in healthy controls (myelin basic protein, 3507 [SD, 2291] versus 127 [SD, 219] pg/mL; neurofilament light, 617 [SD, 207] versus 265 [SD, 187] pg/mL; P < .001). No correlation was found between the clinical data and MR imaging-derived measures. CONCLUSIONS: Multiparametric MR imaging and biochemical indicators demonstrated that demyelination (mainly) and axonal loss led to the white matter integrity loss without gray matter injury in spastic paraplegia type 5.
Authors: R Schüle; T Holland-Letz; S Klimpe; J Kassubek; T Klopstock; V Mall; S Otto; B Winner; L Schöls Journal: Neurology Date: 2006-08-08 Impact factor: 9.910
Authors: Rebecca Schüle; Sarah Wiethoff; Peter Martus; Kathrin N Karle; Susanne Otto; Stephan Klebe; Sven Klimpe; Constanze Gallenmüller; Delia Kurzwelly; Dorothea Henkel; Florian Rimmele; Henning Stolze; Zacharias Kohl; Jan Kassubek; Thomas Klockgether; Stefan Vielhaber; Christoph Kamm; Thomas Klopstock; Peter Bauer; Stephan Züchner; Inga Liepelt-Scarfone; Ludger Schöls Journal: Ann Neurol Date: 2016-03-11 Impact factor: 10.422