Next Article in Journal / Special Issue
High Serum Caspase-Cleaved Cytokeratin-18 Levels and Mortality of Traumatic Brain Injury Patients
Previous Article in Journal
Therapeutic Plasma Exchange in Multiple Sclerosis and Autoimmune Encephalitis: A Comparative Study of Indication, Efficacy, and Safety
Previous Article in Special Issue
Tissue Plasminogen Activator and MRI Signs of Cerebral Small Vessel Disease
Open AccessArticle

Ascending Axonal Degeneration of the Corticospinal Tract in Pure Hereditary Spastic Paraplegia: A Cross-Sectional DTI Study

1
Departments of Molecular Neurology, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-Nuremberg, Schwabachanlage 6, 91054 Erlangen, Germany
2
Neuroradiology, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-Nuremberg, Schwabachanlage 6, 91054 Erlangen, Germany
*
Author to whom correspondence should be addressed.
Brain Sci. 2019, 9(10), 268; https://doi.org/10.3390/brainsci9100268
Received: 5 September 2019 / Revised: 3 October 2019 / Accepted: 5 October 2019 / Published: 9 October 2019
(This article belongs to the Collection Collection on Clinical Neuroscience)
Objective: To identify structural white matter alterations in patients with pure hereditary spastic paraplegia (HSP) using high angular resolution diffusion tensor imaging (DTI). Methods: We examined 37 individuals with high resolution DTI, 20 patients with pure forms of hereditary spastic paraplegia and 17 age and gender matched healthy controls. DTI was performed using a 3 T clinical scanner with whole brain tract-based spatial statistical (TBSS) analysis of the obtained fractional anisotropy (FA) data as well as a region-of-interest (ROI)-based analysis of affected tracts including the cervical spinal cord. We further conducted correlation analyses between DTI data and clinical characteristics. Results: TBSS analysis in HSP patients showed significantly decreased fractional anisotropy of the corpus callosum and the corticospinal tract compared to healthy controls. ROI-based analysis confirmed significantly lower FA in HSP compared to controls in the internal capsule (0.77 vs. 0.80, p = 0.048), the corpus callosum (0.84 vs. 0.87, p = 0.048) and the cervical spinal cord (0.72 vs. 0.79, p = 0.003). FA values of the cervical spinal cord significantly correlated with disease duration. Conclusion: DTI metrics of the corticospinal tract from the internal capsule to the cervical spine suggest microstructural damage and axonal degeneration of motor neurons. The CST at the level of the cervical spinal cord is thereby more severely affected than the intracranial part of the CST, suggesting an ascending axonal degeneration of the CST. Since there is a significant correlation with disease duration, FA may serve as a future progression marker for assessment of the disease course in HSP. View Full-Text
Keywords: hereditary spastic paraplegia; diffusion tensor imaging; fractional anisotropy; tract-based spatial statistics (TBSS); corticospinal tract hereditary spastic paraplegia; diffusion tensor imaging; fractional anisotropy; tract-based spatial statistics (TBSS); corticospinal tract
Show Figures

Graphical abstract

MDPI and ACS Style

List, J.; Kohl, Z.; Winkler, J.; Marxreiter, F.; Doerfler, A.; Schmidt, M.A. Ascending Axonal Degeneration of the Corticospinal Tract in Pure Hereditary Spastic Paraplegia: A Cross-Sectional DTI Study. Brain Sci. 2019, 9, 268.

Show more citation formats Show less citations formats
Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Article Access Map by Country/Region

1
Back to TopTop