Brain MR diffusion tensor imaging in Kennedy’s disease

Author:

Garaci Francesco12,Toschi Nicola23,Lanzafame Simona2,Marfia Girolama A4,Marziali Simone1,Meschini Alessandro1,Di Giuliano Francesca1,Simonetti Giovanni12,Guerrisi Maria2,Massa Roberto4,Floris Roberto12

Affiliation:

1. Department of Diagnostic Imaging, Molecular Imaging, Interventional Radiology and Radiotherapy, University Hospital Tor Vergata, Italy

2. Department of Biomedicine and Prevention, Faculty of Medicine, University of Rome Tor Vergata, Italy

3. Department of Radiology, Athinoula A. Martinos Center for Biomedical Imaging, USA and Harvard Medical School, USA

4. Department of Systems Medicine, Section Neurology, University of Rome Tor Vergata, Italy

Abstract

Introduction Kennedy’s disease (KD) is a progressive degenerative disorder affecting lower motor neurons. We investigated the correlation between disease severity and whole brain white matter microstructure, including upper motor neuron tracts, by using diffusion-tensor imaging (DTI) in eight patients with KD in whom disease severity was evaluated using the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS). Methods From DTI acquisitions we obtained maps of fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (L1) and radial diffusivities (L2, L3). We then employed tract-based spatial statistics (TBSS) to investigate within-patient correlations of DTI invariants with ALSFRS and disease duration (DD). Results We found a significant correlation between low ALSFRS and 1) low FA values in association commissural and projection fibers, and 2) high L3 values in commissural tracts and fronto-parietal white matter. Additionally, we found a significant association between longer DD and 1) low FA in the genu and body of corpus callosum, association fibers and midbrain and 2) high L1 in projection and association tracts. Conclusions The associations between clinical variables and white matter microstructural changes in areas thought to be spared by the disease process support the hypothesis of a multisystem involvement in the complex pathogenic mechanisms responsible for the clinical disability of these patients.

Publisher

SAGE Publications

Subject

Neurology (clinical),Radiology, Nuclear Medicine and imaging,General Medicine

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