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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.

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Published In

Article first published online: May 11, 2015
Issue published: April 2015

Keywords

  1. Kennedy’s disease
  2. fractional anisotropy
  3. mean diffusivity
  4. DTI
  5. TBSS
  6. ALSFRS
  7. disease duration

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PubMed: 25963157

Authors

Affiliations

Francesco Garaci
Department of Diagnostic Imaging, Molecular Imaging, Interventional Radiology and Radiotherapy, University Hospital Tor Vergata, Italy
Department of Biomedicine and Prevention, Faculty of Medicine, University of Rome Tor Vergata, Italy
Nicola Toschi
Department of Biomedicine and Prevention, Faculty of Medicine, University of Rome Tor Vergata, Italy
Department of Radiology, Athinoula A. Martinos Center for Biomedical Imaging, USA and Harvard Medical School, USA
Simona Lanzafame
Department of Biomedicine and Prevention, Faculty of Medicine, University of Rome Tor Vergata, Italy
Girolama A Marfia
Department of Systems Medicine, Section Neurology, University of Rome Tor Vergata, Italy
Simone Marziali
Department of Diagnostic Imaging, Molecular Imaging, Interventional Radiology and Radiotherapy, University Hospital Tor Vergata, Italy
Alessandro Meschini
Department of Diagnostic Imaging, Molecular Imaging, Interventional Radiology and Radiotherapy, University Hospital Tor Vergata, Italy
Francesca Di Giuliano
Department of Diagnostic Imaging, Molecular Imaging, Interventional Radiology and Radiotherapy, University Hospital Tor Vergata, Italy
Giovanni Simonetti
Department of Diagnostic Imaging, Molecular Imaging, Interventional Radiology and Radiotherapy, University Hospital Tor Vergata, Italy
Department of Biomedicine and Prevention, Faculty of Medicine, University of Rome Tor Vergata, Italy
Maria Guerrisi
Department of Biomedicine and Prevention, Faculty of Medicine, University of Rome Tor Vergata, Italy
Roberto Massa
Department of Systems Medicine, Section Neurology, University of Rome Tor Vergata, Italy
Roberto Floris
Department of Diagnostic Imaging, Molecular Imaging, Interventional Radiology and Radiotherapy, University Hospital Tor Vergata, Italy
Department of Biomedicine and Prevention, Faculty of Medicine, University of Rome Tor Vergata, Italy

Notes

Nicola Toschi, Department of Biomedicine and Prevention, University of Rome Tor Vergata, Via Montpellier 1, 00133 Rome (Italy). Email: [email protected]

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