Brain MRI shows white matter sparing in Kennedy's disease and slow-progressing lower motor neuron disease

Edoardo G. Spinelli, Federica Agosta, Pilar M. Ferraro, Giorgia Querin, Nilo Riva, Cinzia Bertolin, Ilaria Martinelli, Christian Lunetta, Andrea Fontana, Gianni Sorarù, Massimo Filippi

Research output: Contribution to journalArticle

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Abstract

The extent of central nervous system involvement in Kennedy's disease (KD) relative to other motor neuron disease (MND) phenotypes still needs to be clarified. In this study, we investigated cortical and white matter (WM) MRI alterations in 25 patients with KD, compared with 24 healthy subjects, 25 patients with sporadic amyotrophic lateral sclerosis (ALS), and 35 cases with lower motor neuron-predominant disease (LMND). LMND patients were clinically differentiated into 24 fast and 11 slow progressors. Whole-brain cortical thickness, WM tract-based spatial statistics and corticospinal tract (CST) tractography analyses were performed. No significant difference in terms of cortical thickness was found between groups. ALS patients showed widespread decreased fractional anisotropy and increased mean (MD) and radial diffusivity (radD) in the CST, corpus callosum and fronto-temporal extra-motor tracts, compared with healthy controls and other patient groups. CST tractography showed significant alterations of DT MRI metrics in ALS and LMND-fast patients whereas KD and LMND-slow patients were comparable with healthy controls. Our study demonstrated the absence of WM abnormalities in patients with KD and LMND-slow, in contrast with diffuse WM damage in ALS and focal CST degeneration in LMND-fast, supporting the use of DT MRI measures as powerful tools to differentiate fast- and slow-progressing MND syndromes, including KD.

Original languageEnglish
JournalHuman Brain Mapping
DOIs
Publication statusPublished - Jan 1 2019

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X-Linked Bulbo-Spinal Atrophy
Motor Neuron Disease
Pyramidal Tracts
Brain
Amyotrophic Lateral Sclerosis
White Matter
Corpus Callosum
Anisotropy
Healthy Volunteers
Central Nervous System
Phenotype

Keywords

  • amyotrophic lateral sclerosis
  • corticospinal tract
  • diffusion tensor MRI
  • Kennedy's disease
  • lower motor neuron disease

ASJC Scopus subject areas

  • Anatomy
  • Radiological and Ultrasound Technology
  • Radiology Nuclear Medicine and imaging
  • Neurology
  • Clinical Neurology

Cite this

Brain MRI shows white matter sparing in Kennedy's disease and slow-progressing lower motor neuron disease. / Spinelli, Edoardo G.; Agosta, Federica; Ferraro, Pilar M.; Querin, Giorgia; Riva, Nilo; Bertolin, Cinzia; Martinelli, Ilaria; Lunetta, Christian; Fontana, Andrea; Sorarù, Gianni; Filippi, Massimo.

In: Human Brain Mapping, 01.01.2019.

Research output: Contribution to journalArticle

Spinelli, Edoardo G. ; Agosta, Federica ; Ferraro, Pilar M. ; Querin, Giorgia ; Riva, Nilo ; Bertolin, Cinzia ; Martinelli, Ilaria ; Lunetta, Christian ; Fontana, Andrea ; Sorarù, Gianni ; Filippi, Massimo. / Brain MRI shows white matter sparing in Kennedy's disease and slow-progressing lower motor neuron disease. In: Human Brain Mapping. 2019.
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