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. 2013 Dec 5;8(12):e82017.
doi: 10.1371/journal.pone.0082017. eCollection 2013.

Extramotor damage is associated with cognition in primary lateral sclerosis patients

Elisa Canu  1 , Federica Agosta  1 , Sebastiano Galantucci  1 , Adriano Chiò  2 , Nilo Riva  3 , Vincenzo Silani  4 , Andrea Falini  5 , Giancarlo Comi  3 , Massimo Filippi  6
Affiliations

Extramotor damage is associated with cognition in primary lateral sclerosis patients

Elisa Canu et al. PLoS One. .

Abstract

Objectives: This is a cross-sectional study aimed at investigating cognitive performances in patients with primary lateral sclerosis (PLS) and using diffusion tensor (DT) magnetic resonance imaging (MRI) to determine the topographical distribution of microstructural white matter (WM) damage in patients with or without cognitive deficits.

Methods: DT MRI scans were obtained from 21 PLS patients and 35 age- and sex-matched healthy controls. All PLS patients underwent a comprehensive neuropsychological battery. Tract-based-spatial-statistics (TBSS) was used to perform a whole-brain voxel-wise analysis of fractional anisotropy (FA), axial, radial (radD) and mean diffusivity (MD).

Results: Ten PLS patients had abnormal scores in at least one neuropsychological test (PLS with cognitive deficits, PLS-cd). Compared with healthy controls and cognitively unimpaired PLS patients (PLS-cu), PLS-cd cases showed decreased FA and increased MD and radD in the corticospinal tract (CST), corpus callosum, brainstem, anterior limb of internal capsule, superior and inferior longitudinal fasciculi, fornix, thalamic radiations, and parietal lobes, bilaterally. Compared with healthy controls, PLS-cd patients showed further decreased FA and increased radD in the cerebellar WM, bilaterally. Compared with controls, PLS-cu patients showed decreased FA in the mid-body of corpus callosum. In PLS, executive and language test scores correlated with WM damage.

Conclusions: This is the first study evaluating the relationship between cognitive performance and WM tract damage in PLS patients. PLS can be associated with a multi-domain cognitive impairment. WM damage to interhemispheric, limbic and major associative WM tracts seem to be the structural correlate of cognitive abnormalities in these patients.

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Conflict of interest statement

Competing Interests: The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. TBSS results: areas of decreased fractional anisotropy (FA, red-yellow) in PLS patients without cognitive impairment (PLS-cu) vs. healthy controls are displayed on a FA template in the Montreal Neurological Institute space.
FWE =  family wise error.
Figure 2
Figure 2. TBSS results: areas of decreased fractional anisotropy (FA, red-yellow), and increased mean (MD, green) and radial diffusivity (radD, blue) in PLS patients with cognitive deficits (PLS-cd) vs. healthy controls are displayed on a FA template in the Montreal Neurological Institute space.
FWE =  family wise error.
Figure 3
Figure 3. TBSS results: areas of decreased fractional anisotropy (FA, red-yellow), and increased mean (MD, green) and radial diffusivity (radD, blue) in PLS patients with cognitive deficits (PLS-cd) vs. PLS patients without cognitive impairment (PLS-cu) are displayed on a FA template in the Montreal Neurological Institute space.
FWE =  family wise error.
Figure 4
Figure 4. TBSS results: positive relationships between fractional anisotropy (FA, red-yellow) and patient performances at the semantic fluency test and at the “Batteria per l'Analisi del Deficit Afasico” action-naming test are displayed on a FA template in the Montreal Neurological Institute space.
FWE =  family wise error.
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MeSH terms

Grants and funding

This work was supported by the Italian Ministry of Health, grant number #RF-2010-2313220; and by the European Community's Health Seventh Framework Programme (FP7/2007–2013), grant number #259867. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.