• Masashi Tsujimoto, Jo Senda, Tetsuro Ishihara, Yoshiki Niimi, Yoshinari Kawai, Naoki Atsuta, Hirohisa Watanabe, Fumiaki Tanaka, Shinji Naganawa, and Gen Sobue.
• Department of Neurology, Nagoya University Graduate School of Medicine, Japan.
• J. Neurol. Sci. 2011 Aug 15; 307 (1-2): 34-40.

BackgroundAmyotrophic lateral sclerosis (ALS) is a multisystem disorder with impairment of frontotemporal functions such as cognition and behavior, but the behavioral changes associated with ALS are not well defined.MethodsTwenty-one consecutive patients with sporadic ALS and 21 control subjects participated in the study. The Frontal System Behavior Scale (FrSBe) was used to assess behavioral change. Voxel-based morphometry (VBM) and voxel-based analysis of diffusion tensor images (DTI) were performed to explore the associations of brain degeneration with behavior. All patients were evaluated before the notification of ALS.ResultsFrSBe scores of ALS patients before notification were significantly increased compared to those of control subjects. Moreover, the FrSBe Apathy score of ALS patients significantly changed from pre- to post-illness (P<0.001). The severity of apathy was significantly correlated with atrophy in the prefrontal cortex, especially in the orbitofrontal (P=0.006) and dorsolateral prefrontal (P=0.006) cortices in VBM, and in the right frontal gyrus (P<0.001) in DTI.ConclusionsALS patients exhibited apathy during the early course of the illness, the severity of which was significantly associated with frontal lobe involvement. These findings support the view that a continuum exits between ALS and frontotemporal dementia.Copyright © 2011 Elsevier B.V. All rights reserved.

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