Behavioral changes in early ALS correlate with voxel-based morphometry and diffusion tensor imaging

J Neurol Sci. 2011 Aug 15;307(1-2):34-40. doi: 10.1016/j.jns.2011.05.025. Epub 2011 Jun 8.


Background: Amyotrophic 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.

Methods: Twenty-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.

Results: FrSBe 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.

Conclusions: ALS 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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Amyotrophic Lateral Sclerosis / epidemiology
  • Amyotrophic Lateral Sclerosis / pathology*
  • Amyotrophic Lateral Sclerosis / psychology*
  • Atrophy
  • Comorbidity
  • Diffusion Tensor Imaging / methods
  • Disability Evaluation
  • Disease Progression
  • Female
  • Frontal Lobe / pathology*
  • Frontotemporal Dementia / epidemiology
  • Frontotemporal Dementia / pathology
  • Humans
  • Image Processing, Computer-Assisted / methods
  • Male
  • Mental Disorders / diagnosis
  • Mental Disorders / pathology*
  • Mental Disorders / psychology
  • Middle Aged
  • Predictive Value of Tests
  • Severity of Illness Index