Objective: We examined narrative discourse in amyotrophic lateral sclerosis (ALS) to assess the role of executive functioning in support of language and the neuroanatomical basis for such support.Methods: We analyzed a semistructured speech sample in 26 patients with ALS and 19 healthy seniors for narrative discourse features of coherence. Regression analyses related a measure of discourse coherence ("local connectedness") to gray matter atrophy and reduced white matter fractional anisotropy.Results: Patients with ALS were impaired relative to controls on measures of discourse adequacy, including local connectedness and maintenance of the theme. These discourse measures were related to measures of executive functioning but not to motor functioning. Regressions related local connectedness to gray matter atrophy in ventral and dorsal prefrontal regions and to reduced fractional anisotropy in white matter tracts mediating projections between prefrontal regions. Conclusion:Patients with ALS exhibit deficits in their ability to organize narrative discourse.These deficits appear to be related in part to executive limitations. Consistent with the hypothesis that ALS is a multisystem disorder, this deficit is related to disease in prefrontal regions. Amyotrophic lateral sclerosis (ALS) is typically viewed as a motor system disorder, but cognitive impairments in ALS are increasingly recognized.1,2 Language impairments in ALS may result in part from deficits in executive functioning, 3-5 but they have also been shown to be at least in part independent of executive dysfunction.6-8 Most reports of language performance in ALS have focused on the comprehension or production of single words. 1,7,9,10 There are few studies of spontaneous, continuous speech-referred to as "connected speech"-in these patients, despite the importance of connected speech in everyday life. In this study, we elicited a semistructured speech sample in the form of a narrative of sufficient length to allow subjects to demonstrate the full range of their linguistic capabilities. We examined the discourse structure of the narratives to determine whether executive impairments were associated with difficulty in telling the story, and we assessed the contribution of motor weakness to narrative production.MRI studies of ALS have demonstrated gray matter (GM) atrophy and white matter (WM) reduced fractional anisotropy (FA) in prefrontal regions, 11,12 consistent with the claim that ALS is a multisystem disorder. 13,14 To elucidate the neuroanatomical basis of narrative discourse deficits in ALS, we related performance to high-resolution GM and WM structural imaging in a subset of patients. Based on previous fMRI work in healthy adults and nonaphasic patients with