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Updated: Apr 27, 2026
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A Protocol for Comprehensive Assessment of Bulbar Dysfunction in Amyotrophic Lateral Sclerosis ALS
Published on: February 21, 2011
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Narrative discourse deficits in amyotrophic lateral sclerosis
Sharon Ash1, Anna Menaged2, Christopher Olm2
1From the Department of Neurology and the Penn Frontotemporal Degeneration Center (S.A., A.M., C.O., C.T.M., A.B., D.J.I., L.M., L.E., M.G.), and Center for Neurodegenerative Disease Research (D.J.I.), Perelman School of Medicine, University of Pennsylvania, Philadelphia. ash@mail.med.upenn.edu.
Neurology
|July 4, 2014
Summary
Patients with amyotrophic lateral sclerosis (ALS) show impaired narrative discourse due to executive functioning deficits. This language impairment is linked to prefrontal brain atrophy and white matter changes in ALS.
Area of Science:
- Neuroscience
- Linguistics
- Neurology
Background:
- Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease.
- Executive functions are crucial for complex cognitive tasks, including language production.
- The relationship between executive function, language, and neuroanatomy in ALS is not fully understood.
Purpose of the Study:
- To investigate narrative discourse abilities in individuals with ALS.
- To assess the role of executive functioning in supporting language in ALS.
- To identify the neuroanatomical correlates of discourse deficits in ALS.
Main Methods:
- Analysis of narrative discourse coherence in a semistructured speech sample.
- Comparison of 26 patients with ALS and 19 healthy controls.
- Regression analyses correlating discourse measures with gray matter atrophy and white matter fractional anisotropy.
Main Results:
- Patients with ALS demonstrated impairments in discourse adequacy, including local connectedness and thematic maintenance.
- Discourse deficits correlated with executive functioning measures, not motor function.
- Local connectedness was associated with prefrontal gray matter atrophy and reduced white matter integrity.
Conclusions:
- Individuals with ALS exhibit difficulties in organizing narrative discourse.
- These discourse deficits are partly attributable to executive limitations.
- The findings support ALS as a multisystem disorder affecting prefrontal brain regions and language.
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