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Syntactic comprehension deficits across the FTD-ALS continuum.

Jody Kamminga1, Felicity V C Leslie2, Sharpley Hsieh3

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Summary

Syntactic comprehension deficits are common in frontotemporal dementia-amyotrophic lateral sclerosis (FTD-ALS) and progressive nonfluent aphasia (PNFA), linked to brain atrophy. Some amyotrophic lateral sclerosis (ALS) patients also show these language impairments.

Keywords:
Cognitive impairmentFTD-ALS continuumSyntactic comprehension deficits

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Area of Science:

  • Neurology
  • Linguistics
  • Neuroimaging

Background:

  • Frontotemporal dementia-amyotrophic lateral sclerosis (FTD-ALS) spectrum disorders can affect cognition and language.
  • Syntactic comprehension deficits are a potential but understudied feature in these neurodegenerative diseases.

Purpose of the Study:

  • To determine the frequency, severity, and neural correlates of syntactic comprehension deficits in FTD-ALS, progressive nonfluent aphasia (PNFA), and amyotrophic lateral sclerosis (ALS).
  • To explore the relationship between syntactic deficits and bulbar symptoms in these patient groups.

Main Methods:

  • Utilized the Test for Reception of Grammar (TRG) to assess syntactic comprehension in 85 participants (20 ALS, 15 FTD-ALS, 27 PNFA, 23 controls).
  • Employed voxel-based morphometry (VBM) to identify neuroanatomical correlates of syntactic comprehension performance.
  • Analyzed data for correlations between TRG scores, disease group, bulbar symptoms, and brain atrophy patterns.

Main Results:

  • Syntactic comprehension deficits were prevalent: 92.9% in FTD-ALS, 81.5% in PNFA, and 25% in ALS.
  • FTD-ALS patients exhibited more severe deficits than PNFA patients.
  • Left peri-insular atrophy significantly correlated with impaired syntactic comprehension.
  • Impaired TRG performance was noted in ALS with early bulbar involvement but not overall bulbar impairment.

Conclusions:

  • Syntactic comprehension deficits are frequent and severe in FTD-ALS, exceeding those in PNFA.
  • Left peri-insular atrophy is a key neural correlate of these language impairments.
  • A notable proportion of ALS patients experience syntactic deficits, though their link to bulbar symptoms requires further investigation.