Syntactic comprehension deficits across the FTD-ALS continuum

Jody Kamminga, Felicity V.C. Leslie, Sharpley Hsieh, Jashelle Caga, Eneida Mioshi, Michael Hornberger, Kirrie J. Ballard, Matthew C. Kiernan, John R. Hodges, James R. Burrell

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To establish the frequency, severity, relationship to bulbar symptoms, and neural correlates of syntactic comprehension deficits across the frontotemporal dementia–amyotrophic lateral sclerosis (FTD-ALS) disease spectrum. In total, 85 participants were included in the study; 20 amyotrophic lateral sclerosis (ALS), 15 FTD-ALS, 27 progressive nonfluent aphasia (PNFA), and 23 controls. Syntactic comprehension was evaluated in ALS, FTD-ALS, PNFA, and controls using the Test for Reception of Grammar. Voxel-based morphometry examined neuroanatomical correlates of performance. Syntactic comprehension deficits were detected in 25% of ALS (p = 0.011), 92.9% of FTD-ALS (p < 0.001), and 81.5% of PNFA (p < 0.001) patients. FTD-ALS was disproportionately impaired compared to PNFA. Impaired Test for Reception of Grammar performance was frequent in ALS with early bulbar involvement but did not correlate with bulbar impairment overall. Left peri-insular atrophy correlated with syntactic comprehension deficits. Syntactic comprehension deficits are frequent in FTD-ALS, more severe than in PNFA, and related to left peri-insular atrophy. A significant minority of ALS patients are impaired, but the relationship between bulbar symptoms and syntactic impairment is not understood.
Original languageEnglish
Pages (from-to)11-18
Number of pages8
JournalNeurobiology of Aging
Early online date11 Feb 2016
Publication statusPublished - May 2016


  • FTD-ALS continuum
  • Cognitive impairment
  • Syntactic comprehension deficits

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