Syntactic comprehension deficits across the FTD-ALS continuum.
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.
Neuroscience Research Australia, Sydney, Australia.
Jody
J
Kamminga
Neuroscience Research Australia, Sydney, Australia; University of New South Wales, Sydney, Australia; ARC Centre of Excellence in Cognition and its Disorders, University of New South Wales, Sydney, Australia.
Felicity V C
FVC
Leslie
Neuroscience Research Australia, Sydney, Australia; ARC Centre of Excellence in Cognition and its Disorders, University of New South Wales, Sydney, Australia; Brain and Mind Centre, Sydney Medical School, The University of Sydney, Sydney, Australia.
Sharpley
S
Hsieh
Brain and Mind Centre, Sydney Medical School, The University of Sydney, Sydney, Australia.
Jashelle
J
Caga
Department of Psychiatry, Cambridge University, Cambridge, UK.
Eneida
E
Mioshi
Department of Neurosciences, Cambridge University, Cambridge, UK.
Michael
M
Hornberger
Faculty of Health Sciences, The University of Sydney, Sydney, Australia.
Kirrie J
KJ
Ballard
Brain and Mind Centre, Sydney Medical School, The University of Sydney, Sydney, Australia.
Matthew C
MC
Kiernan
Neuroscience Research Australia, Sydney, Australia; University of New South Wales, Sydney, Australia; ARC Centre of Excellence in Cognition and its Disorders, University of New South Wales, Sydney, Australia.
John R
JR
Hodges
Neuroscience Research Australia, Sydney, Australia; University of New South Wales, Sydney, Australia; ARC Centre of Excellence in Cognition and its Disorders, University of New South Wales, Sydney, Australia. Electronic address: j.burrell@neura.edu.au.
James R
JR
Burrell
D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology / Q000523:psychology*; D001037:Aphasia; D001284:Atrophy; D002540:Cerebral Cortex / Q000473:pathology; D032882:Comprehension; D018450:Disease Progression; D005260:Female; D057180:Frontotemporal Dementia / Q000473:pathology / Q000523:psychology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012720:Severity of Illness Index
Cognitive impairment; FTD-ALS continuum; Syntactic comprehension deficits