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

doi:10.1016/j.neurobiolaging.2016.02.002

Syntactic comprehension deficits across the FTD-ALS continuum

Neurobiol Aging. 2016 May:41:11-18. doi: 10.1016/j.neurobiolaging.2016.02.002. Epub 2016 Feb 11.

Authors

Affiliations

¹ Neuroscience Research Australia, Sydney, Australia.
² 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.
³ 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.
⁴ Brain and Mind Centre, Sydney Medical School, The University of Sydney, Sydney, Australia.
⁵ Department of Psychiatry, Cambridge University, Cambridge, UK.
⁶ Department of Neurosciences, Cambridge University, Cambridge, UK.
⁷ Faculty of Health Sciences, The University of Sydney, Sydney, Australia.
⁸ 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.

PMID: 27103514
DOI: 10.1016/j.neurobiolaging.2016.02.002

Abstract

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.

Keywords: Cognitive impairment; FTD-ALS continuum; Syntactic comprehension deficits.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adult
Aged
Amyotrophic Lateral Sclerosis / pathology
Amyotrophic Lateral Sclerosis / psychology*
Aphasia*
Atrophy
Cerebral Cortex / pathology
Comprehension
Disease Progression
Female
Frontotemporal Dementia / pathology
Frontotemporal Dementia / psychology*
Humans
Male
Middle Aged
Severity of Illness Index

Supplementary concepts

Frontotemporal Dementia With Motor Neuron Disease