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Connectivity-based characterisation of subcortical grey matter pathology in frontotemporal dementia and ALS: a multimodal neuroimaging study
- Source :
- Brain imaging and behavior. 12(6)
- Publication Year :
- 2018
-
Abstract
- Frontotemporal dementia (FTD) phenotypes have distinctive and well-established cortical signatures, but their subcortical grey matter profiles are poorly characterised. The comprehensive characterisation of striatal and thalamic pathology along the ALS-FTD spectrum is particularly timely, as dysfunction of frontostriatal and cortico-thalamic networks contribute to phenotype-defining cognitive, behavioral, and motor deficits. Ten patients with behavioral-variant FTD, 11 patients with non-fluent-variant primary progressive aphasia, 5 patients with semantic-variant primary progressive aphasia, 14 ALS-FTD patients with C9orf72 hexanucleotide expansions, 12 ALS-FTD patients without hexanucleotide repeats, 36 ALS patients without cognitive impairment and 50 healthy controls were included in a prospective neuroimaging study. Striatal, thalamic, hippocampal and amygdala pathology was evaluated using volume measurements, density analyses and connectivity-based segmentation. Significant volume reductions were identified in the thalamus and putamen of non-fluent-variant PPA patients. Marked nucleus accumbens and hippocampal atrophy was observed in the behavioral-variant FTD cohort. Semantic-variant PPA patients only exhibited volumetric changes in the left hippocampus. C9-positive ALS-FTD patients showed preferential density reductions in thalamic sub-regions connected to motor and sensory cortical areas. C9-negative ALS-FTD patients exhibited striatal pathology in sub-regions projecting to rostral-motor and executive cortical areas. The bulk of striatal and thalamic pathology in non-fluent-variant PPA patients was identified in foci projecting to motor areas. Subcortical density alterations in svPPA patients were limited to basal ganglia regions with parietal projections. Striatal and thalamic changes in FTD exhibit selective, network-defined vulnerability patterns mirroring cortical pathology. Multi-modal cortico-basal imaging analyses confirm that the subcortical grey matter profiles of FTD phenotypes are just as distinct as their cortical signatures. Our findings support emerging concepts of network-wise degeneration, preferential circuit vulnerability and disease propagation along connectivity patterns.
- Subjects :
- 0301 basic medicine
Male
Pathology
medicine.medical_specialty
Cognitive Neuroscience
Thalamus
Neuroimaging
Grey matter
Amygdala
Multimodal Imaging
Primary progressive aphasia
03 medical and health sciences
Behavioral Neuroscience
Cellular and Molecular Neuroscience
0302 clinical medicine
mental disorders
Basal ganglia
Neural Pathways
medicine
Humans
Radiology, Nuclear Medicine and imaging
Prospective Studies
Gray Matter
Aged
business.industry
Putamen
Amyotrophic Lateral Sclerosis
Brain
Organ Size
Middle Aged
medicine.disease
Magnetic Resonance Imaging
Psychiatry and Mental health
030104 developmental biology
medicine.anatomical_structure
Neurology
Frontotemporal Dementia
Female
Neurology (clinical)
business
030217 neurology & neurosurgery
Frontotemporal dementia
Subjects
Details
- ISSN :
- 19317565
- Volume :
- 12
- Issue :
- 6
- Database :
- OpenAIRE
- Journal :
- Brain imaging and behavior
- Accession number :
- edsair.doi.dedup.....7da5632ef28e0daeb816c119bb74e941