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Association of missense and 5′-splice-site mutations in tau with the inherited dementia FTDP-17
Thirteen families have been described with an autosomal dominantly inherited dementia named frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17), historically termed Pick's…
Frontotemporal lobar degeneration
Consensus criteria for the three prototypic syndromes-frontotemporal dementia, progressive nonfluent aphasia, and semantic dementia-were developed by members of an international workshop on frontotem temporal lobar degeneration and ought to provide the foundation for research work into the neuropsychology, neuropathology, genetics, molecular biology, and epidemiology of these important clinical disorders.
Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17
It is demonstrated that in multiple FTD families with significant evidence for linkage to the same region on chromosome 17q21, FTD is caused by mutations in progranulin (PGRN) that are likely to create null alleles and identified mutations in PGRN as a cause of neurodegenerative disease.
Social cognition in frontotemporal dementia and Huntington’s disease
Semantic dementia: a form of circumscribed cerebral atrophy
- J. Snowden
Knowledge of famous faces and names in semantic dementia.
The findings of an examination of famous face and name knowledge in patients with semantic dementia are interpreted as inconsistent with a unitary, amodal model of semantic memory, and lead to argue that the focal syndrome of progressive prosopagnosia is one of the clinical presentations of semantic dementia and not a separate clinical entity.
Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations.
- J. Snowden, S. Rollinson, S. Pickering-Brown
- Medicine, PsychologyBrain : a journal of neurology
- 1 March 2012
Mutations in the C9ORF72 gene may be a major cause not only of frontotemporal dementia with motor neuron disease but also of late onset psychosis, and the behavioural characteristics of patients with C9 ORF72 mutations are qualitatively distinct.
Distinct behavioural profiles in frontotemporal dementia and semantic dementia
- J. Snowden, D. Bathgate, A. Varma, A. Blackshaw, Z. Gibbons, D. Neary
- Psychology, MedicineJournal of neurology, neurosurgery, and…
- 1 March 2001
The findings confirm predictions regarding behavioural differences in frontotemporal and semantic dementia and point to differential roles of the frontal and temporal lobes in affect, social functioning, eating, and compulsive behaviour.
Neuropathological background of phenotypical variability in frontotemporal dementia
Clinical diagnosis of bvFTD does not have a strong relationship to any FTLD subtype or specific pathology and therefore remains a diagnostic challenge, but some evidence suggests improved clinicopathological association of bVFTD by further refining clinical characteristics.
Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria
- M. Strong, S. Abrahams, M. Turner
- PsychologyAmyotrophic lateral sclerosis & frontotemporal…
- 5 January 2017
These revised consensus criteria expand upon those of 2009 and embrace the concept of the frontotemporal spectrum disorder of ALS (ALS-FTSD), which is a re-conceptualisation that neuropsychological deficits in ALS fall along a spectrum.