The DOI matches its Crossref record; 39 of the 39 references it cites were found in a registry or an index.
Frontotemporal Lobar Degeneration: Epidemiology, Pathophysiology, Diagnosis and Management
Open accessAbstract
Frontotemporal lobar degeneration (FTLD) is a clinically and pathologically heterogeneous syndrome, characterized by progressive decline in behaviour or language associated with degeneration of the frontal and anterior temporal lobes. While the seminal cases were described at the turn of the 20th century, FTLD has only recently been appreciated as a leading cause of dementia, particularly in patients presenting before the age of 65 years. Three distinct clinical variants of FTLD have been described: (i) behavioural-variant frontotemporal dementia, characterized by changes in behaviour and personality in association with frontal-predominant cortical degeneration; (ii) semantic dementia, a syndrome of progressive loss of knowledge about words and objects associated with anterior temporal neuronal loss; and (iii) progressive nonfluent aphasia, characterized by effortful language output, loss of grammar and motor speech deficits in the setting of left perisylvian cortical atrophy.The majority of pathologies associated with FTLD clinical syndromes include either tau-positive (FTLD-TAU) or TAR DNA-binding protein 43 (TDP-43)-positive (FTLD-TDP) inclusion bodies. FTLD overlaps clinically and pathologically with the atypical parkinsonian disorders corticobasal degeneration and progressive supranuclear palsy, and with amyotrophic lateral sclerosis. The majority of familial FTLD cases are caused by mutations in the genes encoding microtubule-associated protein tau (leading to FTLD-TAU) or progranulin (leading to FTLD-TDP).The clinical and pathological heterogeneity of FTLD poses a significant diagnostic challenge, and in vivo prediction of underlying histopathology can be significantly improved by supplementing the clinical evaluation with genetic tests and emerging biological markers.Current pharmacotherapy for FTLD focuses on manipulating serotonergic or dopaminergic neurotransmitter systems to ameliorate behavioural or motor symptoms. However, recent advances in FTLD genetics and molecular pathology make the prospect of biologically driven, diseasespecific therapies for FTLD seem closer than ever.
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Cites
Amyloid oligomers exacerbate tau pathology in a mouse model of tauopathy
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Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis
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Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17
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Cited by
Advancing Research and Treatment for Frontotemporal Lobar Degeneration (ARTFL) investigators, Thijssen, E. H., La Joie, R., Wolf, A., Strom, A., Wang, P., Iaccarino, L., Bourakova, V., Cobigo, Y., Heuer, H., et al. (2020) · Nature Medicine · doi:10.1038/s41591-020-0762-2 · Cited by 761
Self-citationA new subtype of frontotemporal lobar degeneration with FUS pathology
Neumann, M., Rademakers, R., Roeber, S., Baker, M., Kretzschmar, H. A., & Mackenzie, I. R. A. (2009) · Brain · doi:10.1093/brain/awp214 · Cited by 722
Neuropathology of Parkinson disease
Dickson, D. W. (2018) · Parkinsonism & Related Disorders · doi:10.1016/j.parkreldis.2017.07.033 · Cited by 623
The evolution and pathology of frontotemporal dementia
Kertesz, A., McMonagle, P., Blair, M., Davidson, W., & Munoz, D. G. (2005) · Brain · doi:10.1093/brain/awh598 · Cited by 609
Behavioural-variant frontotemporal dementia: diagnosis, clinical staging, and management
Piguet, O., Hornberger, M., Mioshi, E., & Hodges, J. R. (2011) · The Lancet Neurology · doi:10.1016/s1474-4422(10)70299-4 · Cited by 496
Metadata
- Authors
- Rabinovici, G. D., & Miller, B. L.
- Venue
- CNS Drugs
- Published
- 2010-05
- Volume
- 24
- Issue
- 5
- Pages
- 375-398
- ISSN
- 1172-7047 (ISSN-L)
- OpenAlex
- W1983209442
- Cited by
- 501
- References
- 39
- FWCI
- 24.13
- Type
- journal article
- Language
- en
- Open access
- green
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- Rabinovici, G. D., & Miller, B. L. (2010). Frontotemporal Lobar Degeneration: Epidemiology, Pathophysiology, Diagnosis and Management. CNS Drugs. https://doi.org/10.2165/11533100-000000000-00000
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