The DOI matches its Crossref record; 8 of the 8 references it cites were found in a registry or an index.
Focal cortical presentations of Alzheimer's disease
Abstract
To determine the frequency of Alzheimer's disease (AD) pathology in patients presenting with progressive focal cortical syndromes, notably posterior cortical atrophy (PCA), corticobasal syndrome (CBS), behavioural variant frontotemporal dementia (bvFTD), progressive non-fluent aphasia (PNFA) (or a mixed aphasia) and semantic dementia (SD); and to compare the age of onset, evolution and prognosis in patients with focal cortical presentations of AD versus more typical AD and those with non AD pathology. From a total of 200 patients with comprehensive prospective clinical and pathological data we selected 120 : 100 consecutive cases with focal cortical syndromes and 20 with clinically typical AD. Clinical files were reviewed blind to pathological diagnosis. Of the 100 patients with focal syndromes, 34 had AD as the primary pathological diagnosis with the following distribution across clinical subtypes: all 7 of the PCA (100%); 6 of 12 with CBS (50%); 2 of 28 with bvFTD (7.1%); 12 of 26 with PNFA (44.1%); 5 of 7 with mixed aphasia (71.4%) and 2 of 20 with SD (10%). Of 20 with clinically typical AD, 19 had pathological AD. Age at both onset and death was greater in the atypical AD cases than those with non-AD pathology, although survival was equivalent. AD is a much commoner cause of focal cortical syndromes than previously recognised, particularly in PCA, PNFA and CBS, but rarely causes SD or bvFTD. The focal syndrome may remain pure for many years. Patients with atypical AD tend…
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“While not apparent in our cases, studies have demonstrated that executive or behavioral dysfunction typically develop after memory deficits in fvAD [1, 22, 23].”
From Diagnosing the frontal variant of Alzheimer’s disease: a clinician’s yellow brick road (2017) · cites this paper as [1]
In the citing paper
“Some studies have suggested that fvAD accounts for approximately 2–3% of AD, [1, 2]”
From Diagnosing the frontal variant of Alzheimer’s disease: a clinician’s yellow brick road (2017) · cites this paper as [1]
In the citing paper
“Conversely, in fvAD memory impairment is the first abnormality in up to 85% of patients (Fig. 4), with the remainder developing memory impairment within 3 years from symptom onset [1, 22, 23].”
From Diagnosing the frontal variant of Alzheimer’s disease: a clinician’s yellow brick road (2017) · cites this paper as [1]
Citations
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Pathologic heterogeneity in clinically diagnosed corticobasal degeneration
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Neural substrates of cognitive and behavioral deficits in atypical Alzheimer's disease
von Gunten, A., Bouras, C., Kövari, E., Giannakopoulos, P., & Hof, P. R. (2006) · Brain Research Reviews · doi:10.1016/j.brainresrev.2005.11.003 · Cited by 75
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McKhann, G. M., Knopman, D. S., Chertkow, H., Hyman, B. T., Jack, C. R., Kawas, C. H., Klunk, W. E., Koroshetz, W. J., Manly, J. J., Mayeux, R., et al. (2011) · Alzheimer's & Dementia · doi:10.1016/j.jalz.2011.03.005 · Cited by 18,657
“Experience has shown that there are several nonamnestic presentations of the pathophysiological process of AD, the most common ones being the syndrome of posterior cortical atrophy [15] and the syndrome of logopenic-primary progressive aphasia [16].”
BackgroundAdvancing research diagnostic criteria for Alzheimer's disease: the IWG-2 criteria
Dubois, B., Feldman, H. H., Jacova, C., Hampel, H., Molinuevo, J. L., Blennow, K., DeKosky, S. T., Gauthier, S., Selkoe, D., Bateman, R., et al. (2014) · The Lancet Neurology · doi:10.1016/s1474-4422(14)70090-0 · Cited by 3,321
Jack, C. R., Albert, M. S., Knopman, D. S., McKhann, G. M., Sperling, R. A., Carrillo, M. C., Thies, B., & Phelps, C. H. (2011) · Alzheimer's & Dementia · doi:10.1016/j.jalz.2011.03.004 · Cited by 2,042
“[5] Alladi S, Xuereb J, Bak T, Nestor P, Knibb J, Patterson K, et al.”
BackgroundRevising the definition of Alzheimer's disease: a new lexicon
Dubois, B., Feldman, H. H., Jacova, C., Cummings, J. L., DeKosky, S. T., Barberger-Gateau, P., Delacourte, A., Frisoni, G., Fox, N. C., Galasko, D., et al. (2010) · The Lancet Neurology · doi:10.1016/s1474-4422(10)70223-4 · Cited by 1,869
Classification and basic pathology of Alzheimer disease
Duyckaerts, C., Delatour, B., & Potier, M.-C. (2009) · Acta Neuropathologica · doi:10.1007/s00401-009-0532-1 · Cited by 1,031
“, studied 12 cases of cortico-basal syndrome (progressive apraxia, alien hand syndrome, unilateral rigidity), among which 6 were related to AD [7].”
Background
Metadata
- Authors
- Alladi, S., Xuereb, J., Bak, T., Nestor, P., Knibb, J., Patterson, K., & Hodges, J. R.
- Affiliations
- MRC Cognition and Brain Sciences Unit (GB)
- Corresponding author
- Alladi, S.
- Venue
- Brain
- Publisher
- Oxford University Press (OUP)
- Published
- 2007-10-01
- Volume
- 130
- Issue
- 10
- Pages
- 2636-2645
- ISSN
- 0006-8950 (ISSN-L)
- 1460-2156
- OpenAlex
- W2142244150
- Cited by
- 581
- References
- 0
- Citations by year
- 2026: 11 · 2025: 20 · 2024: 16 · 2023: 17 · 2022: 23 · 2021: 21 · 2020: 37 · 2019: 28 · 2018: 28 · 2017: 30 · 2016: 20 · 2015: 44 · 2014: 45 · 2013: 47 · 2012: 50
- FWCI
- 15.10
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Brain (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Medical Research Council
- Topics
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
- Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
- Keywords
- Alzheimer's disease, posterior cortical atrophy, corticobasal syndrome, behavioral variant frontotemporal dementia, progressive nonfluent aphasia, semantic dementia, mixed aphasia, age of onset, prognosis, survival, neuropathology
- MeSH terms
- Aged, Alzheimer Disease, Atrophy, Cerebral Cortex, Cognition Disorders, Dementia, Diagnosis, Differential, Female, Humans, Male, Middle Aged, Prognosis, Syndrome, Survival Analysis, Age of Onset, Disease Progression, Aphasia, Primary Progressive
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