Cited by 581Dementia and Cognitive Impairment ResearchAlzheimer's disease research and treatmentsAmyotrophic Lateral Sclerosis Research

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Focal cortical presentations of Alzheimer's disease

Alladi, S., Xuereb, J., Bak, T., Nestor, P., Knibb, J., Patterson, K., Hodges, J. R.·2007·Brain

DOI 10.1093/brain/awm213

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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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“, 2003), (d) behavioral deficits, with atrophy of the prefrontal cortex (Alladi et al., 2007; Johnson, Head, Kim, Starr, & Cotman, 1999), or (e) motor deficits prevalent in corticobasal syndromes (Alladi et al.”
BackgroundInfluential
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Vandenberghe, R. R. (2011) · Brain · doi:10.1093/brain/awr208 · Cited by 6
Progress in the last decade in our understanding of primary progressive aphasia
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“[23,39,62,63] In a recent study of 100 patients with SD, 24 had autopsy – 18 had FTD-U (13/13 TDP-43 positive), 3 had classical tau pathology, and 3 had AD.”
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Parietal Involvement in the Semantic Variant of Primary Progressive Aphasia with Alzheimer’s Disease Cerebrospinal Fluid Profile
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“NA Alladi [10] Histopathology 2/20 (10”
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“This clinical condition has become of increasing interest, since the clinical onset of a relevant proportion of patients with Alzheimer’s disease is dominated by non-typical symptoms [29].”
Focal Cortical Presentations Genetically Proven Alzheimer Disease
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“primarily pathological diagnosis in 34% of the cases (21).”
Background
Understanding atrophy trajectories in alzheimer's disease using association rules on MRI images
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Result
What progressive aphasia says about its neuropathology
Mendez, M. (2010) · Neurology · doi:10.1212/wnl.0b013e3181c719b3 · Cited by 5
The Use of 18F-FDG PET in the Diagnostic Workup of Alzheimer’s Dementia
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Use of Amyloid Positron-Emission Tomography to Diagnose Alzheimer's Disease in Clinical Practice in South Korea: Expert Recommendations
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“…therapeutic choice, it is important to distinguish atypical AD from FTLD, since medications such as acetylcholinesterase inhibitors used in AD are ineffective in FTLD. 68,91,92 Aβ plaques are generally not present in the FTLD pathological spectrum, with low rates of positive amyloid scans (0%– 15”
Influential
The experience of diagnosing Alzheimer’s disease based on the study of cerebrospinal fluid biomarkers
Васильевна, Н. К., Shpilyukova, Y. A., Fedotova, E. Y., Burmak, A. G., Шабалина, А. А., & Illarioshkin, S. N. (2025) · S S Korsakov Journal of Neurology and Psychiatry · doi:10.17116/jnevro202512501191 · Cited by 4
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Is the pathology of posterior cortical atrophy clinically predictable?
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Background
Stratification of patients with Alzheimer’s disease based on longitudinal neuropsychological tests
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Role of [18F]-FDG PET in patients with atypical parkinsonism associated with dementia
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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.
ORCID: Alladi, S.
Xuereb, J.
Nestor, P.
Patterson, K.
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
DOI: 10.1093/brain/awm213
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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Focal cortical presentations of Alzheimer's disease

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