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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Studies have demonstrated that executive or behavioral dysfunction typically develop after memory deficits in fvAD.
“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]
fvAD accounts for approximately 2-3% of AD.
“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 fvAD, memory impairment is the first abnormality in up to 85% of patients, with the remainder developing memory impairment within 3 years from symptom onset.
“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]
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Neuropsychological Predictors of Conversion from Mild Cognitive Impairment to Alzheimer's Disease
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Distinct cerebral perfusion patterns in FTLD and AD
Hu, W. T., Wang, Z., Lee, V., Trojanowski, J., Detre, J., & Grossman, M. (2010) · Neurology · doi:10.1212/wnl.0b013e3181f11e35 · Cited by 189 · see citing works
Biomarker-based staging of Alzheimer disease: rationale and clinical applications
Therriault, J., Schindler, S. E., Salvadó, G., Pascoal, T. A., Benedet, A. L., Ashton, N. J., Karikari, T. K., Apostolova, L. G., Murray, M. E., Verberk, I. M. W., et al. (2024) · Nature Reviews Neurology · doi:10.1038/s41582-024-00942-2 · Cited by 206 · see citing works
“…to amnestic-predominant dementia 144 , but retrospective studies identified AD pathology as the cause of various non-amnestic focal cortical syndromes, including posterior cortical atrophy, primary progressive aphasia, behavioural variant frontotemporal dementia and corticobasal syndrome 145–147 .”
de Souza, L. C., Lamari, F., Belliard, S., Jardel, C., Houillier, C., Paz, R. D., Dubois, B., & Sarazin, M. (2010) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.2010.207183 · Cited by 190 · see citing works
“The most frequent underlying pathology found in autopsy studies, however, is AD.18 Thus, these conditions have been coined focal cortical presentations of AD.18 Biomarkers may be able to identify the biological mechanisms of PCA and PPA and may also be useful for selecting patients who would benefit from new diseasemo…”
InfluentialPrediction of pathology in primary progressive language and speech disorders
Deramecourt, V., Lebert, F., Debachy, B., Mackowiak-Cordoliani, M., Bombois, S., Kerdraon, O., Buée, L., Maurage, C., & Pasquier, F. (2010) · Neurology · doi:10.1212/wnl.0b013e3181c7198e · Cited by 188 · see citing works
Visual assessment of posterior atrophy development of a MRI rating scale
Koedam, E., Lehmann, M., van der Flier, W. M., Scheltens, P., Pijnenburg, Y. A. L., Fox, N. C., Barkhof, F., & Wattjes, M. P. (2011) · European Radiology · doi:10.1007/s00330-011-2205-4 · Cited by 410 · see citing works
“clinical diagnosis difficult [1–3].”
Neural correlates of cognitive impairment in posterior cortical atrophy
Kas, A., de Souza, L. C., Samri, D., Bartolomeo, P., Lacomblez, L., Kalafat, M., Migliaccio, R., de Schotten, M. T., Cohen, L., Dubois, B., et al. (2011) · Brain · doi:10.1093/brain/awr055 · Cited by 177 · see citing works
Clinical and multimodal biomarker correlates of ADNI neuropathological findings
Toledo, J. B., Cairns, N. J., Da, X., Chen, K., Carter, D. R., Fleisher, A., Householder, E., Ayutyanont, N., Roontiva, A., Bauer, R. J., III, et al. (2013) · Acta Neuropathologica Communications · doi:10.1186/2051-5960-1-65 · Cited by 165 · see citing works
“clinical dementia syndromes have degrees of clinicopathological correlation, therefore if clinical diagnosis is used to estimate the accuracy of biomarkers and their cutoffs, inaccurate results may occur [13-15].”
BackgroundEarly-onset Alzheimer's Disease: Nonamnestic Subtypes and Type 2 AD
Mendez, M. F. (2012) · Archives of Medical Research · doi:10.1016/j.arcmed.2012.11.009 · Cited by 167 · see citing works
“Some include the biparietal subtype as PCA and divide PCA into temporo-occipital forms and a basic visual variant (32, 43).”
BackgroundMethodInfluentialProgressive Nonfluent Aphasia and Its Characteristic Motor Speech Deficits
Ogar, J., Dronkers, N., Brambati, S., Miller, B., & Gorno-Tempini, M. (2007) · Alzheimer Disease and Associated Disorders · doi:10.1097/wad.0b013e31815d19fe · Cited by 173 · see citing works
Botha, H., Duffy, J., Whitwell, J., Strand, E. A., Machulda, M., Schwarz, C., Reid, R. I., Spychalla, A., Senjem, M., Jones, D. T. W., et al. (2015) · Cortex; a journal devoted to the study of the nervous system and behavior · doi:10.1016/j.cortex.2015.05.013 · Cited by 171 · see citing works
Alzheimer's pathology in primary progressive aphasia
Rohrer, J. D., Rossor, M. N., & Warren, J. D. (2010) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2010.05.020 · Cited by 175 · see citing works
“…5 research groups have reported PPA patients with either pathologically confirmed AD or a positive PIB-PET scan showing amyloid deposition (Table 4; Alladi et al., 2007; Croot et al., 2000; Davies et al., 2005; Galton et al., 2000; Gorno-Tempini et al., 2008; Josephs et al., 2008; Kertesz et al.,…”
InfluentialElevated CSF GAP‐43 is Alzheimer's disease specific and associated with tau and amyloid pathology
Sandelius, Å., Portelius, E., Källén, Å., Zetterberg, H., Rot, U., Olsson, B., Toledo, J. B., Shaw, L. M. J., Lee, V. M. ‐., Irwin, D. J., et al. (2018) · Alzheimer s & Dementia · doi:10.1016/j.jalz.2018.08.006 · Cited by 191 · see citing works
Frontotemporal Dementia: Implications for Understanding Alzheimer DiseasePreprint
Goedert, M., Ghetti, B. F., & Spillantini, M. G. (2011) · Cold Spring Harbor Perspectives in Medicine · doi:10.1101/cshperspect.a006254 · Cited by 148 · see citing works
“However, a language variant of Alzheimer disease overlaps with LPA (Galton et al. 2000; Alladi et al. 2007).”
BackgroundInfluentialDissecting the clinical heterogeneity of early-onset Alzheimer’s disease
Sirkis, D. W., Bonham, L. W., Johnson, T. P., La Joie, R., & Yokoyama, J. S. (2022) · Molecular Psychiatry · doi:10.1038/s41380-022-01531-9 · Cited by 151 · see citing works
“Unlike PCA and lvPPA, CBS is not highly predictive of underlying AD neuropathology—less than a third of patients with CBS have AD [28, 38, 39]; the majority have underlying frontotemporal lobar degeneration (FTLD), most often the corticobasal degeneration subtype.”
Differential clinicopathologic and Genetic features of late-onset amnestic dementias
Murray, M., Cannon, A., Graff-Radford, N., Liesinger, A. M., Rutherford, N., Ross, O., Duara, R., Carrasquillo, M., Rademakers, R., & Dickson, D. (2014) · Acta Neuropathologica · doi:10.1007/s00401-014-1302-2 · Cited by 150 · see citing works
“These lesions are usually abundant in hippocampus and neocortex; however, atypical distribution of AD pathology presenting with focal cortical atrophy and focal clinical presentations is increasingly recognized [1, 14].”
Clinical features and APOE genotype of pathologically proven early-onset Alzheimer disease
Balasa, M., Gelpí, E., Antonell, A., Rey, M. J., Sánchez‐Valle, R., Molinuevo, J. L., & Lladó, A. (2011) · Neurology · doi:10.1212/wnl.0b013e31821a44dd · Cited by 154 · see citing works
<scp>EFNS</scp> task force: the use of neuroimaging in the diagnosis of dementia
Filippi, M., Agosta, F., Barkhof, F., Dubois, B., Fox, N. C., Frisoni, G. B., Jack, C. R., Johannsen, P., Miller, B. L., Nestor, P. J., et al. (2012) · European Journal of Neurology · doi:10.1111/j.1468-1331.2012.03859.x · Cited by 141 · see citing works
Self-citationAbnormalities of connected speech in semantic dementia vs Alzheimer's disease
Sajjadi, S. A., Patterson, K., Tomek, M., & Nestor, P. J. (2012) · Aphasiology · doi:10.1080/02687038.2012.654933 · Cited by 142 · see citing works
“It is well known that Alzheimer's disease (AD) can present with a principal complaint of language problems (eg, Alladi et al, 2007 ), but the AD patients included in the present study were mild clinically probable cases with a cognitive profile of typical early AD (ie, presenting with memory impairment).”
BackgroundSelf-citationJanocko, N. J., Brodersen, K. A., Soto-Ortolaza, A., Ross, O., Liesinger, A. M., Duara, R., Graff-Radford, N., Dickson, D., & Murray, M. (2012) · Acta Neuropathologica · doi:10.1007/s00401-012-1044-y · Cited by 138 · see citing works
“pathology, including focal cortical degeneration [1, 12, 28].”
Progression of ataxia in patients with Machado‐Joseph disease
Hu, W. T., Rippon, G. W., Boeve, B., Knopman, D., Petersen, R. C., Parisi, J. E., & Josephs, K. (2009) · Movement Disorders · doi:10.1002/mds.22627 · Cited by 136
Frontotemporal Lobar Degeneration: Epidemiology, Pathology, Diagnosis and Management
Seltman, R. E., & Matthews, B. R. (2012) · CNS Drugs · doi:10.2165/11640070-000000000-00000 · Cited by 132 · see citing works
“In contrast, AD pathology has been found in a significantly smaller proportion of SD and PNFA cases (10–30% SD and 16–44% PNFA),([48,51,52]) thus distinguishing LPA from the PPA/FTD variants.”
BackgroundO'Callaghan, C., Bertoux, M., & Hornberger, M. (2014) · Journal of Neurology, Neurosurgery & Psychiatry · doi:10.1136/jnnp-2012-304558 · Cited by 131 · see citing works
Temporoparietal atrophy: a marker of AD pathology independent of clinical diagnosis
Whitwell, J., Clifford, R., Przybelski, S., Parisi, J., Senjem, M., Boeve, B., Knopman, D., Petersen, R., Dickson, D., & Josephs, K. (2009) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2009.10.012 · Cited by 131 · see citing works
“Subjects have been reported with AD pathology that present clinically with an aphasia syndrome (Galton et al., 2000; Alladi et al., 2007; Josephs et al., 2008b; Mesulam et al., 2008), corticobasal syndrome (Alladi et al.”
BackgroundInfluentialSimilar amyloid-β burden in posterior cortical atrophy and Alzheimer's disease
de Souza, L. C., Corlier, F. W., Habert, M., Uspenskaya, O., Maroy, R., Lamari, F., Marie Chupin, A., Lehéricy, S., Olivier Colliot, A., Hahn‐Barma, V., et al. (2011) · Brain · doi:10.1093/brain/awr130 · Cited by 127 · see citing works
“(Renner et al., 2004; Tang-Wai et al., 2004; Alladi et al., 2007).”
Syndromes of nonfluent primary progressive aphasia
Rohrer, J., Rossor, M., & Warren, J. (2010) · Neurology · doi:10.1212/wnl.0b013e3181ed9c6b · Cited by 129 · see citing works
Harmonized diagnostic criteria for Alzheimer's disease: recommendations
Morris, J. C., Blennow, K., Froelich, L., Nordberg, A. K., Soininen, H. S., Waldemar, G., Wahlund, L., & Dubois, B. (2014) · Journal of Internal Medicine · doi:10.1111/joim.12199 · Cited by 120 · see citing works
Distinct clinical and metabolic deficits in PCA and AD are not related to amyloid distribution
Rosenbloom, M. H., Alkalay, A., Agarwal, N., Baker, S. L., O’Neil, J. P., Janabi, M., Yen, I. V., Growdon, M. E., Jang, J., Madison, C. M., et al. (2011) · Neurology · doi:10.1212/wnl.0b013e31821cccad · Cited by 121 · see citing works
Saccade abnormalities in autopsy-confirmed frontotemporal lobar degeneration and Alzheimer’s disease
Boxer, A., Garbutt, S., Seeley, W., Jafari, A., Heuer, H., Mirsky, J., Hellmuth, J., Trojanowski, J., Huang, E., Dearmond, S., et al. (2012) · Archives of Neurology · doi:10.1001/archneurol.2011.1021 · Cited by 123 · see citing works
Transcranial magnetic stimulation distinguishes Alzheimer disease from frontotemporal dementia
Benussi, A., Lorenzo, F. D., dell'Era, V., Cosseddu, M., Alberici, A., Caratozzolo, S., Cotelli, M. S., Micheli, A., Rozzini, L., Depari, A., et al. (2017) · Neurology · doi:10.1212/wnl.0000000000004232 · Cited by 121 · see citing works
Clinical syndromes associated with posterior atrophy
Migliaccio, R., Agosta, F., Rascovsky, K., Karydas, A., Bonasera, S. J., Rabinovici, G. D., Miller, B. L., & Gorno‐Tempini, M. L. (2009) · Neurology · doi:10.1212/wnl.0b013e3181c0d427 · Cited by 252 · see citing works
Nonamnestic Presentations of Early-Onset Alzheimer’s Disease
Mendez, M., Lee, A. S., Joshi, A., & Shapira, J. (2012) · American Journal of Alzheimers Disease & Other Dementias · doi:10.1177/1533317512454711 · Cited by 117 · see citing works
“Some investigators include the ‘‘biparietal’’ variant as PCA and describe temporo-occipital forms and a basic visual variant.(18) It appears from the literature on these different syndromes that nonamnestic EOAD subgroups overlap and may best be differentiated by focusing on the initial, presenting cognitive impairmen…”
BackgroundResearch Criteria for the Behavioral Variant of Alzheimer Disease
Ossenkoppele, R., Singleton, E., Groot, C., Dijkstra, A., Eikelboom, W., Seeley, W., Miller, B., Laforce, R., Scheltens, P., Papma, J., et al. (2021) · JAMA Neurology · doi:10.1001/jamaneurol.2021.4417 · Cited by 116 · see citing works
Paterson, R. W., Slattery, C. F., Poole, T., Nicholas, J. M., Magdalinou, N. K., Toombs, J., Chapman, M. D., Lunn, M. P., Heslegrave, A., Foiani, M. S., et al. (2018) · Alzheimer s Research & Therapy · doi:10.1186/s13195-018-0361-3 · Cited by 130 · see citing works
“PNFA is classically considered within the FTD spectrum, but 10–30% of cases have AD pathology at autopsy [26, 27].”
BackgroundAlzheimer's disease including focal presentations
Villain, N., & Dubois, B. (2019) · Seminars in Neurology · doi:10.1055/s-0039-1681041 · Cited by 113 · see citing works
Diagnosis and Management of Behavioral Variant Frontotemporal Dementia
Pressman, P., & Miller, B. L. (2013) · Biological Psychiatry · doi:10.1016/j.biopsych.2013.11.006 · Cited by 127 · see citing works
“(5) (Supplement: Table S3) In addition, Alzheimer's disease may occasionally mimic FTD with pathology found in the frontal lobes.(44) For each proteinopathy, there are possible genetic contributions.”
BackgroundStamelou, M., Quinn, N. P., & Bhatia, K. P. (2013) · Movement Disorders · doi:10.1002/mds.25509 · Cited by 124 · see citing works
Atrophy patterns in histologic vs clinical groupings of frontotemporal lobar degeneration
Pereira, J., Williams, G. B., Acosta‐Cabronero, J., Pengas, G., Spillantini, M. G., Xuereb, J. H., Hodges, J. R., & Nestor, P. J. (2009) · Neurology · doi:10.1212/wnl.0b013e3181a55fa2 · Cited by 103 · see citing works
“The left caudate nucleus was also observed to be significant in both groups ([ 8 , 14, 2] mm, t value 6.26, z score 5.48).”
BackgroundMethodResultInfluentialSelf-citationAntemortem differential diagnosis of dementia pathology using structural MRI: Differential-STAND
Vemuri, P., Simon, G., Kantarci, K., Whitwell, J. L., Senjem, M. L., Przybelski, S. A., Gunter, J. L., Josephs, K. A., Knopman, D. S., Boeve, B. F., et al. (2011) · NeuroImage · doi:10.1016/j.neuroimage.2010.12.073 · Cited by 108 · see citing works
“Additionally, there can be considerable syndromic heterogeneity, e.g., some pathology confirmed FTLD-TDP cases have prominent anterograde amnesia while some pathology confirmed AD cases have mainly aphasia (Alladi et al., 2007; Josephs et al., 2008).”
The language profile of posterior cortical atrophy
Crutch, S. J., Lehmann, M., Warren, J. D., & Rohrer, J. D. (2012) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp-2012-303309 · Cited by 112 · see citing works
Jicha, G. A., & Carr, S. A. (2010) · Journal of Alzheimer s Disease · doi:10.3233/jad-2010-1237 · Cited by 102 · see citing works
“The clinical phenotype of primary progressive aphasia, fluent as well as nonfluent types, is often a manifestation of underlying AD pathologic involvement of neuroanatomic regions involved in language processing or production in the dominant hemisphere [49,52–56].”
BackgroundFlortaucipir tau PET imaging in semantic variant primary progressive aphasia
Makaretz, S. J., Quimby, M., Collins, J., Makris, N., McGinnis, S., Schultz, A., Vasdev, N., Johnson, K. A., & Dickerson, B. C. (2018) · Journal of Neurology, Neurosurgery & Psychiatry · doi:10.1136/jnnp-2017-316409 · Cited by 104 · see citing works
The identification of cognitive subtypes in Alzheimer's disease dementia using latent class analysis
Scheltens, N., Galindo-Garré, F., Pijnenburg, Y., van der Vlies, A. E., Smits, L., Koene, T., Teunissen, C., Barkhof, F., Wattjes, M., Scheltens, P., et al. (2015) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp-2014-309582 · Cited by 104 · see citing works
- 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
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Health Sciences › Medicine › 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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