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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]
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Usefulness of Biomarkers in the Diagnosis and Prognosis of Early-Onset Cognitive Impairment
Balasa, M., Sánchez‐Valle, R., Antonell, A., Bosch, B., Olives, J., Rami, L., Castellvı́, M., Molinuevo, J. L., & Lladó, A. (2014) · Journal of Alzheimer s Disease · doi:10.3233/jad-132195 · Cited by 39
“The atypical clinical features of EOAD frequently overlap with those seen in frontotemporal lobar degeneration (FTLD) or non-degenerative disorders such as psychiatric conditions leading to frequent misdiagnosis [6–9].”
The TMA-93: A New Memory Test for Alzheimer’s Disease in Illiterate and Less Educated People
Maillet, D., Narme, P., Amieva, H., Matharan, F., Bailon, O., le Clésiau, H., & Belin, C. (2017) · American Journal of Alzheimers Disease & Other Dementias · doi:10.1177/1533317517722630 · Cited by 36
The Neuropsychiatric Examination of the Young-Onset Dementias
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“In contrast, other studies reported that EOAD has shown more heterogeneous features presenting with impairment in language, visuospatial functions, and executive functions [5–7].”
Behavioral and language variants of frontotemporal dementia: A review of key symptoms
Laforce, R. J. (2013) · Clinical Neurology and Neurosurgery · doi:10.1016/j.clineuro.2013.09.031 · Cited by 41
Mez, J., Cosentino, S., Brickman, A., Huey, E., Manly, J., & Mayeux, R. (2013) · Alzheimer Disease and Associated Disorders · doi:10.1097/wad.0b013e31826a94bd · Cited by 36
“Hippocampal sclerosis (%) 1 (5) 0 0 2 (8) 3 (20) 1 (3) Prion disease (%) 0 0 0 0 0 0 Other (%) 1 (5) 2 (18) 5 (18) 0 0 2 (5) Total 22 11 28 26 15 38”
Mez, J., Cosentino, S., Brickman, A. M., Huey, E. D., Manly, J. J., & Mayeux, R. P. (2013) · PLoS ONE · doi:10.1371/journal.pone.0065246 · Cited by 25
“Examples of atypical presentations include logopenic primary progressive aphasia, posterior cortical atrophy/visual variant, and a dysexecutive variant [2,3].”
BackgroundMonitoring Progression of Primary Progressive Aphasia: Current Approaches and Future Directions
Sapolsky, D., Domoto‐Reilly, K., Negreira, A. M., Brickhouse, M. J., McGinnis, S. M., & Dickerson, B. C. (2011) · Neurodegenerative Disease Management · doi:10.2217/nmt.11.2 · Cited by 31
Agosta, F., Mandić‐Stojmenović, G., Canu, E., Stojković, T., Imperiale, F., Caso, F., Stefanova, E. D., Copetti, M., Kostić, V., & Filippi, M. (2018) · NeuroImage Clinical · doi:10.1016/j.nicl.2018.06.013 · Cited by 35
“In vivo biomarker and postmortem pathological data indicate that the vast majority of reported cases of PCA are attributable to Alzheimer's disease (AD) (Alladi et al., 2007; Seguin et al., 2011; TangWai et al., 2004).”
Laforce, R. J., Rosa‐Neto, P., Soucy, J., Rabinovici, G. D., Dubois, B., & Gauthier, S. G. (2016) · Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques · doi:10.1017/cjn.2015.401 · Cited by 34
The behavioral variant of frontotemporal dementia: linking neuropathology to social cognition
Cerami, C., & Cappa, S. F. (2013) · Neurological Sciences · doi:10.1007/s10072-013-1317-9 · Cited by 34
“clinicopathological study of focal presentations of AD [10].”
Reilly, J. J., Rodriguez, A. D., Peelle, J. E., & Grossman, M. (2010) · Cortex · doi:10.1016/j.cortex.2010.05.005 · Cited by 43
“…Evidence from category-specific effects in progressive non-fluen disease in visual association cortex in Alzheimer’s disease and semantic dementia (Alladi et al., 2007; Bonner et al., 2009; Galton et al., 2001; Grossman et al., 2004; Glosser et al., 2002), much of the cortical visual processing…”
BackgroundAmyloid PET imaging in clinical practice
Kolanko, M., Win, Z., Loreto, F., Patel, N., Carswell, C. J., Gontsarova, A., Perry, R. J., & Malhotra, P. (2020) · Practical Neurology · doi:10.1136/practneurol-2019-002468 · Cited by 47
Keage, H., Ince, P., Matthews, F., Wharton, S., McKeith, I., & Brayne, C. (2012) · Journal of Alzheimer's Disease · doi:10.3233/jad-2011-111268 · Cited by 33
“In agreement with these epidemiological studies, suggesting an overlap in the pathological basis of dementia syndromes in the old, a clinic-based study has reported that AD-type pathology often underlies the clinical presentation of rare focal dementias, and that this is more common in those who die later [11].”
Extrapyramidal Signs in the Primary Progressive Aphasias
Kremen, S. A., Mendez, M., Tsai, P.-H., & Teng, E. (2011) · American Journal of Alzheimers Disease & Other Dementias · doi:10.1177/1533317510391239 · Cited by 33
Balasa, M., Gelpi, E., Martín, I., Antonell, A., Rey, M. J., Grau‐Rivera, O., Molinuevo, J. L., Sánchez‐Valle, R., Lladó, A., & Catalan collaborative Study Group for FTLD (2015) · Neuropathology and Applied Neurobiology · doi:10.1111/nan.12194 · Cited by 32
Hodges, J. R. (2012) · Journal of Alzheimer's Disease · doi:10.3233/jad-2012-129038 · Cited by 32
“The second clinico-pathological study [2] looked at the frequency of AD pathology in patients presenting with a greater range of progressive focal cortical syndromes, notably posterior cortical atrophy (PCA), corticobasal syndrome (CBS), behavioral variant fron-”
Self-citationMontembeault, M., Brambati, S. M., Lamari, F., Michon, A., Samri, D., Epelbaum, S., Lacomblez, L., Lehéricy, S., Habert, M., Dubois, B., et al. (2018) · NeuroImage Clinical · doi:10.1016/j.nicl.2018.10.010 · Cited by 31
“…in the absence of ophthalmologic impairment; 2) evidence of complex visual disorder on examination: elements of Balint's and Gerstmann's syndrome, visual agnosia, alexia, apraxia, or environmental disorientation; 3) proportionately less memory loss (Alladi et al., 2007; McMonagle et al., 2006).”
InfluentialSubgroups of Alzheimer's Disease: Stability of Empirical Clusters Over Time
Peter, J., Initiative, A. D. N., Abdulkadir, A., Kaller, C. P., Kümmerer, D., Hüll, M., Vach, W., & Klöppel, S. (2014) · Journal of Alzheimer s Disease · doi:10.3233/jad-140261 · Cited by 40
“Alzheimer’s disease (AD) usually presents with early episodic memory impairment, followed by deficits in attention, visuospatial abilities, and language [1, 2].”
A Patient with Posterior Cortical Atrophy Possesses a Novel Mutation in the Presenilin 1 Gene
Sitek, E. J., Narożańska, E., Pepłońska, B., Filipek, S., Barczak, A. K., Styczyńska, M., Młynarczyk, K., Brockhuis, B., Portelius, E., Religa, D., et al. (2013) · PLoS ONE · doi:10.1371/journal.pone.0061074 · Cited by 33
“The most PCA cases have intact episodic memory and insight early in the course of the disease, which enables differential diagnosis with typical (amnestic) AD [4,5].”
BackgroundMemory Impairment at Initial Clinical Presentation in Posterior Cortical Atrophy
Ahmed, S., Baker, I., Husain, M., Thompson, S., Kipps, C., Hornberger, M., Hodges, J., & Butler, C. R. (2015) · Journal of Alzheimer's Disease · doi:10.3233/jad-160018 · Cited by 31
“The most common underlying pathology is Alzheimer’s disease (AD) [3–5].”
Self-citationDo NIA‐AA criteria distinguish Alzheimer's disease from frontotemporal dementia?
Harris, J., Thompson, J. C., Gall, C., Richardson, A. M. T., Neary, D., du Plessis, D., Pal, P., Mann, D. M. A., Snowden, J. S., & Jones, M. S. (2014) · Alzheimer s & Dementia · doi:10.1016/j.jalz.2014.04.516 · Cited by 28
“Amnesia is the most common cognitive symptom in AD, yet phenotypic variation exists [3].”
BackgroundVisualizing the emergence of posterior cortical atrophy
Kennedy, J., Lehmann, M., Sokolska, M., Archer, H. A., Warrington, E. K., Fox, N. C., & Crutch, S. J. (2011) · Neurocase · doi:10.1080/13554794.2011.588180 · Cited by 28
“The most common underlying pathology in PCA is AD (Alladi et al., 2007; Galton et al., 2000; Hof, Bouras, Constantinidis, & Morrison, 1990; Hof, Bouras, Constantinidis, & Morrison, 1989; Ross et al., 1996; Tang-Wai et al., 2004; Renner et al., 2004; Snowden et al., 2007), however, a minority of cases of PCA have been…”
Clinical comparison of progressive aphasia associated with Alzheimer versus FTD-spectrum pathology
Xiong, L., Xuereb, J., Spillantini, M., Patterson, K., Hodges, J., & Nestor, P. (2010) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.2010.209916 · Cited by 31
“Pathological diagnosis Brains were examined by the same senior neuropathologist (JHX) blind to the clinical information using histological and immunohistochemical methods detailed previously.(4) 5 AD pathology was diagnosed in cases reaching Braak stage 4 or greater, with”
BackgroundMethodResultInfluentialSelf-citationSouza, L. C. D., Bertoux, M., Funkiewiez, A., Samri, D., Azuar, C., Habert, M.-O., Kas, A., Lamari, F., Sarazin, M., & Dubois, B. (2013) · Dementia & Neuropsychologia · doi:10.1590/s1980-57642013dn70100011 · Cited by 31
“The diagnosis of bvFTD was initially established on a clinical basis by the neurologist (BD), as the clinical picture fulfilled the criteria for the disease: [1] a corroborated history of initial progressive decline in social interpersonal conduct, with apathy, affective indifference and loss of empathy; [2] the prese…”
BackgroundMethodSantangelo, R., Coppi, E., Ferrari, L. D., Bernasconi, M. P., Pinto, P., Passerini, G., Comı, G., & Magnani, G. (2014) · Journal of Alzheimer s Disease · doi:10.3233/jad-141122 · Cited by 29
“Conversely, lv-PPA is more frequently associated with Alzheimer’s disease (AD) (amyloid plaques and neurofibrillary tangles) [5, 6].”
Visuospatial deficits in posterior cortical atrophy: structural and functional correlates
Andrade, K., Kas, A., Valabrègue, R., Samri, D., Sarazin, M., Habert, M., Dubois, B., & Bartolomeo, P. (2012) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp-2012-302278 · Cited by 29
Quantitating Severity and Progression in Primary Progressive Aphasia
Dickerson, B. C. (2011) · Journal of Molecular Neuroscience · doi:10.1007/s12031-011-9534-2 · Cited by 35
“As mentioned above, recent post-mortem studies of the pathologic substrates of PPA and related disorders have demonstrated that a substantial portion of patients have AD pathology (Alladi et al. 2007; Mesulam et al. 2008), which has been shown in vivo using PiB PET amyloid imaging (Rabinovici et al.”
Posterior cortical atrophy as an extreme phenotype of GRN mutations.
Caroppo, P., Belin, C., Grabli, D., Maillet, D., de Septenville, A. D., Migliaccio, R., Clot, F., Lamari, F., Camuzat, A., Brice, A., et al. (2015) · JAMA Neurology · doi:10.1001/jamaneurol.2014.3308 · Cited by 27
Nonfluent/agrammatic PPA with in-vivo cortical amyloidosis and Pick’s disease pathology
Caso, F., Gesierich, B., Henry, M., Sidhu, M., Lamarre, A., Babiak, M. C., Miller, B., Rabinovici, G., Huang, E. J., Magnani, G., et al. (2013) · Behavioural Neurology · doi:10.3233/ben-2012-120255 · Cited by 27
“Keywords: Nonfluent primary progressive aphasia, PPA, apraxia of speech, Voxel-based morphometry, PiB-PET, Pick’s disease, Alzheimer disease, Frontotemporal dementia”
BackgroundAtypical Motor and Behavioral Presentations of Alzheimer Disease
Duker, A. P., Espay, A. J., Wszołek, Z. K., Rademakers, R., Dickson, D. W., & Kelley, B. J. (2012) · The Neurologist · doi:10.1097/nrl.0b013e3182675376 · Cited by 38
“Another series reported AD pathology as the finding in 71.4% of those having “mixed aphasia” compared with much lower rates in those having PNFA or SD.18 The variability of these rates likely reflects important differences between series in syndromic definitions, application of pathologic criteria, and unavoidable ref…”
ResultInfluentialAge-at-Onset and APOE -Related Heterogeneity in Pathologically Confirmed Sporadic Alzheimer Disease
Smirnov, D. S., Galasko, D., Hiniker, A., Edland, S. D., & Salmon, D. P. (2021) · Neurology · doi:10.1212/wnl.0000000000011772 · Cited by 25
Diagnostic evaluation and monitoring of patients with posterior cortical atrophy.
Wong, B., Lucente, D. E., MacLean, J., Padmanabhan, J., Quimby, M., Brandt, K., Putcha, D., Sherman, J., Frosch, M., Mcginnis, S., et al. (2019) · Neurodegenerative Disease Management · doi:10.2217/nmt-2018-0052 · Cited by 26
Bisbing, T. A., Olm, C. A., McMillan, C. T., Rascovsky, K., Baehr, L. A., Ternes, K., Irwin, D. J., Clark, R., & Grossman, M. (2015) · Frontiers in Human Neuroscience · doi:10.3389/fnhum.2015.00317 · Cited by 24
“Many imaging studies (Whitwell et al., 2007, 2010b; Lehmann et al., 2011) and autopsy-verified assessments (Alladi et al., 2007; Murray et al., 2007; Pantelyat et al., 2011) underline the distribution of disease in the parietal lobe in CBS/PCA.”
BackgroundGoossens, J., Laton, J., Schependom, J. V., Gielen, J., Struyfs, H., Mossevelde, S. V., den Bossche, T. V., Goeman, J., Deyn, P. P. D., Sieben, A., et al. (2016) · Journal of Alzheimer s Disease · doi:10.3233/jad-160188 · Cited by 43
“Moreover, it is difficult to distinguish between these disorders on a clinical level, as up to 25% of clinical FTLD is actually due to atypical presentation of AD pathology [1, 2].”
Could Clinical Profile Influence CSF Biomarkers in Early-onset Alzheimer Disease?
Koric, L., Félician, O., Guedj, É., Hubert, A.-M., Mancini, J., Boucraut, J., & Ceccaldi, M. P. (2010) · Alzheimer Disease & Associated Disorders · doi:10.1097/wad.0b013e3181d712d9 · Cited by 26
“However, there is to date no evidence for a difference in the frequency of the ApoE4 allele between typical and atypical AD patients.(10,13) Factors other than ApoE4 genotype may mediate the intensity of neocortical changes in atypical AD patients.”
BackgroundArezoumandan, S., Xie, S. X., Cousins, K. A. Q., Mechanic-Hamilton, D. J., Peterson, C. S., Huang, C. Y., Ohm, D. T., Ittyerah, R., McMillan, C. T., Wolk, D. A., et al. (2022) · Acta Neuropathologica · doi:10.1007/s00401-022-02472-x · Cited by 29
“Phenotypic variantsb Probable AD:39 Possible AD:1 PPA:19[4] bvFTD:9[3] CBS:9[2] PCA:2[1]”
BackgroundChaudhry, A., Houlden, H., & Rizig, M. (2020) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2020.116886 · Cited by 33
“In two retrospective studies, 7.1% and 19.7% of bvFTD, and 44.1% of PNFA patients had primary AD neuropathology (Alladi, 2007; Balasa, 2015).”
BackgroundInfluentialDerby, C. A. (2020) · Current Epidemiology Reports · doi:10.1007/s40471-020-00231-8 · Cited by 27
Morgan, B. E., Gross, R. G., Clark, R., Dreyfuss, M., Boller, A., Camp, E., Liang, T., Avants, B., McMillan, C. T., & Grossman, M. (2011) · Neuropsychologia · doi:10.1016/j.neuropsychologia.2011.09.005 · Cited by 29
Li, C., Fan, S., Chen, T., Chiu, M., Yen, R., & Lin, C. (2020) · Brain and Behavior · doi:10.1002/brb3.1548 · Cited by 25
“Memory decline tends to develop earlier and be more severe in fvAD than in FTD (Alladi et al., 2007; Mendez et al., 2013; Ossenkoppele et al., 2015; de Souza et al., 2013).”
BackgroundInfluentialEvaluating pathogenic dementia variants in posterior cortical atrophy
Carrasquillo, M. M., Barber, I., Lincoln, S., Murray, M. E., Camsari, G. B., Khan, Q. U. A., Nguyen, T., Ma, L., Bisceglio, G. D., Crook, J. E., et al. (2015) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2015.09.023 · Cited by 24
“Neuropathologic series of PCA revealed AD pathology in 80–100% of cases (Ala et al., 1996; Alladi et al., 2007; Renner et al., 2004; Tang-Wai et al., 2004; Victoroff et al., 1994).”
BackgroundA Scale of Socioemotional Dysfunction in Frontotemporal Dementia
Barsuglia, J. P., Kaiser, N. C., Wilkins, S. S., Karve, S. J., Barrows, R. J., Paholpak, P., Panchal, H. V., Jimenez, E. E., Mather, M. J., & Mendez, M. F. (2014) · Archives of Clinical Neuropsychology · doi:10.1093/arclin/acu050 · Cited by 28
“Several neuropathology studies demonstrate that individuals clinically diagnosed with bvFTD exhibited primary Alzheimer’s pathology postmortem (Alladi et al., 2007; Mendez, Joshi, Tassniyom, Teng, & Shapira, 2013).”
BackgroundBiological markers of Alzheimer?s disease
de Souza, L. C., Sarazin, M., Teixeira, A. L., Caramelli, P., dos Santos, A. M., & Dubois, B. (2014) · Arquivos de Neuro-Psiquiatria · doi:10.1590/0004-282x20130233 · Cited by 33
Cerebrospinal Fluid Biomarkers in Clinical Subtypes of Early-Onset Alzheimer's Disease
Teng, E., Yamasaki, T. R., Tran, M., Hsiao, J. J., Sultzer, D. L., & Mendez, M. F. (2013) · Dementia and Geriatric Cognitive Disorders · doi:10.1159/000355555 · Cited by 22
Frontotemporal Dementia and Primary Progressive Aphasia: An Update
Kirshner, H. S. (2010) · Current Neurology and Neuroscience Reports · doi:10.1007/s11910-010-0145-z · Cited by 20
“Alzheimer’s disease, by far the most common dementing pathology, has also been associated with syndromes of PPA and FTD in some cases [7, 8, 29 , 68].”
BackgroundSECT and MAST: new tests to assess grammatical abilities in primary progressive aphasia
Billette, O. V., Sajjadi, S., Patterson, K., & Nestor, P. (2015) · Aphasiology · doi:10.1080/02687038.2015.1037822 · Cited by 22
“and presenting feature of AD in which case it falls within the rubric of primary progressive aphasia (PPA) (Alladi et al., 2007; Galton, Patterson, Xuereb, & Hodges, 2000; Gorno-Tempini et al., 2004).”
BackgroundInfluentialSelf-citationHippocampal subfield surface deformity in nonsemantic primary progressive aphasia
Christensen, A., Alpert, K. I., Rogalski, E. J., Cobia, D. J., Rao, J. A., Beg, M. F., Weıntraub, S., Mesulam, M. M., & Wang, L. (2015) · Alzheimer s & Dementia Diagnosis Assessment & Disease Monitoring · doi:10.1016/j.dadm.2014.11.013 · Cited by 18
Further evidence about the crucial role of CSF biomarkers in diagnosis of posterior cortical atrophy
Coppi, E., Ferrari, L., Santangelo, R., Caso, F., Pinto, P., Passerini, G., Comi, G., & Magnani, G. (2014) · Neurological Sciences · doi:10.1007/s10072-014-1644-5 · Cited by 22
Behavioural Variant Frontotemporal Dementia—Defining Genetic and Pathological Subtypes
Rohrer, J. D. (2011) · Journal of Molecular Neuroscience · doi:10.1007/s12031-011-9542-2 · Cited by 19
“In one study looking particularly at focal presentations of AD, 7% of cases with a bvFTD syndrome had AD pathology (Alladi et al. 2007).”
S3‐02‐01: Metacognition in Alzheimer's disease
Cosentino, S. A. (2010) · Alzheimer s & Dementia · doi:10.1016/j.jalz.2010.05.376 · Cited by 22
'The quicksand of forgetfulness': semantic dementia in One hundred years of solitude.
Rascovsky, K., Growdon, M. E., Pardo, I. R., Grossman, S., & Miller, B. (2009) · Brain : a journal of neurology · doi:10.1093/brain/awp100 · Cited by 22
“There have been few SD cases with pathology reported in the literature (Rossor et al., 2000; Hodges et al., 2004; Davies et al., 2005; Shi et al., 2005; Knibb et al., 2006; Alladi et al., 2007; Snowden et al., 2007; Davies et al., 2008).”
BackgroundSchilling, L. P., Balthazar, M. L. F., Radanovic, M., Forlenza, O. V., Silagi, M. L., Smid, J., Barbosa, B. J. A. P., Frota, N. A. F., de Souza, L. C., Vale, F. A. C., et al. (2022) · Dementia & Neuropsychologia · doi:10.1590/1980-5764-dn-2022-s102en · Cited by 29
“It is associated with gait alteration, asymmetric apraxia, alien hand phenomenon, sensory hemineglect, and visual-spatial deficits, besides the more typical symptoms of episodic and visual-spatial memory deficits and aphasia 19,23 .”
BackgroundInfluentialClinical Phenotypes in Corticobasal Syndrome with or without Amyloidosis Biomarkers
Benvenutto, A., Guedj, É., Félician, O., Eusébio, A., Azulay, J., Ceccaldi, M. P., & Koric, L. (2020) · Journal of Alzheimer s Disease · doi:10.3233/jad-190961 · Cited by 34
“Several features were retained as suggestive of AD, including long disease duration, younger age of onset [24], initial memory loss [10, 27], hemicorporeal sensory disorders, hemisensory neglect, memory impairment, visuospatial dysfunction [25, 26], dressing apraxia, and myoclonus [10, 24].”
Wang, X., Lü, H., Shi, Z., Cai, L., Liu, S., Liu, S., Han, T., Wang, Y., Zhou, Y., Wang, X., et al. (2015) · PLoS ONE · doi:10.1371/journal.pone.0134956 · Cited by 20
Biomarkers in the primary progressive aphasias
Grossman, M. (2014) · Aphasiology · doi:10.1080/02687038.2014.929631 · Cited by 25
“, 2005; Knibb, Xuereb, Patterson, & Hodges, 2006) also described histopathologic evidence for AD in several cases with a clinical diagnosis of svPPA (Alladi et al., 2007).”
BackgroundThe interest of amyloid PET imaging in the diagnosis of Alzheimerʼs disease
Vandenberghe, R. R., Adamczuk, K., & Laere, K. V. (2013) · Current Opinion in Neurology · doi:10.1097/wco.0000000000000036 · Cited by 20
“For some of these focal non-amnestic syndromes, in particular CBS and PPA, it may be hard to reliably predict on clinical grounds what the underlying neuropathology will be, Alzheimer’s disease or FTLD [47].”
BackgroundVisuospatial deficits and hemispheric perfusion asymmetries in posterior cortical atrophy
Andrade, K. S., Kas, A., Samri, D., Sarazin, M., Dubois, B., Habert, M., & Bartolomeo, P. (2012) · Cortex · doi:10.1016/j.cortex.2012.03.010 · Cited by 19
Enfermedad de Alzheimer. Guía de práctica clínica
Allegri, R., Arizaga, R. L., Bavec, C. V., Colli, L. P., Demey, I., Fernández, M. C., Frontera, S. A., Garau, M. L., Jiménez, J. J., Golimstok, Á., et al. (2011) · Neurología Argentina · doi:10.1016/s1853-0028(11)70026-x · Cited by 25
Distinct tau PET imaging patterns in typical and atypical Alzheimer’s disease
Sarazin, M., Lagarde, J., & Bottlaender, M. (2016) · Brain · doi:10.1093/brain/aww041 · Cited by 20
Schilling, L. P., Balthazar, M. L. F., Radanovic, M., Forlenza, O. V., Silagi, M. L., Smid, J., Barbosa, B. J. A. P., Frota, N. A. F., de Souza, L. C., Vale, F. A. C., et al. (2022) · Dementia & Neuropsychologia · doi:10.1590/1980-5764-dn-2022-s102pt · Cited by 59
Incidence of frontotemporal disorders in Olmsted County: A population‐based study
Turcano, P., Stang, C. D., Mielke, M. M., Martin, P. R., Upadhyaya, S. G., Josephs, K. A., Boeve, B. F., Knopman, D. S., Petersen, R. C., & Savica, R. (2019) · Alzheimer s & Dementia · doi:10.1016/j.jalz.2019.08.199 · Cited by 20
Martínez, D. B., Soldevilla, M. A. G., Santiago, A. P., & Martínez, J. T. (2019) · Medicine - Programa de Formación Médica Continuada Acreditado · doi:10.1016/j.med.2019.03.012 · Cited by 65
Manning, E. N., Macdonald, K. E., Leung, K. K., Young, J., Pepple, T., Lehmann, M., Zuluaga, M. A., Cardoso, M. J., Schott, J. M., Sebastien Ourselin, A., et al. (2015) · Human Brain Mapping · doi:10.1002/hbm.22999 · Cited by 18
“PCA is most commonly caused by Alzheimer’s disease (AD) [Alladi et al., 2007; Renner et al., 2004; Tang-Wai et al., 2004] and is probably the most common atypical clinical presentation of AD [Dubois et al.”
BackgroundInfluentialUncommon and/or bizarre features of dementia
Cipriani, G., Lucetti, C., Danti, S., Ulivi, M., & Nuti, A. (2015) · Acta Neurologica Belgica · doi:10.1007/s13760-014-0306-y · Cited by 20
“In the past decades, clinical neurologists have recognized that dementia may appear as atypical or variant syndromes, as well as the typical form [1–3].”
BackgroundFrontal variant Alzheimer's disease: A systematic narrative synthesis.
Brown, A., Salo, S. K., & Savage, G. (2023) · Cortex; a journal devoted to the study of the nervous system and behavior · doi:10.1016/j.cortex.2023.05.007 · Cited by 19
CSF placental growth factor – a novel candidate biomarker of frontotemporal dementia
Hansson, O., Santillo, A. F., Meeter, L. H. H., Nilsson, K. H., Waldö, M. L., Nilsson, C., Blennow, K., Swieten, J. C. V., & Janelidze, S. (2019) · Annals of Clinical and Translational Neurology · doi:10.1002/acn3.763 · Cited by 20
The diagnosis of dementias: a practical tool not to miss rare causes
Ferrari, C., Nacmias, B., & Sorbi, S. (2017) · Neurological Sciences · doi:10.1007/s10072-017-3206-0 · Cited by 23
Kaipainen, A. L., Jääskeläinen, O., Liu, Y., Haapalinna, F., Nykänen, N., Vanninen, R. L., Koivisto, A. M., Julkunen, V., Remes, A. M., & Herukka, S. (2020) · Journal of Alzheimer s Disease · doi:10.3233/jad-200175 · Cited by 15
“Of the clinical diagnostic groups, the AD group consisted of 55 subjects with Alzheimer’s clinical syndrome and 9 subjects with atypical variants of AD, including nonamnestic focal cortical syndromes such as posterior cortical atrophy [55], which have been neuropathologically confirmed as AD [2].”
MethodNumerosity impairment in corticobasal syndrome.
Koss, S., Clark, R., Vesely, L., Weinstein, J., Powers, C., Richmond, L. L., Farag, C., Gross, R., Liang, T., & Grossman, M. (2010) · Neuropsychology · doi:10.1037/a0018755 · Cited by 19
Okazaki, K., Fu, Y., Nishihira, Y., Endo, M., Fukushima, T., Ikeuchi, T., Okamoto, K., Onodera, O., Nishizawa, M., & Takahashi, H. (2009) · Neuropathology · doi:10.1111/j.1440-1789.2009.01062.x · Cited by 17
Frontotemporal Dementia Neuroimaging: A Guide for Clinicians
Seeley, W. W. (2009) · Monographs in clinical neuroscience/Frontiers of neurology and neuroscience/Monographs in neural sciences · doi:10.1159/000197895 · Cited by 20
Clinical impact of a second FDG-PET in atypical/unclear dementia syndromes
Bergeron, D., Beauregard, J.-M., Guimond, J., Fortin, M.-P., Houde, M., Poulin, S., Verret, L., Bouchard, R. W., & Laforce, R. (2015) · Journal of Alzheimer's Disease · doi:10.3233/jad-150302 · Cited by 17
“As reported in previous studies [3, 28, 29], a significant proportion of the so-called atypical/unclear dementias further reveal underlying AD pathology.”
ResultLlibre‐Guerra, J. J., Iaccarino, L., Coble, D., Edwards, L., Li, Y., McDade, E., Strom, A., Gordon, B. A., Mundada, N. S., Schindler, S. E., et al. (2023) · Brain Communications · doi:10.1093/braincomms/fcad280 · Cited by 21
“9,21,22 The higher frequency of these atypical presentations in sEOAD suggests that a younger age of onset of Alzheimer’s disease may have different pathogenic drivers or selectively effect different neural networks compared to LOAD.”
Posterior Cingulate Cortex Hypometabolism in Non-Amnestic Variants of Alzheimer’s Disease
Bergeron, D., Beauregard, J., Jean-Guimond, Soucy, J., Verret, L., Poulin, S. P., Matías‐Guiu, J. A., Cabrera‐Martín, M. N., Bouchard, R. W., & Laforce, R. J. (2020) · Journal of Alzheimer s Disease · doi:10.3233/jad-200567 · Cited by 20
Winchester, R. L., & Martyn, K. (2020) · Neurology and Therapy · doi:10.1007/s40120-020-00199-z · Cited by 23
Bertoux, M., Cassagnaud, P., Lebouvier, T., Sarazin, M., Ber, I. L., Dubois, B., Duyckaerts, C., Auriacombe, S., Hannequin, D., Ceccaldi, M., et al. (2019) · Journal of Alzheimer's Disease · doi:10.3233/jad-190124 · Cited by 17
“The lvPPA variant is a progressive decline in language with word finding difficulty, reduced sentence repetition, and diffi-culty comprehending long sentences along with left posterior temporal/inferior parietal dysfunction [5, 19, 20].”
Crossed Cerebellar Diaschisis in Alzheimer's Disease.
Reesink, F., García, D. V., Sanchez-Catasus, C., Peretti, D., Willemsen, A., Boellaard, R., Meles, S., Huitema, R., de Jong, B. D., Dierckx, R., et al. (2018) · Current Alzheimer Research · doi:10.2174/1567205015666180913102615 · Cited by 17
Alberici, A., Benussi, A., Premi, E., Borroni, B., & Padovani, A. (2014) · Current Alzheimer Research · doi:10.2174/1567205011666141107151606 · Cited by 16
Gil-Navarro, S., Lomeña, F., Cot, A., Lladó, A., Montagut, N., Castellví, M., Bosch, B., Rami, L., Antonell, A., Balasa, M., et al. (2013) · European Journal of Neurology · doi:10.1111/ene.12196 · Cited by 17
“However, a significant proportion of cases, up to 30–40% of nfvPPA in some series, present Alzheimer’s disease (AD) pathology at post-mortem [6,7].”
BackgroundAdvances in PET Imaging of Degenerative, Cerebrovascular, and Traumatic Causes of Dementia
Eisenmenger, L. B., Huo, E. J., Hoffman, J. M., Minoshima, S., Matesan, M. C., Lewis, D. H., Lopresti, B. J., Mathis, C. A., Okonkwo, D. O., & Mountz, J. M. (2015) · Seminars in Nuclear Medicine · doi:10.1053/j.semnuclmed.2015.09.003 · Cited by 21
Quispialaya, K. M., Therriault, J., Aliaga, A., Benedet, A. L., Ashton, N. J., Karikari, T. K., Macedo, A. C., Rahmouni, N., Tissot, C., Arias, J. F., et al. (2024) · Neurology · doi:10.1212/wnl.0000000000210211 · Cited by 17
Posterior cortical atrophy: clinical, neuroimaging, and neuropathological features
Best, J., Chapleau, M., & Rabinovici, G. D. (2023) · Expert Review of Neurotherapeutics · doi:10.1080/14737175.2023.2190885 · Cited by 18
“Retrospective studies of PCA have demonstrated that the vast majority of cases are due to underlying Alzheimer’s disease pathology [17].”
Giacomucci, G., Mazzeo, S., Bagnoli, S., Casini, M., Padiglioni, S., Polito, C., Berti, V., Balestrini, J., Ferrari, C., Lombardi, G., et al. (2021) · Journal of Personalized Medicine · doi:10.3390/jpm11010047 · Cited by 16
“Some cases of non-fluent and semantic variants of Primary Progressive Aphasia (nfv-PPA and sv-PPA) could be correlated to AD [22,23].”
BackgroundDifferences in topological progression profile among neurodegenerative diseases from imaging data
Garbarino, S., Lorenzi, M., Oxtoby, N. P., Vinke, E. J., Marinescu, R. V., Eshaghi, A., Ikram, M. A., Niessen, W. J., Ciccarelli, O., Barkhof, F., et al. (2019) · eLife · doi:10.7554/elife.49298 · Cited by 16
“of the progression pattern (Alladi et al., 2007), while neuronal distance from the epicentre domi-”
BackgroundCasoli, T., Paolini, S., Fabbietti, P., Fattoretti, P., Paciaroni, L., Fabi, K., Gobbi, B., Galeazzi, R., Rossi, R., Lattanzio, F., et al. (2019) · Journal of International Medical Research · doi:10.1177/0300060519860951 · Cited by 19
Prodromal posterior cortical atrophy: clinical, neuropsychological, and radiological correlation
Chan, L., Lynch, W., May, M. G. D., Horton, J. C., Miller, B. L., & Rabinovici, G. D. (2013) · Neurocase · doi:10.1080/13554794.2013.860176 · Cited by 16
“While individual cases that conform to these specific categories are reported in the literature (Alladi et al., 2007; Galton et al., 2000), larger cohort studies suggest that most patients with PCA show mixed dysfunction of the dorsal and ventral visual streams as well as primary visuoperceptual deficits when assessed…”
BackgroundResultInfluentialFrom frontotemporal lobar degeneration pathology to frontotemporal lobar degeneration biomarkers
Hales, C. M., & Hu, W. T. (2013) · International Review of Psychiatry · doi:10.3109/09540261.2013.776522 · Cited by 16
PET Neuroimaging in Dementia Conditions
Perani, D., Caminiti, S. P., Carli, G., & Tondo, G. (2020) · Springer eBooks · doi:10.1007/978-3-030-53168-3_9 · Cited by 20
Demencia frontotemporal variante conductual: biomarcadores, una aproximación a la enfermedad
Fernández‐Matarrubia, M., Matías-Guiu, J. A., Moreno‐Ramos, T., & Matías‐Guiu, J. (2013) · Neurología · doi:10.1016/j.nrl.2013.03.002 · Cited by 17
Interpretable differential diagnosis for Alzheimer's disease and Frontotemporal dementia
Nguyen, H.-D., Clément, M., Mansencal, B., & Coupé, P. (2022) · International Conference on Medical Image Computing and Computer-Assisted Intervention · doi:10.48550/arxiv.2206.07417 · Cited by 15
“Besides, people with FTD are often misdiagnosed with AD, although these diseases have different clinical diagnostic criteria [27,33], due to similar clinical symptoms such as a behavior or language disorder [1] and brain atrophy [29].”
Molecular and Anatomical Imaging of Dementia With Lewy Bodies and Frontotemporal Lobar Degeneration
Duignan, J. A., Haughey, A. M., Kinsella, J. A., & Killeen, R. P. (2021) · Seminars in Nuclear Medicine · doi:10.1053/j.semnuclmed.2020.12.002 · Cited by 17
Individual Brain Metabolic Signatures in Corticobasal Syndrome
Cerami, C., Dodich, A., Iannaccone, S., Magnani, G., Marcone, A., Guglielmo, P., Vanoli, G., Cappa, S. F., & Perani, D. (2020) · Journal of Alzheimer s Disease · doi:10.3233/jad-200153 · Cited by 18
“Other possible etiologies are CBD, progressive supranuclear palsy (PSP), frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP), and diffuse Lewy body disease [4, 7–10].”
Tippett, D. C., Thompson, C. B., Demsky, C., Sebastian, R., Wright, A. E., & Hillis, A. E. (2017) · PLoS ONE · doi:10.1371/journal.pone.0183212 · Cited by 14
Marra, C., Villa, G., Quaranta, D., Valenza, A., Vita, M. G., & Gainotti, G. (2011) · Journal of the International Neuropsychological Society · doi:10.1017/s1355617711001287 · Cited by 17
“Some patients with AD pathology present with progressive focal neocortical syndromes (Alladi et al., 2007; Galton, Patterson, Xuereb, & Hodges, 2000) and other patients show isolated defects of episodic and semantic memory (Butters, Lopez, & Becker, 1996; Cappa et al.”
BackgroundClinical and neuroimaging characteristics of primary progressive aphasia
Tippett, D. C., & Keser, Z. (2022) · Handbook of clinical neurology · doi:10.1016/b978-0-12-823384-9.00016-5 · Cited by 19
“Advances in biomarker discovery and diagnostics for alzheimer’s disease”
Bhatia, V., Chandel, A., Minhas, Y., & Kushawaha, S. K. (2025) · Neurological Sciences · doi:10.1007/s10072-025-08023-y · Cited by 20
“Automated tools now enable quantitative assessments, where atrophy patterns differ based on AD presentation, with some focal types sparing the medial temporal region initially [78, 79].”
Day, B. L., Ocal, D., Peters, A., Bancroft, M. J., Cash, D. M., Kaski, D. N., Crutch, S. J., & Yong, K. X. X. (2021) · The Journal of Physiology · doi:10.1113/jp282289 · Cited by 15
“One patient group presented with typical, memory-led Alzheimer’s disease (tAD), the other with posterior cortical atrophy (PCA), often considered the most common atypical AD clinical phenotype (Alladi et al. 2007; Warren et al. 2012; Graff-Radford et al. 2021).”
BackgroundNeuropathologic Associations of Learning and Memory in Primary Progressive Aphasia
Kielb, S. J., Cook, A., Wieneke, C. A., Rademaker, A., Bigio, E., Mesulam, M., Rogalski, E., & Weintraub, S. (2016) · JAMA Neurology · doi:10.1001/jamaneurol.2016.0880 · Cited by 14
Seizures in corticobasal degeneration: A case report
Douglas, V. C., DeArmond, S. J., Aminoff, M. J., Miller, B. L., & Rabinovici, G. D. (2009) · Neurocase · doi:10.1080/13554790902971158 · Cited by 14
“Similarly, AD can mimic CBS clinically (Alladi et al., 2007; Jagust, Davies, Tiller-Borcich, & Reed, 1990), but the absence of PIB uptake or AD pathology on biopsy make underlying AD unlikely in this patient.”
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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