Diagnosis and Management of Posterior Cortical Atrophy
Open accessAbstract
Purpose of review: The study aims to provide a summary of recent developments for diagnosing and managing posterior cortical atrophy (PCA). We present current efforts to improve PCA characterisation and recommendations regarding use of clinical, neuropsychological and biomarker methods in PCA diagnosis and management and highlight current knowledge gaps. Recent findings: Recent multi-centre consensus recommendations provide PCA criteria with implications for different management strategies (e.g. targeting clinical features and/or disease). Studies emphasise the preponderance of primary or co-existing Alzheimer’s disease (AD) pathology underpinning PCA. Evidence of approaches to manage PCA symptoms is largely derived from small studies. Summary: PCA diagnosis is frequently delayed, and people are likely to receive misdiagnoses of ocular or psychological conditions. Current treatment of PCA is symptomatic — pharmacological and non-pharmacological — and the use of most treatment options is based on small studies or expert opinion. Recommendations for non-pharmacological approaches include interdisciplinary management tailored to the PCA clinical profile — visual-spatial — rather than memory-led, predominantly young onset — and psychosocial implications. Whilst emerging disease-modifying treatments have not been tested in PCA, an accurate and timely diagnosis of PCA and determining underlying pathology is of increasing importance in the advent of disease-modifying therapies for AD and other albeit rare causes of PCA.
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A retrospective study suggested that ChEIs may help stabilize cognitive and functional decline in logopenic variant primary progressive aphasia (lvPPA), similar to their effects in amnestic Alzheimer's disease.
“A retrospective study suggested that ChEIs may help stabilize cognitive and functional decline in lvPPA, similar to their effects in amnestic AD.51”
From A 2025 update on treatment strategies for the Alzheimer’s disease spectrum (2025) · cites this paper as [51]
The Pharmacological management section recommends acetylcholinesterase inhibitors for posterior cortical atrophy, while Memory and language notes an overlap between posterior cortical atrophy and lvPPA. Neither section reports a retrospective lvPPA study or says that these drugs stabilize cognitive and functional decline in lvPPA compared with amnestic AD; searches for those terms found no such passage in our copy. (LLM, conf=0.74)
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Putcha, D., Dickerson, B. C., Brickhouse, M. J., Johnson, K. A., Sperling, R. A., & Papp, K. V. (2020) · Neuropsychologia · doi:10.1016/j.neuropsychologia.2020.107391 · Cited by 25 · see citing works
Self-citationPosterior cortical atrophy: visuomotor deficits in reaching and grasping
Meek, B. P., Shelton, P., & Marotta, J. J. (2013) · Frontiers in Human Neuroscience · doi:10.3389/fnhum.2013.00294 · Cited by 24 · see citing works
Navigational cue effects in Alzheimer's disease and posterior cortical atrophy
Yong, K. X. X., McCarthy, I. D., Poole, T., Suzuki, T., Yang, B., Carton, A. M., Holloway, C., Papadosifos, N. N., Boampong, D. K., Langham, J., et al. (2018) · Annals of Clinical and Translational Neurology · doi:10.1002/acn3.566 · Cited by 22 · see citing works
Self-citationFirst Report of PSEN2 Mutation Presenting as Posterior Cortical Atrophy
Tremolizzo, L., Susani, E. L., Mapelli, C., Isella, V., Bertola, F., Ferrarese, C., & Appollonio, I. M. (2014) · Alzheimer Disease & Associated Disorders · doi:10.1097/wad.0000000000000052 · Cited by 19 · see citing works
Yong, K. X. X., Rajdev, K., Warrington, E. K., Nicholas, J. M., Warren, J. D., & Crutch, S. J. (2016) · Neuropsychologia · doi:10.1016/j.neuropsychologia.2016.02.022 · Cited by 18 · see citing works
Self-citationDay, 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 · see citing works
Self-citationMcIntyre, A., Harding, E., Yong, K. X. X., Sullivan, M. P., Gilhooly, M., Gilhooly, K. J., Woodbridge, R., & Crutch, S. J. (2019) · Health & Social Care in the Community · doi:10.1111/hsc.12715 · Cited by 14 · see citing works
Self-citationMcCarthy, I., Suzuki, T., Holloway, C., Poole, T., Frost, C. D., Carton, A. M., Tyler, N., Crutch, S. J., & Yong, K. X. X. (2019) · Healthcare Technology Letters · doi:10.1049/htl.2018.5034 · Cited by 14 · see citing works
Self-citationInspired by chance: valuing patients’ informal contributions to research
Crutch, S. J., Herron, D., Pickett, J., Rosser, S., & Rossor, M. N. (2020) · BMJ · doi:10.1136/bmj.m4478 · Cited by 13 · see citing works
Self-citationVolkmer, A., Farrington-Douglas, C., Crutch, S., Beeke, S., Warren, J. D., & Yong, K. X. X. (2022) · Neurocase · doi:10.1080/13554794.2022.2125326 · Cited by 10 · see citing works
Self-citationReadClear: An Assistive Reading Tool for People Living with Posterior Cortical Atrophy
Gonzalez, A. S., Ocal, D., Pavisic, I. M., Peacock, A., Naessens, M., Ahmed, S., Butler, C., Leff, A. P., Yong, K. X. X., & Crutch, S. J. (2019) · Journal of Alzheimer s Disease · doi:10.3233/jad-190335 · Cited by 10 · see citing works
Self-citationBowen, M. A., Zutshi, H., Cordiner, M., Crutch, S. J., & Shakespeare, T. J. (2019) · BMJ Open · doi:10.1136/bmjopen-2017-020905 · Cited by 9 · see citing works
Self-citationA 63-Year-Old Man With Progressive Visual Symptoms
Mitchell, S. B., Lucente, D. E., Larvie, M., Cobos, M. I., Frosch, M. P., & Dickerson, B. C. (2016) · JAMA Neurology · doi:10.1001/jamaneurol.2016.2210 · Cited by 8 · see citing works
Jackson, S. R., Condon, L. A., Newport, R., Pears, S. L., Masud Husain, B., Bajaj, N. P. S., & O’Donoghue, M. (2017) · Cortex · doi:10.1016/j.cortex.2017.03.023 · Cited by 6 · see citing works
Effects of lighting variability on locomotion in posterior cortical atrophy
Yong, K. X. X., McCarthy, I. D., Poole, T., Ocal, D., Suzuki, A., Suzuki, T., Mengoudi, K., Papadosifos, N. N., Boampong, D. K., Tyler, N., et al. (2020) · Alzheimer s & Dementia Translational Research & Clinical Interventions · doi:10.1002/trc2.12077 · Cited by 6 · see citing works
Self-citationOverman, M. J., Drummond, N., Butler, C., & Ahmed, S. (2022) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp-2021-327501 · Cited by 5 · see citing works
Self-citationImpaired mechanism of visual focal attention in posterior cortical atrophy.
Primativo, S., Crutch, S. J., Pavisic, I. M., Yong, K. X. X., Rossetti, A., & Daini, R. (2020) · Neuropsychology · doi:10.1037/neu0000697 · Cited by 4 · see citing works
Self-citationTang‐Wai, D. F., Lake, A., & Graff‐Radford, N. R. (2014) · Oxford University Press eBooks · doi:10.1093/med/9780199928453.003.0010 · Cited by 4 · see citing works
Burbaite, A., Leeworthy, S., Hirst, L., Mioshi, E., Clare, L., & Ahmed, S. (2023) · Systematic Reviews · doi:10.1186/s13643-023-02187-4 · Cited by 2 · see citing works
Self-citationBurbaite, A., Leeworthy, S., Hirst, L., Mioshi, E., Clare, L., & Ahmed, S. (2021) · Alzheimer s & Dementia · doi:10.1002/alz.057679 · Cited by 1 · see citing works
Self-citation
Flagged first, then by citations
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A 2025 update on treatment strategies for the Alzheimer’s disease spectrum
Wu, C.-K., & Fuh, J.-L. (2025) · Journal of the Chinese Medical Association · doi:10.1097/jcma.0000000000001252 · Cited by 77 · see citing works
“52 Emerging therapies, such as transcranial direct current stimulation (tDCS) combined with language therapy, show some promise for improving word retrieval in lvPPA.”
Volkmer, A., Cross, L., Highton, L., Jackson, C., Smith, C., Brotherhood, E., Harding, E., Mummery, C. J., Rohrer, J. D., Weil, R. S., et al. (2024) · International Journal of Language & Communication Disorders · doi:10.1111/1460-6984.13018 · Cited by 21 · see citing works
Self-citationHarding, E., Sullivan, M. P., Camic, P. M., Yong, K. X. X., Stott, J. C. H., & Crutch, S. J. (2024) · Qualitative Health Research · doi:10.1177/10497323241239487 · Cited by 16 · see citing works
Self-citationLiu, X., Wang, S., Wei, L., Liu, Y., Bian, J., Wang, S., & Du, X. (2024) · Frontiers in Public Health · doi:10.3389/fpubh.2024.1393823 · Cited by 15 · see citing works
Atypical Presentations of Alzheimer Disease
Jones, D. T., Pelak, V. S., & Rogalski, E. J. (2024) · CONTINUUM Lifelong Learning in Neurology · doi:10.1212/con.0000000000001504 · Cited by 13 · see citing works
Diagnosis of Alzheimer's disease by using biological markers in posterior cortical atrophy
Гришина, Д. А., Khayalieva, N. A., Grinyuk, V. V., & Tyurina, A. Y. (2024) · Neurology neuropsychiatry Psychosomatics · doi:10.14412/2074-2711-2024-2-47-53 · Cited by 12 · see citing works
Bader, I., Groot, C., Tan, H. S., Milongo, J.-M. A., den Haan, J., Verberk, I. M. W., Yong, K. X. X., Orellina, J. A., Campbell, S. R., Wilson, D., et al. (2024) · Alzheimer s Research & Therapy · doi:10.1186/s13195-024-01545-1 · Cited by 12 · see citing works
Self-citationSun, Y., Zhang, L., Ye, H., Leng, L., Chen, Y., Su, Y., Ren, P., Lu, H., & Peng, G. (2024) · Alzheimer s Research & Therapy · doi:10.1186/s13195-024-01431-w · Cited by 11 · see citing works
Posterior cortical atrophy: new insights into treatments and biomarkers for Alzheimer's disease
Bejanin, A., & Villain, N. (2024) · The Lancet Neurology · doi:10.1016/s1474-4422(23)00501-x · Cited by 9 · see citing works
Evaluating the effect of kidney function on brain volumes and dementia risk in the UK Biobank
Huang, X., Yuan, S., Ling, Y., Cheng, H., Tan, S., Xu, A., & Lyu, J. (2023) · Archives of Gerontology and Geriatrics · doi:10.1016/j.archger.2023.105157 · Cited by 8 · see citing works
Suárez-González, A., Savage, S. A., Alladi, S., Amaral-Carvalho, V., Arshad, F., Camino, J., Caramelli, P., Comas-Herrera, A., Cook, J., Cooper, C., et al. (2024) · International Journal of Environmental Research and Public Health · doi:10.3390/ijerph21060790 · Cited by 8 · see citing works
Atypical forms of Alzheimer's disease: patients not to forget
Montembeault, M., & Migliaccio, R. (2023) · Current Opinion in Neurology · doi:10.1097/wco.0000000000001182 · Cited by 8 · see citing works
Survival Differences Between Individuals With Typical and Atypical Phenotypes of Alzheimer Disease
Bader, I., Groot, C., van der Flier, W. M., Pijnenburg, Y. A. L., & Ossenkoppele, R. (2025) · Neurology · doi:10.1212/wnl.0000000000213603 · Cited by 6 · see citing works
Self-citationCognitive rehabilitation in posterior cortical atrophy
Gonzalez, A. S., Bier, N., Sauvageau, H., Pelak, V. S., & Ahmed, S. (2024) · Practical Neurology · doi:10.1136/pn-2024-004259 · Cited by 5 · see citing works
Self-citationLin, H., Jiang, Q., Yang, Y., Huang, Q., Zhang, Y., Zhang, Z., Zhu, Y., Lu, J., Wang, J., Wang, M., et al. (2025) · European Journal of Nuclear Medicine and Molecular Imaging · doi:10.1007/s00259-025-07279-y · Cited by 5 · see citing works
Yong, K. X. X., Petzold, A., Foster, P. J., Young, A., Bell, S., Bai, Y., Leff, A. P., Crutch, S. J., & Greenwood, J. A. (2024) · Behavior Research Methods · doi:10.3758/s13428-024-02448-7 · Cited by 4 · see citing works
Self-citationMultidimensional cognitive deficits in the typical and atypical variants of Alzheimer’s disease
Henderson, S. K., Murley, A. G., Cope, T., Bowns, L., Malpetti, M., Patterson, K. E., Rowe, J. B., & Ralph, M. A. L. (2025) · Alzheimer s Research & Therapy · doi:10.1186/s13195-025-01873-w · Cited by 3 · see citing works
Ramusino, M. C., Scanu, L., Gritti, L., Imbimbo, C., Farina, L. M., Cosentino, G., Perini, G., & Costa, A. C. (2024) · Journal of Alzheimer s Disease · doi:10.3233/jad-231123 · Cited by 3 · see citing works
Carton, A. M., Frost, C. D., Poole, T., Yang, B., McCarthy, I. D., Suzuki, T., Holloway, C., Serougne, R., Boampong, D. K., Sullivan, M. P., et al. (2024) · Brain Sciences · doi:10.3390/brainsci14121217 · Cited by 3 · see citing works
Self-citationNeuroimaging in posterior cortical atrophy: an integrative review for clinicians and radiologists
Porto, F. H. D. G., da Silva, M. M., Bravo, C. S. G. J., Porto, G., de Paula, W. A. B. G., Fernandes, S. J. S., Otta, G., & Plant, G. T. (2025) · Frontiers in Neurology · doi:10.3389/fneur.2025.1631245 · Cited by 2 · see citing works
Chiabotti, P. S., Nasuti, M., Rouaud, O., & Allali, G. (2025) · Journal of Alzheimer's Disease · doi:10.1177/13872877251342266 · Cited by 2 · see citing works
FDG PET Imaging Evaluation of Neurodegenerative Dementias
Minoshima, S., Thientunyakit, T., Cross, D. J., & Mosci, K. (2023) · Springer eBooks · doi:10.1007/978-3-031-35098-6_4 · Cited by 1 · see citing works
Mitchell, A., Kelso, W. M., Paynter, C., Hayes, L., Velakoulis, D., & Loi, S. M. (2024) · International Journal of Environmental Research and Public Health · doi:10.3390/ijerph21040513 · Cited by 1 · see citing works
Towards realistic simulation of disease progression in the visual cortex with CNNs
Moore, J. A., Kang, C., Vigneshwaran, V., Stanley, E. A. M., Memon, A., Wilms, M., & Forkert, N. D. (2025) · Scientific Reports · doi:10.1038/s41598-025-89738-y · Cited by 1 · see citing works
Waddington, C., Clements, H., Crutch, S. J., Davis, M., Glenister, J., Harding, E., Thompson, E. H., Walton, J., & Stott, J. C. H. (2025) · Aging & Mental Health · doi:10.1080/13607863.2025.2502793 · Cited by 1 · see citing works
Self-citationAPOE Genotyping in Cognitive Disorders: Preliminary Observations from the Greek Population
Athanasaki, A., Tsantzali, I., Κroupis, C., Theodorou, A., Boufidou, F., Constantinides, V. C., Tzartos, J. S., Tzartos, S. J., Velonakis, G., Zompola, C., et al. (2025) · International Journal of Molecular Sciences · doi:10.3390/ijms26157410 · Cited by 1 · see citing works
Garcia, D. M., Buitrago, C., Forero, L. V., Moreno, M. J., Caballero, C. J. R., Reyes, P. A., & Matallana, D. (2023) · Alzheimer s & Dementia · doi:10.1002/alz.083239
Os tratamentos atuais para a Síndrome de Benson: uma revisão da literatura
Macêdo, J. A., Hermano, A. C., Gomes, S. A., Vaz, M. E. P., & da Silva, L. J. (2024) · Cuadernos de Educación y Desarrollo · doi:10.55905/cuadv16n2-ed.esp.326
Alzheimer’s visual variant: a report of a diagnosis easily missed on ophthalmic examination
Cárdenas‐Belaunzarán, J., Cerrillo-Avila, K. A., & Independent Researcher, M. C. 0. (2024) · International Journal of Ophthalmology · doi:10.18240/ijo.2024.08.24
Preserved musical working memory and absolute pitch in posterior cortical atrophy
Jiang, J., Brotherhood, E., Core, L. B., Hardy, C. J. D., Yong, K. X. X., Foulkes, A., & Warren, J. D. (2024) · Cortex · doi:10.1016/j.cortex.2024.09.009
Self-citationA Case of Posterior Cortical Atrophy in Alzheimer’s Disease
KAFTANIC, C. T., Muddana, N., & Meng, R. (2024) · Cureus · doi:10.7759/cureus.74403
An atypical phenotype is a risk factor for higher mortality in Alzheimer’s diseasePreprint
Bader, I., Groot, C., van der Flier, W. M., Pijnenburg, Y. A. L., & Ossenkoppele, R. (2024) · medRxiv · doi:10.1101/2024.12.16.24319084
Self-citationMultidimensional cognitive deficits in the typical and atypical variants of Alzheimer’s diseasePreprint
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An uncommon variant of Alzheimer’s disease: Posterior cortical atrophy
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Digital dementia and testing of cognitive intervention for degenerating neural networks
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Lecanemab for posterior cortical atrophy: Two contrasting cases
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TOSS-PCA: Tyrannosaurus Optimized Features Based Posterior Cortical Atrophy Classification
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MRI correlates of emotion recognition in vascular dementia: An empty systematic review
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Multi-case review of posterior cortical atrophy in a neuropsychology clinic
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Atypical Alzheimer disease: a multi-axis framework toward defining heterogeneity
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Posterior cortical atrophy: A seven-year single case study of neurocognition and adaptation
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- Authors
- Yong, K. X. X., Graff-Radford, J., Ahmed, S., Chapleau, M., Ossenkoppele, R., Putcha, D., Rabinovici, G. D., Suarez-Gonzalez, A., Schott, J. M., Crutch, S., & Harding, E.
- Affiliations
- UCL Queen Square Institute of Neurology (GB)
- University of Oxford (GB)
- University of California, San Francisco (US)
- Lund University (SE)
- Harvard University (US)
- Corresponding author
- Yong, Keir X. X.
- ORCID
- Venue
- Current Treatment Options in Neurology
- Published
- 2023-02
- Volume
- 25
- Issue
- 2
- Pages
- 23-43
- ISSN
- 1092-8480 (ISSN-L)
- 1534-3138
- PMC
- PMC9935654
- OpenAlex
- W4319596989
- Cited by
- 51
- References
- 97
- Citations by year
- 2026: 10 · 2025: 17 · 2024: 20 · 2023: 4
- FWCI
- 2.33
- Type
- journal article
- Language
- en
- Update notices
- correction (2023-03-21)
- Open access
- hybrid
- doi.org
- Locations
- Current Treatment Options in Neurology (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- Pure Amsterdam UMC (publishedVersion, cc-by)
- Amsterdam UMC (VU Amsterdam) - Institutional Repository (publishedVersion, other-oa)
- CentAUR (University of Reading) (submittedVersion)
- UCL Discovery (University College London) (submittedVersion, cc-by)
- eScholarship (California Digital Library) (submittedVersion, cc-by)
- Lund University Publications (Lund University) (submittedVersion)
- Data Archiving and Networked Services (DANS) (submittedVersion, other-oa)
- Funders
- Alzheimer's Society (AS-JF-18-003)
- NIH Clinical Center (R01EY027964, AG050603, P30-AG062422, R35 AG072362)
- Wellcome Trust (204841/Z/16/Z)
- Alzheimer's Association (ZEN-21-848216)
- ESRC-NIHR (ES/S010467/1, ES/L001810/1)
- NIHR (cov-lt2-0014)
- Economic and Social Research Council (ES/W006014/1)
- Alzheimer Society (AS-PG-14-022)
- 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 › Parkinson's Disease Mechanisms and Treatments
- Keywords
- posterior cortical atrophy, diagnosis, management, Alzheimer's disease pathology, biomarkers, neuropsychological assessment, clinical criteria, misdiagnosis, non-pharmacological treatment, disease-modifying therapy, multidisciplinary care, psychosocial impact
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