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INTRODUCTION: Delay in diagnosis of posterior cortical atrophy (PCA) syndrome is common, and the lack of familiarity with assessment tools for identifying visual cortical dysfunction is a contributing factor. We propose recommendations for the approach to the evaluation of PCA clinical features during the office visit, the neuropsychological evaluation, and the research setting. A recommended screening battery for eye clinics is also proposed. METHODS: Recommendations were developed using results from a web‐based survey of members of Alzheimer's Association International Society to Advance Alzheimer's Research and Treatment (ISTAART) Atypical Alzheimer's Disease Professional Interest Area (PIA), literature review, and consensus by the PCA assessment working party of the Atypical Alzheimer's Disease PIA. RESULTS: Survey results revealed robust agreement for assessment tool preferences for PCA features, and many respondents indicated that they reserve assessment tools for use only when PCA is suspected. For some PCA features, curated tools were preferred over validated battery tools, particularly for the office visit. Consensus recommendations superseded survey preferences for two core cognitive features within the 2017 PCA diagnostic criteria. DISCUSSION: These consensus recommendations provide an evaluation framework for PCA clinical features and can facilitate timely and accurate recognition and diagnosis of PCA. Broader use of these tools should be sought, and development and validation of novel PCA clinical outcome assessments are needed to improve our understanding of atypical AD and other dementias and support the inclusion of those with PCA in treatment trials. [ABSTRACT FROM AUTHOR]

Abstract INTRODUCTION Delay in diagnosis of posterior cortical atrophy (PCA) syndrome is common, and the lack of familiarity with assessment tools for identifying visual cortical dysfunction is a contributing factor. We propose recommendations for the approach to the evaluation of PCA clinical features during the office visit, the neuropsychological evaluation, and the research setting. A recommended screening battery for eye clinics is also proposed. METHODS Recommendations were developed using results from a web‐based survey of members of Alzheimer's Association International Society to Advance Alzheimer's Research and Treatment (ISTAART) Atypical Alzheimer's Disease Professional Interest Area (PIA), literature review, and consensus by the PCA assessment working party of the Atypical Alzheimer's Disease PIA. RESULTS Survey results revealed robust agreement for assessment tool preferences for PCA features, and many respondents indicated that they reserve assessment tools for use only when PCA is suspected. For some PCA features, curated tools were preferred over validated battery tools, particularly for the office visit. Consensus recommendations superseded survey preferences for two core cognitive features within the 2017 PCA diagnostic criteria. DISCUSSION These consensus recommendations provide an evaluation framework for PCA clinical features and can facilitate timely and accurate recognition and diagnosis of PCA. Broader use of these tools should be sought, and development and validation of novel PCA clinical outcome assessments are needed to improve our understanding of atypical AD and other dementias and support the inclusion of those with PCA in treatment trials.

Introduction: Delay in diagnosis of posterior cortical atrophy (PCA) syndrome is common, and the lack of familiarity with assessment tools for identifying visual cortical dysfunction is a contributing factor. We propose recommendations for the approach to the evaluation of PCA clinical features during the office visit, the neuropsychological evaluation, and the research setting. A recommended screening battery for eye clinics is also proposed., Methods: Recommendations were developed using results from a web-based survey of members of Alzheimer's Association International Society to Advance Alzheimer's Research and Treatment (ISTAART) Atypical Alzheimer's Disease Professional Interest Area (PIA), literature review, and consensus by the PCA assessment working party of the Atypical Alzheimer's Disease PIA., Results: Survey results revealed robust agreement for assessment tool preferences for PCA features, and many respondents indicated that they reserve assessment tools for use only when PCA is suspected. For some PCA features, curated tools were preferred over validated battery tools, particularly for the office visit. Consensus recommendations superseded survey preferences for two core cognitive features within the 2017 PCA diagnostic criteria., Discussion: These consensus recommendations provide an evaluation framework for PCA clinical features and can facilitate timely and accurate recognition and diagnosis of PCA. Broader use of these tools should be sought, and development and validation of novel PCA clinical outcome assessments are needed to improve our understanding of atypical AD and other dementias and support the inclusion of those with PCA in treatment trials., Competing Interests: V.S.P.: Research is supported by National Institutes of Health (NIH), the John and Joanne Hare Fund for Accelerating PCA Research, University of Colorado Alzheimer's and Cognition Center, the North American Neuro‐ophthalmology Society, and Institutional support from the Lewy Body Dementia Association; also received support from the American Academy of Neurology for editorial work, royalties from Up‐to‐Date, Inc., the Alzheimer's Association for the Longitudinal Early‐Onset Alzheimer's Disease Study Patient Advocacy Committee, and Biogen and Eisai. B.F.B.: Honorarium for the Tau Consortium. Institutional research grant support: Alector, Biogen, Transposon, Cognition Therapeutics, GE Healthcare; Institutional NIH grant support: P30 AG062677, U19 AG063911, R01 AG038791, U01 NS100620, U19 AG071754, U24 AG056270. Institutional foundation support: Lewy Body Dementia Association, American Brain Foundation. Institutional philanthropic support: Mayo Clinic Dorothy and Harry T. Mangurian Jr. Lewy Body Dementia Program, the Little Family Foundation, the Ted Turner and Family Functional Genomics Program. G.R.: Research support from NIH, Alzheimer's Association, American College of Radiology, Rainwater Charitable Foundation, Avid Radiopharmaceuticals, GE Healthcare, Genentech, Life Molecular Imaging. In the last 36 months, he has served as a paid consultant on scientific advisory boards for Alector, Eli Lilly, Roche, Genentech, GE Healthcare, Merck, and as a paid member of Data and Safety Monitoring Board for Johnson & Johnson. He is an Associate Editor for JAMA Neurology. R.A.T.: Honoraria as an advisory board member from C2N Diagnostics. G.S.D.: Research is supported by NIH (K23AG064029, U01AG057195, U19AG032438), the Alzheimer's Association, and Chan Zuckerberg Initiative. He serves as a consultant for Parabon Nanolabs Inc, as a Topic Editor (Dementia) for DynaMed (EBSCO), and as the Clinical Director of the Anti‐NMDA Receptor Encephalitis Foundation (Inc, Canada; uncompensated). He is the co‐Project PI for a clinical trial in anti‐NMDAR encephalitis, which receives support from Horizon Pharmaceuticals. He has developed educational materials for PeerView Media, Inc., and Continuing Education Inc. He owns stock in ANI Pharmaceuticals. Dr. Day's institution has received support from Eli Lilly for Dr. Day's development and participation in an educational event promoting early diagnosis of symptomatic Alzheimer's disease. B.D.C.B.: Receives funding from Alzheimer Nederland (#WE.15‐2019‐13, #WE.03‐2021‐15). C.U.O.: Research support from Alector, Transposon, and Denali, and consulting work from Acadia, Reata—consulting. H.S.: Full‐time employee of the Alzheimer's Association and spouse employed by Abbott Laboratories. K.Y. is an Etherington PCA Senior Research Fellow and is funded by the Alzheimer's Society, grant number 453 (AS‐JF‐18‐003) and a NIH grant (R01EY027964), and reports support from Roche outside the submitted work. All other authors report no conflict‐of‐interest disclosures. Author disclosures are available in the Supporting Information., (© 2023 The Authors. Alzheimer's & Dementia: Diagnosis, Assessment & Disease Monitoring published by Wiley Periodicals, LLC on behalf of Alzheimer's Association.)