Yong Keir X X, Graff-Radford Jonathan, Ahmed Samrah, Chapleau Marianne, Ossenkoppele Rik, Putcha Deepti, Rabinovici Gil D, Suarez-Gonzalez Aida, Schott Jonathan M, Crutch Sebastian, Harding Emma
Dementia Research Centre, UCL Queen Square Institute of Neurology, Box 16, Queen Square, London, WC1N 3BG UK.
Department of Neurology, Mayo Clinic, Rochester, MN USA.
Curr Treat Options Neurol. 2023;25(2):23-43. doi: 10.1007/s11940-022-00745-0. Epub 2023 Feb 8.
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 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.
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.
本研究旨在总结后皮质萎缩（PCA）诊断和管理的近期进展。我们介绍了目前为改善PCA特征描述所做的努力，以及关于在PCA诊断和管理中使用临床、神经心理学和生物标志物方法的建议，并强调了当前的知识空白。
近期的多中心共识建议提供了PCA标准，对不同的管理策略（如针对临床特征和/或疾病）具有启示意义。研究强调了PCA背后原发性或并存的阿尔茨海默病（AD）病理的优势。管理PCA症状方法的证据主要来自小型研究。
PCA诊断常常延迟，患者很可能被误诊为眼部或心理疾病。目前对PCA的治疗是对症治疗——药物治疗和非药物治疗——大多数治疗选择的使用基于小型研究或专家意见。非药物治疗方法的建议包括根据PCA的临床特征——视觉空间特征而非以记忆为主导、主要为青年发病——以及社会心理影响进行跨学科管理。虽然新兴的疾病修饰疗法尚未在PCA中进行测试，但在针对AD和PCA其他罕见病因的疾病修饰疗法出现的情况下，准确及时地诊断PCA并确定潜在病理变得越来越重要。
