Diagnosis and Management of Posterior Cortical Atrophy

Abstract

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.

Keywords: Alzheimer’s disease; Atypical Alzheimer’s disease; Posterior cortical atrophy; Treatment; Visual processing; Visual variant Alzheimer’s disease.

Fig. 1

A T2 axial FLAIR MRI demonstrating parieto-occipital atrophy in a patient with PCA. B Fluorodeoxyglucose (FDG)-positron emission tomography (PET) scans with statistical maps showing regions of significant hypometabolism relative to age-matched controls (GE cortex ID): i PCA patient with predominantly right occipital-parietal-temporal hypometabolism with prominent environmental agnosia and dressing apraxia and ii PCA patient with predominantly left occipital-parietal-temporal hypometabolism with prominent Gerstmann features including acalculia. C Tau-PET (flortaucipir) scan with predominant parieto-occipital tracer uptake in a patient with PCA.