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.

Atypical forms of Alzheimer's disease: patients not to forget

“…Two main clinical variants of PCA have been described, depending on the main location of the brain damage: the biparietal variant, anatomically corresponding to an impairment of the dorsal visual pathway; the occipitotemporal variant corresponding to an impairment of the ventral visual pathway [15][16][17] (Fig. 2).…”

Potential ocular indicators to distinguish posterior cortical atrophy and typical Alzheimer’s disease: a cross-section study using optical coherence tomography angiography

“…Posterior cortical atrophy (PCA) is a neurodegenerative disease characterized by a gradual and selective functional impairment and structural alternation of the parietal and occipital lobes, with an early onset between 50 and 65 years. Visual impairment and cognitive failure associated with parieto-occipital cortical atrophy such as dysmetria, alexia, and ignorance are the main symptoms in PCA patients, while the routine ophthalmic clinical examination frequently cannot find obvious abnormalities of ocular optic structures [ 1 , 2 ]. Although PCA patients frequently share similar pathologic characteristic with Alzheimer’s disease (AD), they can exhibit different patterns of clinical manifestations and structural and/or functional neuroimaging [ 3 ].…”

Peer Support for Caregivers of People Living with Posterior Cortical Atrophy in Melbourne, Australia: A Feasibility Study

“…PCA is most commonly associated with pathology related to AD; however, other rare associations include Lewy body disease, corticobasal degeneration (CBD), and prion disease [3,4]. In the natural history of PCA, the earliest clinical findings include the aforementioned visuospatial and visuoperceptual deficits, followed later by deficits in language, executive function, and memory, as well as progression to functional blindness [1,5]. Diagnosis of PCA involves neurological and neuropsychological evaluation, as well as structural brain imaging, usually via magnetic resonance imaging (MRI).…”

“…Diagnosis of PCA involves neurological and neuropsychological evaluation, as well as structural brain imaging, usually via magnetic resonance imaging (MRI). Functional imaging such as fluorodeoxyglucose positron emission tomography (FDG-PET) and single-photon emission computed tomography (SPECT) may be utilised if further imaging is required [5]. Cerebrospinal fluid confirming the presence of AD pathology, low amyloid-β (Aβ1-42), and raised total-and phosphorylated-tau or plasma biomarkers may be useful in both ruling out alternative causes of symptoms or confirming the underlying pathological process driving PCA [5].…”

“…Functional imaging such as fluorodeoxyglucose positron emission tomography (FDG-PET) and single-photon emission computed tomography (SPECT) may be utilised if further imaging is required [5]. Cerebrospinal fluid confirming the presence of AD pathology, low amyloid-β (Aβ1-42), and raised total-and phosphorylated-tau or plasma biomarkers may be useful in both ruling out alternative causes of symptoms or confirming the underlying pathological process driving PCA [5]. The most recent international consortium criteria include clinical features (insidious onset, gradual progression, early disturbance of visual with or without posterior cognitive features); at least three cognitive features (such as Gerstmann's syndrome-acalculia, right-left disorientation, finger agnosia, agraphia, apraxia, alexia and simultanagnosia); and relatively spared memory function, speech and non-visual language function, executive function, and behaviour and personality, with predominant occipitoparietal or occipitotemporal atrophy or hypometabolism on MRI/FDG-PET/SPECT [6].…”