ABSTRACT
Impairments of object recognition are core features of neurodegenerative syndromes, in particular posterior cortical atrophy (PCA; the 'visual-variant Alzheimer's disease'). These impairments arise from damage to higher-level cortical visual regions and are often missed or misattributed to common ophthalmological conditions. Consequently, diagnosis can be delayed for years with considerable implications for patients. We report a new test for the rapid measurement of cortical visual loss - the Graded Incomplete Letters Test (GILT). The GILT is an optimised psychophysical variation of a test used to diagnose cortical visual impairment, which measures thresholds for recognising letters under levels of increasing visual degradation (decreasing "completeness") in a similar fashion to ophthalmic tests. The GILT was administered to UK Biobank participants (total n=2,359) and participants with neurodegenerative conditions characterised by initial cortical visual (PCA, n=18) or memory loss (typical Alzheimer's disease, n=9). UK Biobank participants, including both typical adults and those with ophthalmological conditions, were able to recognise letters under low levels of completeness. In contrast, participants with PCA consistently made errors with only modest decreases in completeness. GILT sensitivity to PCA was 83.3% for participants reaching the 80% accuracy cut-off, increasing to 88.9% using alternative cut-offs (60% or 100% accuracy). Specificity values were consistently over 94% when compared to UK Biobank participants without or with documented visual conditions, regardless of accuracy cut-off. These first-release UK Biobank and clinical verification data suggest the GILT has utility in both rapidly detecting visual perceptual losses following posterior cortical damage and differentiating perceptual losses from common eye-related conditions.
Subject(s)
Alzheimer Disease , Humans , Female , Male , Aged , United Kingdom , Middle Aged , Alzheimer Disease/physiopathology , Alzheimer Disease/diagnosis , Adult , Vision Disorders/diagnosis , Vision Disorders/physiopathology , Biological Specimen Banks , Aged, 80 and over , Neuropsychological Tests , UK BiobankABSTRACT
Posterior cortical atrophy (PCA) describes a neurodegenerative syndrome characterized by progressive difficulties in cortical visual and other posterior cortical functions consistent with parieto-occipital and occipito-temporal involvement. It is increasingly recognized that many patients develop difficulties with other aspects of daily living, in particular, with language and communication. We present a case emphasizing how language difficulties may emerge in PCA. Difficulties are interpreted as arising from interacting effects of linguistic deficits and impaired detection of nonverbal (particularly, visual) turns that normally facilitate, schedule, and disambiguate the exchange of verbal messages between speakers. We propose that relatively simple speech and language therapy interventions may hold promise in addressing language and communication difficulties as secondary features of PCA by targeting the behaviors of both the person with PCA and their communication partners.