title: The oral spelling profile of posterior cortical atrophy and the nature of the graphemic representation.
abstract: Spelling is a complex cognitive task where central and peripheral components are involved in engaging resources from many different cognitive processes. The present paper aims to both characterize the oral spelling deficit in a population of patients affected by a neurodegenerative condition and to clarify the nature of the graphemic representation within the currently available spelling models. Indeed, the nature of graphemic representation as a linear or multi-componential structure is still debated. Different hypotheses have been raised about its nature in the orthographic lexicon, with one positing that graphemes are complex objects whereby quantity and identity are separately represented in orthographic representations and can thus be selectively impaired. Posterior cortical atrophy (PCA) is a neurodegenerative condition that mainly affects visuoperceptual and visuospatial functions. Spelling impairments are considered part of the disease. Nonetheless the spelling deficit has received little attention so far and often it has been interpreted in relation to peripheral impairments such as writing difficulties associated with visuoperceptual and visuospatial deficits. In the present study we provide a detailed characterization of the oral spelling profile in PCA. The data suggest that multiple deficits underpin oral spelling problems in PCA, with elements of surface and phonological dysgraphia but also suggesting the involvement of the graphemic buffer. A large phenotypic individual variability is reported. Moreover, the larger proportion and the specific nature of errors involving geminate (i.e., double) as compared to non-geminate (i.e., non-double) letters suggest that a further central impairment might be associated with the abstract graphemic representation of letter numerosity. The present study contributes to the clinical characterization of PCA and to the current debate in the cognitive literature on spelling models; findings, despite not definitive, support the hypothesis that graphemic representations are multidimensional mental objects that separately encode information about grapheme identity and quantity.
authors.affiliation: Dementia, Research Centre, UCL Institute of Neurology, University College London, London, UK. Electronic address: s.primativo@ucl.ac.uk.
authors.forename: Silvia
authors.initials: S
authors.lastname: Primativo
authors.affiliation: Dementia, Research Centre, UCL Institute of Neurology, University College London, London, UK.
authors.forename: Keir X X
authors.initials: KX
authors.lastname: Yong
authors.affiliation: Dementia, Research Centre, UCL Institute of Neurology, University College London, London, UK.
authors.forename: Timothy J
authors.initials: TJ
authors.lastname: Shakespeare
authors.affiliation: Dementia, Research Centre, UCL Institute of Neurology, University College London, London, UK.
authors.forename: Sebastian J
authors.initials: SJ
authors.lastname: Crutch
mesh_terms: D000368:Aged; D000381:Agraphia / Q000000981:diagnostic imaging / Q000209:etiology / Q000523:psychology*; D001284:Atrophy; D001921:Brain / Q000000981:diagnostic imaging; D001927:Brain Diseases / Q000150:complications / Q000000981:diagnostic imaging / Q000523:psychology*; D005260:Female; D006801:Humans; D007807:Language Tests; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D019636:Neurodegenerative Diseases / Q000150:complications / Q000000981:diagnostic imaging / Q000523:psychology*; D009483:Neuropsychological Tests; D013060:Speech
keywords: Geminate words; Graphemic representation; Oral spelling; Posterior cortical atrophy