Emergence and progression of 'non-semantic' deficits in semantic dementia

Although semantic dementia (SD) is defined as a selective disruption of conceptual knowledge, a number of group studies have now demonstrated that SD patients also show impaired performance on tasks not usually considered to have a high semantic load (e.g., reading words aloud and lexical or object decision). The aim of the current study was to document the relative deterioration, over time, of a number of semantic and so-called 'non-semantic' tasks in LF, a single case of SD for whom - by virtue of his work as a published cartoonist - we also have extensive data regarding his pre-morbid linguistic and drawing skills. In five testing rounds over a period of five years we administered semantic tests of object naming and object definition (on both of which LF was progressively impaired, as expected for a diagnosis of SD), plus verbal and non-verbal 'non-semantic' tasks of reading aloud, spelling, object and lexical decision, and delayed copy drawing. Initially, his only striking 'non-semantic' deficit was in the domain of spelling - a pronounced surface dysgraphia in an individual with demonstrably superior pre-morbid spelling skill. Over time, and in line with his declining semantic system, LF's performance gradually deteriorated on all of the 'non-semantic' tasks. The most vulnerable items on most tasks were those with low frequency and an atypical form. This report adds to the growing body of evidence that a number of cognitive processes not usually considered to be 'semantic' in their demands rely on the integrity of semantic knowledge for successful execution. Furthermore, it provides the first indication that these non-semantic deficits might emerge in an order predictable from the typicality structure of the relevant domain. Crown Copyright (C) 2008 Published by Elsevier Srl. All rights reserved.