A multiple case study of verbal short-term memory in velo-cardio-facial syndrome

Background: Velo-cardio-facial syndrome (VCFS, 22q11.2 deletion) is characterized by severely delayed language development. The current study explored the integrity of verbal short-term memory (STM), a cognitive function critically involved in language development, in eight children with VCFS. Methods: Using a multiple case study design, we presented a series of STM tasks exploring immediate serial recall for word and non-word lists to eight children with VCFS (aged 8 - 12 years) and to chronological-age-matched control groups. A first task assessed the integrity of phonological coding in verbal STM by comparing recall for phonologically similar and dissimilar words. Subsequently, the interaction between verbal knowledge and STM capacity was investigated by comparing recall for high- and low-imageability words, for high- and low-frequency words, and for words and non-words. A final task assessed short-term serial order recognition for digit sequences. Results: When computing the number of items recalled in the word recall tasks, independently of their serial position, only one child presented consistent difficulties. Short-term recall of non-words was normal in each child. Phonological similarity and verbal knowledge influenced STM performance to a similar extent in children with VCFS and controls. On the other hand, when applying a strict serial recall criterion, difficulties with the word and non-word recall tasks were observed in most children. Half of the patients were also impaired in the serial order recognition task. Conclusions: Despite mild intellectual disability, it is possible for short-term retention capacities for verbal item information to be at an age-appropriate level in VCFS. However, STM for serial order information could be impaired more specifically