[en] Many researchers have recently explored the cognitive profile of velocardiofacial syndrome (VCFS), a neurodevelopmental disorder linked to a 22q11.2 deletion. However, verbal short-term memory has not yet been systematically investigated. We explored verbal short-term memory abilities in a group of 11 children and adults presenting with VCFS and two control groups, matched on either CA or vocabulary knowledge, by distinguishing short-term memory for serial order and item information. The VCFS group showed impaired performance on the serial order short-term memory tasks compared to both control groups. Relative to the vocabulary-matched control group, item short-term memory was preserved. The implication of serial order short-term memory deficits on other aspects of cognitive development in VCFS (e.g., language development, numerical cognition) is discussed.
Adams, A. M., & Gathercole S. E. (2000). Limitations in working memory: Implications for language development. International Journal of Language and Communication Disorders, 35, 95-116.
Avons, S. E., Wragg, C. A., Cupples, L., & Lovegrove, W. J. (1998). Measures of phonological short-term memory and their relationship to vocabulary development. Applied Psycholinguistics, 19, 583-601.
Bearden, C. E., Woodin, M. F., Wang, P., Moss E., McDonald-McGinn, D. M., Zackai, E. H., Emanuel, B. S., & Cannon, T. D. (2001). The neurocognitive phenotype of 22q11.2 deletion syndrome: Selective deficit in visuo-spatial memory. Journal of Clinical and Experimental Neuropsychology, 23, 447-464.
Brock, J., & Jarrold, C. (2004). Language influences on verbal short-term memory performance in Down syndrome: Item and order recognition. Journal of Speech, Language and Hearing Research, 47, 1334-1346.
Brock, J., McCormack, T., & Boucher, J. (2005). Probed serial recall in Williams syndrome: Lexical influences on phonological short-term memory. Journal of Speech, Language and Hearing Research, 48, 360-371.
Brown, G. D. A., Preece, T., & Hulme, C. (2000). Oscillator-based memory for serial order. Psychological Review, 107, 127-181.
Burgess, N., & Hitch, G. J. (1999). Memory for serial order: A network model of the phonological loop and its timing. Psychological Review, 106, 551-581.
Carlson, C., Sirotkin, H., Pandita, R., Goldberg, R., McKie, J., Wadey, R., Patanjali, S. R., Weissman, S. M., Anyane-Yeboa, K., & Warburton, D. (1997). Molecular definition of 22q11 deletions in 151 velo-cardio-facial syndrome patients. American Journal of Human Genetics, 61, 620-629.
Chochon, F., Cohen, L., van de Moortele, P. F., & Dehaene, S. (1999). Differential contributions of the left and right inferior parietal lobules to number processing. Journal of Cognitive Neuroscience, 11, 617-630.
Content, A., Mousty, P., & Radeau, M. (1990). BRULEX. Une base de donnees lexicales informatisée pour le français ecrit et parle. [BRULEX: A computerized lexical database for the French language] Année Psychologique, 90, 551-566.
Dehaene, S. (2000). Cerebral basis of number processing and calculation. In M. S. Gazzaniga (Ed.), The new cognitive neurosciences (pp. 987-998). Cambridge: MIT Press.
De Smedt, B., Swillen, A., Ghesquiere, P., DeVriendt, K., & Fryns, J. P. (2003). Pre-academic and early academic achievement in children with velocardiofacial syndrome (del22q11.2) of borderline or normal intelligence. Genetic Counseling, 14, 15-29.
Driscoll, D. A., Salvin, J., Sellinger, B., Budarf, M. L., McDonald-McGinn, D. M., Zackai, E. H., & Emanuel, B. S. (1993). Prevalence of 22q11 microdeletions in DiGeorge and velocardiofacial syndromes: Implications for genetic counselling and prenatal diagnosis. Journal of Medical Genetics, 30, 813-817.
Dunn, L. M., Thériault-Whalen, C. M., & Dunn, L. M. (1993). Echelle de vocabulaire en images Peabody. Adaptation française du Peabody Picture Vocabulary Test. Toronto, Canada: Psycan
Eliez, S., Schmitt, J. E., White, C. D., & Reiss, A. L. (2000). Children and adolescents with velocardiofacial syndrome: A volumetric MRI study. American Journal of Psychiatry, 157, 409-415.
Eliez, S., White, C. D., Menon, V., White, C. D., Schmitt, J. E., & Reiss, A. L. (2003). Functional brain imaging study of mathematical reasoning abilities in velocardiofacial syndrome (del22q11.2). Genetics in Medicine, 3, 49-55.
Gathercole, S. E., & Baddeley, A. D. (1990). Phonological memory deficits in language disordered children: Is there a causal connection? Journal of Memory and Language, 29, 336-360.
Gathercole, S. E., & Baddeley, A. D. (1993). Phonological working memory: A critical building block for reading development and vocabulary acquisition? European Journal of Psychology of Education, 8, 259-272.
Gathercole, S. E., Frankish, C. R., Pickering, S. J., & Peaker, S. (1999). Phonotactic influences on short-term memory. Journal of Experimental Psychology: Human Learning and Memory, 25, 84-95.
Gathercole, S. E., Willis, C. S., Emslie, H., & Baddeley, A. D. (1992). Phonological memory and vocabulary development during the early school years: A longitudinal study. Developmental Psychology, 28, 887-898.
Gerdes, M., Solot, C., Wang, P. P., Moss, E., Larossa, D., Randall, P., Goldmuntz, E., Clark, B. J., Driscoll, D. A., Jawad, A. et al. (1999). Cognitive and behavior profile of preschool children with chromosome 22q11.2 deletion. American Journal of Medical Genetics, 85, 127-133.
Glaser, B., Mumme, D. L., Blasey, C., Morris, M. A., Dahoun, S. P., Antonarakis, S. E., Reiss, A. L., & Eliez, S. (2002). Language skills in children with velocardiofacial syndrome (deletion 22q11.2). Journal of Pediatrics, 140, 753-758.
Goldberg, R., Motzkin, B., Marion, R., Scambler, P. J., & Shprintzen, R. J. (1993). Velo-cardiofacial syndrome: A review of 120 patients. American Journal of Medical Genetics, 45, 313-319.
Golding-Kushner, K. J., Weller, G., & Shprintzen, R. J. (1985). Velo-cardio-facial syndrome: Language and psychological profiles. Journal of Craniofacial Genetics and Developmental Biology, 5, 259-266.
Gupta, P. (2003). Examining the relationship between word learning, nonword repetition and immediate serial recall in adults. Quarterly Journal of Experimental Psychology, 56A, 1213-1236.
Gupta, P., & MacWhinney, B. (1997). Vocabulary acquisition and verbal short-term memory: Computational and neural bases. Brain and Language, 59, 267-333.
Henry, J. C., Van Amelsvoort, T., Morris, R. G., Owen, M. J., Murphy, D. G. M., & Murphy, K. C. (2002). An investigation of the neuropsychological profile in adults with velocardiofacial syndrome (VCFS). Neuropsychologia, 40, 471-478.
Henson, R. N. A. (1998). Short-term memory for serial order: The start-end model. Cognitive Psychology, 36, 73-137.
Henson, R., Hartley, T., Burgess, N., Hitch, G., & Flude, B. (2003). Selective interference with verbal short-term memory for serial order information: A new paradigm and tests of a timing-signal hypothesis. Quarterly Journal of Experimental Psychology, 56A, 1307-1334.
Kok, L. L., & Solman, R. T. (1995). Velocardiofacial syndrome: Learning difficulties and intervention. Journal of Medical Genetics, 32, 612-618.
Lindsay, E. A., Morris, M. A., Gos, A., Nestadt, G., Wolyniec, P. S., Lasseter, V. K., Shprintzen, R., Antonarakis, S. E., Baldini, A., & Pulver, A. E. (1995). Schizophrenia and chromosomal deletions within 22q11.2. American Journal of Medical Genetics, 56, 1502-1503.
Majerus, S., Barisnikov, K., Vuillemin, I., Poncelet, M., & Van der Linden, M. (2003). An investigation of verbal short-term memory and phonological processing in four children with Williams syndrome. Neurocase, 9, 390-401.
Majerus, S., Glaser, B., Van der Linden, M., & Eliez, S. (2006). A multiple case study of verbal short-term memory in VCFS. Journal of Intellectual Disability Research 50, 457-469.
Majerus, S., Poncelet, M., Elsen, B., & Van der Linden, M. (2006). Exploring the relationship between new word learning and short-term memory for serial order recall, item recall and item recognition. European Journal of Cognitive Psychology, 18, 848-873.
Majerus, S., Poncelet, M., Greffe, C., & Van der Linden, M. (2006). Relations between vocabulary development and verbal short-term memory: The relative importance of short-term memory for serial order and item information. Journal of Experimental Child Psychology, 93, 95-119.
Majerus, S., & Van der Linden, M. (2003). Long-term memory effects on verbal short-term memory: A replication study. British Journal of Developmental Psychology, 21, 303-310.
McCormack, T., Brown, G. D., Vousden, J. I., & Henson, R. N. A. (2000). Children's serial recall errors: Implications for theories of short-term memory development. Journal of Experimental Child Psychology, 76, 222-252.
Michas, I. C., & Henry, L. A. (1994). The link between phonological memory and vocabulary acquisition. British Journal of Developmental Psychology, 12, 147-164.
Moss, E. M., Batshaw, M. L., Solot, C. B., Gerdes, M., McDonald-McGinn, D. M., Driscoll, D. A., Emanuel, B. S., Zackai, E. H., & Wang, P. P. (1999). Psychoeducational profile of the 22q11.2 microdeletion: A complex pattern. Journal of Pediatrics, 134, 193-198.
Murphy, K. C. (2004). The behavioural phenotype in velo-cardio-facial syndrome. Journal of Intellectual Disability Research, 48, 524-530.
Murphy, K. C. (2005). Annotation: Velo-cardiofacial syndrome. Journal of Child Psychology and Psychiatry, 46, 563-571.
Poirier, M., & Saint-Aubin, J. (1996). Immediate serial recall, item identity and item position. Canadian Journal of Experimental Psychology, 50, 408-412.
Ravizza, S. M., Delgado, M. R., Chein, J. M., Becker, J. T., & Fiez, J. A. (2004). Functional dissociations within the inferior parietal cortex in verbal working memory. NeuroImage, 22, 562-573.
Ryan, A. K., Goodship, J. A., Wilson, D. I., Philip, N., Levy, A., Seidel, H. et al. (1997). Spectrum of clinical features associated with interstitial chromosome 22q11 deletions: A European collaborative study. Journal of Medical Genetics, 34, 798-804.
Scambler, P. J., Kelly, D., Lindsay, E., Williamson, R., Goldberg, R., Shprintzen, R., Wilson, D. I., Goodship, J. A., Cross, I. E., & Burn, J. (1992). Velo-cardio-facial syndrome associated with chromosome 22 deletions encompassing the DiGeorge locus. Lancet, 339, 1138-1139.
Scherer, N. J., D'Antonio, L. L., & Kalbfleisch, J. H. (1999). Early speech and language development in children with velocardiofacial syndrome. American Journal of Medical Genetics, 88, 714-723.
Scherer, N. J., D'Antonio, L. L., & Rodgers, J. R. (2001). Profiles of communication disorder in children with velocardiofacial syndrome: Comparison to children with Down syndrome. Genetics in Medicine, 3, 72-78.
Service, E. (1992). Phonology, working memory, and foreign-language learning. Quarterly Journal of Experimental Psychology: Human Experimental Psychology, 45A, 21-50.
Shprintzen, R. J., Goldberg, R., Golding-Kushner, K.J., & Marion, R. W. (1992). Late-onset psychosis in the velo-cardio-facial syndrome. American Journal of Medical Genetics, 42, 141-142.
Shprintzen, R. J., Goldberg, R. B., Lewin, M. L., Sidoti, E. J., Berkman, M. D., Argamaso, R. V., & Young, D. (1978). A new syndrome involving cleft palate, cardiac anomalies, typical facies, and learning disabilities: Velo-cardiofacial syndrome. Cleft Palate Journal, 15, 56-62.
Simon, T. J., Bearden, C. E., McDonald-McGinn, D. M., & Zackai, E. (2005). Visuo-spatial and numerical cognitive deficits in children with chromosome 22q11.2 deletion syndrome. Cortex, 41, 145-155.
Smyth, M. M., Hay, D. C., Hitch, G. J., & Horton, N. J. (2005). Serial position memory in the visual-spatial domain: Reconstructing sequences of unfamiliar faces. Quarterly Journal of Experimental Psychology, 58A, 909-930.
Solot C., Gerdes M., Kirschner R., McDonald-McGinn D., Moss, E., Woodin M., Aleman, D., Zackai, E. H., & Wang, P. P. (2001) Communication issues in 22q11.2 deletion syndrome: Children at risk. Genetics in Medicine, 3, 67-71.
Swillen, A., DeVriendt, K., Legius, E., Eyckens, B., Dumoulin, M., Gewillig, M., & Fryns, J. P. (1997). Intelligence and psychosocial adjustment in velocardiofacial syndrome: A study of 37 children and adolescents with VCFS. Journal of Medical Genetics, 34, 453-458.
Swillen, A., Vogel, S. A., Devriendt, K., & Fryns, J. P. (2000). Chromosome 22q11 deletion syndrome: Update and review of the clinical features, cognitive-behavioral spectrum, and psychiatric complications. American Journal of Medical Genetics, 97, 128-135.
Tezenas Du Montcel, S., Mendizabai, H., Ayme, S., Levy, A., & Philip, N. (1996). Prevalence of 22q11 microdeletion. Journal of Medical Genetics, 33, 719.
Todd, J. J., & Marois, R. (2004). Capacity limit of visual short-term memory in human posterior parietal cortex. Nature, 428, 751-754.
Tubach, J. L., & Boe, L. J. (1990). Un corpus de transcription phonétique. France: Telecom.
Vicari, S., Carlesimo, G., Brizzolara, D., & Pezzini, G. (1996). Short-term memory in children with Williams syndrome: A reduced contribution of lexical-semantic knowledge to word span. Neuropsychologia, 34, 919-925.
Wang, P. P., Woodin, M. F., Kreps-Falk, R., & Moss, E. M. (2000). Research on behavioral phenotypes: Velocardiofacial syndrome (deletion 22q11.2). Developmental Medicine and Child Neurology, 42, 422-427.