A Longitudinal Study of Cognitive Abilities in Williams Syndrome

Williams syndrome is a rare genetic disorder characterized by a unique physiological and behavioral profile, involving excessive sociability and relatively spared linguistic abilities in spite of mild to moderate mental retardation. The present study examines the narrative development of children with Williams syndrome and, for the first time, compares their performance to typically developing chronological-age matched children to examine the development of both structural linguistic abilities as well as the use of evaluation to elaborate and enrich narrative. Thirty children with Williams syndrome (5- through 10-years-old) and 30 typically developing age- and gender-matched comparison children were asked to tell a story from a wordless picture book. Results indicated that as a group, children with Williams syndrome committed significantly more morphological errors and used less complex syntax than comparison children, not surprising considering their language delay and impaired cognitive abilities. Significantly, children with Williams syndrome greatly exceeded comparison children in their elaboration and use of evaluative devices and showed particular preference for types of evaluation which serve as social engagement devices, reflecting their profile of excessive sociability. (Williams syndrome, Narrative, Evaluation)

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Age-Associated Memory Loss: Initial Neuropsychological and Cerebral Metabolic Findings of a Longitudinal Study

International Psychogeriatrics ◽

10.1017/s1041610294001596 ◽

1994 ◽

Vol 6 (1) ◽

pp. 23-44 ◽

Cited By ~ 22

Author(s):

Gary W. Small ◽

Anna Okonek ◽

Mark A. Mandelkern ◽

Asenath La Rue ◽

Linda Chang ◽

...

Keyword(s):

Alzheimer’S Disease ◽

Alzheimer's Disease ◽

Longitudinal Study ◽

Cognitive Abilities ◽

Mood State ◽

Familial Risk ◽

Memory Loss ◽

Subjective Memory ◽

Age Related ◽

Positron Emission

To determine the relationships between clinical and brain function in persons with a familial risk for Alzheimer's disease, the authors assessed subjective and objective cognitive abilities, mood state, and cerebral glucose metabolism (using positron emission tomography) in 43 persons with age-associated memory impairment, with and without first-degree relatives with a clinical diagnosis of Alzheimer's disease. Subjective complaints of memory loss, mood state ratings, and objective memory measures were similar in persons with a family history of Alzheimer's disease (n = 29) compared to those without such a history (n = 14). Metabolic ratios in the frontal regions correlated with a decrease in a specific type of subjective memory complaint (mnemonics usage; p < .001) and some mood state ratings. These results indicate that parietal and temporal hypometabolism is not evident in persons with mild age-related memory complaints, even when such subjects have a familial risk for Alzheimer's disease. Moreover, self-reports of mnemonics usage may be sensitive indicators of decreased frontal lobe function. Longitudinal study will determine whether such clinical and metabolic measures will predict eventual disease progression.

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Music Affinity and Emotion in Williams Syndrome

Music and Medicine ◽

10.47513/mmd.v12i2.686 ◽

2020 ◽

Vol 12 (2) ◽

pp. 122

Author(s):

Philip To Lai

Keyword(s):

Williams Syndrome ◽

Cognitive Abilities ◽

Genetic Disorder ◽

Cognitive Profile ◽

Typically Developing ◽

Spatial Skills ◽

Moderate Intellectual Disability ◽

Reasoning Abilities ◽

Neuropsychological Profile ◽

Conceptual Reasoning

Williams syndrome (WS) is a neurodevelopmental genetic disorder. Of interest to cognitive scientists is the uneven cognitive profile that is characteristic of the syndrome. In spite of mild to moderate intellectual disability, a complex pattern of strengths and weaknesses is found in their cognitive abilities. Strengths are seen in linguistic abilities and facial processing whereas weaknesses include visuo-spatial skills and conceptual reasoning abilities. Numerous anecdotes exist regarding musical abilities in individuals with WS. To elaborate on the neuropsychological profile of WS and particularly to better understand the affinity to music in individuals with WS, the present study examined the perception and production of music in this population. Results revealed that overall, individuals with WS were more expressive than typically developing individuals when they were involved with music. This study serves to further characterize this unique social and cognitive profile of individuals with WS, adding an affinity to music as a characteristic of their phenotype.

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Cognitive Abilities in Old Age: Results from the Zurich Longitudinal Study on Cognitive Aging

Swiss Journal of Psychology ◽

10.1024/1421-0185.67.3.177 ◽

2008 ◽

Vol 67 (3) ◽

pp. 177-195 ◽

Cited By ~ 21

Author(s):

Daniel Zimprich ◽

Mike Martin ◽

Matthias Kliegel ◽

Myriam Dellenbach ◽

Philippe Rast ◽

...

Keyword(s):

Longitudinal Study ◽

Cognitive Ability ◽

Old Age ◽

Processing Speed ◽

Cognitive Abilities ◽

Cognitive Aging ◽

Verbal Learning ◽

General Cognitive Ability ◽

Cross Sectional ◽

Verbal Knowledge

The Zurich Longitudinal Study on Cognitive Aging (ZULU) is an ongoing longitudinal study on the structure and development of cognition in old age. At the first assessment, the N = 364 participants had an average age of 73 years (age range: 65-80 years), and 46% were female. In total, a battery of 14 cognitive tests, including five consecutive verbal learning trials, were administered and adequately described by a measurement model of six first-order factors (processing speed, working memory, reasoning, learning, memory, and verbal knowledge) and one second-order factor of general cognitive ability. The cross-sectional age relations of the six cognitive abilities were, apart from processing speed and verbal knowledge, mediated by the general cognitive ability factor. From a conceptual perspective, these results imply that cognitive aging is not a completely uniform process driven by a single causal variable.

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Mediators of the Effect of Childhood Socioeconomic Status on Late Midlife Cognitive Abilities: A Four Decade Longitudinal Study

Innovation in Aging ◽

10.1093/geroni/igy003 ◽

2018 ◽

Vol 2 (1) ◽

Cited By ~ 9

Author(s):

Asad Beck ◽

Carol E Franz ◽

Hong Xian ◽

Eero Vuoksimaa ◽

Xin Tu ◽

...

Keyword(s):

Socioeconomic Status ◽

Longitudinal Study ◽

Indirect Effects ◽

Cognitive Abilities ◽

Leisure Activities ◽

Cognitive Outcomes ◽

Abstract Reasoning ◽

Multiple Mediation ◽

Childhood Socioeconomic Status ◽

Late Midlife

Abstract Background and Objectives Childhood socioeconomic status (cSES) is found to predict later-life cognitive abilities, yet the mechanisms underlying these associations remain unclear. The objective of this longitudinal study was to examine the direct and indirect paths through which cSES influences late midlife cognitive outcomes. Research Design and Methods Participants were 1,009 male twins in the Vietnam Era Twin Study of Aging (VETSA). At mean ages 20 and 62, participants completed a standardized test for general cognitive ability (GCA). The age 62 cognitive assessment also included in-person tests of processing speed, episodic memory, abstract reasoning, working memory, verbal fluency, visual-spatial ability, and executive functions. At mean age 56, participants were interviewed regarding their own and their parents’ education and occupation, and completed questionnaires about cognitive leisure activities and sociodemographic information. Multiple mediation analyses were conducted to examine the direct path effects and indirect path effects of cSES through age 20 GCA, adult SES, and cognitive leisure activities on seven cognitive outcomes at age 62, adjusting for age, ethnicity, and non-independence of observations. Results Total (direct plus indirect) effects were significant for all measures with the exception of executive functions. Men from lower cSES backgrounds had poorer cognitive functioning in late midlife. The direct effect of cSES was partially mediated for abstract reasoning, and was fully mediated for the remaining six cognitive outcomes. Total indirect effects accounted for at least half of the total effects in each model, with paths through age 20 GCA explaining most of the total indirect effects. Discussion and Implications cSES predicted cognitive functioning in late middle age Using multiple mediation models, we show that lower cSES predicts poorer cognition in late midlife primarily through young adult cognitive ability and to a lesser extent through SES in adulthood and engagement in cognitively stimulating activities.

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