Charles Dickens and Barnaby Rudge: The First Description of Williams Syndrome?

Williams syndrome, a disorder caused by a genetic deletion and characterized by moderate intellectual disability with relatively strong language skills and a hypersocial personality, was first described in the medical literature in 1961. However, 120 years earlier, Charles Dickens wrote the novel Barnaby Rudge, which follows an "idiot" through London's Gordon Riots of 1780. We propose that Dickens based this character on a person he knew with Williams syndrome. Common features include an "elfin" face, decreased cognitive ability and dependence on a caretaker, strong language skills with emphatic and perseverative speech, anxiety, and an empathetic, overly trusting personality. In the novel, these traits lead the character Barnaby to be duped into actively participating in the riots, which nearly results in his hanging. This example of fiction providing a description of a disorder more detailed than that of medical journals more than a century later should encourage physicians to look to sources beyond traditional scientific articles for valuable clinical information.

John C. P. Williams of Williams-Beuren syndrome.

John C.P. Williams of New Zealand, whose name is associated with Williams-Beuren syndrome, spent his known professional career primarily in cardiovascular research. His disappearance in the mid-1970s and his later life remain a mystery.

Williams-Beuren syndrome: historical aspects.

Williams syndrome, also known as Williams-Beuren syndrome (OMIM database entry 194050), is now known to be commonly associated with a hemizygous chromosomal deletion at 7.q11.23. The way in which the condition came to be recognized historically is reviewed along with some biographical details of the people involved.

Emanuel Miller Lecture: Attachment insecurity, disinhibited attachment, and attachment disorders: where do research findings leave the concepts?

BACKGROUND: Despite the evidence on anomalous attachment patterns, there has been a tendency to interpret most of these as reflecting differences in security/insecurity. METHODS: Empirical research findings are reviewed in relation to attachment/insecurity as evident in both infancy and later childhood, disorganised attachment, inhibited attachment disorder, and disinhibited attachment disorder. FINDINGS: Substantial differences are found in the correlates and meaning of these different features, as well as in the patterns associated with conditions such as autism, psychopathy, and Williams syndrome. CONCLUSIONS: It is seriously misleading to view all of these patterns through the lens of security/insecurity. This heterogeneity in social relationship features necessarily has implications for the assessment measures for social relationships that need to be used.

Williams syndrome: 15 years of psychological research.

Williams syndrome is a rare genetic disorder caused by a microdeletion of about 20 genes on chromosome 7q11.23. The importance of this syndrome for theories and models of cognition became apparent about 15 years ago, when Bellugi, Sabo, and Vaid (1988) argued that Williams syndrome was a paradigm case of the independence of language from cognition. In particular, it was argued that individuals with Williams syndrome have "intact" language despite severe mental retardation. More recent findings indicate a more complex relation between language and characteristics associated with this syndrome. The articles in this special issue form 3 overlapping clusters: articles concerned with language development relative to cognitive development, articles concerned with other aspects of cognition, and articles concerned with interpersonal relations and personality. Together, these articles provide strong evidence of the importance of the study of neurodevelopmental genetic disorders for enhancing understanding of the complex manner in which initial genetic differences impact both behavior and processing strategies from infancy through adulthood. Our hope is that this issue will motivate further studies, informed by the genetic-developmental approach, on both Williams syndrome and other neurodevelopmental genetic disorders.

[History of Williams syndrome]. / História Williamsovho syndrómu.

History of the Williams syndrome (WS) represents a process of detailed analysis of phenotypic markers and of attempts to reveal their origin. This demanding tasks have brought many valuable findings, which were employed in different fields of medicine, namely in cardiology, metabolism, genetics, psychology and cognitive neurosciences. Discovery of the genetic basis of the disease closed the first period of the syndrome analysis. Genetic studies have been proceeding and WS can be taken as a model syndrome for the behavioral genes identification. Similarly the description of the pathogenesis of vascular anomalles represents the key for understanding of the pathogenesis of other, more common vascular diseases. The article brings the review of the history of the WS.