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

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

Pediatr Ann. 2016 Feb 1;45(2):e67-e69

Authors: Eblovi D, Clardy C

Abstract
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. [Pediatr Ann. 2016;45(2):e67-e69.].

PMID: 26878187 [PubMed - as supplied by publisher]

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