Reply: Attenuated variants of Lesch-Nyhan disease: the case of King James VI/I
Author(s) -
H. A. Jinnah,
David J. Schretlen
Publication year - 2010
Publication title -
brain
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.142
H-Index - 336
eISSN - 1460-2156
pISSN - 0006-8950
DOI - 10.1093/brain/awq157
Subject(s) - disease , psychology , psychiatry , recall , psychoanalysis , lesch–nyhan syndrome , pediatrics , medicine , biochemistry , hypoxanthine guanine phosphoribosyltransferase , chemistry , mutant , cognitive psychology , gene
Sir, In the 13th century, Jacobus de Voragine described how God punished the murders of St Thomas. The perpetrators were inflicted with a terrible syndrome that included self-injurious behaviour, compulsive–aggressive acts, intellectual deficiency, gout and renal failure. This syndrome is so highly characteristic of Lesch–Nyhan disease that Beck speculated Jacobus must have had first-hand experience with a patient with the disease (Beck, 1991).Now P. Garrard et al. (2010) recall the medical disorder of King James VI/I. As a child, there was evidence of motor and speech delay with awkward coordination that persisted without worsening throughout adulthood. His condition has been compared to cerebral palsy, with prominent writhing …
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