Literature DB >> 1764145

Recurrent coma and Lesch-Nyhan syndrome.

B J Lynch1, M J Noetzel.   

Abstract

A patient with Lesch-Nyhan syndrome has had 3 recurrent episodes of coma, each associated with an acute illness. Extensive investigation for known causes of coma has failed to yield a diagnosis. Although coma is not generally recognized as a feature of Lesch-Nyhan syndrome, similar patients have been reported previously. This and other episodic phenomena observed in Lesch-Nyhan syndrome may be explained by the disruption of cellular energy metabolism due to purine depletion, consequent to lack of the purine salvage pathway normally provided by the hypoxanthine-guanine-phosphoribosyl-transferase enzyme.

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Year:  1991        PMID: 1764145     DOI: 10.1016/0887-8994(91)90073-t

Source DB:  PubMed          Journal:  Pediatr Neurol        ISSN: 0887-8994            Impact factor:   3.372


  4 in total

1.  Regional brain volume abnormalities in Lesch-Nyhan disease and its variants: a cross-sectional study.

Authors:  David J Schretlen; Mark Varvaris; Tiffany E Ho; Tracy D Vannorsdall; Barry Gordon; James C Harris; H A Jinnah
Journal:  Lancet Neurol       Date:  2013-12       Impact factor: 44.182

2.  Sudden death in Lesch-Nyhan disease.

Authors:  Vladimir Kostadinov Neychev; H A Jinnah
Journal:  Dev Med Child Neurol       Date:  2006-11       Impact factor: 5.449

Review 3.  Delineation of the motor disorder of Lesch-Nyhan disease.

Authors:  H A Jinnah; Jasper E Visser; James C Harris; Alfonso Verdu; Laura Larovere; Irene Ceballos-Picot; Pedro Gonzalez-Alegre; Vladimir Neychev; Rosa J Torres; Olivier Dulac; Isabelle Desguerre; David J Schretlen; Kenneth L Robey; Gabor Barabas; Bastiaan R Bloem; William Nyhan; Raquel De Kremer; Gary E Eddey; Juan G Puig; Stephen G Reich
Journal:  Brain       Date:  2006-03-20       Impact factor: 13.501

Review 4.  Update on pediatric dystonias: etiology, epidemiology, and management.

Authors:  Emilio Fernández-Alvarez; Nardo Nardocci
Journal:  Degener Neurol Neuromuscul Dis       Date:  2012-04-11
  4 in total

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