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Gilles de la Tourette syndrome: Further studies and thoughts
Author(s) -
Singer Harvey S.,
Pepple James M.,
Ramage Ann L.,
Butler Ian J.
Publication year - 1978
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410040105
Subject(s) - tourette syndrome , psychology , dopamine , medicine , endocrinology , psychiatry , genetics , biology , neuroscience
A possible association between the Gilles de la Tourette and Lesch‐Nyhan syndromes has recently been postulated. Fourteen patients with Tourette syndrome demonstrated no similarity to Lesch‐Nyhan based upon patterns of inheritance, behavioral changes, or alterations of purine metabolism. Despite a strong male predominance, a sex‐linked pattern of inheritance could not be confirmed. Self‐mutilating behavior was found in 4 male patients but was readily differentiated from that characteristic of the Lesch‐Nyhan syndrome. Quantitation of hypoxanthineguanine phosphoribosyltransferase and isoelectric focusing of its isoenzymes produced results that were indistinguishable from those in controls. We speculate that, pathophysiologically, Tourette syndrome represents an imbalance between the central neurotransmitters dopamine and serotonin rather than an alteration in purine metabolism.