{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T14:47:58Z","timestamp":1791902878404,"version":"4.3.4"},"reference-count":17,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2003,2]]},"abstract":"<jats:p content-type=\"fine-print\">\u2713 Lesch\u2014Nyhan syndrome (LNS) is an X-linked hereditary disorder caused by a deficiency of hypoxanthine-guanine phosphoribosyltransferase. Patients with this syndrome are characterized by hyperuricemia, self-mutilation, developmental retardation, and movement disorders such as spasticity and dystonia. The authors performed bilateral chronic stimulation of the globus pallidus internus for control of dystonic movements in a 19-year-old man with LNS. His self-mutilating behavior unexpectedly disappeared after chronic stimulation. This is the first case of LNS that has been successfully treated with deep brain stimulation. The findings indicate that neurobehavioral features of this syndrome are either mediated in the basal ganglia pathways or secondary to the dystonia.<\/jats:p>","DOI":"10.3171\/jns.2003.98.2.0414","type":"journal-article","created":{"date-parts":[[2009,5,13]],"date-time":"2009-05-13T17:55:36Z","timestamp":1242237336000},"page":"414-416","source":"Crossref","is-referenced-by-count":89,"title":["Disappearance of self-mutilation in a patient with Lesch\u2014Nyhan syndrome after bilateral chronic stimulation of the globus pallidus internus"],"prefix":"10.3171","volume":"98","author":[{"given":"Takaomi","family":"Taira","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomonori","family":"Kobayashi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomokatsu","family":"Hori","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1","series-title":"Neurology","doi-asserted-by":"publisher","first-page":"557","DOI":"10.1212\/WNL.57.3.557","article-title":"Bilateral pallidal deep brain stimulation for cervical and truncal dystonia.","volume":"57","author":"Andaluz","year":"2001"},{"key":"b2","series-title":"Psychopharmacol Bull","first-page":"353","article-title":"Dopamine deficiency in self-injurious behavior.","volume":"25","author":"Breese","year":"1989"},{"key":"b3","series-title":"Ann Neurol","doi-asserted-by":"publisher","first-page":"184","DOI":"10.1002\/ana.410310209","article-title":"Striatal D2 receptor status in patients with Parkinson's disease, striatonigral degeneration, and progressive supranuclear palsy, measured with 11Craclopride and positron emission tomography.","volume":"31","author":"Brooks","year":"1992"},{"key":"b4","series-title":"Neurology","doi-asserted-by":"publisher","first-page":"73","DOI":"10.1212\/WNL.35.1.73","article-title":"Validity and reliability of a rating scale for primary torsion dystonias.","volume":"35","author":"Burke","year":"1985"},{"key":"b5","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"713","DOI":"10.3171\/jns.1998.89.5.0713","article-title":"Efficiency and safety of bilateral contemporaneous pallidal stimulation (deep brain stimulation) in levodopa-responsive patients with Parkinson's disease with severe motor fluctuations: a 2-year follow-up review.","volume":"89","author":"Ghika","year":"1998"},{"key":"b6","series-title":"Lancet","first-page":"338","article-title":"Self-mutilation in Lesch-Nyhan disease is caused by dopaminergic denervation.","volume":"1","author":"Goldstein","year":"1985"},{"key":"b7","series-title":"Brain Res","doi-asserted-by":"publisher","first-page":"93","DOI":"10.1016\/0006-8993(90)90439-I","article-title":"Autoradiographic studies in animal models of hemi-parkinsonism reveal dopamine D2 but not D1 receptor supersensitivity. 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