Electrophysiological characteristics of limbic and motor globus pallidus internus (GPI) neurons in two cases of Lesch-Nyhan syndrome.
作者： E. Pralong , C. Pollo , P. Coubes , J. Bloch , E. Roulet
DOI: 10.1016/J.NEUCLI.2005.12.004
关键词: Globus pallidus internus 、 Gynecology 、 Medicine 、 Anesthesia
摘要: Objectifs. - Le syndrome de Lesch-Nyhan est une pathologie rare, caracterisee par dystonie accompagnee d'actes d'automutilation. Pour mieux comprendre la physiopathologie cette maladie, nous avons enregistre l'activite electrophysiologique chez deux patients lors pose stimulateurs profonds (DBS) dans le pallidum. Methode. Des enregistrements unitaires ont ete effectues sur 162 neurones long 4 trajectoires en direction du pallidum interne (GPI) anterieur (limbique) et posterieur (moteur). Resultats. Quel que soit l'agent anesthesique utilise (propofol ou sevoflurane), les montrerent des frequences decharge similaires quatre regions etudiees, externe (GPE) moteur limbique GPI moteur. Chez patients, decharges etaient GPE (12.2 ± 1.8 Hz, n = 38) (13.2 1.0 83) alors dechargeait a frequence plus elevee (23.8 2.7 18) (12.5 1.4 23). Conclusions. Ces resultats montrent qu'une legere anesthesie au propofol sevoflurane modifie maniere similaire Les electrophysiologiques suggerent pourrait etre caracterise analogues dechargerait avec Il donc tentant penser symptomes observes maladie correspondraient inhibition GPI. Signifianc Cette observation va sens modele Albin Delong fonctionnement noyaux gris centraux mouvements anormaux.
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