Neurophysiologie clinique = Clinical neurophysiology
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Lesch-Nyhan syndrome is a rare and debilitating condition characterized by dystonia and self-mutilating behavior. In order to shed light on the pathophysiology of dystonia, we report the pallidal electrophysiological activity recorded in two patients during deep brain stimulation surgery (DBS). ⋯ This observation may confirm the Albin and Delong's model of the basal nuclei in hypokinetic and hyperkinetic disorders.