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Acute extrapyramidal movement disorders have rarely been reported in uremic patients. We had previously presented three cases of acute movement disorders with bilateral basal ganglia lesions in uremia, and had proposed that it is not a rare condition. The objective of this study is to establish a more accurate clinical profile of this rarely described clinical syndrome, and to call attention to its common occurrence. We prospectively studied six patients we encountered from March 1996 to June 2001. We also reviewed the clinical records of a large population of uremic patients and identified six more cases. The clinical manifestations, laboratory findings, neuroimages, and clinical outcomes of these 12 patients were analyzed. When possible, each patient was followed up to the present time. Twelve patients had acute onset of movement disorders and bilateral basal ganglia lesions. All of the patients were diabetic. They had acute-onset Parkinsonism or dyskinesias, together with various symptoms such as consciousness disturbance, dysarthria, dysphagia, or ataxia. The main laboratory test results of abnormalities consisted of elevated blood urea nitrogen, creatinine, and metabolic acidosis. They had uniform neuroimaging findings of symmetrical bilateral basal ganglion changes. These changes regressed or disappeared during follow-up. The clinical prognoses were diverse. We believe that this group of patients represents a well-demarcated clinical syndrome, which is not uncommon but has previously been rarely addressed. The underlying mechanism of such lesions may be associated with metabolic, as well as vascular factors.