Acute bilateral basal ganglia lesions and chorea in a diabetic-uremic patient on dialysis.
Department of Neurology, The University of Texas Medical School at Houston, 6431 Fannin, Houston, TX 77030, USA.Archives of neurology (Impact Factor: 7.58). 02/2010; 67(2):246. DOI: 10.1001/archneurol.2009.323
- Journal of Neurology 10/2012; · 3.58 Impact Factor
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ABSTRACT: Diabetic uremic syndrome (DUS) is an increasingly reported acute neurometabolic cerebral disease with characteristic clinical and imaging features. Clinical spectrum includes a wide range of movement disorders such as acute parkinsonism. Imaging studies show reversible (with hemodialysis) bilateral lesions in the lenticular nuclei. DUS pathophysiology has not been entirely clarified yet. Our case study shows certainly that LN lesions are characterized with increased lactate peak with MR spectroscopy and decreased perfusion in computerized tomography perfusion along with increased diffusion with apparent diffusion coefficient (ADC) mapping in the subacute phase of the syndrome. Abnormalities were almost normalized quickly after metabolic control by hemodialysis. Together with reports indicating that a deficit of glucose use exacerbated with acute increase of uremic toxins in bilateral LN, observed changes (lactate peak and hypoperfusion) led us to state that a primary metabolic depression may cause this syndrome. Metabolic depression is probably due to uncompensated uremic toxin accumulation related mitochondrial supression and/or dysfunction. This definition fits well to the other elements of DUS such as ADC evolution and marked lesion regression. Our single case study is not supportive of other previously credited mechanisms such as microvascular dysfunction related focal ischemia or hypoperfusion, prolonged uremic toxin related histotoxic hypoxia, central pontine myelinolysis-like demyelination and posterior leukoencephalopathy spectrum disorder related vasogenic edema.Metabolic Brain Disease 08/2013; · 2.33 Impact Factor
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ABSTRACT: A rare syndrome of acute symmetrical bilateral basal ganglia lesions in diabetic dialysis patients that manifests clinically with headache, dysarthria, and gait and movement disorder has been described almost exclusively in patients of Asian descent. The pathophysiology of this condition has not been established. Of the 28 cases reported, 3 patients have been from North America. In the context of magnetic resonance imaging showing dramatic resolution of lesions of the basal ganglia, this report describes a fourth case from North America of a 47-year-old Hispanic woman with diabetes on dialysis therapy who presented with headache, unsteady gait, and slurred speech. We also consider presymptomatic metabolic abnormalities in the pathogenesis of this disease.American Journal of Kidney Diseases 10/2013; · 5.29 Impact Factor
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