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.
References
Bas DF, Oguz KK, Topcuoglu MA (2008) Atypical reversible posterior leukoencephalopathy syndrome in thrombotic thrombocytopenic purpura. Intern Med 47:1931–1934
Cheng SJ, Hsueh IH, Po HL, Huang JK, Tzen CY, Huang FC (2001) Neuroimaging characteristics of basal ganglia lesions in diabetic patients: correlation with clinical features. Acta Neurol Taiwana 10:14–20
Cupidi C, Piccoli F, La Bella V (2006) Acute reversible parkinsonism in a diabetic-uremic patient. Clin Neurol Neurosurg 108:601–603
Dicuonzo F, Di Fede R, Salvati A, Palma M, de Mari M, Baldassarre GD, Di Renzo B, Tortorella C (2010) Acute extrapyramidal disorder with bilateral reversible basal ganglia lesions in a diabetic uremic patient: diffusion-weighted imaging and spectroscopy findings. J Neurol Sci 293:119–121
Hegde AN, Mohan S, Lath N, Lim CC (2011) Differential diagnosis for bilateral abnormalities of the basal ganglia and thalamus. Radiographics 31:5–30
Hung SC, Hung SH, Tarng DC, Yang WC, Huang TP (2001) Chorea induced by thiamine deficiency in hemodialysis patients. Am J Kidney Dis 37:427–430
Juryńczyk M, Rozniecki J, Zaleski K, Selmaj K (2010) Hypoglycemia as a trigger for the syndrome of acute bilateral basal ganglia lesions in uremia. J Neurol Sci 297:74–75
Kim TK, Seo SI, Kim JH, Lee NJ, Seol HY (2006) Diffusion-weighted magnetic resonance imaging in the syndrome of acute bilateral basal ganglia lesions in diabetic uremia. Mov Disord 21:1267–1270
Kiryluk K, Khan F, Valeri A (2008) Acute chorea and bilateral basal ganglia lesions in a hemodialysis patient. Kidney Int 73:1087–1091
Lee PH, Shin DH, Kim JW, Song YS, Kim HS (2006) Parkinsonism with basal ganglia lesions in a patient with uremia: evidence of vasogenic edema. Parkinsonism Relat Disord 12:93–96
Lee EJ, Park JH, Ihn Y, Kim YJ, Lee SK, Park CS (2007) Acute bilateral basal ganglia lesions in diabetic uraemia: diffusion-weighted MRI. Neuroradiology 49:1009–1013
Li JY, Yong TY, Sebben R, Khoo E, Disney AP (2008) Bilateral basal ganglia lesions in patients with end-stage diabetic nephropathy. Nephrology (Carlton) 13:68–72
Lim CC (2009) Magnetic resonance imaging findings in bilateral basal ganglia lesions. Ann Acad Med Singap 38:795–798
Lin JJ (2011) Generalized chorea in the syndrome of acute bilateral basal ganglia lesions in patients with diabetic uremia. J Clin Neurosci 18:1266–1268
Nishimura Y, Shibata K, Funaki T, Ito H, Ito E, Otsuka K (2013) A case of subacute parkinsonism presenting as bilateral basal ganglia legions by MRI in diabetic uremic syndrome. Rinsho Shinkeigaku Clin Neurol 53:217–223
Nzwalo H, Sa F, Capela C, Ferreira F, Basilio C (2012) Reversible acute parkinsonism and bilateral Basal Ganglia lesions in a diabetic uremic patient. Case Rep Neurol 4:156–158
Park JH, Kim HJ, Kim SM (2007) Acute chorea with bilateral basal ganglia lesions in diabetic uremia. Can J Neurol Sci 34:248–250
Renard D, Castelnovo G, Taieb G, Verd A, Lionnet C, Labauge P (2011) Bilateral basal ganglia lesions in a diabetic-uremic patient with dystonia. Acta Neurol Belg 111:166–167
Sheu YL, Cheng SJ, Chen YM, Hseuh IH (2007) The syndrome of bilateral basal ganglia lesions in diabetic uremic patients presenting with a relapsing and remitting course: a case report. Acta Neurol Taiwan 16:226–230
Singhal AB, Topcuoglu MA, Koroshetz WJ (2002) Diffusion MRI in three types of anoxic encephalopathy. J Neurol Sci 196:37–40
Tajima Y, Mito Y, Yanai M, Fukazawa Y (2012) Unusual basal ganglia lesions in a diabetic uraemic patient proven to be demyelination: first pathological observation. BMJ Case Rep 2012
Topcuoglu MA, Oguz KK, Buyukserbetci G, Bulut E (2009) Prognostic value of magnetic resonance imaging in post-resuscitation encephalopathy. Intern Med 48:1635–1645
Wali GM, Khanpet MS, Mali RV (2011) Acute movement disorder with bilateral basal ganglia lesions in diabetic uremia. Ann Indian Acad Neurol 14:211–213
Wang HC, Cheng SJ (2003) The syndrome of acute bilateral basal ganglia lesions in diabetic uremic patients. J Neurol 250:948–955
Wang HC, Brown P, Lees AJ (1998) Acute movement disorders with bilateral basal ganglia lesions in uremia. Mov Disord 13:952–957
Wang HC, Hsu JL, Shen YY (2004) Acute bilateral basal ganglia lesions in patients with diabetic uremia: an FDG-PET study. Clin Nucl Med 29:475–478
Yalcin G, Ozgen B, Varli K, Topcuoglu MA (2008) Diabetic uremic syndrome. J Neurol 255:1415–1416
Yaltho TC, Schiess MC, Furr-Stimming E (2010) Acute bilateral basal ganglia lesions and chorea in a diabetic-uremic patient on dialysis. Arch Neurol 67:246
Yoon CH, Seok JI, Lee DK, An GS (2009) Bilateral basal ganglia and unilateral cortical involvement in a diabetic uremic patient. Clin Neurol Neurosurg 111:477–479
Zazulia AR, Diringer MN, Videen TO, Adams RE, Yundt K, Aiyagari V, Grubb RL Jr, Powers WJ (2001) Hypoperfusion without ischemia surrounding acute intracerebral hemorrhage. J Cereb Blood Flow Metab 21:804–810
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Acar, N.P., Arsava, E.M., Gocmen, R. et al. Diabetic uremic syndrome studied with cerebral MR spectroscopy and CT perfusion. Metab Brain Dis 28, 711–715 (2013). https://doi.org/10.1007/s11011-013-9427-x
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DOI: https://doi.org/10.1007/s11011-013-9427-x