Gülay OKYAY ULUSAL, Ezgi YENİGÜN COŞKUN, Atakan PIRPIR, Osman ERSOY, İ. Safa YILDIRIM

Portal-systemic encephalopathy with hypermanganesemia: A case report and review of the literature

Hepatik ensefalopati (HE), kronik karaciğer hastalarınında izlenen nöropsikiyatrik bir sendromdur. Amonyak düzeylerine ek olarak beyinde artmış manganez düzeyinin de HE patogenezinde rolü olduğu düşünülmektedir. Karaciğer sirozlu hastalarda Manyetik Resonans incelemenin (MRI) T1 ağırlıklı kesitlerinde globus palliduslarda simetrik hiperintens görünüm, manganez depozisyonuna ilişkin karakteristik bir bulgudur. Biz bu makalede porto-sistemik ensefalopatili bir vakayı, tipik MR görüntüsü ve artmış kan manganez düzeyi ile sunmaktayız.

Hipermanganesemili portosistemik ensefalopati: Olgu sunumu ve literatürün gözden geçirilmesi

Hepatic encephalopathy (HE) is a neuropsychiatric syndrome of patients with chronic liver disease. In addition to ammonia levels, increased manganese levels in the brain are also considered as having role in the pathogenesis of HE. On cranial T1-weighted magnetic resonance imaging (MRI), hyperintense and symmetrical globus pallidi linked to the manganese deposition are characteristic for patients with cirrhosis of the liver. We presented here a case of portal-systemic encephalopathy demonstrated with typical MR images and increased blood manganese concentration.

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1. Spahr L, Butterworth RF, Fontaine S, et al. Increased blood manganese in cirrhotic patients: relationship to pallidal magnetic resonance signal hyperintensity and neurological symptoms. Hepatology 1996;24(5):1116-20.
2. Riordan S.M, Williams R. Treatment of hepatic encephalopathy. N Engl J Med 1997;337 (7):473-9.
3. Choi Y, Park JK, Park NH, et al. Whole blood and red blood cell manganese reflected signal intensities of T1-weighted magnetic resonance images better than plasma manganese in liver cirrhotics. J Occup Health 2005;47(1):68-73.
4. Hauser RA, Zesiewicz TA, Rosemurgy AS, Martinez C, Olanow CW. Manganese intoxication and chronic liver failure. Ann Neurol 1994;36(6):871-5.
5. Krieger D. Manganese may be of importance in the pathogenesis of chronic hepatic encephalopathy. Biomed Pharmacotherapy 1996;50(2):93-6.
6. Gospe SM Jr, Caruso RD, Clegg MS, et al. Paraparesis, hypermanganesaemia, and polycythaemia: a novel presentation of cirrhosis. Arch Dis Child 2000;83(5):439-42.
7. Rose C, Butterworth RF, Zayed J, et al. Manganese deposition in basal ganglia structures results from both portalsystemic shunting and liver dysfunction. Gastroenterology 1999;117(3):640-4.
8. Krieger D, Krieger S, Jansen O, Gass P, Theilmann L, Lichtnecker H. Manganese and chronic hepatic encephalopathy. Lancet 1995;346(8970):270-4.
9. Fitsanakis VA, Zhang N, Garcia S, Aschner M. Manganese (Mn) and iron (Fe): interdependency of transport and regulation. Neurotox Res 2010;18:124-31.
10. Weissenborn K, Ehrenheim C, Hori A, Kubicka S, Manns MP. Pallidal lesions in patients with liver cirrhosis: clinical and MRI evaluation. Metab Brain Dis 1995;10(3):219-31.
11. Pomier-Layrargues G, Spahr L, Butterworth RF. Increased manganese concentrations in pallidum of cirrhotic patients. Lancet 1995;345(8951):735-7.
12. Ikeda S, Yamaguchi Y, Sera Y, et al. Manganese deposition in the globus pallidus in patients with biliary atresia. Transplantation 2000; 69(11):2339-43.
13. Kashida H, Kondo M, Fukunaga T, et al. Reversal of portalsystemic encephalopathy by shunt-preserving disconnection of portal and systemic circulation. Nihon Shokakibyo Gakkai Zasshi 1996;93(2):96-103.