Neuropediatrics 2010; 41(1): 12-17
DOI: 10.1055/s-0030-1254157
Original Article

© Georg Thieme Verlag KG Stuttgart · New York

MR Findings of the Brain in Children and Adolescents with Portal Hypertension and the Relationship with Blood Manganese Levels

R. B. Pinto1 , P. E. Fröehlich2 , E. H. Pitrez3 , J. A. Bragatti4 , J. Becker5 , A. F. H. Cornely6 , A. C. R. Schneider7 , T. R. da Silveira8
  • 1Pediatric Gastroenterology Unit, Hospital de Clínicas de Porto Alegre (HCPA), Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, Brazil
  • 2School of Pharmacy, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil
  • 3Radiology Service, Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil
  • 4Encephalography Department, Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil
  • 5Neurology Service, Pontifícia Universidade Católica do Rio Grande do Sul (PUCRS), Porto Alegre, Brazil
  • 6Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil
  • 7Pediatric Nutrition, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil
  • 8Pediatric Liver Transplant Department, Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil
Further Information

Publication History

received 21.10.2009

accepted 05.05.2010

Publication Date:
22 June 2010 (online)

Abstract

Background: Few studies have evaluated abnormalities on brain magnetic resonance imaging (MRI) in children and adolescents with chronic liver disease.

Aims: The aim of this study was to investigate the presence of T1 hyperintensity in the basal ganglia of pediatric patients with portal hypertension and its association with blood manganese levels.

Methods: A case control study of 22 patients with portal hypertension (14 Child-Pugh A cirrhosis, 8 non-cirrhotic portal hypertension) and 15 controls was conducted from 2006 to 2007. Blood manganese levels were measured using atomic absorption spectrophotometry. Brain MRI scans were performed using a 1.5 Tesla (Philips) scanner.

Results: Blood manganese levels were 26.01±12.82 μg/L for patients with portal hypertension (cirrhotic: 22.73±11.67 μg/L, non-cirrhotic: 32±13.32 μg/L) and 15.64±6.61 μg/L for controls (p=0.003). 14/22 patients with portal hypertension presented T1 hyperintensity in the basal ganglia [6/14 cirrhotic; 8/8 non-cirrhotic (p=0.018); zero controls (p=0.001)]. Mean blood manganese levels of patients with liver disease and normal vs. abnormal brain MRI scans were 18.45±8.38 μg/L and 30.47±13.07 μg/L, respectively (p=0.04).

Conclusions: Brain MRI showed a high frequency (64%) of T1 hyperintensity in the basal ganglia of patients with portal hypertension, which correlated positively with blood manganese levels. This abnormality was found in 100% of the patients with portal hypertension and in 43% of those with mild cirrhotic disease.

References

  • 1 Ballauff A, Engelbrecht V, Voit T. Hyperintense lesions of the globus pallidus on MRI in children with chronic liver disease.  Eur J Pediatr. 1994;  153 802-806
  • 2 Devenyi AG, Barron TF, Mamourian AC. Dystonia, hyperintense basal ganglia, and high whole blood manganese levels in Alagille's syndrome.  Gastroenterology. 1994;  106 1068-1071
  • 3 Genovese E, Maghnie M, Maggiore G. et al . MR imaging of CNS involvement in children affected by chronic liver disease.  Am J Neuroradiol. 2000;  21 845-851
  • 4 Ikeda S, Sera Y, Yoshida M. et al . Manganese deposits in patients with biliary atresia after hepatic porto-enterostomy.  J Pediatr Surg. 2000;  35 450-453
  • 5 Inoue E, Hori S, Narumi Y. et al . Portal-systemic encephalopathy: presence of basal ganglia lesions with high signal intensity on MR images.  Radiology. 1991;  179 551-555
  • 6 Klos KJ, Ahlskog E, Josephs KA. et al . Neurologic spectrum of chronic liver failure and basal ganglia T1 hyperintensity on magnetic resonance imaging. Probable manganese neurotoxicity.  Arch Neurol. 2005;  62 1385-1390
  • 7 Krieger D, Krieger S, Jansen O. et al . Manganese and chronic hepatic encephalopathy.  Lancet. 1995;  346 270-274
  • 8 Krieger S, Jauss M, Jansen O. et al . MRI findings in chronic encephalopathy depend on portosystemic shunt: results of a controlled prospective clinical findings.  J Hepatol. 1997;  27 121-126
  • 9 Mínguez B, García-Pagán JC, Bosch J. et al . Noncirrhotic portal vein thrombosis exhibits neuropsychological and MR changes consistent with minimal hepatic encephalopathy.  Hepatology. 2006;  43 707-714
  • 10 Mizoguchi N, Nishimura Y, Ono H. et al . Manganese elevations in blood of children with congenital portosystemic shunts.  Eur J Pediatr. 2001;  160 247-250
  • 11 Nolte W, Wiltfang J, Schindler CG. et al . Bright basal ganglia in T1-weighted magnetic resonance images are frequent in patients with portal vein thrombosis without liver cirrhosis and not suggestive of hepatic encephalopathy.  J Hepatol. 1998;  29 443-449
  • 12 Pugh RN, Murray-Lyon IM, Dawson JL. et al . Transection of the oesophagus for bleeding varices.  Br J Surg. 1973;  60 646-649
  • 13 Pujol A, Pujol F, Graus F. et al . Hyperintense globus pallidus on T1-weighted MRI in cirrhotic patients is associated with severity of liver disease.  Neurology. 1993;  43 65-69
  • 14 Rose C, Butterworth RF, Zayed J. et al . Manganese deposition in basal ganglia structures results from both portal-systemic shunting and liver dysfunction.  Gastroenterology. 1999;  117 640-644
  • 15 Shah NJ, Neeb H, Zaitsev M. et al . Quantitative T1 mapping of hepatic encephalopathy using magnetic resonance imaging.  Hepatology. 2003;  38 1219-1226
  • 16 Skranes J, Evensen KI, Lohaugen GC. et al . Abnormal cerebral MRI findings and neuroimpairments in very low birth weight (VLBW) adolescents.  Eur J Paediatr Neurol. 2008;  12 273-283
  • 17 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 1116-1120
  • 18 Uchino A, Noguchi T, Nomiyama K. et al . Manganese accumulation in the brain: MR imaging.  Neuroradiology. 2007;  49 715-720
  • 19 Verbruggen KT, Meiners LC, Sijens PE. et al . Magnetic resonance imaging and proton magnetic resonance spectroscopy of the brain in the diagnostic evaluation of development delay.  Eur J Paediatr Neurol. 2009;  13 181-190
  • 20 Yanai S, Minami T, Sonoda K. et al . Patent ductus venous associated with a hyperintense globus pallidum on T1-weighted magnetic resonance imaging and pulmonary hypertension.  Eur J Pediatr. 1995;  154 526-529

Correspondence

Themis Reverbel daSilveira 

Rua 24 de Outubro 1181

Auxiliadora

CEP 90510-001 − Porto Alegre

RS

Brazil

Phone: +55/51/3330 4942

Fax: +55/51/3330 3308

Email: tsilveira@hcpa.ufrgs.br

    >