Manganese Deposition in the Globus Pallidus in Patients With Biliary Atresia

Ikeda, Shinji; Yamaguchi, Yasuo; Sera, Yoshihisa; Ohshiro, Hajime; Uchino, Shigehiko; Yamashita, Yasuyuki; Ogawa, Michio

doi:10.1097/00007890-200006150-00021

Cited by 47 publications

(30 citation statements)

References 29 publications

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“…AHD may accompany any form of chronic liver disease associated with portosystemic shunting and has been described in the setting of viral hepatitis, alcoholic cirrhosis, alpha-1 antitrypsin deficiency, nonalcoholic steatohepatitis, autoimmune hepatitis, primary and secondary biliary cirrhosis [67,114], biliary atresia [51], Byler's disease [108] and cryptogenic forms of cirrhosis [8]. Occasionally, AHD occurs in patients who have portosystemic shunts without underlying hepatocellular disease, such as those with hereditary hemorrhagic telangiectasias, surgeries or portal vein thrombosis [9,30,93,100,127,144,164].…”

Section: Pathogenesismentioning

confidence: 99%

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Acquired hepatocerebral degeneration

2009

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Cirrhosis and its co-morbidities may cause a variety of neurological complications, the most common being bouts of toxic metabolic encephalopathy. A proportion of patients with chronic liver disease develop acquired hepatocerebral degeneration (AHD), a chronic progressive neurological syndrome characterized by parkinsonism, ataxia and other movement disorders. This article reviews the clinical spectrum, pathophysiology, neuroimaging features and differential diagnosis of AHD along with emerging treatment options.

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Section: Pathogenesismentioning

confidence: 99%

“…Symptoms of AHD usually begin in adulthood, often in the fifth to sixth decade of life [8, 67,114,154]; however, AHD occasionally affects children [51,108], so the age at onset alone cannot distinguish AHD from Wilson disease (Table 1).…”

Section: Introductionmentioning

confidence: 99%

Acquired hepatocerebral degeneration

2009

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“…There is post-mortem evidence for manganese accumulation within the globus pallidus in patients dying with established cirrhosis. 13 14 Furthermore, marked hyperintensity of the globus pallidus (GP) on T 1 weighted magnetic resonance imaging (MRI) has been reported both in patients with cirrhosis 13 15-19 and in patients with hypermanganesaemia due to occupational exposure, 20 biliary atresia, 21 and total parenteral nutrition. 22 We hypothesised that the fatigue experienced by patients with PBC, in the absence of cirrhosis, may occur as a consequence of increased GP manganese concentrations as a result of impaired biliary excretion.…”

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confidence: 99%

Fatigue and primary biliary cirrhosis: association of globus pallidus magnetisation transfer ratio measurements with fatigue severity and blood manganese levels

Forton

2004

Gut

107

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Background and aim: Fatigue is the commonest symptom in primary biliary cirrhosis (PBC), affecting individuals at all stages of disease. The pathogenesis of fatigue in PBC is unknown although rat models suggest a central nervous system (CNS) cause. We examined the hypothesis that a CNS abnormality related to cholestasis, rather than cirrhosis per se, underlies this symptom. Patients and methods: Fourteen patients with precirrhotic PBC (stage I-II disease), four patients with stage III-IV PBC, and 11 healthy women were studied using cerebral magnetisation contrast imaging and proton magnetic resonance spectroscopy (MRS). Results: The globus pallidus magnetisation transfer ratio (MTR), a quantifiable tissue characteristic that may be abnormal in the presence of normal magnetic resonance imaging, was significantly reduced in precirrhotic PBC patients compared with healthy controls. These measurements correlated with blood manganese levels and were more abnormal in the more fatigued subjects. There were no differences in MRS measurements between the three study groups, suggesting that the abnormal MTR was not related to hepatic encephalopathy. Conclusion: This study suggests that impairments in liver function in PBC may adversely affect the brain long before the development of cirrhosis and hepatic encephalopathy, possibly as a result of altered manganese homeostasis within the CNS.

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“…4,10,11 Extrapyramidal symptoms may result from a toxic effect of manganese on basal ganglia dopaminergic functions. 12 Spahr and colleagues 1 have reported that there was no significant correlation between blood manganese levels and extrapyramidal symptoms. In our patient, although the blood manganese level was elevated significantly, he only presented with flapping tremor without any other signs of extrapyramidal dysfunction.…”

Section: Discussionmentioning

confidence: 98%

Hipermanganesemili portosistemik ensefalopati: olgu sunumu ve literatürün gözden geçirilmesi

Okyay¹

2012

Dicle Med J

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ÖZETHepatik ensefalopati (HE), kronik karaciğer hastalarının-da izlenen nöropsikiyatrik bir sendromdur. Amonyak dü-zeylerine 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.Anahtar kelimeler: Kronik karaciğer hastalığı, manganez, T1 ağırlıklı hiperintensite ABSTRACTHepatic 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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Manganese Deposition in the Globus Pallidus in Patients With Biliary Atresia

Abstract: Manganese accumulates in the brain during cholestasis associated with biliary atresia and disappears after hepatic transplantation. Manganese deposition is likely to be subclinical and reversible but may be associated with some age-related neurologic symptoms.

Cited by 47 publications

References 29 publications

Acquired hepatocerebral degeneration

Acquired hepatocerebral degeneration

Fatigue and primary biliary cirrhosis: association of globus pallidus magnetisation transfer ratio measurements with fatigue severity and blood manganese levels

Hipermanganesemili portosistemik ensefalopati: olgu sunumu ve literatürün gözden geçirilmesi

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